Article
A subtype of sporadic prion disease mimicking fatal familial insomnia.
Neurology - 10 Jun 1999
Parchi P, Capellari S, Chin S, Schwarz H B, Schecter N P, Butts J D, Hudkins P, Burns D K, Powers J M, Gambetti P
Abstract excerpt
OBJECTIVE: To establish a variant of sporadic prion disease as the sporadic form of fatal familial insomnia (FFI). BACKGROUND: FFI is a recently described prion disease characterized clinically by severe sleep impairment, dysautonomia, and motor signs, and pathologically by atrophy of thalamic nuclei, especially the medial dorsal and anterior ventral, and of the inferior olive. FFI is linked to the D178N mutation...
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