Article
An autopsy case of Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting with pathological laughing and an exaggerated startle reaction.
Neuropathology : official journal of the Japanese Society of Neuropathology - 1 Dec 2017
Iwasaki Yasushi, Mori Keiko, Ito Masumi, Akagi Akio, Mimuro Maya, Kitamoto Tetsuyuki, Yoshida Mari
Abstract excerpt
A 78-year-old Japanese woman presented with slow progressive disorientation and memory disturbances. Pathological laughing was observed at an early disease stage and continued for several months. Around the same time, the patient began to exhibit an exaggerated startle reaction and mild myoclonus. The pathological laughing and startle reaction disappeared before the patient reached an akinetic mutism state...
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