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STAGES OF SYPHILIS

CONTACT ( I/3 BECOME INFECTED)

PRIMARY CHANCRE

SECONDARY SYPHILIS

EARLY LATENT -------------- RELAPSING

LATE LATENT

REMISSION ( 2/3) TERTIARY (1/3)

LATE BENIGN ( 16%)


CARDIOVASCULAR ( 9.6%)
NEUROSYPHILIS ( 6.5%)

TERTIARY SYPHILIS
WHEN CALLED AS LATE SYPHILIS: AFTER 2 YEARS OF THE DISEASE.
CHARACTERISTICS OF TERTIARY SYPHILIS:
• NON CONTAGIOUS : ORGANISMS CANNOT BE DEMONSTRATED
EXCEPT BY ANIMAL INOCULATION.
• THIS STAGE COMMENCES WITH A PERIOD OF LATENCY.
• Approximately ONE THIRD of the patients with untreated latent syphilis
DEVELOP TERTIARY SYPHILIS, while the other TWO- THIRD remain in
PERPETUAL LATENCY.
• THE MORTALITY AND MORBIDITY OF SYPHILIS ARE MAINLY DUE TO
THE OCCURRENCE OF ITS LATE MANIFESTATIONS IN SKIN, BONES,
CNS OR VISCERA, PARTICULARLY HEART AND GREAT VESSELS.
• LATE LESIONS ARE CHRONIC AND DESTRUCTIVE
• CHARACTERISTIC LESION IS GUMMA ( localised/ diffuse). IT
REPRESENTS A MAXIMAL INFLAMMATORY RESPONSE (ALLERGIC) TO
A FEW ORGANISMS.
• THE MOST TYPICAL FEATURE OF A LOCALISED GUMMA IS CENTRAL
NECROSIS, WITH THE FORMATION OF A STRINGY MASS ( or
‘gum’,hence the name gummata) SARROUNDED BY A ZONE OF
GRANULATION TISSUE WITH A PERIPHERAL NARROW ZONE OF
TOUGH FIBROUS TISSUE. Microscopically the zone of granulation consists
of lymphocytic and plasma cell infiltration around small blood vessels, giving
an appearance of perivascular cuffing. The blood vessels show changes of
ENDARTERITIS OBLITERANS. Epitheloid cells and gaint cells are also
present. Fibroblasts are seen in the periphery of the lesion.

LATE LATENT SYPHILIS:


• CHARACTERISED BY NO CLINICAL S/S BUT POSITIVE SEROLOGICAL
TESTS FOR SYPHILIS.
• HOW IT GETS DETECTED?
 birth of a syphilitic child
 during antenatal blood tests
 testing before blood donation.
• LOOK FOR EVIDENCE OF PAST INFECTION:
 scar of primary chancre
 leucoderma colli
 macular atrophy ---- scar of secondary syphilic papules
• ALWAYS CHECK THE CSF to exclude asyptomatic NEUROSYPHILIS and do
an X-RAY of the aorta for evidence of aortitis.

LATE BENIGN (TERTIARY) SYPHILIS: It includes any symptomatic syphilitic


manifestations after the secondary and relapsing stages that DOES NOT
INVOLVE THE CARDIOVASCULAR AND NERVOUS SYSTEM.
• USUALLY SEEN 3-10 YEARS AFTER THE PRIMARY STAGE ( HOWEVER,
VARIABLE).
• THE LONGER THE INTERVAL BEFORE THE APPEARANCE OF THE SKIN
LESIONS, THE MORE SOLITARY AND DESTRUCTIVE IS THE PROCESS.
• THE LESIONS ARE CAUSED BY CMI RESPONSE TO A SMALL NUMBER
OF TREPONEMES PRESENT IN THE AFFECTED TISSUES.
• At the beginning of the twentieth century, the incidence in untreated men
and women was 14.4 and 16.7 per 100,000 population respectively but now
this stage is very rare due to widespread use of antibiotics for other
infections---- FITZPATRIK.
• THE MORE COMMONLY INVOLVED ORGANS ARE:
 SKIN (70%)
 MUCOUS MEMBRANE ( 10.3%)
 BONES ( 9.6%). But gummas can occur in any organ.

