Abstract Prion disorders are rare neurodegenerative disorders resulting from proteinaceous infectious particles and are universally fatal. It can be sporadic, genetic, or acquired. Globally, sporadic Creutzfeldt-Jakob disease is the most commonly reported prion disorder, as well as in India. To delineate the Indian perspective, this study identified and analyzed Indian studies on Prion disorders using the PubMed and Scopus databases. Several Indian case reports and case series suggest a higher prevalence of prion disorders than expected. These disorders commonly affect individuals in the sixth decade of life or later. They are accompanied by rapidly progressive dementia and movement disorders in the form of myoclonus, ataxia, and parkinsonism. The diffusion-weighted sequence of brain magnetic resonance imaging has the highest sensitivity for confirming suspected prion disorders. Short-interval periodic complexes in electroencephalograms are a supportive feature for diagnosing these disorders. Although brain biopsy is the gold standard for their diagnosis, to date, this approach has been performed in <50 Indian cases. Although the results of real-time quaking-induced conversion are comparable to those of histopathological diagnosis, it is not available in India. A pan-India prion disorder registry should be established for the extensive evaluation and systematic collection of data on prion disorders.
Reddy et al. (Fri,) studied this question.