Spongiform degeneration of cerebral coex occurs in –
Correct Answer: Creutzfeldt-Jakob disease
Description: Spongiform Encephalopathy (Creutzfeldt-Jakob Disease) Spongiform encephalopathy, also called Creutzfeldt-Jakob disease (CJD) or mad-cow disease, though included under the group of viral encephalitis but is caused by accumulation of prion proteins. Prion proteins are a modified form of normal structural proteins present in the mammalian CNS and are peculiar in two respects: they lack nucleic acid (DNA or RNA), and they can be transmitted as an infectious proteina- ceous paicles (Dr Prusiner was awarded the Nobel Prize in medicine in 1997 for his discovery on prion proteins). Majority of cases occur sporadically though familial predisposition with autosomal dominant inheritance has also been repoed in 5-15% cases. Other methods of transmission are by iatrogenic route (e.g. by tissue transplantation from an infected individual) and by human consumption of BSE (bovine spongiform encephalopathy)-infected beef, also called as mad-cow disease. Clinically, CJD is characterised by rapidly progressive dementia with prominent association of myoclonus. CJD is invariably fatal with mean survival of about 7 months after diagnosis.Grossly, the changes are too rapid to become noticeable but brain atrophy may be seen in long-standing cases. Microscopically, the hallmark is spongiform change i.e. there are small round vacuoles in the neuronal cells. These changes are predominantly seen in the coex and other grey matter areas. Spongiform changes result in neuronal loss and glial cell proliferation but significantly without any inflammation or white matter involvement. Ref:TEXTBOOK OF PATHOLOGY 6th EDITION - HARSH MOHAN PAGE NO:878-879
Category:
Pathology
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