(A) CUTANEOUS LESIONS:


Tertiary skin lesions can be divided into 3 types:
• Granulomatous nodules
• Psoriasiform granulomatous plaque
• Gummata
There is yet another variant called “PRECOCIOUS LESIONS” .
These develop within the first 2 years after the resolution of the
secondary stage. They comprise of groups of infiltrated papules ( localised
to one area/ generalised) . They tend to ulcerate. THESE PAPULES HAVE
FEATURES OF BOTH SECONDARY STAGE LESIONS AND TERTIARY
STAGE GRANULOMAS. They heal with little or no scarring .
NODULAR/NODULOULCERATIVE lesions consist of superficial firm
rounded,dull-red PAINLESS nodules varying in size from a pinhead to a pea
TENDING TO FORM GROUPS AND GET COALESCED INTO PLAQUES.
Usually the surface is smooth and shiny. Occasionally the skin breaks down
to form small punched out ulcers covered with crust. Over weeks or
months, central healing and advancing borders produce plaques with annular,
arciform, serpiginous, or polycyclic configuration. Healing leaves behind
pigmentation or scars of tissue-paper type,or skin may be normal.
PSORIASIFORM/SQUAMOUS LESIONS are the same but larger
lesions with scaling over the surface. Autpitz sign negative. Little ulceration
or scarring.
A GUMMA IS A DEEP GRANULOMATOUS PROCESS IN THE
SUBCUTIS INVOLVING THE EPIDERMIS SECONDARILY. These present
as rounded , painless subcutaneous nodules ( single/multiple). At first the
skin over the lesion is not attached and not discolored. The swelling
increases in size , develops gummy consistency, the overlying skin shows
dull-red discoloration and breaks down to form a PUNCHED OUT,
DESTRUCTIVE ULCER WITH ROUNDED OR POLYCYCLIC OUTLINE.
WALLS—VERTICAL, FLOOR--- CLEAN LOOKING WITH DULL RED
GRANULATION. NECROTIC TISSUE IS ATTACHED TO THE WALLS AND
FLOOR FOR SOME TIME IN THE FORM OF TOUGH , YELLOWISH-
WHITE SLOUGH, CALLED THE “WASH-LEATHER SLOUGH”. The intact
skin at the margin of the ulcer shows dull-red discoloration and adherent
crust. They favour sites of trauma and are more common over PRETIBIAL
AREA, SCALP, FOREHEAD, BUTTOCKS, PRESTERNAL AND OVER
STERNOCLAVICULAR JOINT. As the central gumma heals , new lesions
may develop at the periphery. Gumma is very indolent and enlarges
horizontally as well as vertically sometimes to expose the underlying bones
which also necroses. More superficial gumma heal with noncontractile
atrophic scars whereas deeper lesions leave thickened, ridged scars.
PSUEDOCHANCRE REDUX is a solitary gumma of the penis at the site
of original chancre.
(B) MUCOUS MEMBRANE LESIONS
Gummatous lesions of the mucous membrane may be LOCALISED OR
DIFFUSE. Localised gummata are most likely to involve the submucous tissue of the
mouth and throat, the palate, the pharynx, the larynx or the bony and cartilaginous
structures of nasal septum. They begin as painless swelling and later break down to
form characteristic PUNCHED OUT ULCER WITH A WASH LEATHER SLOUGH.
They lead to such complications like:
 Palatal perforation.
 Saddle nose, perforation of the nasal septum
 Destruction of uvula
 Pharyngeal and laryngeal stenosis
 Leukoplakia ---- malignancy of the oral cavity.
The tongue may be involved by a solitary painless swelling which
undergoes necrosis and ulceration or by a diffuse gummatous infiltration
( commoner) that makes the tongue look big. The end result may be-----
 DEEP IRREGULAR FISSURING OF THE TONGUE ---- this is due to
interstitial fibrosis and subsequent contraction of the fibrous bands
reaching the mucous surface of the tongue.
 LEUKOPLAKIA ----- These are irregular white patches on the surface of
the tongue. The lesions are precancerous.
 CHRONIC SUPERFICIAL GLOSSITIS ---- The sides and tip of the tongue
are smooth due to the loss of filiform papillae. This causes sudden onset
discomfort and pain in the tongue in response to irritants. IT SHOULD BE
REGARDED AS A RESIDUAL EFFECT WHICH APPEARS AFTER A PHASE
OF GUMMATOUS ACTIVITY. A PRECANCEROUS CONDITION.

(C) BONES: Although gumma is a destructive pocess, it may be hidden in the bones
by the OSSEUS OR PERIOSTEAL REACTION. They usually occur 5-20 years
after the original infection. Slightly more common in males. Bones most
commonly involved are :
 TIBIA, CRANIAL BONES, SHOULDER GIRDLE, FEMUR, FIBULA,
HUMERUS, BONES OF FOREARM.
Lesions usually commence in the fibrous layer of the periosteum
where chonic inflammatory cells infiltrate alongwith endarteritis obliterans of
the vessels ---- this stimulates the adjacent ‘osteogenic layer’ with new bone
formation that lacks the orderly cortical pattern. This leads to thickening of
the cortex and roughening and irregularity of the surface. This process
extends into the cortex via Haversian canals causing erosion and destruction
followed by laying down of new bone. THE NEW BONE IS MORE SCLEROTIC
THAN NORMAL AND THERE IS ABRUPT TRANSITION OF THE NORMAL
AND SCLEROTIC BONE. Due to this, the long bones affected by the
gummatous process are thickened. There is no tendency to bending and
pathological fracture. Occasionally the gummatous process starts in the
medullary canal and erodes the bone from within ----- SYPHILITIC
OSTEOMYELITIS. In the bones of the vault of the skull, nasal septum and
hard palate, destruction outspaces the new bone formation and the periosteal
reaction is slight. There are rounded areas of bone loss . in the skull, known as
‘WORM EATEN SKULL”.
The main symptom is deep seated boring pain at the site of
involvement, usually worse at night. If the bone is subcutaneous, the patient
may become aware of the swelling which may be tender at first. Sometimes
gummatous ulcer may lead from the bone to the skin.

