From Harvard Guide to Psychiatry Harvard Universisy Press, 1988 A, Nicoli (BAiter} CHAPTER 5 * Neural Substrates of Behavior: The Effects of Brain Legions upon Mental State ef ‘M.-Marsel Mesilam Neurological diseases that sas behavioral iat pairments assume a substa wal relevance inthe Tierential diaprosis of pychitee disease. Eur thermore, the associated clnieal syndromes pro wide important insight iro the biological sub Theses of anencalfation. The behavioral features and pathophiysiolgical mechansins of these sydvoies have alrezdy become the focus ff extemive monographs. The purpose of this Chapter to provides highly elective introdue- tio this subject Brain Organization “The neurons of she central nervous. sytem (CNS) are engaged an 3 major operations: (1) fecoption ann registration of sensory. still feorn enusie and from witbin (input; (2) plan ning snd execution of complex motor ats (l= jut and (3) intermediary processing interposed fetween input and output. Thought, language, memory, self-awareness, and ever many aspects ‘of oud aud affect constinte different manifes- ations of intermediary processing. The neural Substrates for these interiediagy processes are located principally within he ine system and Comical association atens. From a behavioral point of view, therefore. the cerebral hen Fpheres can be divided into 4 major compo ness primary sensory cortex, primary motor torte, assoiation cortex, at the limbiesystem {Fie 5:1; Table 5.1). fs the later 2 compos hems chose associated with imermediary pro sing at are moat elerane vo the substance ‘ofthis chapter, OF these, the nbc eomponent isprimarily involved in affective inception and Inemoni processing, whereas the association Componcits more involved in multimodal se Sory elaboration and motor planning. “Fie function ofthe CNS iso gather, process, iegrate, store, and abstract informacion about the internal nie and the excrapersonal envi ronment This work is done through hierarchi Cally organized neural interconnections that form distributed cerebral necworks. The hypo- thas ste ead ganglion ofthe internal mni- Tien and the chet coordinator of instincts and rive, Among the steuctes listed in Table 5.1, ‘nly limbie regions have substantial monosyn- pic itercompections with the byporhalamms ‘The limbic system therefore polarized toward the internal envionment and ts requirements [At the other pole of tht continuum, the senson fd motor systems enter ito direct eontact with the outside word: the sensory areas provide portals forthe entry of information aout ext peetonal events, while the moror areas coordi- fate the anipuation of the enviroment. ‘Neitet core Tinie structures nor hypo thalamic nuclei bave substancial direct conc: tions with primary sensory and motor cortical reas. This erangementensutes that Motor pro" ciency ad sccurate sensory analysis of exter fal reality ate noe nduly tlenced by sudden Shifts in the emotional state of the individual ‘Although the sensorimotor apparaius and he Inypothalous Jo communicate with each other tivough smultisynaptie pathways, this occurs through obligatory rclays within limbic and as- feetation cortex, The limbic and association rePART Twocuarren 5 Table $1. Some behavioral ese ofthe [MESULAM /NEUMAL SUBSTRATES OF BEHAVIOR 93, cerebral ees Frmarysevnry cortex "Vinal ores 170 Figure 5.1) ‘diory eas 41,82) Somatonemory (areas 3, 1, 2, but moyen 3) Prinery motor cortex ' ices cout pat af cen 6 Tinamadal war (cone aen 6 cand area 8 rea 44) {nmol viel erent 18, 19,2052 37) Unima anory ore 22) Uhumadal ematozerry (area Sosa ares 7) Hetero pele (reas seroma paretoremporal areas 38, 40, Linke system forticel and subcortical component) Marne cir nas ermporoplar comix aren ‘nate vomplex-ateas 23,24, 33, 31,26, 2; paolbctor ep pocampal ccten—ateag 26, 34,33, 30) 10,11, $5, 46,47, coral area 8, roselare 12, oneal ea 32) rial en 7, bake of ypetio tenpora sul 36336) 38; caudal orbitoona comten-—enedal aren 12,13: 35, cual me 32; paca ETE aon hippocampus, syed, sobstanca innominate, septa ede, pyetorm olfactory ‘nen un enol Tot haben) Teal ganglia an elated suctre (moles accumbens, medi lobus pallidus, ventral epmental 112 han niin nae, atrior suber vee lterodoral nucleus, dorsomedial meus, medial ina cles) ypodialamie nce Hirmorialamigmncl ions therefore provide neural bridges that me- ‘iar betwee the inner urges of the individual find she contingencies ofthe extrapesonal envi fonment (Mesulam, 19853). Tneemediary processing increases the feibil> iry of behavior ao that drives can be stisfied ac- ‘ovding tote linitations and epportuniies that teks within the extepersonal world. Avimals Comparatively lower in the phylogenetic scale fave relatively underdeveloped finbic and ass {ation avens and display rewsaekable behavioral Fi. In these animals specific stil can a= omateally set in motion predetermined 1 Sponses that are desribed as instinctive. For ex- ipl arkey hen with a newly hatched brood twill attack every moving objec within the nest That does nor utter the specie "peep" of ts hicks, Ia turkey hen ie rade deat, wil pro- feed 10 kil i own progeny (Scileidt and Sclleide, 1960), The behavioral repertoire of higher species, especially humans, is eharacter- ized by much greater flexibly The neural sab- strate for ouch ofthis xii is provided by the interposition of Timabie and ssociation areas between simul and responses, between hypo thal urges and external rea. These iter. iediary area ofthe brain acta: ond gates and rgats ina programing boare. Thus Wentical Stimuli can srigger warty iflerent responses de pending onthe stuntonal conte, past exper- nee, and present needs of the individual. Ie i the phylogenetic development o! this interned Sty processing that i responsible for choice Sntong options, exible shift way fom unsuc- esl responses, adaptation tonew situations, Selay of prema gratification, and even for self-awareness, thought, and ply behavior. As in so many other aens of bicloy, however, ad ‘vantages rarely come without special vullnerabi Ines In this cate ineemediary processing could besa 0 Bring with tthe swept for ap= preheasion, doubt, sel/-consciousness, rumina-94 BRAIN AND HEUAVION tion, and excessive inhibition. Some of there snore speculative aspecte of brain helavior fn crete mor yer accessible co direct analysis by the neurologieal approach ut the examina tins of patients with lesions a Tibi anid mere atin areas has provided uber sosights into the aeuesl substrates of many other cont plexhehsviors. Limibie Syndron “he te tiniest i wad designate 2 Scenpeer tf erucuran be bret Socios stan us force and tones ae. eur Sand 3) Foor ma Sprain ca be Miedo mpg tee ruses tna 9 singe system (0) The cone at mae pe bein aly noconec ive mesyoaptcrettaye {pred nthe 150 Pape pte ou at ie ren few. convection thn cpr feroed = dosed’ anmiletilane-cnglr Iirrecinpermsany hops hi rc ow inns some, ny sal err rane tin hve cr deed set ten sd Vopr ao reine ha he apex ol teed many sen lps (2) Coop woot links syem ay lo cons Sermon Immunologie reperice or oe Ste hres apie na has ele {UT any ar he evel epee of he Inlet Conca hau eeopint nn aie hated yt co ‘lets Lane ens sho day com fn Hhaeacolops pipe. Bor ea, ice opener nd opi wae Comer fh lnc cn ane nm be stain Thin ees Soria bese eon lve moet ney ‘ede cto te prow unre ded to suet 0 po pl ers fence Ge Fe.) Te Finn ans comer sco to Be tore dey asec with mony fn, Shown the cena Sods pbs te Pant Two more closely elated to the alective componente of experience. in animals, severe sniesins occur tly afer leone in bot the Kppocsinp xt and the amygeaol elruie Mishkin 1942) I fu mans, amygdaloid involvement is epparendly not necessary for the emergence of amnestic Memory and Ammesia Memory permeates all expects of mental fife, CConomon sense may therelore lead to the as sumption Fac thie fculyie relatively protested, Pehaps inpaited only by the largest of brain Tesone. The fac, bower, lead to 2 dierent ‘conclusion: severe diturbances of memory are ‘not uncommon snd arise as a consequence of relatively small lesions, longa the damage is Tate wii the Fmibie system, “The amuestic syndrome is the mast severe formof memory disturbance and isthe follow ing 5 lineal characterises: (1) The patient is alert, attentive, and modvated. (2) There is 2 Iuanifest retrograde avmesie: information 2¢- aquired before the onset othe its it not aces- sible to the patiem, Ths retrograde armesi ‘obeys temporal gradient, also kuown a8 Rie Bots law so that anemorins acquired just before the onset ofthe armesis se lss accesible than. temporally more distant ones. his probably ee- Meee he fact that older information i highly ‘overlecned and more extensively incorporated Ineo the contents of consclousness. Thus patients ‘wih the ares form of senile dementia have Felatiely litle difienley remembering trina Metals of distant eveits whereas more recest ‘vents are forgotten, Th fac that anaes pv ince almost never forget their own names: oF Ihethplacee is also consizent with Ribor’ law. {G)"The patient hae severe anterograde amnesia, which interferes with the acquisition of new learning. For examnpe, de patient may nt reall ha he had to eat few minwtes ago oF whe tisted him the day belore. Disorientation te time and also to person and places new tothe Dien aries as 9 consequence of this ater Frade amnesia because the necesary for tion cannot be incorporated into the working:cHlanren 5 J & { &, O % ans ians/ NEURAL SUBSTRATES OF # Massena Wide Stare! + Zcosratin seca Feria wwe fasom NERE segs, yaePuk 4 em eiavion 9F PAPEZ CIRCUIT HuppaRa r NSUL gaeeam CINGULATE bic connections. Onl Figure 52, Some li ty select paways ae demonstated io iin she Paps cei. The erm bppacemna i wd Tascnor only Armas en Bat ls the forse, amma a8 memory stores. Thete patiets are extremely TMncrable wo distraction. Akhough they can re [hi informadion for many minutes if undis- Tavbed, even the briefest staeton lead 10 the Toss ofthe pertinent memory eraces. (3) One of thomas colorful consequences ofthe amnesia is Bofabulation. For example, if the examiner Sook the ptien, “Do you know who Lam?” the patent may provide A most elaborate confabu Froeyeespone, such a8: “OF cours I know you, ‘leer you the salesman who sold me 9 exe?" Some say. that thie confabolation (a false postive renieal response) aceues beease the Psene is not flly aware of his memory problem Bhd also because he Tacks the mechanisms for Pubelar somplex. Abbreviations: critically evaluasing whether retrieved item fis the present comext and his past experience. CConfapuation + nor 3 necessary farure and is Greve mestly daring the sce stares of the erspenicayndonre, (9) In conerast vo devastated Memory faneton, her coguitie facies seh setangiage and vinwxpatial skills are quite i thet In fk severely ast patients ay 0+ tain supers ures 010 te “The patiene wth anamnestic syndrome [0ks perfectly healthy and aay appear sel-voniident Fea aeay inact dh eouese of exsualcOn- ‘enatian, The conv, however, lau ts Were and incapacitating Hiseatons in diy toe Ing atts the pasent completely aware96 DRAIN AND UxttAWOR ‘of arene news items he cannot leaf his way to the bathroom or hack wis bedrooin; he can- rot find personal tens within the houses he may leave the stove on or the water runing he ay pay the same bill several tes or nota all The acute atnnes syndrome isnot compatible with independent existence ard almost invariably leads to instcuionalzatios either permanently ‘or until some recovery take place. When the amnesic syndrome occurs ia iol tion, one ean always conclude that a lesion ex ie inthe ime syste ely sie ln bilateral, Occasionally the damage may be uni Tavera, and in toe case ir almost always 00 the ll side; sch patient aually recover rather rapidly, whereas patients vith bilateral lesions ‘may remain amnesicindefiiely. Not al the lim Bec structures shown in Faure 5.2 bave vo be involved to produce an anesl. to fat, in most ninestic eases only very few of there sructres fare damaged. For example bilateral infarctions in the limbic thalamus, biateral amerior tem ‘poral lobectomy, bilateral damage to the sep ‘or basal forebrain, sid biteral medial den ‘ephalic lesions (2 in the Wemicke Kossakolf syndrome) have ead) been associated with Amnesic stares (Signoces, 1985). Although the Site of damage does iallyee some clinialfea= tures of the amnesia, the major aspect ofthe ammnestic state are present in most ofthese p= Even the mot severe amnesia ig never com- plete Alhough the ality to give a verbal ac count of newy acquired experience (declarative tvemory) i almost always stverey inipaited, the sneniing of procedures al habits (Proceddsl ficmory) i tlatively preserved. For example, the patient can learn new motor and pereepual lls even though he does nat seem to be con= sciously aware of acquiring these skills Thus 3 mocoreoordination ask suchas the tot pu suicest, an armnesicpadentipay show a learning ‘eurveindiscinguishablefrom that of normal sub= pects. Ar the beginning ofeach daily raining se sion, hstever, the same patent may deny haw ig had any exposure to this task (Miler, 1962, Emotionally landed experiences also seem to be retained, though not necessarily at the verbal level of consciousness. For example, patients who havea condition known as proso: agnosia inability to recognize amine faces by Sight) emie an autonomic response pattern ind cating, consierable arousal when shown the face af an emotionally signin pers though they give no veal indeaton of having recognized that individual (Teanel snd Damasio, 1985), This finding gests that the patient can recreve the sutonomie component ofthe recog. tition fom memory stores hut sot the ae tiated verbal label A sindar sonclsion merges fom s clinical aed reported hy the French neurologist Cloparede (1911). He was taking care of several ameesic pavents who showed no signs of recognizing him despite his dedicated daly visits. Apparendy annoyed at this ingratitude, Claparede concealed sharp ject in his hand and gave one of his paiens 3 powerful prick during is eustomary daily hand ‘Shake. The next morning the patient sil dened knowing him but this me was most reluctane to shake his hand. Cleaty the painfl conse. quence of the handshake had been stored at some nonverbal level of meniory and inuenced subsequent behaviog although no verbaizeble naciouses was associated with i These con- Aitions may provide 2 biological mode for ‘esigating the proces of unconscious mem Partial anmmesat. The alledged amnesic sy rome is muliimodal and global except for mo to¢ and emotionally loaded tasks. Occasionally, when the limbic damage is unilateral, material: pec and other partial amnesiaeatize. Kiht- Sided lesions may slecvely tpi memory for complex perceptusl pattems, whereas lesions Confined to llesided structures may preferen= Sally impale memory for word Its The veal snonesia of lfsided lesions is usually snore se- tere than dhe amnesia for nonverbal material thar occurs after right sided lesions (Signoret, 1985) This may inlcate ltt hemispere dom. ‘nance for memory. Altecnaively, it