1-1-05. Electroencephalographic diagnosis of prion disease in Japanese CJD surveillance

Publication date: June 2017
Source:Clinical Neurophysiology, Volume 128, Issue 6
Author(s): Yoshiyuki Kuroiwa, Ichiro Takumi, Hiroyuki Murai, Kensaku Kasuga, Yoshikazu Nakamura, Kimihiro Fujino, Takashi Kurokawa, Yasuhisa Baba, Katsuya Sato, Masashi Harada, Tetsuyuki Kitamoto, Tadashi Tsukamoto, Masahito Yamada, Hidehiro Mizusawa
The electroencephalographic finding of periodic synchronous discharges (PSDs) is a basic clue for the diagnosis of Creutzfeldt-Jacob disease (CJD). We proposed grading of PSDs. We defined grade A as typical PSDs, grade B as PSDs of relatively longer periodic intervals, grade C as PSDs of relatively rare appearance, grade D as PSDs of rudimentary appearance, and grade E as absent PSDs. The occurrence rate of PSDs in Japanese CJD surveillance was 62% in total CJD (490/794), 72% in sporadic CJD (441/610), 24% in genetic CJD (41/171), and 61% in dura CJD patients (8/13). Among 171 genetic CJD patients, the occurrence rate of PSDs was 0% in P105L mutation CJD (0/2), 3% in V180I mutation CJD (3/90), 14% in P102L mutation CJD (4/29), 70% in E200K mutation CJD (16/23), and 71% in M232R mutation CJD patients (17/24). The occurrence rate of MRI changes in both cortical and basal ganglia was significantly higher in the s-CJD group with PSDs than in the s-CJD group without PSDs. The occurrence rate of PSDs in non-CJD patients who needed differential diagnosis of CJD was 10% (37/355). Non-CJD diseases associated with PSDs were status epilepticus, diffuse Lewy disease, Hashimoto encephalopathy, Wernicke encephalopathy and uremic encephalopathy.



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