2024-03-29T06:01:21Z
https://niigata-u.repo.nii.ac.jp/oai
oai:niigata-u.repo.nii.ac.jp:00017966
2022-12-15T03:49:16Z
453:456
471:537:538:1127
Machado-Joseph病の神経生理学的特徴に関する研究
Machado-Joseph病の神経生理学的特徴に関する研究
Neurophysiological Findings in Machado-Joseph Disease in Japanese Families
近藤, 浩
Machado-Joseph disease (MJD)
auditory brain stem response (ABR)
somatosensory evoked potential (SEP)
visual evoked potential (VEP)
Machado-Joseph 病
聴性脳幹反応
体性感覚誘発電位
視覚誘発電位
To clarify neurophysiological aspects of Machado-Joseph disease (NJD), we studied auditory brain stem response (ABR), somatosensory evoked potential (SEP), visual evoked potential (VEP), electromyography (EMG), nerve conduction velocity (NCV) and electroencephalography (EEG) in the 29 patients with MJD in Japanese families. ABR was abnormal in 21 of the 26 patients studied. Wave I was relatively normal, but the peakes after wave II could not be evoked clearly. Median nerve SEP was abnormal in 17 of the 27 patients and posterior tibial nerve SEP was abnormal in all of the 27 patients studied. Peripheral nerve conduction was almost normal but the central conduction was impaired. In VEP, the latency of P100 was prolonged in 2 of the 6 patients studied. In EMG, neuropathic changes were found in 16 of the 20 patients studied. In NCV, motor nerve conduction was delayed in 3 of the 22 patients and sensory nerve action potential could not be evoked in 2 of the 22 patients studied. In EEG, slow activity was found in 8 of the 19 patients studied, but no epileptic discharge was found in all patients. As has been revealed by neuropathological studies, MJD is a multisystem disorder. This study clarified the multisystem abnormality electrophysiologically, and a high incidence of abnormalities was found in EMG, ABR and SEP, especially in posterior tibial nerve SEP, compared with other electrophysiological markers. Electrophysiological examinations, especially EMG, ABR and SEP, are considered to be useful for diagnosing MJD.
新潟医学会
1996-08
jpn
departmental bulletin paper
http://hdl.handle.net/10191/43254
https://niigata-u.repo.nii.ac.jp/records/17966
AN00182415
00290440
新潟医学会雑誌
新潟医学会雑誌
110
8
326
336
https://niigata-u.repo.nii.ac.jp/record/17966/files/110(8)_326-336.pdf
application/pdf
1.9 MB
2019-08-08