may reflec the fact that memorization even of nonverbal Teems inay be verbally medited ‘Memory processes require extensive samoryCHAPTER 5 timbie integration. Lesions thac give cise to the Teste syndrome divetly damage. limbic ftevctues and disrupt such interactions. Some Testons outside the Hib xyter, however cn Selectively imeerupt the low of information Song individual sensory pathways into limbic essere, Such lesions may eesult in modality” pecfc amnesia. The most dramatic condition eis cacgory is prosopagosia, which has ak ready bem mentioned. Although visual-el de {ts ate uwally also present, prosopagnosic patients possess the percepual ability for distin: uishing one face from another (os long as chey fe both simultaneously in view). They know they ate looking aca face and can determine its fpe and gendes, bur not its identity, even if it belongs toa very close family member, uch 288 child or 2 spouse The defi eeflects a problem inthe associative processing of relatively suble visual distinctions. Thus when poneisal infor Imation (for example the sound of 8 voie) ora particulary salen visual ene (a hat with a char~ Sets eolor or a tie With a special patern) becomes available, che patie ean ceach the proper identification, AS noted above, pros Cpaznosie patients may sill produce powerful uronomic responses when showa a familiar face but not when shown an unfamiliar one “Therefore i appenc thatthe visual stim ean evoke emotional but not verbal associations Ta most settings prosopagnasia i the most dramatic, but not the only, manifestation of & ‘more widespread modaltyspecic partial visual “invests (Damasio, 1985) This contin sus Sly sssociced with a rclagively generalized in Siliey to recognize individual members from mong a class of similar objects shar dif from fone another by claivly subi visual cus. Example, patients with this syndrome may easily iemtly an objet 98 2 shoe, bur they may not recogoine that i belongs to them. This i not 3 peveenual problem, esas they ean easily di Tinga 2 sessile by eve nino deal “The pralany i tha they ctr associate the imyrind aubile visual cute (age, color shape, State f pai) ith the speci individ mern- ‘orice hat form the basis fora more precise ien- ‘ication. The bran damage in prosopagnosiais MESULAM / NEUKAL SUBSTRATES OF BEMAVIOR 97 atest always bilateral and involves che ventral Temporoocerpital regions of the brain, This ‘Tamage sents in a paral visualimie discon teetign, so that visual information can 0 longer Uf the fll range of relevant associaions. Ler ions that inerfere with auditorylimbie oF omacosensory lini interactions cam also ead fo feaetonal amesias i these modalities (Ros, 1980), “Tansient global amesia is 9 most dramatic syndrome usually sen in middle-aged indvid= Sie especially after a period of physical stress {Gehl activity. a pin cold water, exercise, and soon) The onset ofthe memory loss is quite Shade. A component of retrograde amnesia 0 Shes and can encompass a peril caning from ‘ew hours to several years preceng the onset Of the ictus, Daring dhe rus the patient manic fess severe anterograde stnnesia similar ro the tne described forthe amnesic state, Tal dis- ‘ntneaion is the rule ante patient may even forget biographical information. In contrast £0 the amnesic syndrome, there i sully conside cable anvety an even agitation. The patient Continuously take what is happening 1 bin, fet he is dong ere, and wh the other perso Ja The episode may last from 12 0 72 ons and Sometimes even longer. Folewing recovery the patent usually has no recall of events that ac here during the iets. Vascular insficiency in ‘he mel temporal teeitory othe postesior ee febral anery, migraine, epilepsy, and even brain tumorhave been associated as etologieal factors for this syndrome, In most instances there is 8 single episode, without recurtence, This sy Crome, however cou also be the harbinger of i oclusve stroke in the posterior cerebral cee ulation, Transient global amnesia can easly be tmisinerprete as having psychogenic cases. Epileptic and narcleptic memory disturbances. “Temporal Inbe epilepsy may resi in powerful feelings of div (losin of Fama) or fae tnais wo (lsiow of waiaiy), Profonged panes of jamais ws could give ese to sates luring which the patient denies being ac iquainted with tania persons, places, andl {cts This condition may pve the impression of98 MKAIN AND nEHAVION ‘anamnestic state. When dhese paientsafe ques- tioned, however, they wil eadiy disingush the Fecing of unfamiliarity from 3 tre los of fae ‘ual memory. “Temporal lobe epilepsy can als lead to di sociatve fugue stares. The patient may low ‘memory for past information, including bio frephicsl data and ideniy: During the fopie State—which cowl Last from mynwtes to bout fd im unusual circumstances even days—the Dtient may engage in complex acts ranging From neural vo criminal. Exterial appearance say be quite unremarkable. Occasionally be- hhavioe scems automatic, driven, and inflexible. Despite the macked retrograde amnesia, che pa tient may show ile any anterograde aninesis, 40 new Information can be acquired dating the fetus. Following the episode, the patient may not fetain any koowledge of events that gceuctel ‘using the fugue state, Some patients may com plain that they “lose time Similar, but usualy Uriefer. ave nates are also seen In areclepy. Distorted memory. Reduplicative paramnesia and the Capgras syndeome are closely allied Condions of distored. memory (Alexander, Stuns, nd Benson, 1979). Redplicstve rar: resia (aterm coined by Arnold Pek) refers (0 the delusional hei that a place (sual new to the patient) ie ncroly situated n'a diferene and wsuily more familia geogrophic ste, For ex- ample, 4 resident of Providence hospitalized at theeth lz Hospital tn Boston may insist that he is atthe Providence Beth Israel Hospital ‘When confronted with the information thatthe sign atthe entrance indicates this tobe the Bos- ton Beth Israel Hospital and that Providence floes not have a Beth Lael Hospital, or when shown evidence that his present surroundings txacly ft those of Boston, the patient may ex- press wonderment but remain tshalken ibis Bole. He may suggest har there could fe an exact duplicate or branch ofthe Boston Beth I fel Plogpitalsitaced in Providence, perbaps bul just recently. Sometimes the posit may wonder why anybody has gone o sue lengths 45 to reprodice the Boston skyline in Prov ‘ence. Although the patient is eapable of com prehending the implausibly of such circum Frances, logicelangements usually have lle if fy eet sm sltcrige hs belie, Sometimes che faien confess tae he “Hows” this hein possible fe tht i soneteles “feels” that way. Patienss wits redupicaive parammesia usually Ihave no tudinal choughr disturbances, del sions, oF hallainations. Temporal disrienta- tiom may be presen, Paints with thie syndrome do noe have anterograde amnesia. They ea learn new mteriah which makes even more pling thse persistence inthis delusional mis: cation of thee environment. "The Caparas syndrome is sill more dramatic and refers tothe strongly held delusional belie! hae others (analy family members) and sowve- fines even the self have been replaced by doubles or impostor Often the patient believes fai underlying pl ie motivating the impostor. [tore mesmo paranoid fenees are preset. I one elinieal case dhar was reported, x patient insted that lis wife and children were re placed by doubles He nsumed that his original Ivifehad dese him, and he was very thank that she fad been dhoughfal enough vo find ¢ replaceasent. When the pant was asked 10 © Pin how 2 ete ofS cilren could have been Fired by him, especaly since he had et is eee Cond wife so recenl e agreed that this was dit Fenle to explsin ut refused o alter his convic- tion (Alexa, Sts and Benson, 1979). It has been poineed eu tae the Capgeas syndrome it hot based on a perceptual problem, since he pa- tien wil lay state thar the puraive impostor look “exaal like” the exginl person. There fore, asin reduplicative pararmesi, the prob- lem i mostly one of cisoried belie associated swith memory aces (erson, 1983). In newologesl pracdee the most common esting for replicative paramvesia and the Capgras syndrome occurs patients with rla- tively severe hend trauma who iniially develop an ame syndrome (indicative of Tie in ‘olvement) bit who subsequently recover most memory fanevions. Ii addon, seb patients Usually have bifeonral brain dase tha i al tugs always more sever in the eight side Conceivably the aranestic pero inrodvees accuawren 5 isconinity inthe fabsie of awareness an the omseaences ofthe additional rain damage in the Frontal lobes prevent the proper illngsin of the gans when menory improves, hus if poe titi old that is 2 ew place but bas 0 tnpetience of geting ther, he may tend 10 di ts this fact ana co conclade cht he iat af ilar site despite the weight of comteaditing evidence. In the Capgras syndeome a sight Change in a femiliae person’ routine oF appeat se may Hea co dhe coneree(stimulas-bound) deal sele-perperuating impression dhe cis is 2 dilerent person, The silty to che original ers, however, also Teads cO the conclusion tae this mast e an impostor In norial waking ite, experiences associaced with Faiiae persons and places do change from Tie to ime, The ines Bea tends wo assume continuity ard to ute thit new information eo only 10 update the eolevant associations. 10 Petes wha caver from anamnestic sare ad ‘wo also have bron lesions, the mechanisms recesory for ensuing such continuity and for Crieally assessing contextual plasibity seem to be dstypted, leading tothe dramatic occ fence ofthe Capgeas syndrome and reduplca- tive parampesia tn incl practice these cond tione must be etingwishe frum experiences of inna whi ea lead eo transient Feelings of Colamiiarity associated with places and per- Depression, aging, and memory function. De ress! patients frequently complain about ‘merory fale. tn some indivals this merely represents the process of sel-deprecation, and ‘jective testing fils to eeveal memory dificul tie In others dhe syndrome of enlogenons ‘epresion contains # bona fide component of ‘reony loss. The deft isnot as severe asin the annvetie syndrome not as specie asthe parcial Srinesins described alive, Some heal guide Tins lp to distinguish ths ype a wenory dis turbunce from other annexe In cunteost cose patient with the amuestic sire eve whe ster enn hs it fiscion with depression are very much eon serned abot sis dist Testing eveal thatthe SMUSULAN / NEURAL SUBSTRATES OF BEMAVION 99 memory losis due mainly ro iefecive encod tng and regiscation, "The patient does por seem torspend the necessary efor the ste of en ceding (Weingartee et al, 1981). If addtional sili isallowed atthe stage of egiteation, the gf is usually overcome, whereas thememocy Ics of dementia or rhar seen inthe amnesty rome is usually impervious to this maneuver. It harp contrast to other types of amiesa where ‘onabulacion may be a central feature, patients ‘who have dhe meinory loss of depresion rend to tive excessive false negative responses in che form of «| don’ kone” hn fet, when the patient ‘coaxed to provide an answer, the memory lose torn out to beless severe than reported. es my reesion thatthe memory loss of endogenous Aspression is particulsely marked i the aged in tdival. Young patients with depression usually ‘donot have signeant cognitive des. Ieis important ro keep in mind thar many mo: alice of eremimens fr depression (eles wulsive therapy, lithium sls, eicycieanti> Aepressants with anticholinergic elles, and so fon) ean sho lead to memory impairment Hence tnrmanaing a depressed patient who cemplains of faulty inemory, one must decide wheter the symptom is stociated wih the undedring dis- tate or with its therapy. Treatment ofthe depres: fon or withdrawal ofthe ollending medication isoten associated with a complet restiution of ‘ensoryfaetion i many ofthese patients. ‘Agingitself may lead to decrease in memory capacity, but thie deficit ie usually mild and farely interferes with age-appropriate vail Hiv ing activities (Mesulans, 1985¢). This eype of memory disturbance has been ealled beng se- hnescen orgefulneev et it apart foe the peo irene and malignant amnesia of demenia (ra, 1962). In general, the benign forgtflness of auing impedes mocly the ability to remember haines and dates rather than evens. Ar most of the difiuley appeses to be atthe stage of re tseval, peripheral ees in te forms of notes and dae books canbe very helpful 'No fixed steuctural brain lesion bas been associated with the memory dlisticbaees. of ‘lpression al ang, hut deve i ease 0 be lieve thar cholinergic transmission (ir aging)and norepinepheine innervation (a depression) Veith the Tse stem aay he die these conditions, Poychogeric ammesias. Many diparate sates Pant 78 ‘emporotinbic Epilepsy and Peychiatrc Spudiomer Play and Poy The second major hehavinral alfition of the Iinabie system ie ithe reals of emotion, A ‘hough this relationship har been suggested have been included under the rubne of payer f since the time of Paper, new evidence has more onic ammesia. At ove extreme ae individuals ‘who tend to reain lle if any conscious reco lection of unpleasane or traumatic events, The process of repression is invoked to expla this [henomenon. The memory discontinwity ean sometimes be eliminated by hypnosis, sada amytal injections, or dicing” peychotherapy ‘These pavents have iealted Ineunae in meinory hr no other manifestations of ames. In com test to patente with fugue sates, the memory ss are for specific events rater than for Gime pesies ‘Atte other extreme is the malingerer who Isa vested interest i ailing vo recall certain event. ly definition secondary yan is derived Irom his denial. Some malngeringmay take the form of “theatieal (ot Hollywood) arimesia,” vwhetehy a persons suddenly found to have lost All prior memory including wh be i (Signore, 1983). The pers giver no evidene of ening anteroyrade anmesta, however, and can acuite new inerinaion quite normaly Thi condition needs to be ditingished from lugve satex, ‘which usually last for sore perindeand rately i ever (or days om ed. The bes of information Shout personal wlentiy isan apertant difer. ‘niatng feature because patents with organic Snesag (with the pole exception of fugue ‘tes andthe bt snges of demi) ordinarily flo not forget thor cx nate, braces, oF Jandates Pesogeic scsi do et bt in portant to avoid dhe edeney «0 make this di Sunoss just hocaese the clsical crore looks tunusual.”Akhug patents with jamais faye, tansien global amsesin, raluplicatve parainncsa, and the Capgras syadraine have ‘rama lial pietrse chat aut easily end temrelves to psychiatric anterpretaton, the el rican must he aware of the nla causes fssocatgd with these coudyions recently heen introduced. For example, depth tleetrodes inpatients who are being invesigted forte surges! tentment of intractable epilepsy show that strong emotional experiences are s- Ssoeiced much sore commonly with discharges fran imbie stractares (expecially the amygdala) than with