Article
Gerstmann-Straeussler-Scheinker disease with P102L prion protein gene mutation presenting with rapidly progressive clinical course.
Clinical neuropathology - 1 Jan 2000
Iwasaki Yasushi, Mori Keiko, Ito Masumi, Nokura Kazuya, Tatsumi Shinsui, Mimuro Maya, Kitamoto Tetsuyuki, Yoshida Mari
Abstract excerpt
We describe an autopsied case of a Japanese woman with Gerstmann-Straeussler-Scheinker disease (GSS) presenting with a rapidly progressive clinical course. Disease onset occurred at the age of 54 with dementia and gait disturbance. Her clinical course progressively deteriorated until she reached a bedridden state with myoclonus 9 months after onset. Two months later, she reached the akinetic mutism state. Nasal...
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