(D) CARTILAGE: perichondritis of the costal cartilage, external ear and the
cartilagenous nasal septum.
(E) MUSCLE: gumma is rare but may occur from extension of the bone and
subcutaneous tissue and later lead to contracture and joint fixity.
(F) JOINTS, BURSA AND TENDON SHEATHS: the sites most exposed to stress
and strain such as the knee joint and prepatellar bursa are affected leading to
SWELLING, JUXTA ARTICULAR NODES ALONG TENDON SHEATHS.
(G) STOMACH: Rare. X-Ray examination shows a filling defect or distortion of the
cardiac end of the stomach. The symptoms are of chronic indigestion..
Elsewhere in the GIT, it can involve the parotid gland ( parotitis, gumma),
esophagus , intestine.
(H) LIVER AND SPLEEN: GUMMATOUS INVOLVEMENT OF THE LIVER IS THE
MOST FREQUENT TYPE OF ABDOMINAL SYPHILIS. It can occur as
DIFFUSE INTERSTITIAL CIRRHOSIS( a form of portal cirrhosis) or LOCAL
GUMMATOUS CONDITION progressing to an irregular fibrosis the so called
‘hepar lobatum’ ( localization--- capsule, parenchyma). Secondary amyloidosis can
occur later on. S/S include----
 Jaundice, weight loss , remittent or intermittent fever.
 Pain in the right hypochondrium. Palpable liver. ( LEFT LOBE IS
AFFECTED MORE THAN RIGHT)
 S/S of portal hypertension.
 Abnormal liver function test.

Gumma of the spleen and pancreas are rare but reported.


(I) LUNG: Rare. Gumma may heal to result in fibroid lung and bronchiectasis. There
may be no symptoms or there may be cough and sputum with perhaps low grade
fever and weight loss. X-Ray shows localised shadows suggesting tumor
formation or tuberculosis.
(J) URINARY TRACT: gumma of the bladder, kidney and prostate is reported.
(K) REPRODUCTIVE ORGAN: gumma of the female genital tract is very rare but
reported. On the otherhand, gumma of the testis is seen occasionally. It may
take the form of localised gummata or diffuse gummatous infiltration. On
healing, it causes diffuse interstitial fibrosis ---- alteration in the consistency
of the testis, loss of testicular sensation, a dragging sensation --- the testis is
called “BILLIARD BALL TESTIS” ( HEAVY , PAINLESS, SMOOTH TESTIS,
UNILATERAL, WITH OR WITHOUT SECONDARY HYDROCELE). The
epididymis is rarely involved. Gummata of the body of the testis does not
involve the overlying skin and does not ulcerate to the surface.

DIAGNOSIS OF THIS STAGE IS BASED ON-----


 Characteristic organ involvement
 Histopathology
 Serology
 Response to adequate antibiotic therapy.

CARDIOVASCULAR SYPHILIS:
 The incidence of cardiovascular syphilis in the case of late, untreated
syphilis is probably 10%.
 Manifestations usually develop 15-30 years after the initial infection.
 Most patients are 40-55 years.
 Men: female = 3:1
 Reported incidence in India = 0.1 – 2.6 %.
 In 40% cases, it is accompanied by neurosyphilis.
 The MOST COMMON LESON IS AORTITIS . THE CHANGES ARE MOST
MARKED IN THE ASCENDING AORTA AND AORTIC ARCH.

Classification: 3 MAIN CATAGORIES------


 SYPHILIS OF HEART
 SYPHILIS OF GREAT VESSELS
 SYPHILIS OF MEDIUM SIZED VESSELS.: cerebral, spinal A, carotid A,
hepatic A, mesenteric A, renal A, ileal A, femoral A.

Pathogenesis: T.pallidum presumely reaches the heart during early stages of blood
borne dissemination --- then invade the vasa vasorum of the aortic wall and cause a
chronic low grade inflammation that eventuates in endarteritis and periarteritis.
This results in necrosis of the muscular and elastic tissues and scarring. This leads
to ----
• Atheromatous plaques in the intima with calcification of the intima.
• Aneurysmal dilatation.