discharges from the nonlimbie com povents of the temporal late (Gloor et al, 1982). Moreover, sensory stimuli have been showen co cause amygdaloid and hypothalamic sedvation only if they are aswociated with an ‘ional response (Le Doux eal, 1983) Each component ofthe limbic system seems to lave some involvement in the modulation af mond and affect. This eorcelation is paticulay strug in the ease ofthe amygdala and the ten poropelar, intl, orbitofrontal and anterior ‘ngular regions with which ii interconnected (see Figure 5.2), The amygdala and elated coe- tical hmbie areas ate aefociated not £0 much ‘with the generation af dhe emotion itl (his it probably eoordinsted ehely by the hypothala: ‘nus but eater sith difeting ito the proper ‘object and pelias to the proper mental com tents For example, Downer (1962) showed that visual scl tigered the appropriate emo- tional response i ehenus monkeys oly if the ‘igual information bad access ro amintactannyge dale. This finding provides an explanation for the pathogenesis ofthe Kliver-Duey sjdrome, wherein isteral damage tothe amygdala and urontiing temnporopolar cortex in monkeys results by 3 dramatic Behavioral chaiger ince sant and inappeopriate mouthing of inedible ob: jects, attenirs 10 copulate sof the sme sex and even” with ‘nainate objects, tnd ai wncharseterisie fiendlinese to human beings. Although the drives that are being ex pressed ave quite norival forthe behavioral rep- nti ofthe anna, they ae no longer directed to the proper extrapersoual target Ths changeccHarran 5 elects an interruption of sensory limbic iner- ‘etons by dhe lesions ther eause tis syndrome {Geschivind, 1965). Some components of the Kliver-Bucy syndrome ean be seen in mans who develop bilateral degeneration of the ten poral pole (asin Pek’s date or limbic ecep- Sts seconalary to herpessimplex “The linbie componzns of the temporal lobe (arnygdala, hippocampus parshippocampal gy- rus, temporal pole) and elated paralimbic areas ine insta, the orbitoleoutal cortex, and the Cingulate pyr have alow seizure chresold and Frequently become the source of epileptic activ ity. In some patients such seizures ae associated vith characteristic automatism such as string, Tip smacking, head torning, forced walking, ru= ‘ing (epilapsia cursive, hallucinations (espe Cally olfactory snd gusitory), and epigastric Sensations, When such spnptoms are described tothe physician, there i eave le dewey in considering temporoimbic epilepsy a8 3 d gnostic possibility. But in other paints tem porolimbie epilepsy results excesively in sy Chie phenomena. The extraordinarily diverse be- hhavioeal manifestations of remporolimbic epi- lepsy can be divided ino those that are brie (jaa) and those tht aremore prolonged iver- fetal Spies ea, 1985 Terai manifestation include dei va, jamais vv, flings of nreaity Sepersonaiaation, fear, panic attacks (with or without associated aut omic discharges), elaion, erotic sensations, ‘pression and forced shots that may take fbsessive-compulsive peoparcions. Its the Sudden, teansient, unprovoked, repetitive, and Sereoryped nature of the sympeomatology shat nay provide a cae to the underlying etobogy. Th reac deal has bese weiter about agers sion sind epilepsy. Some patiens wich cemporo- Timbie eilepay do show sudden and unpro- voked aggression, occasionally associated with prising feats of sre. Sometimes these fonursis can have ajar criminal “conse- (quences Such epvodes usualy eur without premeiation, but this isnot a general eu. Ane trata forthe episode nay not he 2 consistent Feature, In contrast te psychopathic cnitions whete the patient may experience ite gu, p= MESULAM / NEOKAL SUDSTRATES OF BEHAVIOR 101 ems wh ate arsine in coianesion with ‘Seporac elepy todo be vey remorse taLti addon to ial seen oubor, whi are peel sate te, temporal “pipe nay seb octal wh peaoalty SE ese in Btn of exloee Nehavios, inanimate aryglaid damage See t hve an ovr aming ell, whee “jut! simon ay tee amp! sche agyessive stacks These obser Ise te oppo thatsome abnormal earenive inl ay have amyloid Sete (ethaps in the ov of eave Soe fire andi srg alain of the any Talay have sherpa eles nhs rou (Mark and Erving 1970) Tis 3 vry conto (erst ae hat dteret nore ation. Ts tle ens andra how a sen epic shares ins brain en be a Sat hast feta nent re. Sine parents howevetenporolinbi=ppsy fray sao be socaed wah longer bebe Tor changes Some ofthese take he fom of Sere peony vty wheres others may remibe comenconal payee syndromes vedo cron wel 5 quate Sie ave ped ona eae racer ister fehl te hs patents Some ems wih eporlinbie peey 2 Sacer wrk were snarl wth ese rosa glance Tey may oka sos of Tumor bce eri eran reodng e ‘Shpexcsive pisos ete at bse: ‘Seles and 9 cvs tendency ‘the Cael ay show ili Tighe pom drag emerson, become at ieminble ds and psy hr ‘ei seman of hough pens Fan moy matt wide mood wings oc Sonaly"upecticatic agree exon, shales in seman fom of Innosnoty a a! eal pre, The feten anton etsy ad 0 She sing sy of erpersna erin thus een decbd steer aco ty Theres good del of Iba wheter hse tsar pear to tcp eps tdconnie a specific ier! ebanorl sy re So fas yenrani se festre that Sea to fave the Cet mpc fe otc pny (len 1975; Bee 199%, Sherer 380) Tbe ave, oo al patios with pari bicep hve iss of beatin at Cones nal pacts whee ern say have tooporolnc epee. Bar wen ths behavior ne ke esa of ruony, byperelgiosy yposeal ier) aed dinate he apie pe tute cnian shoal hve a igre of ‘tion for the presen of tempol ip lis iportoe to understand baths de scion das wot eet inves bap perio the wring or have song Telipoes fe but rater totaly extraordinary devi tons rm normal bchavior. For sane some ii tsc paren wl ave male eli coe ‘ern fom ove ble eaten fo anther wth Ulamenealyopponel views Orsay mnie ta extant nse co pose exredingy ong ites (at they may ever sai forte ee peatre of wei. A potent nt developed emporio epepay Teme age and had wo por tendency for ex Sane witincexlsned ashe urge exes Fetus fg wring ae tne that se would wit = pee er had trom of s ped emake tre tht aobing orth weg dows would be mie Aver ren cece to copy. Webster ditionary racy poe ia lnghand ih adit to thee iebavioral ria, emo ronbicenlpay ba to en decid co Iomtion with tore conventional pyciaic Indes sch shiner poy, Micive dee, wile personaly, comer din eacions ahd chstssiscompaive disor de The arongst ascii with Seba fret conltons and depesson. The ‘ouch dstnce ml higher ‘polities tian nthe pe ra pelo (Sever a Pead, 1963; Me S13 Cams and Uso, 98) conree te idee opens patient with the trenton encom of temporainhie equlepsy generally lack 2 fanily Wstory of psy hosts, ely shove temporal detectration, tend omaiatain adequate interpersonal contact, and {do not show Hltening of affect. Bue these pa tiene do have hallucinations, delusions, and dit- tincly iigryneratic paranoid ideation. There is some evidence indienting that left-handed women who develop terporaimibiceilepsy on the basis of cystic oF hamartomatnus lesions in the Tet Ievispere are the mon prone to develop sehizophireniform reacions’ (Fiylos, 1975). Clinical observations suggest that in addition to patient with depression and sebizepheni foram states there may also he a subset of ps tems with multiple personality, hysterifrm ‘conditions, and obsessive-compulsive disease in ‘von dhe temporotimble eiepsy may play an innportan etiological role, This isan important cosideration because some (bur nora ofthese Patients may show behavioral improvement ‘wish antiepileptic medication ‘Women with the paychiatrie manifestations of temporolinic epilepsy usualy show a charac tergtic exacerbation of synproms in synchrony with the mensteual eye. Sometimes tis elect is very much exaggerated and ean asist in ris- ing the suspicion of underlying temporolimbie pilepay. Paints with temporoliniie epilepsy ay also develop major abnormalities in the Uypoxhalamopitstary regulation of endocrine faveian Thir should not come as surprise, be cause dc amygdala, which is very fequently the site of abnoenal epileptic discharges, is mono- fynaptcally connected to. the ypothalanms Consequently patients with temporalimbie ep lepsy may develop inletiy, testicular acophy, decreased sperm mori, ecomettosis, or ey tic mast and other maniestations of poly Cptic ovatian disease (Herzog etal, 1986), Sometimes these endocrinological abwormalites Improve on antiepileptic medication. The char facteriaic hypocenualty and some of the other Fehavioral manifestations in patiens with ce porolibic eilepey are undowitedly influenced br thete endocrinological problems. ‘The almormal physiological state ofthe ep- leptc focus i= mot confined ro the ital dis-ccHarrun 5 Mt charge. Evidence obtained with positeon emis sie tomgraphy shows tha the epilepi fos isin an abmorial hypumetabulic stave even dr Jing aerial yates (Ral etal, (980). Patints ‘with temporainbic eplepsy therefore have @ fontinno line dysfunction characterized by Sibrupe Nations that eange foun cereal storms to abnormally low levels of metabolic e- tivity Mocause the lnc system plays a pivral foie in directing emotion to the appropriate ‘menial cantenty these patients are subjext to an Cnpeedictable, distort, and inappropriate ‘mapping of the affective one eno chongh and xpetiense (Mesulam, 1981). The resleant li fupron in he fabric of mental Bie and abu the ts attempts a reintcaducing fnterna cy nay vullectively Tend to dhe associated paychincc sympomarcgy. In some patients the major outeume of the tempocliobie Tepsy is to intensify the affective coloring of ex- perience, Ti state, whic has bec descebed as inc hyperconnceaviy” ea fuel some ofthe Inchavioral ais (hypercligiosiey, bypergraphia, and s0 on} described above (Beat, 1979). In ther patients the affective mapping may bein hgruous and clatorted and may lead co the Schizophreniform and dissociative sates Epileptic discharges ea result From many dit ferent causes that ater the electra stability of the neuron In moet eases we Jo noc understand what eriggers 2 specific seizure eve. Ina small number of patients, however, epileptic dis Charges are triggered by very specific sensory Simul This condition & known as reflex ep Tepsy ln some patients dhe tigger may bea ee tain color ora certain visual pattem In thers in extremely specific, nach 38 a verain piece of Imusic o the chiming of a particular chuzeh bel, ‘When sich complex triggers exist, the focus of the eplepey ie commonly located within the temporal lobe ‘Sometimes the effects ofthe sultan pile ischarge are eely confine to the behavioral teal, For example, in a now famous ease the Sigh of s safety pin innced epileptic dachanges that were assosatd oth intensely pleasure frome felings (Machel, Faleoner, and Ul, 1954). ime, ooking a dking aout the LAM /NUUKAL SUUSTRATES OF BEUAVIOK 103, safety pin became a maja source of sexual grat iicnton for the patent. This clinical case sug tests that eflex eplepry could hecde an etior Joical facia in ftsisn. Anosher example is provided by 2 patient who complained of abrupt jand unprovoked tied Muctuations. She ce- Forced fling intensely depressed and cearil Irn listening Wa cermin pee of dance mse, Since the imisie did not sound paricuacy Inowaly to ether observers, ad since it was not fssovinted with any paniclaely sad event in her iif an eleetecencephslogramn (EEG) was ob tunel while she was Fite rit The onset of Irsic wae aaocineed wih intense EEG spike UTscharges, depression, al cryin There were no exer sensory, moter, er autonomic maniles: tations of eplepoy. At Teast some of the mood Swings in this patient could therefore be ex plied on the basis of comples reflex epilepsy. tia very small group of patient the reflex epi Jepsy leads toa feeling of sation. Such paienss nny seek the triggering event and work 10 i vce seizures (Ames sind Safer, 1983; Faught et Sl, 1986). In other patents the eeflex epilepsy an be ttggered not by external stimuli but by inwernal mental event, such a¢ reading, Joo- ‘ing, or mental arithwetic (Anderson and Wal lis, 1986). “Temporalimie epilepsy i 2 major imperson- savor of poychiatrie wnditions. A high index of Sspicion forthe wndelying neurological diag rossi important ei necessary to realize that the foci in temporolinbie epilepsy, because of their Tocaion i the basal and medist pares of the brain, are particulary dificule o detect with the help of eoutine sieface EEGs, Therefore, fr thorough workup should include 2etivaion Drocedures (for exsiple, sleep tacing) and ‘ho specialized nasopharyngeal and sphenoklal Teas which are beter at sampling emporolin= Ie tracures, Obtaining che tracing during pe- Fouls of pariclaly intense eympomatelogy is flso sels Negative EEGs do ot atomaiclly fle ot temporolimbse epilepsy ie & possible that he patent wae steed ate when spk fog astivity was absen or that the placement of the lecrrade was mot opi T have seen patients with temporotimble epifepsy in whom antiepiteprie medication fae f feped to alleviate depression, panic tink, sind Hallucinations, Sach sneication 6 asely ies tpt for the scophreiforns al iss ioive conditions. In general, patiants with the cnjantion of enpanobnicepleey aod psy diate symiptoniatology ed inetd program of reatne tha inches atepleic toanagement, paycoderapy, soa psrcisctive twedicaton, Croup therapy or pacts. wth femporatinibic epilepsy may be paricary fecive for shaving highly smal experiences and dispelling the sense of uniqueness. The pysican who arsnages sich pate wselly fas base inthe mide of ot alli svina-budy eons. Wher dealing sith al tiring symptomatology the patient may fre: qui ak, Hs has ne eis x eizre?” Te Answer to thie very ict question neds Wo be Individualized foreach paticn Comtral of Autonomic Function Emotional states are associated with specific paderns of autonomic responses. I is therefore tot sueprising tat nbc eeyons shoul al partsipate in the relation of sutoniic tone The amygdala aia hypetbatunes have po eeful influence on soto function, electing the lget connections they have with autnonnic fall the rinses, Elsi sation the eorial components of the Kashi systems (Gorexaiple, anterior insula, ent oxbitotrn- tal eortex, temporal pole, cingulate gyrus) als Fess in marke! an consent atone Spouses. (Kaas, 1960). Ins stimulation tends to. prodice gastroiniestinal responses, whereas simulation of the other cot eon oents lls to cardvascatar and resiestory hangs, Sonne of hese respons are ite d+ mati an ay include nbbiion of pase per inal, respiratory arrest, snd bloal pressure changes of as el as 1 mum of never. Be duulfocal cardiac secs can he obuinel ie eardirasclar sven soe with min Aisese receive electrical stmnlatian ine the aula exburontl etex (Holland Cons 1979), 1 ko hat vil emstions, ao «ven difeten eopsitivestates may he narocated ‘wil relatively speci pattern of auton ae Livation, The inponaace of Haters be tsecen inet state ann aunaonic acvaton pattern i el owe etc practice, For ample, niestal ates ean increme Dh pres sure, pridote the loratiear of uleees, Lead social esepeayeat sol ma eve imdace pte Teal eardie scythe the Senee of etek wasenla pedis