All the following are disease phenotypes of Prion diseases Except
Correct Answer: Leigh syndrome
Description: Prions are abnormal forms of a cellular protein that cause rapidly progressive neurodegenerative disorders that may be sporadic, familial or transmitted. Disease phenotypes include Creutzfeldt-Jakob disease (rapidly progressive dementia), Gerstmann-Straussler-Scheinker syndrome (progressive cerebellar ataxia) and fatal familial insomnia.Leigh syndrome is a disease of infancy characterized by lactic acidemia, an arrest of psychomotor development, feeding problems, seizures, extraocular palsies, and weakness with hypotonia.Ref: Robbins and Cotran Pathologic Basis of Disease; 9th edition; Chapter 28; The Central Nervous System; Page no: 1284
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Pathology
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