SYPHILIS OF HEART: (2.4%)


• Takes the form of DIFFUSE MYOCARDITIS or GUMMATA OF THE
HEART.
• Gumma can occur in the conduction tissue of the heart, myocardium,
heart valves, pericardium and origin of the pulmonary artery.
SYPHILIS OF GREAT VESSELS:
• LESIONS MAY OCCUR IN THE AORTA ( MOST COMMON), GREAT
VESSELS ARISING FROM THE AORTA AND IN THE PULMONARY
ARTERY.
• The results are ----
 Uncomplicated aortitis = 27-36%. This mainly affects the ascending
aorta.
 Coronary osteal stenosis = 25-30%
 Aortic valvular incompetancy = 30%
 Aortic aneurysm = 20%. Of these (a) 60% develop in the ascending
aorta and (b) 25% develop in the arch of aorta. Abdominal aneurysm
usually develop above the origin of renal arteries. Focal weakenng of
the wall results in saccular aneurysm whereas diffuse weakening
produces fusiform involvement of the ascending and transverse aorta
with dilatation of the aortic ring.
In syphilis, SACCULAR ANEURYSM IS MORE COMMON.
FUSIFORM ANEURYSM IS MORE DIAGNOSTIC OF
ATHEROSCLEROSIS.
• UNCOMPLICATED AORTITIS: FEATURES INCLUDE------
 Retrosternal aching pain
 Loud and tambour like S2 ( bruit de Tabourka)
 Soft systolic ejection murmer.
 CXR: linear eggshell calcifications of the anterolateral aortic wall.
 Angiocardiography: allows better visualization of the first part of
aorta which is normally obscured by the heart shadow.

• CORONARY OSTEAL STENOSIS: the involvement of the ostia of the


right and left or both coronary arteries by the syphilitic process may
produce narrowing or occlusion. IN MOST CASES, THE LUMEN IS
STILL PATENT BUT LEADS TO FEATURES OF CORONARY
INSUFFICIENCY. ACUTE MYOCARDIAL INSUFFICIENCY RARELY
RESULTS SINCE THE NARROWING OCCURS SLOWLY AND BY THIS
TIME THE COLLATERALS DEVELOP. Patients usually have aortic
regurgitation and aneurysm of the ascending aorta. Features are----
 angina pectoris initially brought on by effort, emotion and cold and
later occurs even at rest.
 ECG: ST segment depression with T wave inversion.
 Treadmill.

• AORTIC REGURGITATION: Occurs due to aortic root dilatation or


involvement and destruction of the cusps in the process of intimal
fibrosis. Features include:
 Dyspnoea on exertion / paroxysmal nocturnal dyspnoea ( DUE TO
LEFT HEART FAILURE)
 Pedal oedema, raised JVP, pulsatile liver ( due to progression to
RIGHT HEART FAILURE)
 Shifting of the apex beat with forceful apex beat ( due to LVH )
 COLLAPSING PULSE.
 HIGH PULSE PRESSURE
 CAPILLARY PULSATION : nail bed, lips, fundus
 PISTON SHOT sound over the femoral arteries. ( due to high
pressure during systole and collapse during diastole)
 Dancing carotid.
 Murmers-----
 Blowing aortic diastolic murmer : HIGH PITCHED, better heard
with diaphragm, onset is after the S2. often transmitted over
the precordium and may be maximal at the aortic area or down
the left side of the sternum particularly in the third or fourth
interspace. It is accentuated if the patient leans forward and
holds his breath in full expiration. It is decrescendo type. It
lasts through most of diastole.
 An associated AORTIC SYSTOLIC MURMER occurs due to
dilatation of the ascending aorta and increased stroke output.
The systolic and diastolic murmer are together described as ‘to
and fro’ type.
 An apical mid-diastolic murmer is heard in severe aortic
regurgitation ( AUSTIN FLINT MURMER). This is due to blood
regurgitating into the the left ventricle impeding the opening of
the anterior cusp of the mitral valve and so causing functional
obstruction.
 Occasionally a patient may have a loud musical or ‘dovecote’
diastolic murmer which may be audible to the examiner even
without a stetho. It is usually due the rupture, perforation or
retroversion of an aortic valve cusp.

 X-Ray shows linear calcification of the ascending aorta + enlargement


of the left ventricle.
 ECG: left axis deviation. ST segment and T wave changes may occur
due to left ventricular strain or sometimes ischaemia.

• ANEURYSM: It is dilatation of the aortic wall due to weakening of the


media by syphilitic endarteritis. They are diagnosed by X-Rays ( PA &
OBLIQUE views) and angiography. Barium swallow can be used to
delineate the oesophagus.
Aneurysm of ascending aorta (ANEURYSM OF SIGNS):
 Commonest site of syphilitic aneurysm
 If bulges laterally ------- compresses on the right lung or superior
vena cava : diminished rt sided breath sounds + engorgement of veins
of head and neck.
 If bulges anteriorly ------ compresses the ribs and sternum : bony
erosion and pain.
 If bulges medially ----- compresses the trachea / pulmonary artery.
 If bulges posteromedially ---- causes pressure symptoms and
pulsation of the left upper chest.
 Loud S2 ( AORTIC component)
 Rupture of aneurysm may cause sudden death. If ruptures into
pericardium : CARDIAC TEMPONADE. If ruptures into right lung :
HEMOTHORAX. If ruptures into rt. bronchus or lungs :
HEMOPTYSIS. It may also rupture through the skin.
(2) Aneurysm of arch of aorta ( ANEURYSM OF SYMPTOMS):

 Causes a pulsating mass in the suprasternal notch.