feta. le decd distuthaneeia the relationship of mental onent uo anionic aiviy is thought tobe a nugial Ineoe i the genesis of ney psychosr tet daca. Cental the illuence of al state spon awionontic activation i bot ‘nora mil abuoraal snore is coordinated princely by the paalinic arene ofthe bea ature resorch may wel devrsiae har these rnarevof the Brin prvi potential anand substrate for peychosomaic dvense extemal iypertension and eran types heat disease These ave sree of the cnsieratine hat awe ted ws the lierative designation of the ie sytem as “he iseeral vain” (MaeLean, 1949; Mesutan, 1959). Left Hemisphere Syndromes The oltwate product of yrental stv boi, Lanjarge one of te most nape vehicles dnl which thong is encoded, node sl tested Teer fend an eve sive descr comparing hon to Api lod that shale a shower of words. ho the boloical uy of dh its ere Fee, great sides have heen made inthe stay ‘of worl and Fangs, especaly theo the xaminatin of brain sous that give fise 10 aplasia (em ad Gesell, 1985). Aphasias and Related Conditions fn ait all cera al abe 60 percent of sinisae, the Fle hemaphece i deatinane for bana nese inivida amage ve the lel Iremispce ykls severe aul acensonally pe t debcte uf Langage (hat i poe),cuawren 5 whereas even lange right henninphere sions have ho such effect, Soe individuals display 2 pat tem of mined dominance, so that damage 1 titer hemaphere may give ese veaphasas: Very few individeals have 3 dearcut right hem spheric dominance far language fanctions. Be huse close to 90 percent of all ndividuas are Srongly tighthanded, the cerebral hemisphere that ts dominant for complex movements is ffi (but noe always) alu dominant for fan~ fuage. This hemispheric asymmetry for haml- ‘ness and langage factions s mnecof dhe mest Fundanmental biological fais about the hua Inain There is at Test one anatomical bass for this bebaviocal asymmetry: the posterior fai fs ‘iehelelshemiaphereis anatomy Fenger than the eqivalent cegion inthe right [hemisphere in abut 60 percent cf ight-handers (Geschornd and Levitsky, 1968). Although this feature may provide at avatomiclsabsrat for language dominance, the strucral basis fr handedness emiins a enigma, Froma practical print of view lefchanders and even nividals Whose fanly contains Ieftchanders havea etter Prognosis for recovery fem aphasia. In dese Uivduats the right hemisphere may be more apt tw take on lnguage functions Wernicke’s area and aphasia, The most widely sceepted neurological approach co aphasia is hse om an anatomical network containing 2 intereonnecte centers, Werickesarea and Bro- to ares (ee Pure 3.1 and able §.2). Were bickes aren aceupies the posteion thi of the fpenor temporal gyrus (audiory association Cortex area 22) alan adjacent rim of ee in fere pital lol (prannanginal and sn lar yt areas 40 and 39) [na figurative sense emmicke' aren can be concepoalized as a re ton that i mocerney for decaing the lexical Eontent of sensory input (or example, speech Sounds the shapes of writing) and also for sociaigshonghts and complex percepts with theie corresponding word equialets, Damage te this rejann gives acto 9 elaractrite y= tonne known as Wernicke’s aplasia Langonge tutpotin these patents manautsa normal me tady and chythin but is usually quite excessive APSULAM / NRUKAL.SUASTRATES OF BEHAVIOR 0s, (iogorthes) and lio remarkably devoid of tneaningfal content, The speech i replete with poraphasins words that ate neologistic or erro~ ets) and ckicent content words. The pa tion ses eicumfocutiong, fillers, and an excess ‘staal fanction words, For instance a 76-yeat tld man was brought to the emergency room ibeanse he tase to tlk “funny” while playing ards nthe following passage he is rying {Tceribe how his wife accidentally theew away Something importan, perhaps his densures (Me- pulses, 1979} \We don't ned i anymore, she says. And wit en Uses downs ras my teehick vt “Teale ay dana Al they happened oe Jiang So How eoul ths have happened? Hwtuul' ng ket happen nS Se says esa needitanymere’ sale ink we'd use Ai wif ave soy peablems yy coming ‘ooh frm se hae moss from now, OF or trea new deni. Whete my ame (eet ace fda ha se hat 1 i ges ste bons ewe ing ti Ste rend here and se an thw thet sway. (p818) Inadulition to being unable so expres thought fn the Form of language, the patient has equiva Tene difeulty in understanding other people's Tangages Testing comprebension in hese. pa- ‘Gens svt altogether simple. If asked to ease 8 Thand, touch an ear or perform other actions ‘with he Tins, he patient gives no indication of Comprehension. If asked simple questions that Fequurea yes oF no answer, such as "Can 2 dog fy?" the patient answers a andom fa al. fe wil follow conomands for whole boly move- tnens or mover Hat se axial mwsculaure {ch ag "Closerapen your eyes stand upg tur round") with extreme care and rapidity the Ceaminer confined the testing opening and ‘hsing he eye, the existence of severe compre- Fension deficits could be overlooked. itis sid thar this dramatic disodation occurs because the ability to follow whole body and axial com mands is subaerved by pathways that remain ttre the hase language network Geschwind, 1965). Ae akersanive possbily is thar these movements ate coordinated by the right hemi= Sphere. In either ense this vissoeation empha106 okaWW an aEnavion vant Two aie §.2, Chalet of apbass tl rele Yate legen Symdrane Output Comprehension Repetition Naming Wing —_ Reading ‘Wensakes Avent parade. Enpaied excep Uopaied Inspared —Inyaived Unpaid sphass —Grcumloctionsl, lors com . ™ fooniy a eonten’ tan Bocas Nowluem reduced, tat except unpaied Impaired pated — pais for rkasa phy for comple ‘pamela ersinmatic geared words Global Nth, Iipsied paced paced Inpaced—Impaizee ‘nhac mies rte ‘Gomiicaon Five, paras, nace Iwi Inpaied Usually face compre phos orcunlocutis inysted. "henson Transertcal Fes pavaphasc, paired encepe Intact mypied —Inpaved)impatee omy ccinloetonal feral rosa cna “Tanscotcal Noullgn pithy ane tact Sewetines Soneimes Intact come sie inpaved —inpaed — hesion| ras ‘nove vent paraphasie, nae Aovact—Impaiced Usually Ural irae ‘phan acilostional Topsite foreword loach, Unpaid paied tact nach Iat teresa tact, Ter Toney tgetexepe Intact lpaied oe clone ives that “commpechension™ is wor a fnental phenomenon, that ea be fa swith bes damage, and thatthe examiner bas {olook im several categories of compeeinsion ‘ite independent fom one another. nition to the tent Hogorehea and impaired compre bens, these patients so have difculty ve peating speech, nang objets, reading. and teriing-The language deley heeft ml Tonal Te cleus the various spheres of langue ha vir ned note identical in eves: ity Some patents wh Wernick’ aphasia have tore eificulty with reading (ord- bd su typed: ethers aay have more cliulty with tiderstanding speech (word-deaf subtype). The paraphase lgorehes of these patints (oon ab cll jon aphoin) een Soll full of neologine and may reeemble the Word salad of rchizoplrenia or the pressured speech of manic patients. Especially in the acute hae, patents with Wernicke's aphasia may dleobe agitated eligerent, and paranoid. Since there patients may have no elementary neuro= Togicl findings on the bedside examination, the incoherent language in an agitated individual nay occasionally lead to psychiatric diagnoses. one paye atenion tothe features described shove, such ersocs are enly avoided. In time, pratenis with Wersicke's aplasia ae key 0 de- elon rather iniferent attitude toward “iar and may be seen pouring ove massive ‘volumes of completely incomprehensible Tame fuage without realizing that they are not com- Shucating thee shoughts. The question soften raised whether patents with Wernicke aphas inte ahnormal tough patterns lsofor asin trospecton can be trusted, careful questioning ff patents who have sullered transient episodescuarrer 5 of Wesnickes aphasia indizace that though pat ferns ean be preserved during the aphasic pe fi Broce’s area ad aphasia, Broes's aren occupies ‘hepneterioe aspect ofthe inferior fron ays ‘This can be concsived at 2 region that fran forms the neues} eepresenation of words (v= trieved through dhe meciation of Wernicke’s area into the proper articulatory ouput, word Sequence, and grammatical structre. Damage ‘enter around this region gives rise o labored, Chysstthri, nonflvent, and telegraphic speech, Comprehension of spoken aid wetten longuaye is generally preserved. In soneas wo those with Wemicke’s aphasia, the patient uses few gram smatiel funetion words. He chooses content ‘words appropriately, however, and has 2 rather pithy way of expressing tought. In che follow ing passage 2 4S-yearold man with Broca's aphasia is answering she question “What brought you rhe hospital?” lacie date dor ees, G0 Iospira Dotor kept we beside To, ce day, (ctor eed me Boe Despite the prominert dysarthria, Brocas aphasia isnot jest problem of motor output because patents who hive extemely labored Speech may have no dificult using the same truscles Tor singing. The dicrepaney between Singing and speech is ore of the teasons why roca’ aphaies are sometimes destined 38 hsving an apraxia for speech. But roca’ phasio ie more than sr apraxia, becae p= {lens seem co have sped difclty withthe grammatical aspect of guage. In adicon to theveayrannmaties speech outp, these patie Soo have a relatively sectvedifclty i eo prchenling complex grasimatialconstuctions End even in reading grarmatcal words. When Srked questions that havea complex grammat al snore (or example, "If tiger i eaten by $n, which anal stays alive?) they show Considerable dificil. reading they have litle Tike Wenulying long sul onto mons fnd verbs bur eannot Weatiy small function Swoeds such 35 Wor abe, Henge it appeats that Mestad / NEURAL SUBSTRATES OF RENAVION tor the role of roca area in word sequencing nd iramnnatical siuctureexteus to chestage of de oding aswel ao thae of encoding, fn addition to the features described above, pasiens with Thoca's aphasia have dificult naming objets, repeating speech, and weting (even with their Jac lle gnu During epic they have he ‘mont dificulty with stall grammarical words Patients with Broca’ aphasia almost always Ihave an associated right heniplepa, because this region iss0 dose to the awror cartes In contrast torpatients wth Wernicke’ aphasi, chose with bres aphasia are usally very frstrated and depressed and readily display the carasrophic reaction during examination. Giobat aphasia. Some patients sues damage to both Brocas and Wernickes areas. They develop 23 very severe aphasia chareteried by an ainost tora fail of speech ouput and comprehen- Son. Even some of these ratients, however, a uentvey follow commends aimed. 3¢ axial musculature. This syndrome is designated 33 lobal aphasia, “The disconnection apasias. Some apasas o¢- arr not because of deer damage to Weenicke's fr Broca’s area but becaie the interconnection Of hese areas with enh eer ad with the est Gf the brain i inpaired. le eondueton aphasia. the lesion i located ether ia fiber bundles (such ts the arcuate fasciculus) shat iterconnect We rickes ates to Brocs's ares in intervening co tial ares that ae likely te act asrlays between the two (Such as the neu, patel operculum, “and inferior parietal Fable). Sine the 2 maior Seeates™ af the language network are inact, these patents have relatively fee difcities with Tangaue cm prchension o wit speech fvency Bar they do have paraphase speech that is ela tively erty of eancent an! des noe express the eating they intend 10 convey. This probably fects becase there eruption inthe cor fespondence, between ihe words retieved though Wernickes area and those articulated Mirongh the mediation of Broa acea. During the examination, paeats with conduction aphasia have the most difeulty when they arefo nuain ann mesavion saked to repent speech especialy sal twords (resample, Nei, ands hte ‘Ocensinally damage the dorselaters pa iene sabect of heel hemisphere leaves Wensche's ares aniact but diconmecte i front secierasroiaton eins of che bean. These p> Went, whi are sid 0 have a transcrtial sen- sory aphasia, experience severe dilinis in amprehending language and expressing thir theughs: Because she 2 cortical centers an their interconnections are antact, however, they have no probes repesting speech, 'hs analogons ls te dorsolateral fon tal lone ncerfres wit dhe interactions between Brouss area and the rex of the Frontal lo, i= dading. the medially sitwoted supplementary Ino area, This eon give is 0 2 ens Comical motor apbasia, Patients show dra trate paucity af spoaeaneous speech output {When foneed speak, the oaeput w generally telegraphic and pithye Several features of uit typeof aphacia sre reminiscent af teocas Shasta I becate locas ates and its inter ane with Werke’ aren are stacy, the abi fy vo repent is preserved (see Table 5.2). ‘Anomic ales, Nawsing sone of the most se ‘tive tests of Fanguangefenetion. A disturbance fi naming isnot avery spicing, hovrere Iecauge Hie afeatre of verally all heapiasias described above and ea even oeeur in eonje= tion with ight hemisphere damage or metabolic ‘neephalopathies, When naming i te nly fa fuape dsordce that exit, the patient is sid to Fave an anonie aplasia. Such patients bave wo rablem wt compeehassion of fepetiion and they are able to expeess themselves reasonably trell except for cersiona parapias. Rat they Fail in eats of nang especially in those that tmplasize small parte of common objects, sich Gribe exgstal of wach, the hein of a coat, oF the up of a pene. Writing say or may no be Impatted, Left hemisphere lesions shat do wot fave ise to one of the aphasie syndromes de- Eerihed above ate usualy assocaced with an an- Simic aphasia. Anomic aphasia is also the ost anmaon type of aphasia seen ater head inary Shin the dementias ofthe Alzheimer and Pick types Athougl the presgace of aie ie not 3 ‘ery specie dignoaic sul ii very eowative Indicator of lle hemisphere dyslenci he agua gyrus andl the Gevetnann a9 drome, The ole parietal lobule sone ofthe tunst-anportant highvorderassociatlon teas ‘where complex multimodal sssocations occur Sone lesions (not at extensive 26 those giving rise to transcorieal sensory aphasia) destoy parts ofthe inferioe parietal lobule while leaving Wrercke aren mony inact. The wnsequence of such damage may include dfcultes with naming, reading, witing, and calculations, col- Tecively refered to a8 the angler gyrus st drome. Comprehension of spoken langage is fznerlly inact, whereas feeding comprehen Sion soften inipaired. On cession however an ‘nomic aplasia may be the only linical mani- festation ofan agar gyrus lesion. Soine patients develop a combinadion of 4 symptonsdificaley with efeaations, impaired ‘weting Gysgeapi}, an inability co name fin ft (get agnosie), and an inability to dstin= ph fe ro ight This eead is known asthe Gerstmann syndrome. When it oceuts i isch tion i Frequently associated with a left i [evr patctal lobule lesion, The correlation of Siner-haning cliffeltes with ealulaion dil ities sido elec he importance of fingers inthe acquisition of