Expert Consensus on Clinical Diagnostic Criteria for Fatal Familial Insomnia

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A proposal of new diagnostic pathway for fatal familial insomnia

BACKGROUND In absence of a positive family history, the diagnosis of fatal familial insomnia (FFI) might be difficult because of atypical clinical features and low sensitivity of diagnostic tests. FFI patients usually do not fulfil the established classification criteria for Creutzfeldt-Jakob disease (CJD); therefore, a prion disease is not always suspected. OBJECTIVE To propose an update of ...

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Fatal Familial Insomnia: An Overview

Fatal Familial Insomnia (FFI) is an insidious prion disorder that tends to manifest itself as a patient reaches middle age following a pattern consistent with autosomal dominance. A wide range of symptoms are represented, many related to motor function and autonomic regulation, but degeneration of certain areas of the thalamus is present in every case. Genetically, the condition is transmitted ...

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ژورنال

عنوان ژورنال: Chinese Medical Journal

سال: 2018

ISSN: 0366-6999

DOI: 10.4103/0366-6999.235115