 May press on the trachea producing stridor and a brassy cough.
 May press the left recurrent laryngeal nerve causing hoarseness.
 May press and displace oesophagus ( but dysphagia is less)
 May press the left bronchus causing TRACHEAL TUG with each
heart beat.
 May press the cervical sympathetic chain : HORNERS SYNDROME.
 May press the innonimate vein or left common carotid vein :
asynchronism of pulses and blood pressure. Dizziness due to
cerebral anoxia.
 May press and occlude the subclavian artery : STEAL SYNDROME.
 May press and erode the bodies of 4th to 6th thorasic vertebra.
 May press the nerve roots and cause severe root pain.
 May press phrenic nerve causing diaphragmatic palsy.
 Patient may suddenly die from rupture of an aneurysm.
(3) Aneurysm of descending thorasic aorta ( ANEURYSM OF NO SIGNS AND
NO SYMPTOMS):

 Rare and usually symptomless.


 Rupture is rare.
 Deep seated and continous pain in the back may result from pressure
on the bodies of the 7th to the 11th thorasic vertebra.
(4) Aneurysm of Abdominal aorta:
 More rarer. Below the origin of renal arteries is unlikely to be due to
syphilis.
 Pain in the abdomen or back.
 Root pain.
 Palpable mass with expansile pulsation in the abdomen. There may be
associated thrill and murmer.
 Radiography shows calcium deposition in the walls and sometimes
erosion of the 12th thorasic and 1st and 2nd lumbar vertebra.
(5) Aneurysms at other sites: the INNONIMATE, LEFT COMMON CAROTID
and LEFT SUBCLAVIAN ARTERIES.
NEUROSYPHILIS:

 Involvement of the CNS occurs in all stages of syphilis. The very early and
frequent invasion of the meninges by T.pallidum during systemic
dissemination of infection has been confirmed by the isolation of t.pallidum
in the CSF and by CSF abnormalities in 4-24 % of patients with early
syphilis.
 Both treponemal isolation and CSF abnormalities appear to be more common
in PATIENTS WITH EARLY SYPHILIS THAN LATE SYPHILIS suggesting
that in some patients CSF ABNORMALITIES MAY RESOLVE WITHOUT
TREATMENT.
 Although the true incidence of neurosyphilis is not known , the successful
treatment of early syphilis in the antibiotic era has made this an uncommon
disorder in many parts of world.
 The incidence of neurosyphilis in STD patients in INDIA has been reported
to vary from 0.1% to 5.8%.
 After resolution of the secondary stage, THE SPIROCHAETES PRESUMELY
REMAIN DORMANT IN THE CNS OF UNTREATED OR INADEQUATELY
TREATED PATIENTS.
 In absence of treatment , approximately one third of those with early
invasion will develop signs of neurosyphilis.
 Symptoms occur from 5-35 years after the initial infection.
 Neurosyphilis is less common in women than in men and in negroes and Asians
than whites.
 CLASSIFICATION:
 ASYMPTOMATIC NEUROSYPHILIS: C/B
------ no S/S
------- CSF changes like
 Elevated cell count ( > 4 lymphocytes per cu.mm ) : FIRST
INDICATION OF CNS INVOLVEMENT.
 Elevated protein in the CSF ( > 40 mg%) . the level of IgG and IgM in
CSF may be increased and quantitative determination of these may
help in assessing the activity of the disease.
ABNORMALITIES OF CELL COUNT AND PROTEIN ARE THE MOST
SENSITIVE INDICES OF ACTIVITY OF DISEASE PROCESS. AFTER
SUCCESSFUL TREATMENT, THEY ARE THE FIRST TO RETURN TO NORMAL.
TESTS FOR REAGIN AND COLLOIDAL GOLD CURVE REMAIN
ABNORMAL FOR LONG PERIODS.
 POSITIVE REAGIN TESTS GIVE A FIRM INDICATION OF THE
PRESENCE OF SYPHILIS OF THE NERVOUS SYSTEM. FALSE
POSITIVE RESULTS ARE VERY RARE IN CSF ( this sometimes may
occur in meningitis due to other causes due to passage of reagin from
the blood stream through the choroid plexus.
 POSITIVE SPECIFIC TESTS OF SYPHILIS IN THE SERUM except
in very long standing burnt out cases of tabes syphilis. If this test is
negative in serum, it is unlikely to give a positive result in CSF.
 Specific tests are positive in the CSF.
 COLLOIDAL GOLD TEST : It is reported in the form of
• Graph into 3 zones : first zone ( paretic) , midzone ( leutic) and end zone
( meningitic)
• By a series of numericals: 0-1 : normal ; 2-5 : abnormal
This test is not diagnostic since it reflects the proportion of albumin
and globulin. Thus , paretic or leutic curve may be found in disseminated
sclerosis. Therefore always confirm by serological tests, cell count and protein.
 Asymptomatic neurosyphilis is classified as:
TYPE 1 : minimal change of cell and protein, negative reagin and normal Lange
curve.
TYPE II: Raised cells and proteins, weakly positive reagin and mid zone Lange
curve.
TYPE III: raised cell and protein, strongly positive reagin, first zone Lange
curve. It is significant since it warrants that without treatment , patient is likely to
develop GPI.
 SYPHILIS OF THE BRAIN:
(1) MENINGEAL SYPHILIS:
 Meningeal involvement can occur in the secondary stage of the
disease or at any time in the later stages. Manifestations include:
 Headache, Nausea, vomiting and stiffness of the neck :
MANIFESTATIONS OF ASEPTIC MENINGITIS.
 It may be associated with COMMUNICATING HYDROCEPHALUS
( one third). There may be papilloedema.
 The underlying CORTEX may be involved causing ----- convulsions,
aphasia and mental confusion. Monoplegia and hemiplegia can occur.
 BASAL MENINGITIS can affect the cranial nerves MOSTLY
OCULOMOTOR, ABDUCENS AUDITORY AND FACIAL NERVES.
 ARGYL- ROBERTSON PUPIL -------- where the light redlex is lost
but accomodation reflex is retained. The pupils are irregular and
unequal in size.
 The DIAGNOSIS is based on ---- POSITIVE SEROLOGICAL TESTS,
CHANGES IN CSF, PRESENCE OF PUPILLARY ABNORMALITIES
AND IN SOME CASES, THE RESPONSE TO TREATMENT.