arithmett skills ure word denies. Deficits in the major apa tias.are_multimodsl, Under certain circum Habces, however lesions disconnect the {a network fron only one sensory snodaliy ade eelativey rave conditions give rise 10 the bamatie manifestations of re word deafness fd pure alexia without agraphia xr word deals, either bilateral lesions Strategically placed unilateral lesion inthe Ie superior temporal gyrus prevents auditory information from teaching Wericke’ area Tinse patients are not deat, sine they ave 00 culty registering and comprehending cov foumental sounde=they wil open the door tipon heating a knock and wil pick up che phone wien ings. Furthermore these videnarren 5 uals ae nor aphasia, since they have no problem in understanding writen language. Ye they ap pear deaf in response to spoken language, 28 che Shdwory information bas ne access xo. Wer- ike’ area, where iis toe deced. This 8 patent who picks up the tlephone as i ings frill assume a puzued and occasionally fms: trated expression when realizing bis inability 0 Comprehend what is being said nthe other end Sf the ine. The language outpa is almost en trely normal. Ocestonaly, belgeren and ‘anol reactions are associated with Gis sym- rome. Because these patienss may have no def in the outine newologial examination, they ean easily be mistaken as having primaily poyehiatec dasurhanes: The disociginn her fore the comprehension of written and of spo- Ken language is diagnostic and distinguishes these patients From thase with psychiatric dix Pure alexia without agraphia. The alexis that ‘curs with she angular gyrus syndromes called conta alexi beente it represents the deste- tion ofa brain region necessary for the relevant ‘multimodal ineractions. Ths typeof slexia is linost always associated with dysurapi. Pure leva, in contrat is a disconnection syndrome. ‘Most characterially, this syndrome is seen ‘when 2 region, each supplied by the posterior Cerebral artery, are simukaneonsly damaged. ‘One componene ofthe damage occurs inthe left ‘occipital cortex, By elf his only creates ight FRomonymous hemianopis, ad sch patients are not alexi because visual information can reach the right hemisphere and then eross the spl ‘ium ofthe corpus callosum to convey the rle~ ‘ane visual information to Wernicke's rea it the Tete hemisphere. If patient with left osepital damage aso sustains an infarction ofthe sple~ ‘ium (this part of the corpus callosum is sup- plied by the same artery that supplies the occip> Heal cortex) then Werniekes. area. becomes completely disconnected from visual inp “These patents have no aphasia, because the an gage network is intact, They derstand and prenlice spoken langage quite norway. They fan write wety well bu they cannot rel even seca massa amun¥iOn 109 what ave win sects yo ene Geemetasres or dening. These parents ey aetee generale color naming dot caer hninon ete nonanope par of cael el Pant hi oation ste ay fee treading mers han 2 Tanscallosal anornia, information trom the Fie hemphere ees to be ransferred across the eoxpos ellos wo dhe lfc hemisphere in Dorder tb elst appeupriate language behavior. Spatien witha corpus eallosur lesion is blind folded and given an ject to palpate with the right hand, he wil have no prablem naming the tject or dscibing ts woe since somatosensory pathways are eroned and rect emvey the ine foemation frm te ight hand eo the et hemi sphere Bt whens siar objects placed in the fete hand, ie-cannor be named, sine the so- tosenso°y infirmation reaches. the eight hremiaphere ut anwar be conveyed co the lle side af the bran. Such a patient could demon- teat with his felt hana how to use the object, ths indicaing that he “knows” hae the object, fs. His inahty o name the objec, however, Couldabo eal tthe mference that he “does not Know its identity, This syndrome shows that Inrain lesions can Fran mental proceses and thor know edge fs mat necessarily a uniery phe rromenon (Geschwind, 1965), The most dri mati inseances of this syulrome are seen with aseular sions in the teretory of the anterior Cerebral aneries ‘Manis, Absence of speeh is deserved 26 mt fm. Mite patients need note aphasic. In a Sion to Baer lesions that direcly damage ‘ropharyngeal mor parkway, mis i also ‘seen with nial fontal damage in ether hem Sphere (inthe region of the supplementary mo- for aces) ar wih baal yangls, white mater, tind frontal cortex lesions of the left hemisphere. ‘The absence of aphasia can he demonsteate by the feta seme of these patents have mo dif fuaty oi Tangaage Gomprchension or oi wring. The stages of rounery from moist may bichide whispered, breathy and eventual dysarihrie speect On seems, the, terns aphenia is sel to describe ths syle m Subcortical aptsasis. phasis semdeanss can louie wi less th pins ie, satal Hor ves I sora to the syndromes esrihad hve, the coe spondence berween the details of the aphasia and the ste ofthe lesion is less well understood, Apraxia Apraxia cane deed as a cateyry-specificin- pairment of sill and purposetil movement in the absence af elementary motor defies Hen- ‘motor apraia is sai to occur i patent su able 1o convert spoke commands that he can ‘easly gndersand inte the corresponding rep resentational movements. For example, the Po tient can be asked 9 show how he would tse 4 hammer, stamp out a cigarette, and sce through a saw. In the absence of the appro priate objects (hammer, cigateve, and straw), lis response o these commands enables the ex: miner w assess ideomoter pravis inthe wrper limb, lower lah an buccofacal mesculate, respectively ‘When 3 patent eannoe perform a command sed to test deoinotor apraxia, the examiner fan test compreoeasion by perkorming several movement including the correct one. I the pas tient identifies the coveece movenent, une tay assume he has understood the command, The ‘xauiner ea tet the fee thatthe pale has > relevant motor impediment veker by spn ‘neous observation or, much more eet, hy pesformiyg the correct movetnunt and ashing the patent 20 signe it. Sues, imitation thoughe nor to requie the language network and is often preserve in patents with ideo motor apvaxis. Occasional dete patients ca ot imitae, and in such cates one may ase that the engrams for the pertinent complex ‘svovemencs (even for their isto) ate lac ied in de paris of the eft hemisphere that have Been dang, Mconoor aptais 6 pevernly axsocaed wv ko that ascot the Lagat staat ol the Nema Mente wth. Were phase tet he sey sane sanporea fens ae asked to perform wholedbody move ‘ments sch 8 *Shovw me how a honerstande” foe *Shiow me howe you woul dance” they have rach les dlficuly. In weder to explain thi, has been assumed thar the coordination of whole-body movements are Iss dependent on the movor cortex ofthe left hemisphere. Patients with deomoror apraxia illustrate the pitfalls a sociated with using lib commands a the sole rmemis of assessing comprehension in patents swith ein damage, Agnosia Agnsia ean be defsed as 2 percept that is Stripped ofthe assosations that ive ve mem, (euber, 1964; Danio, 1985). One examine, Prosopagnosia, wat described eaclie, Some pot tients nee agosie wor ly far faces but forall visually prescnred nbjecs, These patients are sai ro have a modalty-specifi visual agi, ‘Thar percept is relatively preserved exm be show by their ability today the aes or ree ‘aqize those that are soe appeacaves, Av lami patents exer name the het but can alist always serie ie purpose and we. Pa tients with visual agnenisexanor name the ob Feet and cannot deere its wey Dut fey con demonstrate the ws ofthe objcet and eve vn iif allowed to roneh it. Ocsionally the apne ‘in fe malinodal, Therefor, allowing the mdi vidual co handle che object does nor improve rec ‘ition. Visual agnosis xd multinol oheet saunas ost commonly acer wth flan te sims in the acciptoremporal region ef the Iain, Steaegically placed, siloyerat kets lesions cam als extn this ayndsome. Lesions thar cause agnoia scone the sensory sex ation regions of the brain fear the langngecuarren 5 serwork al ali fom the sie sytem. Ths conor inloriation abut bjecs fa evoke the appropriate aiciations, memories, an words. Right Hemisphere Syndromes Paris with ight senphere njry de to lina diypay iit with oem ange functions, Upon rapid. bedside examvnatin thee patients may therefore seem to have be tavioral impairments that ate substanvaly nie hao the fed apasic a superficially inulerent patents wi felt hemisphere ing. Uh lstnetion ha ec the designation of the iaphre a the rigor or nooulinant ch, lever, Trai. Subsequent re Shaws that he right hemisphere docs have roe in several wary important of ichavior These areas include attention, vis Twspatial sil, faa! reuogpition, affective be havior, aud. paralngistic communication, ontast eee eager damage, whi ends to aphasia, acai, and apraxia, damage 10 the eight hemisphere i characterize by fem spatial nego, anesognoss (denial of ines), inapprupriateafccive a paling hela inwseehnstrvcionaldefits (eosseuctional apraxia, vistospatildisrbances, al dic ts wih dressing (tessing apron. “Tis lifeence inthe hchavioral speciale sions ofthe 2 bemisperes has generated ber of dichoxonves, Fae example, 9 ees 0 the loca aa! vera Left heoipler, the eight Irinpere a evn sharacteriel onan, processes that ae steaive, ante ad hol tic, Fonthermore, some hivestigators ave su fev das the igh emipiere may ete site APrmetneceus prin There i hile supe pore for these sweeping genetaliations, which fhove permeated nvich al the papular terse fon hemopherc spvialeration, "The aseciaion ff de right hemisphere weit sncnscin pre ech atiae Irom fhe taken pression that taverbal mental uperations ca Be exited ‘vith those hat areuncoscony, The one lestly stabs ft i hat the 2 hewnapheres bee willy sivergent havior ypeiaiations al sunstn.an /UAR SHS THAMES OF MEVAVIOR LT ‘hat tis ivergece hase om illerences in the Syautts sel fr inematinn praceaning. Left Iamispere processngis edited oy words and their inerrlaineship, whereas he beri Spiere processing is neied by 2 hetero ‘ans tf ogi sy Attention and Confusional States ‘The waking inva sjete vast an this of senny input and to ge supply of thongs generated bythe Ii self Meeause the aint of aaa formacon i key fncet the procensing expaciy of the nervous System, aly segments the sina hase ean feel wih at any glen ne. Furthermore, the part of he sci comple thar ts most tle ont for achieving goals wf irmedkate impor tance (nd hese cu range fin ln een ing revsling deta dlesmas) keeps sfifing trom ne nnn 0 another ia mat that ells de inser neds he nvida, the dictates ofthe crvonent, and the expe ‘ee pane in the past Hence deze nee for Pwstlating 9 neural econ thar regulates the foes nf cnmciousnens. The wor alention israel sea gneric tern designates family oF ann ose areay that ito ap ‘al Froth stn 1198S, ‘went cin be vide into 2 maj esac Hrs, tere i A’ matriy or sate Fonsi that egies the poster of concentra ‘oF vince the efciency of dee tectum, ad the siguabienove rai, This class ft atienional processes has heen associate with the rete setrating system. fn seo 10 fnaeonakery nthe bance, his ste ie Cndes thalamic compmnents (epectlly” dhe evil and inane le) al also pais tlhe cerebeal coco, expel the pret fal riya the Beare. The acc cass of at ovenaly dstaeting ial pens cui orp #8 vicki that ep Tae he dieevion an age of ett oe ofthe my ebay eleva spaces (ext rerum nem, ean, visors, a Sefoci. This component i we attention and ener plex network of cortical srwctives anal te Eubeortica! connections, There i renin he love thatthe sigh hemisphere is specaliaa for both type of atontional processes, but expe> ily Yor the vesor aspact of direc seneey in lea prseice ce freely enconens istarhances In the matrie sepeet of stein “Teter confusiona states is rwmenines ed 0 designate these conditions. The clinical pretare fof a patient 1 an acute confasional stare i f= miliar4o most physicins, Atenion either wan ders aimfessly ori suddenly focused on an ite vant stimule that becomes the source of Gistesctiom, Thonght and sled movement also become vulerable to interference. The paient may volumtcer that “concentration” and “think- ing sraigit” require great eflot. The stream of thought loses its coherence hecause of frequent incrusions by competing thoughts and sensa tions, Skilled-movement sequences, even those 2 automatic as dialing the telephone oF using tating utensils, lose their coherence and show signs of Usimegraion, perseveration, and petsstence, When asked to recite the months of the year in reverse order the patient mays "Deter, November, Octohee, September. October, November, December, Jannary” sho ing the ality wo inhibi neraion fro cus omary bu inappropriate response tendencies. “This cnet desertion highlights she 3 major features of confusional site: I) disturbance of vigilance and heightened disteactbiliy,imper= Sitence, anni perseveration; (2) inability to Imaingain a coherent stream of thought; ane (3) Inability to eaery oot a sequence of goa-drccte ‘Aduitional mental-state dees ae also com monn confusional states Percent dstortons tnay lead to ilusons and even halludnations. “The pavem i often, bt nox always, dieiented an shows evidence of fuley merry. Mi a fom, dysgraphia, dyscaeulia, and estrus tional deeite are commer. Jedgrment ay he Faulty, insight appears Bhoted, a llect ie qui labile, with » curious tendeney foe face” as tn, Se fh te ps fees the patient sales ‘ei the asysition sage a ear ak cng anproven, Calcagnie aprede tenstatel when tote mentally may. pree Sve accurate who ae patient llowe oti Fron and pape, Oke dette eal sate exsinpy poor jac aoa Tallies Tina) nye atesed ndepenly by. the “nderying poy tha ves re to dhe come fasion sae. Lee important co realize cha in confusional states there addtional dees are by Aelintion of fee importance than the atten- tions difelis, “The most common cause of 2 confasional state is'2 tow oF metabolic encephalopathy (cowsed by polypharmacy, renal falar, ard she like). Some toxic metabatc encephalopathies, specially those aseociated with withdrawal sxates, may lead vo agitated and psychotiedelic~ is. When aspects such as hallacinations del slows and agitation become more prominent than the attentional deck in 2 metabolic cephalopathy, then the designation of “deli ium” of ctoxie payehosie™ may he mre apro- priate than. thar of 4 *eanfusional ate: Metabolic encephalopathies aee nor the only nice of confasional states, They may als aise betheaule manifertuion of sshdealhemecoma, seins, sifoca hein disease (such as sys teiclaps erythematess, femal, vasa, for degenerative deena), al even Toca cere br nfarcion. Two major eypet of Coca strokes give ise to coafusinsal sates. Fis strokes in the medial emporo-occiptalconcx of exter hemisphere an trigger agitated confsinal states. These pa tiem sally also have wensbield dei. In nher group af potiews nnagtated ate Confusion states may ase asthe major mani Fesotion of srwke inte inferior parietal ible and the inerioe Fromtal jes ofthe ght hemi Shere: These patients may have very fee i any, livia! newrologial signs, aid the