MulTiple AV MaLformations: A Case

J.C. Aquino MD
in need!
Department of Internal Medicine
Davao Medical Center
The Case
 S.C.
 48/ Male
 Married
 R.C.
 Ilang, Davao City


 Chief
Complaints:
 memory
History of Present Illness
1 year PTC
 disproportionate gait
 limping and dragging the right side
of his body.

A year later. .
 inability to walk straight

 frontoparietal headache
 throbbing, pain scale of 6, occuring at
night.
 memory lapses
 (-) blurring of vision
 (-) nausea/vomiting

 Pla in C o n tra st


Ct SCAN
 A P V ie w -1 - R ig h t A P vie w -2 -R ig h t


`4 Vessel Angiogram: AV MAL

 La te ra lV ie w -1 La te ra l V ie w -2


Angiography
 A P V ie w - 1 -Le ft A p vie w -2 -le ft


angiography
Multiple dural
fistulae with feeding
vessels originating
from the bilateral
opthalmic,
frontopolar and
internal frontal
branch of the
anterior cerebral
arterieswith multiple
dilated draining
superficial cortical
veins.

Multiple arteriovenous malformations

Past Medical History


 (-) HPN, (-) DM, (-) BA



 March 31, 2006: History of head trauma

Personal and Social


 Non smoker
 Alcoholic beverage drinker

 History of taking cannabis ( Marijuana)

and “cough syrup” at age 20-22
 Married with 2 girls and a boy
Educational History


 High School Graduate



 First year BS in Commerce

Employment History


 1995-96: Sales man ( Felcris)



 1996-2000: Farm Manager



 2000-present: Stevedore ( Davao Port

Authority)
Heredofamilial Disease


 Father: CVA (2000)

 Mother: CVA(1995)

 2 sisters: HPN,
Review of System
 Skin: (-) lesion, brown hair
 Lymph node: (-) lymphadenopathy
 Bones, Joints and Muscle: (-) fractures, (-)
dislocations (-) swelling
 Head: (+) headache, (-) seizure
 Eyes: (-) blurring of vision, (-) discharges
 Ears: (-) deafness, (-) discharge, (-) pain

 Nose: (-) discharge, (-) epistaxis
 Neck: (-) enlarge lymph nodes
 Respiratory: (-) cough, (-) dyspnea
 Cardiovascular: (-) chest pain, (-) exertional
dyspnea, (-) orthopnea
 Gastrointestinal: (-) diarrhea, (-) nausea, (-)
vomiting
 Nervous System:
 Cranial nerves: (-) photophobia,(-) blurring of
vision, (-) limitation in motion of neck

 Allergies: None
Physical Examinations
 Gen Survey: conscious, coherent,
ambulatory, not in CR distress
 BP=120/80; CR-84;T=37; RR=18
 HEENT: pink palpebral conjunctiva, anicteric
sclera, (-) tonsillo pharyngeal congestion, (-)
CLAD (-) bruit

Precordium: adynamic, PMI at 5th ICSMCL,
(-) murmur, (-) heaves
 CHEST: equal chest expansion, clear breath
sounds, (-) retraction, (-) lagging
 Abdomen: flat, soft,NABS, non tender, (-)
organomegaly
 Extremities: full equal pulses, (-) edema
Neurologic Examination
 Mental Status Exam: Seen an adult male,
standing, above average built and height,
good grooming.
 Manner of speech is noted to be soft and
monotonous with normal rate.
 Good eye contact towards the interviewer.
 Mood was depressed with decrease range of
affect.
 Oriented to person, place time and situation.
 Coherent, logical and goal directed.
 Mini Mental Status xam
Maxi Patient’s Questions
mum Score “What is the year? Season? Date? Day of the week? Month?”
5 4
Score
5 5 “Where are we now: State? County? Town/city? Hospital?
Floor?”
3 3 The examiner names three unrelated objects clearly and
slowly, then
5 2 Iaskswould
thelike you to
patient to count backward
name all three from 100. The
of them by sevens .” ’s(93,
patient
86 , 79,
response
3 1 Earlier
“72 , 65, IisStop
…) told after
you the names
five of three
answers . things. Can you tell
used
me for scoring
what those were? WORLD backwards.” (D-L-R-O-until
. The examiner repeats them patient
Alternative
learns all of : “ Spell W)
them, if possible. Number of trials: ___________
 Show the patient two simple objects, such as a wristwatch
2 2 and a pencil,
Repeat
ask the
the phrase : ‘No
to ifs , ands
them, . or buts.’”
1 0 “and patient name
3 3 “Take the paper in your right hand, fold it in half, and
1 put it on the floor.”
1 Please
“(The read this
examiner givesandthedopatient
what ita says Written
.” (of
piece blank paper.)
instruction is “Close your eyes.”)
Make up and write a sentence about anything.” (This
1 1 sentence must
1 0 Please copy
“contain a nounthis
andpicture .) (The examiner gives the patient
a verb.”
a blank
 piece of paper and asks him/her to draw the symbol below.
All 10
 angles must be present and two must intersect.)