(2) VASCULAR SYPHILIS: It is caused by endarteritis of the medium and large


arteries ( Heubner’s arteries) or small arteries and arterioles (Nissl arteritis) that
results in thrombotic infarction . because the symptoms are focal, the clinical
picture resembles atherosclerotic occlusive disease. Weeks or months before the
abrupt onset, there is prodorme like dizziness, headaches, insomnia , memory loss
and mood changes. Depending on the artery involved, patients may be present with
almost ay neurologic deficit. THE MOST FREQUENT IS HEMIPARESIS OR
HEMIPLEGIA FOLLOWED IN FREQUENCY BY APHASIA AND SEIZURES.
Therefore this diagnosis should be kept in mind in adult onset epilepsy or stroke.

ARTERIES CLINICAL FEATURES

ANTERIOR (A) MENTAL RETARDATION (B) CONTRALATERAL


CEREBRAL HEMIPLEGIA & HEMIANAESTHESIA.
ARTERY
MIDDLE (A) CONTRALATERAL HEMIPLEGIA AND
CEREBRAL HEMIANAESTHESIA (B) APHASIA
ARTERY
POSTERIOR (A)CONTRALATERAL HOMONYMOUS HEMIANOPIA (B)
CEREBRAL SPONTANEOUS PAIN & ATHETOSIS due to involvement
ARTERY of thalamus.
CEREBELLAR (a) giddiness due to vestibular nuclei (b) dysphagia due to
ARTERY( usually nucleus ambiguous (c) crossed hemianaesthesia with sensory
the loss of the face on the same side ( spinal tract of trigeminal
posteroinferior nerve) and anaesthesia of the opposite side of the body
artery): ( spinothalamic tract ) (d) horner’s syndrome ( involvement
Wallenburg of sympathetic pathways in the medulla). (e) cerebellar
syndrome ataxia on the same side.
BASILAR ARTERY WEBER’S SYNDROME: partial III nerve palsy with crossed
( usually small hemiplegia of face and legs.
arteries arising
from the upper
part of the artery
are affected).

(3) PARENCHYMATOUS INVOLVEMENT ( GENERAL PARESIS OF INSANE): A


chronic meningoencephalitis severly disturbs the cerebrocortical functions and
results in gross atrophy of the FRONTAL AND PARIETAL LOBES , widened
cerebral sulci and hydrocephalus.
 The disease begins about 15 to 20 years after infection.
 Depending on the damaged area, the symptoms may be psychiatric,
neurologic or, more commonly both.
 SEQUENCE OF MENTAL CHANGES-----
(A) PERIOD OF ONSET:
> May be insidious and gradual progressive ( psychoneurosis) ---- C/B
inability to concentrate, forgetfulness, irritability,headache, impaired judgement.
Patient is ill- kept .
> May be sudden onset ( congestive attack: transient bouts with progressive
detoriaration) ---- convulsion, confusion, aphasia, hemiplegia, monoplegia.

(B) PERIOD OF FULL DEVELOPMENT: C/B various PSYCHOSIS ( Grandiose/


dementic (M.C) / manic ) and MOOD ALTERATION ( usually depressed and
confused). Patient becomes emotionally labile.