presence ‘fa cerehrovaselae accident canbe overlooked The necurrence of eoafusional sates ith uot Jateral sees inthe eight side of the rain hascuarren 5 t fed tothe suggestion tha the eight hemisphere smay have a specialized fanction in regulating the atti aspect of attention. Sn farther support of this hypothesis, some investigators fave re ported that pavents wth right lemisphere dan ge tend ro show greater deficits in vigilance (arousal) fonctions than chose with equivalent lef hemisphere damage (Boller et, 1986) Hemispatial Neglect “The evidence in favor of right hemispheric spe- alization forthe mix aspects of stenton Suggestive but not definive In eonteast, conch five evidence shows that the right hemispheres ‘Sominant in determining the distebuion of se leeive semory attention within the extaper: sonal space (Mesular, 1985b). This conclusion ie principally based on observations of pacients who develop contralateral neglect a5 2 conse ‘quence of unilateral brain damage ‘A convincing body af evidence shours dat the left hemispatia! neglect resulsing, from right brain damage is far more common, lasting, and severe than the analogous tight hemispatial ne- leet thar emerges after injry t0 the lft hem: fpliere. These observacions lead to a model of Femispherie specialization whereby the left Ihemigphere contains the neural mechanisms for atending only 0 the contralateral righ hemi= space, whereas the right hemisphere contains the mechanisms for atending nor only to the cane tealatral left hemispace but also co the ipsilas~ tral right side of space, According to this mode, lef hemisphere damagelends relatively minor centrilatcral neglet hessuvethe pilates ‘ion mechanisms ofthe right hemisphere eake lover attentional processes within the right hemi= space, However, ight hemisphere damage Feds to severe lle unlateral neglect beens the Tee hemisphere Joes not have ipalnteal stenion nechaninms In keeping with this model of damn inance, right hennsphereiney also ea to some infin uf rected attention within the rig hemispace (Weintraub and. Mesulam, 1387), The emergence of this ipsilateral neglect, suppoes the presence of right hemisphere spe ita theta of atemto ea the extapersenal space, The right hemisphere Sminance forthe disibuton of tention 8 prcbubly as powerfal au fundamental asthe Tominanerof he eft hemisphere for he formal tse lange. The le ele pow tay prowide an anatomical substrate forts ex Ihara aspmnety (idelberg and Gelaburda, 18. Sever lef wiles eget sono the mast dhamave,oseurenen in cia! neurology Some pavens wih this syndome may Tal to trom, prow, or shove the lade a the body they ay fai et foun onthe ee side of the tras and they may fo real he fet side of Senence. In mille ees tbe egies may he Conlned to te phenomenon of extineion that ‘Scone daring the bedside maneuver of bist tral simultanaos simoftion, Hemspaial ne- fect can suri the shee of primary motor Ur sensry dices the patent ignores Sensory rents within he fe hemispace eventhough the tecesary senor information has acess iat the panes ban and even consciousness. Thos bemtapatial neler refers 2 fre of looking, lsening, and reaching but not one of sei, tearing, or moving. Its an actenional dit thar cannot he explained on the basis of more tlomenary svsny or motor fre he p= Tints ake draw the fave of a clack or 10 copy a spe igre, he ay mie dtl on the fetid; Waste to rea, he may ignore the worn the lsd of ee nsf gen pen Ind paper al asked to write, he map scree che word ono she right side of the Page Tests hued om te detection and eaneelion of tantsom a page ring nt nelet Behavior and belo quam entont (Fre $3). Arlene feral omponents sh been tied n nls. Hin thera seaory compo nc: ents acueing within hele hemipaee tae a diniysted impact on aware, pe: Gilly H competing events ate sotaneonaly taking ple fn the ig a, Thin hes den Cntrated wish phenomenon of tino. tn sh mane he pt en dete ine ie prec waa, When the114 BRADY AND URHAVIOR ‘@ awe ake itl al she ‘As: Altgh the patent ha vail defect he had mashed elt neg same stimalaion is presented simultaneously from both sides of space, the patent Fie to te- port the stimulus onthe lef sie. Lesser degrees of extinction can 30 occur in Be coment af other nical spideomes (for example, callsal diseoancetion oF sensory dysfunction), But when extinction multimodal or severe when it emenges in eonivnccion with auher manifest ‘inns of spatial inattention, i reflects the eneory omponent ofthe neglect syndrome The second component of nelec isin the relay of motor tutpet: the patient shows 2 eeluctance to diet orienting and explersory movements toward the lec hemispace. This ear be demonstrated by Iindfoding dhe paent and asking him to e- treve a small target rom the tp of tale eth the unaffected eight arm Revival under these ‘conditions is muds snore ecient on the sight side of the tale wlen compared with ae lle scl. Tied, shee i» imbie or ero eo ‘ponent weglec: the patient behaves aa noe ing ofiemportance could he expected to cen in the lef side of space. I seers aif he lef ike ‘of space hecomes subjected wo an affective deal tation. AS part af thir devaluatin, some po tients develop the caioas phenunnenn of ane sigosia, mentioned enter. They devy the existence a left hemiplegia and sometines even xpress the delusional hele! thatthe arm be longs 0 anothee person. The plienanarnn of anosognosia may prowde a ne! Tor studying, the bolegiealbaecr of denial sates le keeping withthe hel astral complexity of negler behaving, investigators have sown that the brain eepons involved in neglect makenp complex cerebral network. The 3 mah artical components of this network are tn the fra cortex (including the Frm eye Bly area sce Figure $.1}, the posterior parish cones (areas 39, 4,7), and the cingulate yrs. Se etic ebmponents are found in the baal ya si, she sala, and the beastem retowlarcenawren 5 foxmation. A diferent represemation ofthe eng weitnnene may even wan enc of the msi “Conical components of hi networks #530") ‘Shrcsenttiel mapof the extrapersonat work! inh posterior pata cortex, 2 map forthe {Taurbutgn of mor exploration inthe frontal lateg, aa a moiational map fo the dit bution of eevance che cngulce gyrus. These 3 coral regions 2% interconnected by mon jyapti pathways. Thetnterations among cr al componcoas and cated subeores truce tures may, be wcenary for an eflectve aenbuuon of atecion within the ex nal pace, Damage to any one of these siti {hte or we tie merconocerons may Bite f= toineglec This powbityexpiins wy next etre can ocer after lesions nar oni the fait ore bt als in he cingulate region, {he Troe Tobe, tt basal ganglia, and the tha rus, The multiply of leone that ean give tae to neglect represen nota chaos organ {ation fr this very importa behavioral anc ton but complex organization nthe form of W patty dstibue nerconnected nerwork inteslam, 1985). Complex Pecepnal Task and Facial enlfcton Patents with ight bran lesions especialy in the posterior aspects of the hemisphere, have & fs greater impament in complex visuospa- Gal casks than those with equivalent fsions in the lee hemisphere, In keeping with this el tionship, it ean be shown thatthe eight hem Splere ofthe neat brain fs moe effective the processing of comolex nonlinguisie percep! formation, For sxaniple, experiments based ‘on dichotic listening show 9 consistent ight ear {and therefore lef hemisphere) advantage for the recopidon of wordy and numbers but a felt far advantage fo tmelody ienication (Ki Iura, 1973). Tachstoscopic experiyents have Shown a Kft visaleld (and therefore eight hemisphere) superionty for depth pereeption, spatial localization, andl the identification of Complex geomeme shapes (Springer and Deutsch, 1981) SuSULAM / NEUMAL SUISTHATES OF BEAVION 115 Llenifeation of faces isa most complex per cena tak that i ls of great bola i= france A lef visnal-eld (and theretore eight Remisliere) superiority as been demonstrated forthe KdeniBeaton of waa faces. This ele: fsaymmetty aires potential behavior ance ecanse mist faces are distnty asyi- Inerrcal‘hos, when a experimental subjece is Briel exposed wa new face, he inirmarion in the ff sul Red (coming from ve right side i the other pers Fae) can he stown to play the mas important rte inthe store of pert= rent information. For exannple, i he subject is Shscaenly, presented with 2 photographic ‘composites, one cutsating ofthe 2 eft aides and {heather ofthe 2 eight ides of the ame Tac, he {wil tend vo conclude tia the composite made OF the right side (wich had been viewed Aheough the fe vstal fel) more losely re- fevsbles the original fee. Despite sis crucial tole ofthe right hemisphere in leaning 0 en fp ew face, severe provopagnosi (inability 0 recoprize fannie faces or lexen nilenify new fone) does not develop unless bilateral lesions fit in the brain, naggesting tha: Iu eric Spheres take pact in dhe recognition and ident- Fesuon of fe. “The lfc hemispatial neglect and complex per ceputa deficits contribute ro the energence of 3 Sudivonal clinical fearures of the right bemi- Sphere syndeome: (1) constructional apraxia that i, majors io copying simple Fi tres, especially if they also teclnde chee dimensional perspectives (2) dresing aprixia, fallcring the combined manifestation of elect forthe felt aude of the body ane lificnlty in ling the nly axis with the aos of the Ear tn (lensing apeaaia ean he demonstrate by ppeesenting te parc with a coat dae has heen Fhverte or with a sleeve cee tside-out: une tle w rearange the garment i the proper trian, the patent may keep looking tit ‘wih » pura expression while successfully Tumbling with ity and (3) dienes an spacial “osncaon alin ote fing ren in ior Steronraings. Some diicutes vith construe tional taaks may also emerge afer left hemi= Shere damage in mich ale forN16 BRAIN AN nEntaIOR Emotion t Ina previows section | stressed he role of she Tbe system iemotins, But meric pare ticipation, especially by the right hemaphere, is abo important in the aunutation of alfet nd emote. in the neurologically intact dual ene generally argues that the enor expressed through body language (conveyed "through tone of voice, gesures, and facial ex- pression) if consitent with what that person Ieee: In ations sth brain Jomepe, however, experience and expression can be distociated from each other. For example, in peudabulbar aly and related conditions, the patent may splay crying or faughing behavior thac bears ltl f any relationship ro underlying sadness oF happiness. The potential for such dissociation indieates thar dhe expression and the experience (of emocion shoul be considered independenty. Recent observations suggest that marked hemispheric asymmetries may occur she cere bral organization of emotional experience and is expression. The lcerature on dis subject can he divided into 2 eannps. Some authors suggest that each hemisphere may introduce diferent allecive perspective to experience and behavior. Ones, however, suggest thatthe right hemic sphere may be dominant fr the experience al expression of ailemtions, A cose examination ofthese 2 positions shows shat the divergence of ‘opinion is more apparent than rel Consistent hemispheric eifferences in alfective responses were Brst detected through obeeres tions of patients who bad suifered unlacera ttrokes, For example, he lack of concern and ¥en inappropriate joculanty (anosognesi) in response 10 hemiplegia isa ttiking festute of some paiens with right hemisphere infarctions bur is lmost never seen after damage tothe left Femisphere. tn Reeping with this chives! ohsec~ ‘vation, experiments in normal volunteer have Shown dha the right hemisphere ofthe icaet ‘individuals anor closely associated with the ex- pevence and expeesion of negative (dysphoric) ‘otons whereas the felt hemisphere sees to impact a more positive (euphori) perspective experince (Dimond, Farrington, and Johnson, 1976). ln she lotact brain che modsteten of morioual perspective could elect 2 comple Elance hereon those 2 relencie The gh Iemispere lees conld rece the negate omponient of thi equim aad Tend to ie Appropriate joclseity as 2 manifestaon of ex- ‘esivelefe hemisphere activity. Converely, left Fheiephere damage could lead to depression by reducing the positive component ofthis putative aietive equilibria “Twa misjor difiulies ate associated witht line of rvanning. Firat the expression af fo larity i let heipleics may bave very fe ve- Intionship tothe underying ste of feding be= ceise major discrepancies may ate between ‘evotional state and Hs expression Inpatients swith bran damage. Infact ate examvnation hows that despite their apparent joclarty, some left hemiplegics are severly depresied (oss, 1985). Second, te assimption is usualy rade thatthe despondency in response to right hemiplegia is analogous to the jculaity th cenierges afte left hemiplegia, excet that itis of {he opposite valence. Burone could also arge that the despondency of the right hemiplegic is an entirely appropriate reaction toa devastating event, whereas the expression of jocularity by the patient with lfc hemiplegia is always inap propriste. This reasoning suggests that proper tmvtional perspective (oF ts expressions more ely to be disrupted after cg hemisphere le “This interpretation shifts the emphasis say {om an interhemispherie balance berweet 209° posing emotional tendencies and toward a rein tively greater tight hemisphere specialization in thie domain, Indeed, severa lines of investiga tion suppor thie conclusion, For example, pa siengs with right lemsphere damage may show profound deficits in their ability to express emo tonal state throvgh variations #n the tone of voice (emovional prosody), facial expresion, Inody pastare, and hand gestures. Sometins the ‘most salient defi in the entire elniea plete fof» patient with right hemisphere ny wil be 2105s of emoxional prosody and an inability to impart she prope alleciverone onto behavior {oss and Mesularn, 1979; Ross, 1985). Acurren 5 teacher with this kind of lesion bitsy cop watt he oul no loge iain ca Miia her easroom, which he had previously aan ea rply hrogh changes her tone of rote patent ras toght £9 BENE ON: ae peer Uepressed beeose bis demean sider ice nced the proper variations is and Mesulam, 1979) ea an to the inability 0 impart lecive on lian, patent wth eight hemisphere ee Pay a have difeuey wndeseanding con al cxpresions of exhrs (Henan ‘Sihote and Watson, 1975) Equivalent difical st gaen ater analogns damage the Tee Ue ofthe bran, Des i she encoding re sh of emotionel expression ae aso ee ame ore aerin Fon pars of eGR hemiophere, whereas temporopac sae Tae tend tx disrpt the decoding (0m Setading) of emotional expresions (Ress Seis dhe neal eta for the encode Jarek decoding of aleve expression shows sal organization tha parle the ora aan Ot formal Lingua fanexons i te lee Payers, These encoding and decoding dfs ae Neied to the expression of lately ee erve rodulaions Outbursts of &% ae eee angen Hower, ay emai 9 sea tae ie primary else seb ated array within imbi - fore (Ros, 1985), “elspa wih hese observations on paints