NIME Next Batch PGI Quest in Delhi from 10 August to 20 August
A. Caused by JC virus
B. Diffuse cortical matter involvement
C. Disease of white matter
D. Disease of grey matter
E. All of the above
PML true A/E:
A. Caused by JC virus B. Diffuse cortical matter involvement
C. Disease of white matter
D. Disease of grey matter
E. All of the above
Ans. A. Caused
by JC virus ; (C) Disease of white matter.
• Progressive multifocal leukoencephalopathy (PML) is
a progressive disorder characterized pathologically by multifocal areas of
demyehnation of varying sizes and distributed throughout the NS
• In addition to demyelination, there are
characteristic cytologic alterations in both astrocytes and oligodendrocytes
• The disease is caused by JC polyorina virus
(Jamestown Canyon)
• Almost all patients have an underlyiiig
immunosuppressive disorder e.g. HIV, Lymphoproliferative disorder,
myeloproliferative disease, granulomatous diseaseetc.
• Pathologically, lesions consists of patches of
regular ill defined destruction of white matter ranging in size from few mm to
extensive involvement of entire lobe of brain.
• White matter lesions are
asymmetric multifocal coalescing located periventricularly in the entrum semi
oval, in the parietal-occipital region and in cerebellum.
• Clinically present with visual impairment (typically
homonymous hemianopia) and mental impairment (dementia, personality changes,
confusion) and later develop motor weakness in 75% of cases.
•
CSF typically normal, although mild elevation of protein and/or IgG may be
found.
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