(C) PERIOD OF DECLINE IN MENTAL AND PHYSICAL DECLINE: C/B


> Dementia
> Tremors of hands, lips, tongue
> Convulsions of any type
> Vegetative state, emaciation,
> Death from pneumonia, infected bed sores, septicaemia ( within a
average of 2-12 years).
CLINICAL SIGNS INCLUDE -----
 POOR SCORES IN TESTS OF MENTAL STATUS.
 HANDWRITING IMPAIRMENT .
 DYSARTHRIA
 LARGE PUPILS WHICH REACT POORLY TO LIGHT AND
CONVERGENCE.
 PARETIC FACIES ( expressionless).
 Trombone tremor ( tremors in lips and tongue on protrusion)
 MILD SPASTIC PARAPLEGIA WITH INCREASED KNEE AND ANKLE
JERK. EXTENSOR PLANTER RESPONSE AND LOSS OF ABDOMINAL
REFLEXES.
 OCULOMOTOR PALSIES / OPTIC ATROPHY.

COMBINATION OF HEMIPARESIS, CONVULSIONS AND APHASIA has been


termed LISSAUER’S TYPE OF GPI.
The clinical spectrum is summarized in the Holmes pnemonic:
P = personality disorder
A = affect ( flat)
R = reflexes hyperactive
E = eye changes ( unequal pupils that react poorly to light)
S = sensorium
I = intellect impairment
S = speech dysarthria.

Diagnosis is based on C/F, Serum and CSF serologic tests, type iii CSF CHANGES,
good response to antibiotics ( HALTS PROGRESSION IN 80% CASES).

Optic atrophy can occur as an isolated finding. Penicillin does not restore
vision but prevents further progression.

 SYPHILIS OF THE SPINAL CORD:


(a) MENINGEAL INVOLVEMENT :
 It is likely to result in spread to the underlying nerve tissue with
TENDENCY TO INVOLVE THE PYRAMIDAL TRACT AND OTHER
MOTOR TRACTS. This produces LMN palsy.
 The meninges are mainly affected in 3 distinct areas. Viz.
DORSOLUMBER REGION involving pyramidal tracts
( ERB’S SPASTIC PARAPLEGIA);
CERVICAL REGION involving spinal nerve roots and spinal
tracts. ( HYPERTROPHIC CERVICAL PACHYMENINGITIS)
COMBINATION OF TWO ( SYPHILITIC AMYOTROPHY).
 ERB’S SPASTIC PARAPLEGIA : It is characterized by ------
• Gradual onset
• Spastic weakness of legs so that the patient drags feet while walking (due to
involvement of the corticospinal tract . their fibres have a superficial
location in the lateral spinal cord rendering them susceptible to external
compression)
• Irritable bladder with small capacity ---- increased frequency of micturition.
• Increased tone and reflexes in the legs ---- ankle clonus, extensor planter
response.
• Paraplegia is of extensor in type.

 HYPERTROPHIC CERVICAL PACHYMENINGITIS

• Gradual onset
• Headache
• Bulbar cranial nerve lesions.
• Redicular Pain in the neck shoulder and upper limb
• Lower motor neuron lesions of the shoulder girdle and arms with lost
reflexes, flaccidity and wasting. Sensory loss from posterior nerve root
compression can occur.
• Upper motor neuron palsy and sensory loss below the level of lesion may
occur due to compression and ischaemia of spinal cord.

(b) VASCULAR INVOLVEMENT: This results in infarction of the spinal cord.


The spinal cord is supplied by one anterior and two posterior spinal arteries.
The former supplies the anterior two third area of the cord and when this
vessel undergoes thrombosis, onset is sudden like transection of the cord.
Affects vary according to the level at which artery is thrombosed .
USUALLY IN THE THORASIC AND LUMBER REGIONS.
Suddenly there develops paraplegia or quadriplegia with urinary and
fecal incontinence and dissociated sensory loss affecting pain and temperature
but sparing vibration and pressure sense. The paralysis is at first like spinal
shock with lost reflexes and muscle tone , this is replaced within 2-3 weeks by
spascicity and increased reflexes. There is involuntary contraction of the flexor
muscles of legs and abdomen and ultimately there PARAPLEGIA IN FLEXION
( due to damage of pyramidal + extrapyramidal tracts).
Thrombosis of a lateral branch of the anterior spinal artery produces
sudden weakness followed by muscle wasting of the affected spinal segment.
Thrombosis of a posterior spinal artery gives rise to so localised
lesion that it is hardly diagnosed during life. There may be affection of the
posterior columns and pyramidal tract with manifestations below the level of
lesion.
(c ) PARENCHYMATOUS INVOLVEMENT : Caused by infection of the parenchyma
of posterior columns and dorsal root of the spinal cord. Develops 10 -20 years
after the onset of the disease

 The three stages are PREATAXIA, ATAXIA AND PARALYSIS.