wilt eo fecinna, am inreasing body of ev Seen oma thames mpgs tha he a tig hemaphere i more eflectiv in both insti and decoding emorional expression. Free onal exprenins are aecenated 00 Teas ofthe face hich i predominantly srettied by the righ Hemisphere and ee Sfoateld advantage exis freind WFcmotional expresions (Sackeim, Gur, and Shaye 1976; Hele and Levy, 198). This sym ware) leads to a potential paradox. For ex Pein facenace encom the mone eS eae ell ofthe faces ely ofall within Tighe val eld fhe observer. This won erat eeate the seing fora nie nel- Btn inthe communication of affective ater sesuans/ NEURAL SUMSTRATES OF BEHAVIOR 117 ‘pecause the right visual felis analyzed primar Ty the sde ofthe rain lef) hat hate es, seeoped ables for decoding emotional sections. Posy, however his ar@ngement ext efcc the survival valve of mang one's ‘oorions less than perfectly obvious ‘premidignn 10. these observations of the exhrenion of emotions, indirect evidenes #5 Slr ight hemisphere specialization for the Se ence of emotions For example, ives caret that aonomi fesponses 10 E10 we eal were lost in patents with right eeteere lesions bue nor in dose with lfc rerntchere injury (Zoccolar Seabiri 30d Viow rn You2) Ina diferent and rather emarkable ta ments mewroogically inact subst were capt ro atmulat hemes ino sexual clin ante their EEGs were being monitored. There wi how that EEG amplicude dveng orgzse™ ie gremer over the Fight hemisphere (Cohen, Meee iad Guldsten, 1976). These ests have Rese ten cited as supporting the specialised oof the ght hemisphere in the experience of re arther support of # parative right hemi splere specialization for morionl behavior Fear aparchers indicate thot affective csease ea pemore closely associated withdfunetion rPlne igh hemisphere, For exam some 8, Sear char in temporolimbie epilepsy, tees Fee ioe more Tikely 10 be assccited with thought disorders, whereas rightsde foci are rponbtfkely ro be associated wit fleeive ds Tt gd Fadi 1977) tn individuals with soctered nonverbal behaviors: Unilateral Parietal Lobe Syndromes “The consequerees of elt parietal injury were de- reribed inthe seston on lft hemisphere syn Aromes unde the designation of the angular fyrus syndrome and the Gerstmann syulrome. Bisrarbancee of language and ealulations dom- nave dhe inal pate “The most widely recognized right parietal lobe syndrome fs said to sce neglect forthe Teeside, dressing apraxia, enastructon apraxia, and denial of Hines (anosognosi). Ths clinica picture was delineated on the hasis of very large Fesions Gwostly mors al bead trasna) shat most ceaily eamcnded se confines of the parietal lobe In Fact, small infaecions eonined fo the postenor parietal cortex may have rel steSi/LAM / NEUMALSUNSTRATES OF NEUAVION 119 ively subile wanifestarions—the extent nf ne {foot may be slight and dessng apraxia may be iment Some clinicians believe tharcontealseral Tegleet is more prone after frontal Tbe = ‘fons than afer parietal lesions, Therefore one ould avoid the auromatic tendency of dig fring a lesion inthe rgb paral fe on che boss ofthe neglet ‘ter cnical syndromes asocired wih sight pagal lesions refers wll ecoginel. AS described shove, some patients develop am ate famfnsinoal sane se the major manifestanon of Sigh parietal infarction, Other foealizing sigs fonch ae neglect or ateregesis) may eabsent tract to clic, Anorher even fess rerized cinne of right tempocoparictal strokes i 29 Slnte psyehonte state with profi! depression often secompavied hy hallucinations an dela Sons (Price and Mesulam, (985). This can Sometimes he the only manifestation of the p= ‘ea lobe damage. Hence the onset in miller Tatelfe fan acto payline state withont prior psychiatric history precipitating environmen Tal stem shoul rage the pessibity of argh parietal lohe lesion, Aldongh patients may ae Fave subtle neglecr and cortical somatoensory eft onthe lft sid, these sigs may e difie tat cic inthe face of dhe rid pyshiasie symptomatology. Diparietal Syndromes Iiloeral damage eo she parietoocciiat area tes ive oa dramatic lial pitare known as Tialts syndrome, These patients have pea l= fenfty integrating visual information, They ean hho san he visa environment ina sptematic Wray a conlition designated as an oeuomotor urea When viewing 4 comptes vinil suene tie large elect patient teu use lee ‘ication excoavely on the feature cha fall ‘within he centeal pac ofthe visual eld wit Being able to oegrate his formation with che more peripheral aspects of dhe stimu Fi ‘Ths, when faxing snr dhe pain may ie till detce the dro alae al verbally ry to reason on hae daanol would eds hy ssl, eaching the ennetsion thar‘he mst he loking st a door. This aspett of Bak ines syaronve i also known 45 sinlianagns 42a, hoover lente isthe inability urease toe band apprnpristey ta visa anger This onegh reaching dilicuty, opie ata, srs eee ‘he underying esion disconect vind nee, tion coctex fom motorareas a the femal fae, Faients with Balin’s symone wsualy shes hve severe spacial disorientation as well a¢ se, vere dressing apraxia. All consewtional ane complex visuospatial tasks are impaired. Ae ‘hough some visual ell detcis may be peace ‘bey are wot sulicient to explain the profound Adsruption of visuospatial feson ‘The clinical manvesaions of Want’ syne rome help to highlight the anatomical sa be havior divxon of wiatagal pathware ines dorsal and vente components (Mlishhes Use {ether and Macko, 1983). The dorsal visuoie fal ptiny is lrectal town the parietal gt frontal lobes and is important foe visnospavat and siswomotor organization, A bilateral inter ‘pion of this component i associated. with Halin’s syndrome. The ventral pathway, how ‘ver telays visual information neo the nls sytem a ina) languoge areas, Destruction of his ventral componee i sesoeated wit: visual anmesis (or example, prosopagnonn), coher ‘ravablinbie Jscomecton syndrower, pe alexis, and visual agnosis, A relatively severe Jatal-limbie disconnection syndrome takes the for of ie Klivee vey spndmonie deseoed tacher In milder instances damage tothe ventral Ysvofagal pathways cove lel to sinoron whete visi information is niente cope ‘ively ut fails to elcc the appropriate tens onal response, Frontal Lobe Syndromes the frontal lobes occupy approximately one. third ofthe human cerebral hemisphere, The terms preftontal cortex ad the associated from, tal lot synivenne yenerlly teler only to the Paxalbic (caudal exhitofeovesl amesion cre rate, and parolfactry areas) and the hereon, ‘add (areas 9, 10, 11, 12, 45. 46, and 49 tn Figuee 5.1 components ofthe fond loben Lan Pane-rwo sions that involve chese components ar of spe. al socerest because they can mie psyehiantc tlaorders, The chnial esse of Pliness Gage {also known asthe Boston evowhae ase) oe ‘eribed more than 2 century ago by Talons (1868), hasbeen used asa adel fr the Teo lobe syndrome. Gage wat a rehable and conse “tious foreman who became profave, rasa, and iresponsble folowing an accident during which a taming rod wae blown through bie frontal lobes, The many reports published since Hariow's paper have provided additonal supe port forthe conclusions derived front the cae of Phineas Gage, namely, chat frontal love damage «an fend to dramatic alterations of personaly and conduct while laving many enpins we Sensorimocor funtion relive innace AA wide range of bebavioral Changes can be ‘served in patents with prefrontal lesions (Mes slam, 1986). Some heenme puerie, profane, slovenly, fcetous,irespomue, geandione; aa inoscibies thers lose spontaneity curing nd iniioive and develop an spatiene blaniay of fecin, deve, memati, ac behavior (aban thers show an erosion of foresight, mene, and insight and lose the ably co delay prac sation and often the eapacity for eimone, Sti ‘others show an impairnene of ahneae reas ing, exeatviey, peeblamslving, a mena fe, Alig: temp to premanare conclusions sad hes some excessively conerete or siimeles bound The orderly planning and sequencing of com: Hex behaviors the ability to attend to sever] components snultaneousy and then ey st, fer the foeus of concentration, the capacity for sFasping the contexs and gist ofa comple ste ation, the resistance to distraction and infor, ce, the ability io folw nthisep natocrngs, ‘he inhibition of inasedite but inappmopting respome tendencies and the ably to reag behavioral ouput withow perseveration may exch become mackedly disrupted ‘On neuropsycholagieal investigation, tients with prefrontal damage may show defies ie tasks of enncenration (sels as cig som one Pex ateional fetes (the poo gated he Stroup test) tests of motor sequencing, sel Mexiiiy (he visuaverbal ves, aud hepetioacharren 5 formation (he Waconsin card sor ask). Seo Memtraub and Mesulam, 1985, for adie iow thse tests) The eas of standard rx tng are nx always impressive fact, sme po tush sable froma lobe lesions and severe feheworaldiarbances ay have routine net {Glial and even neuroprybologiel exam isn thar sre_quite remarkable. Tis Pavey of “objectives dings sometimes = Toni forthe incon oveloking the po “Boy of large and enable brain lesions some patents wih rml syndromes. Even ane wih a hsory of major behavioral i Ses associte with froma le damoge may shave impecably inthe ofc, This in beep ine with the scion that such patient re most impaired unde cieumstnces with iainal eX teal conta of behavior: he oie Sting may incest enough eternal roc to sup fos. some ofthese behavioral tendencies Frermore the sane patient who gives perfect, rowers questions about hypovbetieal soda fr morl dilemmas may act wi a trl nck of fdgment wien faced. with the rea situation The clinical adage that dgment and complex omporunent cannot be tested in the ofc fariclary pertinent to the evaluation of pax Tens wit ron tbe damage ‘Aug the tem font syndrome is used vo designate heen sper o these behavioral cach patent may havea diferent die fof the saieardefits and this de ion may even fntiate from one exam. ination fo the next The specif patern of behavioral dein probably determine bythe Sie site trai. natre, and temporal couse tthe lesen and peshap also bythe pas ee sonality of the print and the age of onset Moscinvestigeors believe that lesions inthe me Gia! and basal parlinbie components of the Komal lobe lead to afective dmteration, whereas dorsolateral lesions, involving mort the igi-endersiociaton component ofthe pretontl cores a to Ueno ene Bex: Try and rensonog, The more dramaie mani festaions of (roel ibe damage ar sen afer bateralinvlvement. In conta thedeiits 5- socal wit unilateral lesions can be vite ch sive and pose 8 major challenge for diagnosis Numerons cise reports describe patients with mmassive franal lobe parbology (for example, meningioma, lipoma, oF carmopharyngioma) teh have vartied payehatrc diagnoses for many years In genera, che abulia and apathy fan be misinterpreted a3 depression, wheress he Tabile, inappropriate, and impulsive behaviors nay taine the possiblies of mania, hysteria, ychoss, oF character disorder, Such mis Tiagnones en have grave consequences, beenise Some of the wnueriying frontal lobe lesions are treatable fderected early. Physiological absereaions in Inboratory pri ater provide some dives about frontal lobe function. Many prefrontal neurons readily ce- spond to sensory inp, some in 9 single pee Feered modi, others in several modalities But these neurens are far less responsive to the spe ‘ic pliysia dimensions ofthe sensory stimalias than ate narons in the modality specific areas of the brain. For example, visual cortex (area 17) neuron sensitive 10 4 certain stimu ori~ entation wil always fie when an object in that ‘rienation enters thie receptive fi. Im con~ trast, many ofthe visually espoasive neurons in the prefrontal cortex have live specificity for olor, size, orientation, oF movement, Instead, these neurens ae very sensitiv to the behaviors relevance of the envionsental event, Thus 3 reuron tht eesponds briskly t a stimu asso- fiat with reward may dastclly alter ity c= Sponse co ihe same visual stimutns when i be- Comes asaciated with an aversive or neutcat focome (Kujima, 1980; Thorpe, Rolls, and Maddison, 1983), These nenrons provide mechanism whereby identical physical scm ‘may cli differen resstons that rellece context fd past expevence e could be suggested thar {his seni to the behavioral relevance rater than eo the physical dimensions of simul prox vides the cuca substrate for realising that ll is nor yole tha glee.” Without this type of ‘neuron, given stimolus would automatically Call up's predetermined expose cegnelese of Sonex, st that either autonomy fon the em vironment nor abstract thinking would be pose sible Mesalam, 1986)Despite all we have learned in the fast few decals, a cera sense of uniqueness i sil ase sociated with frontal lobe fanetion, te is quite feinarkable, for example, that sizable Fatal lohe lesions can remin clinically sien for manny years. Even after massive. Infontal lesions, change can often he deteced only m compara wih the previous personaly of the inivideal rather than in eeference 10 any set of ahsolate Uoehavioral standard. Infact, many ofthe ater ations assocated with prefrontal lesions appear {o overlap with the range of normal hina be havior Avast numberof improvilent, respon sible, inappropriate, and facetious individuals, for example, give no evidence of demonstrable hain damage. In contrast, the Lack uf isle damage to the pertinent cercbral aren is rare ‘oceurtencein indivals with apiasia, amnesia, apraxia, or unilateral neglect, Perhaps this ‘means tat the prefrontal cortex underes fone tions that are much less “hardwired” and that aes predominantly ae an orchestrator loi tegrating er eorial areas snd for calls up behavioral programs appropiate o de context Damage to this pare ofthe heain would thus te: sulein behavioral deits that ae context depen dene rather than stati Other Neurobehavioral Syndromes Pure Degenerative Dementia Dementia is said to exist when a grata detine of mental function, enused hy disease of the brain, interferes with diy living activites ape propriate ro one's age al hackground. Several egenerative diseases ofthe brain lead to pre dementia without any other sensory oF motor impairment” The mart commu eanse of pe degenerative dementia is Alzheimer's disease. A ual memory loss sway the intial event Jn cine, aphasic and visuospatial deficits de- velop. Socal praces and concen fr external ap. pearance may initially appear ceatively pre served. The patient may look healthy and alert, uml the terminal stages. tn keepin, with this clinical pctare che pathology is intially mest severe in libie strctares at soon inves pa Fietotemporal and eventually ontal assoc conte. True depression ean be sea ad show be treated. Not infrequently, however, the ape thy ofthe later stages can be asinerpresed 35 depression Uther dementias that are not 36 well tinder stond at Alaheime'sdisgnte imny primatly a fect he fromel Lobes, yving rise 4 progressive frontal fohe syndrome, Pick’ disease is one of the conditions that lead to such dementias. tn patiens with this disorder