PML true A/E | PGI Frequently Asked Medical PG topics

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.