 The early symptoms are recurrent attacks of paresthesia and
paroxysms of lightening pain. The pains usually occur in the legs ( less
often in the back and arms). The pain is usually SUDDEN, BRIEF ,
INTERMITTENT AND STABBING in character ( may also be burning
or gripping). Hyperalgesia and vasodilation may occur in the affected
area. Remissions from pain are absolute and usually tend to grow
longer as time passes. Paresthesia occurs as muscle cramps,
numbness, tingling or aching sensation of feet, legs or body. There is
a sensation of walking on cotton wool. A girdle like sensation may be
felt round the abdomen. STABBING PAIN CAN ALSO OCCUR IN
VISCERAL ORGANS LIKE ABDOMEN, RECTUM AND LARYNX -------
simulating SURGICAL EMERGENCIES. Gastric crises ( abdominal pain
and vomiting) , rectal crises ( tenesmus, loose motion) and laryngeal
crises ( stridor) can occur. As the disease progresses, the patient
may develop impotence , urinary retention ( due to damage to the
posterior roots the patients loses the sensory supply of the bladder
and becomes unaware that the bladder is full ------ overdistention of
bladder, UTI), visual loss ( due to optic atrophy), constipation ( due to
loss of tone of lower gut and the musculature of the pelvic floor.)
 Locomotor ataxia begins with stumbling and difficulty in walking.
BECAUSE THE POSITION SENSE IS LOST, THE ATAXIA IS
MORE PROMINENT IN THE DARK or WITH EYES CLOSED e.g.
washing face early in the morning.
 SIGNS INCLUDE:
• PUPIL ---- papillary reaction becomes sluggish. This is earliest. Later the
typical argyl Robertson pupil develops C/B
 Pinpoint and irregular pupils ( due to oculosympathetic paralysis)
 React to accommodation but not light ( there is gliosis involving the light
reflex tracts of midbrain )
• ATONY AND AREFLEXIA MAINLY OF THE LOWER LIMBS.
• POSITIVE ROMBERG’S SIGN which means that the patient falls when the
feet are held close together with the eyes closed.
• GAIT --- the ataxic patient walks with his feet somewhat apart and his toes
turned out. He lifts his feet unduly high and tends to stamp them on the
ground. Depends on vision for standing and walking. He has difficulty in
turning and does so in a series of careful and deliberate steps. He tends to
lose concentration in crowds and becomes UNSTEADY.
• Patient is unable to perform the HEEL- KNEE TEST with eyes closed.
• LOSS OF POSTERIOR COLUMN SENSATION LIKE ---- DEEP PAIN
( tested by sqeezing the Achilles tendon) , POSITION SENSE and
VIBRATION SENSE.
• Palpable distended bladder . when bladder fills the usual detrusor reflex is
gone . this is detected by CYSTOMETROGRAM which measures
intravescical pressure with varying vescicle content.
• If tabes is associated with basal meningitis, there may be unilateral or
bilateral ptosis and opthalmoplegia due to involvement of 3 rd, 6th and perhaps
4th nerve palsies.
• CSF CHANGES ARE USUALLY OF TYPE II . In 11% CSF does not show any
abnormality. In one third the bllod reagin tests are negative. Occasionally
the specific tests are negative.

• 2 TYPES OF TROPHIC CHANGES CAN OCCUR :


(A) CHARCOT’S ARTHROPATHY
(B) PERFORATING ULCER ( ‘mal perforans’)

 CHARCOT’S ARTHROPATHY: Loss or impairment of the sensation of


pain in the joints may lead to severe degenerative effects especially
if the patient sustains trauma to the joint with the formation of a
loose body and due to continous use of the limb, the cartilage and
subchondral bone gets eroded and destroyed with the formation of
more loose bodies. The ligaments are also destroyed. The periosteum
reacts to the insult by new bone formation ------ osteophytic
outgrowth and gross sclerosis at the ends of the bone. The end
result is a grossly deformed joint that is hypermobile , swollen but
PAINLESS. Loints mostly involved are : KNEE, ANKLE, HIP, FOOT
AND SPINE. CHARCOT CHANGES MAY ALSO OCCUR IN
SYRINGOMYELIA, DIABETES, SPINAL CORD OR PERIPHERAL
NERVE INJURY.
 PERFORATING ULCER OCCURS DUE TO loss of deep pain sensation
---- callus formation over the pressure areas from trauma beneath
which is a trophic ulcer .

• PRIMARY OPTIC ATROPHY is found in 15-20 % of tabetics. Gradual


diminution of vision often begins in one eye, progresses to a certain point
and then begins in the other eye. The EARLIEST FEATURE IS
CONTRACTION OF VISUAL FIELDS . there may be central and peripheral
SCOTOMA. Opthalmoscopy shows funneling of the optic disc with vessel
obliteration. Loss of central vision occurs when the papillomacular bundle is
affected. End result = BLINDNESS .