inappropriate and bi 2atre behavior or apathy may dominate the lin eal picture inthe aeence of ammesin, asi ‘or agnosia, This Is the one group of patents for whom erroneous primary psychiaenecaghogee re most likely toe fox. In contrast to par tense with Abpheimer’ disease, who sally Aisplay suorexia and decrested libido, those with fonral dementias may manifest overt ng and lypersexuaity, Basal Ganglia Disorders ‘Almost every disease of che basal ganglia isa sociated with major changes in mental state ‘One possibility ie thae the basal gangla are sect involved in complex belive. in keeping with cis suggestion, ii wellknown that eoey Donents of che basal ganglia (for exsenple, the ‘caudate andthe nucleus aecurnbens) have exten sive connections with agsciation and limbic simuerures. In-monkeys, furthermore, lesions onfined co the head of the caudate mies may Wve ise to deficits in cognitive tasks witout ‘necessarily imefering with mt function. eis also important 10 realize however, that many degenerative basal ganglia diseases are aioe slated witha certain degree of cortical pathol ‘ogy thae could count for the mental sam alterations. The degenerative bass ganglia dts fees associate with major mentastate alter: tions include Huathgton’s chorea, Parkinson's disease, Wilson's dissate, supranacear op {holmoplegia (Stele Olerewski Richardson sym rome), olvopontocerehelle degeneration, and Halleevorden-Spats disease, Amvong these, the mmenta-sare alterations of Uuntigton's and Barkin diseases are the hes studi, ly wana Iwleads unington’s disease tr genet tei in an autosomal dominan fabiochianren 5 to severe stophy ofthe caudate mol 0d Beles o core cll oes 2 maior ein {eTeEiponene ave chores and menabsae a terocane, The changes in mental ae 89 PE Cie the corn by many Yo The meno anger ae usally very simian the compo seer the tena lobe syndrome deste ove Evenuali patents show defi ivandfedgmens snd uy develop persve Site af epoy and bul Depression aso ommon and ay represent another logis! Snfaration ot he unesyng, nesologal nate depeertve change of Han {Git dace Become cole the Uprssion tendo blend impos ate of apathere deen "lease onefourth of elely patients with rains’ ines develop progronive me fare dang, Tite mos ceomory change cm SE sgnted a badyphtene a inden Src owing of meration. Ts retardation ses stone! eft apd also secondary fection wih lost al cognitive fnctions Bag tar aleware the mor components of Parkinsons dene ae sey whl for ee IRguhe mental changes Ocersorally an amne cation sma to Alzheimers deena Tray develop, bt this probably associ Wiah the ememitont csc of etal Futbol change choracerse of Ale Aimer inae Inthe te singe the disease, ua alloiations of ghtening nate can ‘our Pens may nor vole th forma Gn ‘anfes they ave specifiy qusione Depression is very common, Some hve are thar oeaeve depcstn #03 dabling ease: Ores have ped out thot the mir dls tocw curls wich prone he mo Srnec inmervton of the eclencenalon, Shows agenesvon in Parkinson's dete ad diac tis ay provide a biologi shatare or thedersionAnderessans are se the tmanagemen of these patent The Gilles dela Tourette Syndrome “The Tourette syndrome consins of a character: ‘ane ead: (1) motor tes the neck, face, and $omesimes lower nbs (2) vocal ues, which cam Fang leon throat clearing to snorting, backing, MesULAM / NEUNA-SUUSTRATES OF MENAVION 123 ating, and profanity (coprolaliys and (3) Tecinecompriive sympromarologs. which fnclades recurring thowghes (for example, that family memiers wil et inco an aciden, repet- itive touching cllecting, obsessive orderinss, tnd sometimes compulsive self-mudiaion, The ive end iy present in less than a third of the population wt ths syndrome. Generally the iagnosisrequces one ofthe Fst 2 components to be present fers vnacivable, however, that 2 ‘ied u pariens wih Tonrete] syndrome will Thave sient obsesive-compuisive component while the tes may be subtle or perhaps even Shaene, This possibilty intcoduces additional Considerations «0 the diferental diagnosis of hessive- compulsive isoeders There fa fan iar pattern if occurrence, even though the mode ‘of nerance as not been determined. This dsr teder is more common in wales and ha 3 typ fal age of onset between 6 andl 20 years. But iis important to este thatthe syndrome extenels Jno adiihood. “The colorful sympromarology in these pas viens may have grave consequences. The tn tleney for coprolalia or for enitting bizare noises an make a extremely difieult to com tinne with schooling o to Keep a jab. Oezasion- ily the copra el leat alterations with pssetady wr sith th poles. meinen the i fan be so violent that they lead to cervical tise disease and major dental problems. ‘The tbsessve-compulsive tendencies are sometimes ‘marked. One patient almost face divorce be ‘ause of bis eomnpusvetendeney to keep pinel ing his wifes ches, With the exceprion of these Specie behavioral problems, parents with Toure’ syndrome tel 40 be bright, intelli gent, and insightfl. Treatment of the Tourette Syonirome is usally very suecessal, proving felef te approximately 80 peroent of patients “The meinsuy af therapy is dopamine blocking nedraletics, Success in isolate cases has aso been repre wit vasety of other agents, peal elonatne. Sleep Disorders ureolpay Ine 4 shavactsntie Fetones (1) am ieee impale for daytine naps, which maycevr dasing inappropriate circumstances such 3 eating, driving, or at work; (2) catplesy—a sudden los of all usc ene, scaly est hing in falls that ocears fn response to laher aad ‘other trong emotions; (3) sleep paealysis, which ‘can occu just before falling asleep or just aiet awakening, a whic time dhe patie relies he ‘annot move (asually a bref euch by souneove clse is enough to break this paralysis): and (4) Ihypnagogic hallucinations, which may peecede ‘or accompany the sleep paralysis or the bref daysime naps. These hallucinations can be quite terifying to the patent, As in the exse of the Tourette syndrome, p= tiens with this condition may elude appropriate Aiagnoss for many years and receive napprvr Date teatment. Although definitive treatment for narcolepsy is, not yet available, analeeies and antdepressarc do provide major symptom atic relief Patents with narcolepsy may have pe Fiods of automatic behavior for whiel they re later ampesi. During these periods the patient tay be involved in complex activites, such as riving or transacting barness, Hence te clin sian must include natcokepry inthe difeental diagnosis of fugue rates. “The Kleine-Levnt syndrome ie sen in male adolescents and is characterized by episodes of hypersomnin that may range from inerensed noetuenal sleep to sleeping for many days. on ‘nd. These episodes occue 34 times a year. The peviod of sleeping is interrupted hy perils of Iyyperactvicy, overeating. (bulimia), Increased ‘mastucbation, and outbursts of belligerence, sometimes associated with hallucinations. I has been argued thar the Kleine-Levin syndrome ‘ould represent an incressedsenskivity to male hormones, such as testosterone, No specific ‘weatment is avaiable, although analeptics on ‘oyccs may help. Sometimes no specific path. tenes can be found. in other instances the pa Yieat-may have a history of encephalitis oF temporal lobe epilepsy. Corpus Calloston Syndromes The corpus callosum provides a lrRe shite matter system for interconnecting the 2 hemi pant Two spheres of the ain, When this co mnissure is inact, dhe brain functions as a whole inthe cor ordination of complex behavioral aperations Dantige 10 the ep alts leads to discon Destion syndromes (such as anomia for objects placed inthe left hand or Wleomocor apraxia in {he left libs). Another and most desmatie syne ‘rome of eallosal disconnection has been desig: nated as dhe “alien have.” In this condition the patient may ceport chat one had (silly the Jef is out of his control and behaves indepen ently, sometimes in ways that ease physic hagtn 6 the patient himself. These elinical eon- sitions illustrate how the Fabre of volition can be fragmented inpatients with brain damage Hallucinations Hallucinations may occur ia a large variety of neurological syndromes. For example, halle’ nationsare nt infrequent in tone metabolic or cephalopathies, epectally those associated with {ever infection, crag overdose, and withdrawal syndromes. As mereioned above, hallucinations are common in editions such as Parkinson's disease aed nareclepsy. Hallucinations ate also ‘eon in several otter coniione that ean be di vided int 2 lass: ital hallucinations and fe lease hallucinations. feel llucnations are sociated with petal epilepsy, expecially inthe ‘ccpital and temporal oe, These en accu in ‘any modality, but the visual and olfactory hal. lucinations are pdbably the most common. I the se ofthe eplipac discharge isin the oe ital lobe, then the sisal hallucinations may tke the fori of flashes, sinple geometric shape, and colors. If the ste ifn the temporal labe, ‘specially inthe eght bemiepiere, comple itm ages and scenes ate hallucinate. lta halluel- nations are brief serewtyped, an! sometimes lo alized co a certain part of the visual field, ‘Shi haltainatins tay also occur patients wish grain, especially as pat ofthe arn, Release bullcintions cxcut. ether. whe there isa majoepreblen with the penpheral sen, sory anparat (leiog so sensory deprivation) ‘fr hen thet central pathology inthe bein fr bor (MeNamaea, Heros, and Wallen, 1982)cHarren 5 Loss of peripheral acuity, 1 fa progrenive deat pes, macular degeneration, and cataracts, may [ead to nivid visual and auditory hallucinations in eWery individual, even when no oxer ev idence of psychiatric disturbance exists. The vir ‘ual hallucinations may igelode small animals fand complex colorful seers, and the auditory Fallucinations may eange Fom continvously 16 eating jingles and tunes eo entice songs In con- rast tothe ital hallcintions, these ate Con- Simuowe and repesive. Inaly. dese elease hallucinations are associated with fear, bit even- tually they est in aonoyance and then res ‘ation. The combination ef pathologies alter fioninthe peripheral sensery apparatis aid aso inthe CNS appears to be assnciated sah he Trost ord hallucinations, On rare occasions {ema lesions alone, espeill inthe iscibu- fiom of che basilar artery, may result release hallucinations {for exon, pedrncalae ball nosis). In those instanes Investigators. have Suggested that sch lesions permanently release Iiypotherical mechanisms that inhibit the day- time occurence of dreaming Perhaps a physi Toglea disturbanee ofthe same mechanisns eiies the hypnagogic hallucinations sezn in tarcolesy, Ths bref suey shows that halle Cinatins can be cated by many diverse cond tions that nee 0 be eonsiered fn the process ‘of diferenial diagnosis Relevance of Neurological Disease 10 Psychiatric Practice “The relevance of nearologea disease ro psychi sic practice can be fevewed fom the vantage point of atleast 4 patient geoups. The great m= forty of patients who come co cletive paychiat= fictrearment have no deronstrable strtural or ‘Physical abmormaity in the bean. Povents Trthis Bs gmp sek balp either because they face intolerable circumstances or beease they Ihave acquired maladaptive response patterns 0 life experiences. Except for the symptomatic presen of medizatio, the biological ap Broach to brain fmeion sf relatively fie a> Fistance inthe manayernenc ofthese patent Recent observations suggest the existence of AIiSULAM / NEURAL SUMSTIATES OF MHIEAVIOK [35 aposher group of ivi in whom geste Posse or devclopmenet pease {hen organaton cml impede mor Raa an choral murano eat ewig her aca sc brain So (einen and Meso, 1983), om the at vantage point individ hs yn SESSr econ group be nor be eas i ference rom tne the fit, They 3s were ely develop ately tye irate thn ave genera em espns i thd pn consis patents with the nai apc oer chartering po Baraca pn, al pes sas ‘Empire doer: Ines wen Sig thee thee acne eg “eects of brain pyslogy (ch a ae naerge dyatnton zee, an ‘Ranunrnre semi deren) De ta purse cereal otey eth the fr’ groups cn faa co have newolap eesti the wodional ese fhe er "Fis impr foc on yea. Forth arom of pats who fer sores mea secant bhai inconjoneton wth cars Neng dase, Somenmes fe presence of the umderiing ba anes nei = owen rere he figs on {futeaubac or een eve. The er gene ston common sso wt proms 8 {hs peop of pens. Thi cheer as ene ore ws thts petra of these 90 Uromes i encedingly wie ha theatre of tke son erin te sca sires of the tehawiort del, and tat an weaning of thercevan bane erase {Eifornecrte ileal pan sa lee eng tae behavioral rales tht ee te anamteiyng nels esse am i fran onree fr the sk of he pyc {Bhs oc el thre Eva pert wih aoe fpe bs apne seen el y= Ria nan so prong sursf sch eset cade eal Itty fr cong the ny sd p> tape sve fo cementing En the tenet andthe aly aa te Beleral Plone that are the meine emcees Iain age ls beg esa fv tial aeons ets of i a dee The prydunir ces 0 ree, howe twtr cha teal payne ls of mental foneton and pspshotherapy are only partly applicable w te popelation of wi thi with merle deste hthe couse of trea these pent, fe exapey the thera Pts nonverbal messages the form ofthe 3p Proving disapproving, o questioning “hn Ravan" aye miscnstrveh the patent tone ‘of woe may have Inte eli to state feling the mechani for sight may be at sre sg affect may rele sure scity tates than peyeholoiclamecedent thea iy fr abaracion may e deficient emecy sy be fly an iareangoge may notre ier a0 underying hone darsne. ta Aion to these ial eoniderations, new a8 finicnons may need be entertained pre fering prychoactive meteston. The tse of Seuruleptcs and antidepressant i tempol ioe eles for example, mst rake imo ae count the putative fle of thee agents sre thvesll (Clifford es, 1983). Despite exe potential abtactes, the pyc aun eaten of indvidoale wh neologial diseme can bec 3 rewarng espera tr the pot wll forthe heaps. The ex ference or ranaping thse patients ay Evenly Tel to te eben of a al Cheptnary apeilty haved om eases era Sion erwen newroley and paychintry. he in Shin kei to rerge from thie hteracton Co hen pave de way for ipa aeons inthe veg of metal Iaetn the References Moxa, M.ED, St dD Feo, 199, Cipgrassyadeome: redhat pene, Neal 2934-439, ‘ves ead D. Salle 1983. The sunflower sy “hone Neo. Se 39:01 sero NK and, Be Wali 1986. Aetvaton st epepsn aeity by meal arte Arch, Neo 4184-826, ean D-M, 1979 lemporat abe eens: sy “ome of sence hypercontecn. Crier wsas7. than Dando, 1977. Qrantveaalsiof ‘mirc beenr ntl lobe ely Arc. enol MSe487 Bente Le, DM, Hea, hy Rosenal, MoM, TMesulan, E) Zak, and 2. 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Neupiyeol 414-180. “The secsons on sight hemp and ona lb syedaes contin parapet goattions fo ‘Mestlan 1988s an 1986. thank FA. Davis an ne, oe especie oe perio sea nae Recommended Reading (Cle, M1953. The portals, Landon: Ae rol ee D. 1876, Thefmeton ofthe bran, London ‘Sh, er Gaschw, N1974, Select! papers on longuote ‘nl the en, Won: ee Nicen J. M. 1936, Ags, prota, New “ork Heche Peal W, amd. . rch, 1971. Eplepayanl ‘tela lain, Spennikh s Thoms