2. Q.1.What is the diagnosis?
Q.2.Where are the probable
lesions?
Q.3.What are the expected
examination findings considering
his complaints?
3. INTRODUCTION
Infection of nervous system by T.pallidum
The incidence has reduced drastically, since the introduction of
penicillin
However, in more recent years, the number of reported cases of
early syphilis has increased, both in non-immunocompromised and
compromises
The organism usually invades the CNS within 3 to 18 months of
inoculation.
The initial event of meningitis, which occurs in approximately 25% of
all cases
If the nervous system is not involved by the end of the 2nd year,
there is only 5% that the patient will develop neurosyphilis as a result
4.
5. ASYMPTOMATIC NEUROSYPHILIS
From a clinical point of view, asymptomatic neurosyphilis is
perhaps the most important form
As, if adequately treated, the symptomatic varieties would be
prevented in most instances
In this condition, there are no symptoms
In rare cases, Argyll Robertson pupils may be found
The diagnosis is based entirely on the CSF findings
6. MENINGEAL SYPHILIS
May occur at any time after infection
Usually occurs within the first 2 years.
The patient is afebrile, unlike the case in tuberculous meningitis.
Most common symptoms are headache, stiff neck, cranial-nerve
palsies,confuion
Occasionally, signs of increased intracranial pressure may present as well
The CSF always has a lymphocytic reaction,
With adequate treatment, the prognosis is good.
The symptoms usually disappear within days to weeks
7. MENINGOVASCULAR SYPHILIS
The most common form of neurosyphilis.
Usually occurs 6 to 7 years after first inoculation, but may occur
anytime.
In the past, strokes associated with 10 % of cases , which is now
around 35 %.
Pathogenesis - inflammation and fibrosis of meninges ;small arteries
(Heubner arteritis)
Infarctions occur in the medium- and small-caliber lenticulostriate branches
Common site: Internal capsule, Basal ganglia, periventricular region
Adequate treatment will prevent further vascular episodes.
If repeated strokes occur despite adequate therapy, consider the
possibility of non-syphilitic vascular disease of the brain.
9. PARETIC NEUROSYPHILIS
Usually occurs 15-20 years after the primary infection
Once a major cause of various forms of mental illness, progressive
dementia
Often associated with paretic symptoms
Hence called General Paresis of insane/ Dementia Paralytica
The clinical picture in its fully developed form includes progressive
dementia, dysarthria, myoclonic jerks, action tremor, seizures, hyperreflexia,
Babinski signs, and Argyll Robertson pupils
However, a greater importance attaches to diagnosis at an earlier stage
10. CONT.
The insidious onset of dementia and disorders of speaking and writing can
be early manifestation (mimics FTD)
As the deterioration continues into the paralytic stage, intellectual function
progressively declines, and aphasias, agnosias, and apraxias intrude
themselves
All these disabilities lead eventually to a bedridden state; hence the term
paretic.
Dilution and mania, such symptoms are late features along with paresis.
Psychiatric syndromes that can be differentiated by cognitive decline,
neurologic signs, and CSF findings
13. HIGH SIGNAL INTENSITY IN BOTH MESIAL TEMPORAL REGIONS AND HYPER INTENSE RIM
SHAPED LESION ATTACHED WITH THE FALX IN THE LEFT FRONTAL LOBE .THE TEMPORAL
HORNS OF THE LATERAL VENTRICLE ARE DILATED DUE TO ATROPHY OF THE MESIAL
TEMPORAL REGIONS.
THERE IS ALSO GENERALIZED ATROPHY OF BRAIN
(PARETIC NEUROSYPHILIS)
FLAIR T2 T1
14. TABES DORSALIS
•Usually develops 15 to 20 years after the onset of the infection.
•The major symptoms are -
Sharp, radicular pains, absent tendon reflexes
Urinary incontinence(overflow incontinence)
Impaired vibratory and position sense in feet and legs, sensory ataxia and a Romberg sign.
Power, by contrast, is fully retained in most cases.
Perforating foot ulcers , and Charcot joints are characteristic complications of the tabes
•In most cases now seen, the CSF is normal when the patient is first examined
(so-called burned-out tabes).
17. Spinal Syphilis
Tabes Dorsalis
Infection and
inflammation of
dorsal roots +
Dorsal column
Arefelxia
Sensory Ataxia
Loss of sense of
position and
vibration
CSF usually
normal
syphilitic
meningomyelitis
chronic fibrosing
meningitis
subpial loss of
myelinated fibers
and gliosis
Usually spastic
paraperesis
CSF abnormal
spinal
meningovascular
syphilis
Heubner arteritic
anterior spinal
artery syndrome
CSF abnormal
18. TABES DORSALIS
T2WI : hyperintensity along the
dorsal column in thoracic
segment
T2WI : hyperintensity along dorsal
part of spinal cord
19. SPINAL MENINGOVASCULAR
SYPHILIS
T2WI demonstrates increase signal
intensities signal from T1-T3
T2-weighted MRI , axial section
demonstrates high signal intensities in
anterior two third of the cord
20. SYPHILITIC
MENINGOMYELITIS
T1WC+: shows multiple nodular foci of
enhancement throughout the thoracic cord
T2WI: diffuse T2 hyperintensity throughout
the thoracic cord, slight irregularities
corresponds to foci of enhancements
24. SYPHILITIC OPTIC ATROPHY
Progressive blindness in one eye and then involving the other
Pathophysiology- Peri-optic meningitis ,occasionally vasculitis may cause it.
