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Rapidly progressive dementia a case of sporadic creutzfeldt-jakob disease (scjd)

Sporadic CJD is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. It is a rare cause of dementia with an estimated incidence of the disease in one patient per million per year worldwide, however, no data is available for Pakistan. As it is a rare cause of dementia, so rarely considered in differential diagnosis of rapidly progressive dementia. C A S E R E P O R T INTRODUCTION: CJD is a rare degenerative brain disorder which is almost always fatal, it is characterized by progressive brain dysfunction.1 CJD falls into four categories, sporadic, iatrogenic, familial and variant.2 Sporadic CJD is most common and accounts for 85% of all cases. It occurs worldwide and its incidence ranges from 0.5 to 1.5 cases per million per year. Of the remaining 15% of cases, 10 percent (%) is familial and iatrogenic and variant comprise 2 to 5 percent. CJD should be suspected in patients who presented with cerebellar signs, myoclonic jerks, and rapidly progressive dementia. However, these symptoms and signs are usually preceded by Psychiatric symptoms, which could be easily ignored.