 Mini Mental Status Examination: 22


 Sensory Examination:
 Can differentiate sharp and dull
objects
 Can identify the location of touch
 Can identify the direction of
movement



Modified Hachinski Score


POINTS
 Abrupt onset of symptoms 2
 Stepwise deterioration (eg, decline-stability-decline) 1
 Fluctuating course 2
 Nocturnal confusion 1
 Personality relatively preserved 1
 Depression 1
 Somatic complaints (eg, body aches, chest pain, headache) 1
 Emotional lability 1
 History or presence of hypertension 1
 History of stroke 2
 Evidence of coexisting atherosclerosis (eg, PAD, MI) 1
 Focal neurologic symptoms (eg, hemiparesis, homonymous
hemianopia, aphasia) 2
 Focal neurologic signs (eg, unilateral weakness, sensory loss,
asymmetric reflexes) 2

 *Total score is determined:
 < 4 suggests primary dementia (eg,
Alzheimer's disease)
 4–7 = indeterminate
 > 7 suggests vascular dementia

 Coordination: No tremors
 Can button his shirts and write
word ligibly
 Slight loss of balance when
allowed to stand without hands support.
 Finger to nose: Normal
 Rapid Alternating Movement: can
pronate and supinate the hand, however
slowing when ask to change the
direction.
Cranial Nerve Examination


 CN I – can smell
 CN II, III – pupils equally reactive to light
 Fundoscopy:

OD OS
ROR (+) (+)
DISC clear disc margin clear disc margin
AVR 1:3 1:3
Venules Normal Normal
Exudates (-) (-)
Hemorrhage (-) (-)
 CN III,IV, VI: intact extraocular muscle
 CN V: (+) corneal reflex
 (+) can clench teeth
 CN VII: (-) facial asymmetry, wrinkling of the
forehead are equal and symmetrical
 CN VIII: can hear
 CN IX: (+) gag reflex
 CN X: (+) gag reflex
 CN XI: can shrug shoulder
 CN XII: tongue is at the midline

 Nuchal rigidity(-)
 Brudzinski: (-)
 Kernigs: (-)
 Babinski: (-)
 R L
R L

5 /5 4 /5 100% 100%

5 /5 4 /5 100% 100%

m o to r sensory
++ +
+

++ +
+
DTR
Diagnostics

 CBC: N ECG: Sinus

Rhythm

 RBS: 5.9
 Crea: 85.20
 Na: 151.00
 K: 4.0

 Chest Xray: Normal
Salient Features
 Motor: 4/5 on the


 48/M right side

 memory loss of
recent event
 Headache

 Limping gait

 Alcoholic bev
drinker
 (+) history of
substance abuse
(cannabis)
 LOCALIZATION: a lesion in the
Right cortical
 area



Differential Diagnosis
 1.Vascular Dementia
 Stepwise, sudden deterioration in cognition; episodes of
confusion and recent memory loss, aphasia, slurred
speech, focal weakness
 2. Other Forms of Dementia:
 Gradual onset of short-term memory loss and functional
impairment in more than one domain:( Dementia
Alzheimer disease, Parkinson dementia, Lewy body
dementia, Pick's disease, alcohol-related dementia,
Creutzfeld-Jacobs disease )
 I. Executive function (finances, shopping, cooking,
laundry, transportation)
 II. Basic activities of daily living (feeding, dressing,
bathing, toileting, transfers)

 3.Depression:

 complains of memory loss, decreased

concentration, impaired judgment


 4. Stroke


 Diagnosis:
Vascular Dementia
Secondary to Multiple AV
Malformation S/P Craniectomy.
Discussion
 Dementia: impairment of memory
and at least one other cognitive
domain,
 aphasia .
 apraxia
 agnosia
 executive function

 Types of Dementia:
 Alzheimer disease
 Vascular dementia
 Dementia with Lewy bodies
 Parkinson disease with dementia
 Frontotemporal dementia
 Reversible dementias
 Vascular Dementia:
 19th century- Binswanger and
 Alzhiemer
 - multiple infarctions and chronic
ischemia
 - later part of 20th century,

 progressive ischemic injury on

cognitive decline reemerged

 Vascular cognitive Impairment
 TI - National Institute of Neurological Disorders and Stroke-
Canadian Stroke Network vascular cognitive impairment
 harmonization standards.
AU - Hachinski V et al
 SO - Stroke. 2006 Sep;37(9):2220-41. Epub 2006 Aug 17.

 Vascular Dementia – a
heterogenous syndrome rather
than a distinct disorder, in which
the un derlying cause is
cerebrovascular disease in some
form and its ultimate
manifestation is DEMENTIA
Epidemiology
 second most common form of dementia
after Alzhiemers disease
 10-20% of cases
TI - Prevalence of dementia and major
 subtypes in Europe: A collaborative study of
population-based cohorts. Neurologic Diseases
 in the Elderly Research Group.
AU - Lobo A et al
SO - Neurology 2000;54(11 Suppl 5):S4-9.


 reversed in Japan and China

TI - Increased prevalence of vascular dementia in J
a community-based epidemiological study.
AU - Ikeda M;
SO - Neurology 2001 Sep 11;57(5):839-44
Etiology and PathoPhysiology

 Threecommon pathological entities contribute
substantively to vascular dementia (VaD)


 Large artery infarctions, usually cortical, sometimes

also or exclusively subcortical in location.

