Creutzfeldt-Jacob disease: An autopsy case report in tertiary care hospital.

Sporadic Creutzfeldt-Jakob disease (CJD) is the most common prion disease. It is a rare, fatal neurodegenerative disease caused by an infectious protein called prion. The diagnosis can be confirmed only by histological examination of brain tissue. Because of the transmissible nature of the disease, autopsy or brain biopsy cannot be performed at many institutions. Histology shows spongiform changes, neuronal loss, reactive astrocytic proliferation, accumulation of pathologic protein occurring in three general forms: Sporadic, familial, and acquired form, including a variant form of CJD. It clinically presents as predominantly progressive dementia with a rapid onset, myoclonus, cerebellar, pyramidal, extra pyramidal and visual signs. Occurrence of periodical spikes in electro-encephalogram, observation of cortical signal alterations in magnetic resonance imaging (MRI) studies, and detection of protein 14-3-3 in cerebrospinal fluid substantiate diagnosis. Autopsy case is presented of a 50 year old woman with progressive dementia, typical neurological symptoms, MRI findings and confirmation of CJD on histology and immunostaining.

Sudden, unexpected and natural death in young adults of age between 18 and 35 years: A clinicopathological study.

Context: To identify various causes, risk factors, age and sex distribution associated with sudden and unexpected natural deaths (SUNDs) in young adults of age between 18 and 35 years. Materials and Methods: Retrospective analysis of autopsy reports and medical records of all SUNDs that occurred instantaneously or within 24 hours of onset of symptoms in young adults, between 2001 and 2009. Result: Of the total 6453 deaths autopsied during 2001-2009, 64 (0.99%) were SUNDs in young adults, chiefly in males between 30 and 35 years of age. Non-cardiac causes significantly predominated (73.4%) over cardiac causes (7.8%). Most of the SUND cases were due to preventable causes, including infections (54.6% cases), cerebrovascular accidents (9.37%) and ischemic cardiac causes (6.25%). Sudden adult death syndrome (SADS) accounted for 18.75% deaths. Conclusion: SUND in young adults is preventable. A meticulous post-mortem examination with special attention to the conduction system of heart and detailed toxicological analysis can pinpoint the cause of death in SADS.