Occur within months of the primary infection or as a later manifestation.
Constriction of the visual fields, but scotomata may occur in rare cases.
The prognosis is poor if severe and bilateral.
If only one eye is badly affected, sight in the other eye can usually be saved.
CSF is almost invariably abnormal,
In exceptional cases, visual impairment may progress, even after the CSF
becomes negative.
25. SYPHILITIC NERVE
DEAFNESS AND
VESTIBULOPATHY
Vertigo, with or without hearing loss
Some of the characteristics of vestibular neurosyphilis are identical to those
of Meniere disease.
There is seldom a history of clear primary syphilitic infection.
The pathology, mainly endarteritis in the cochlea and labyrinths,
So, syphilis serology should be tested in patients with cryptic
vestibular dysfunction
26. GUMMA
Non-caseating granuloma resulting from the tertiary stage
of syphilis but can occur in early stages as well.
Cerebral syphilitic gumma is very rare and can only be
histologically confirmed following surgery
caused by a reaction to spirochaete bacteria in the tissue
Menigeal
syphilis
Spirochaete
In subpial
tissue
Infiltration
of lymphocy
tes and plas
ma cells
fibroblast
Granulaoma
formation+
Central
coagulative
necrosis
Gumma
27. GUMMA OF NEUROSYPHILIS
T1C+: A dural-based, peripheral
enhancement of the lesion
T2wI: A dural-based, peripherally
hyperintense and centrally hypointense lesion
located lateral to the left frontal lobe
28. INVESTIGATION
CBC with ESR
S. Creatinine
S. electrolyte
LFT
RFT
U.R/E
CXR P/A view
S. VDRL
S. TPHA
FTA-abs(Flurocent treponemal ab
absorbtion)
MRI of brain & spinal cord with
contrast
CSF study
29. CSF
The CSF is a sensitive indicator of the presence of active neurosyphilis.
The CSF findings are consist of
Protein: raised, from 40 - 200 mg/dL;
Glucose: normal
Cell: pleocytosis more common in early , up to 100 cells/mm3,mostly
lymphocytes
(May be normal patients with HIV and those with leukopenia);
an increase IgG index usually with oligoclonal banding;
VDRL/RPR
Treponemal test-TPHA/FTA-abs
30. PEELING, R., MABEY, D., KAMB, M. ET AL. SYPHILIS. NAT REV DIS PRIMERS 3, 17073 (2017).
HTTPS://DOI.ORG/10.1038/NRDP.2017.73
33. CONT.
Caution
The Jarisch-Herxheimer reaction,
Special situation
If co-infection with HIV, longer treatment and surveillance for relapse
Symptomatic therapy
Radicular pains may respond to gabapentin or carbamazepine
Atropine; phenothiazine derivatives are said to be useful in the treatment of visceral crises.
Neuropathic (Charcot) joints require bracing or fusion
35. After completion
of treatment
Symptom free
CSF -Pleocytosis- absent
Reduction in protein
Reduction in gamma globulin
Reduction in Serology titers
Yes
No further
treatment
No
Repeat another
cycle of therapy
Examination
Every 3-6 monthly
CSF study
Every 6 monthly
A persistent weakly
positive serologic
(VDRL) test after the
cells and protein
levels have returned to
normal does not
constitute an
indication for
additional
treatment.
36. Site Clinical Features
Meninges Meningitis (acute or chronic) , CN palsy(2,6,7,8)
Blood
vessels
Heubner arteritic stroke,
Brain oDementia paralytica’ /general paralysis of insane
Dementia + spastic paraparesis(cortical degeneration)
Neuropsychiatric symptoms(dilutions of grandeur)
o Gumma /mass lesion : seizure , tremors, Ataxia
Spinal cord oTabes Dorsalis: Degeneration of dorsal column + dorsal roots + dorsal root
ganglia
sensory ataxia, radicular pain, areflexia ,bladders symptoms
o syphilitic meningomyelitis : spastic paraparesis
o spinal meningovascular syphilis : anterior spinal artery syndrome
Eyes Optic atrophy (peri-optic meningitis)
Argyll Robertson pupil( pretectal lesion)
Others Neuropathic joint/ Charcot joint, Deafness and Vestibulopathy
37. Q.1.What is the diagnosis?
Q.2.Where are the probable
lesions?
Q.3.What are the expected
examination findings considering
his complaints?