 Small artery infarctions or lacunes, exclusively
subcortical, in the distribution of small penetrating arteries,
affecting the basal ganglia, caudate, thalamus, and internal
capsule as well as the cerebellum and brainstem.

 Chronic subcortical ischemia occurring in the
distribution of small arteries in the periventricular white
matter and leading to selective loss of tissue elements in
order of their selective vulnerability - neuron,
oligodendrocyte, myelinated axon, astrocyte, and endothelial
 Strategic Sites


 Cortical:hippocampus, angular gyrus,

gyrus cinguli, frontal lobe
 Subcortical: thalamus, fornix, caudate,
globus pallidus, internal capsule
 “Strategic Infarct Dementia”
TI - Confusion and memory loss from capsular genu
infarction: a thalamocortical disconnection syndrome?
AU - Tatemichi TK; Desmond
SO - Neurology 1992 Oct;42(10):1966-79.
 Arteriovenous Malformation: a
tangle of blood vessels in the brain or
on its surface bypasses normal brain
tissue and directly diverts blood from
the arteries to the veins.
 - discovered incidentally either at
autopsy or treatment for an unrelated
disorder.
Clinical Manifestations of AV Malformation

 Seizures and Headache

 Paralysis
 Ataxia
 Visual disturbances
 Dizziness
 Aphasia

AV Malformation
 Pathophysiology of AVM:
 1. ischemia
 2. hemorrhage
 3. Compression
Diagnostic for AVM
 CT Scan
 MRI
 Angiography
 SurgicalManagement
 1. Conventional Surgery
 2. Endovascular embolization
 3. Radiosurgery

Clinical Features of Vascular Dementia

 Cortical:
 Medial Frontal: executive dysfunction,
abulia or apathy
 Left parietal: aphasia, apraxia or
agnosia
 Right parietal: confusion, agitation,
visuospatial and constructional difficulty.
 Medial temporal: anterograde amnesia
 Sub- cortical:
 Focal motor signs
 Early presence of gait disturbance (marche a
petit pas or magnetic, apraxic gait or
Parkinsonian gait)
 History of unsteadiness and frequent,
unprovoked falls
 Early urinary frequency, urgency, and other
urinary symptoms not explained by urologic
disease Pseudobulbar palsy
 Personality and mood changes, abulia, apathy,
depression, emotional incontinence
 Cognitive disorder characterized by relatively
mild memory deficit, psychomotor retardation,
Diagnosis
 Patients with dementia may have
difficulty with one or more of the
following:
 Learning and retaining new
information
 Handling complex task
 Reasoning
 Spatial ability and orientation
 Language and Behavior

 Sensitivity and specificity of diagnostic tests for
dementia

 Sensitivity
Specificity
 Mini-Mental State Exam* 87 82
 Short Portable Mental Status Questionnaire*
 Any dementia 82
92
 Mild dementia 55
96
 NINCDS criteria 92
65
Diagnosis of dementia
Diagnostic Criteria for VASCULAR
dEMENTIA
 Diagnostic and Statistical Manual of Mental
Disorders (DSM-IV)
 State of California Alzheimer's Disease
Diagnostic and Treatment Centers
(ADDTC)
 National Institute for Neurological
Disorders and Stroke-Association
Internationale pour la Recherche et
l'Enseignement en Neurosciences
(NINDS-AIREN)
 International Classification of Diseases,
tenth edition (ICD-10)
Management
 Medical:
 a. Antihypertensive
 SYST-EUR trial
 Progress trial
 b. Diabetes Management

 c. Acetylcholinesterase inhibitor
 Donepezil
 Galantamine
 Rivastigmine
 d. N-Methyl-D-Aspartate receptor
antagonists
TI - Efficacy and safety of memantine in
 Memantine
patients with mild to moderate vascular
dementia: a randomized, placebo-controlled trial
(MMM 300).
AU - Orgogozo JM; Rigaud AS; Stoffler A;
SO - Stroke 2002 Jul;33(7):1834-9.
Summary and Recommendations


  While considerable uncertainty continues to surround vascular
dementia (VaD), the following summarizes current understanding
and offers some pragmatic suggestions for evaluation.
 The presentation of cognitive impairment in VaDmay be quite distinct
from Alzheimer disease (AD), especially early in the disease course,
with prominent deficits in executive dysfunction causing significant
disability, even while memory impairment is quite mild and before
the patient reaches criteria for dementia.
 Neuropsychological testing can be helpful to better profile the nature
and severity of the cognitive deficits and chart disease course in
VaD.
 There is considerable overlap between AD and VaD with regard to
comorbidity as well as shared risk factors and even pathogenesis.
The combination of pathologies may be more common than either in
isolation.
 There are no uniform diagnostic criteria for VaD. Evidence of prominent
executive dysfunction, a stroke history, vascular risk factors, and a
high should suggest either the diagnosis of VaD or AD with
cerebrovascular disease and prompt a neuroimaging study.
GOOD Evening!!!!!