Transient Myoclonic State with Asterixis: Primary Motor Cortex Hyperexcitability is Correlated with Myoclonus

• Hitomi Takefumi

  Department of Neurology, Kyoto University Hospital, Japan Department of Respiratory Care and Sleep Control Medicine, Kyoto University School of Medicine, Japan

• Ikeda Akio

  Department of Neurology, Kyoto University Hospital, Japan

• Inouchi Morito

  Department of Neurology, Kyoto University Hospital, Japan

• Imamura Hisaji

  Department of Neurology, Kyoto University Hospital, Japan

• Nakagawa Tomokazu

  Department of Neurology, Kyoto University Hospital, Japan

• Fumuro Tomoyuki

  Department of Neurology, Kyoto University Hospital, Japan

• Matsumoto Riki

  Department of Neurology, Kyoto University Hospital, Japan

• Takahashi Ryosuke

  Department of Neurology, Kyoto University Hospital, Japan

抄録

Objective To clarify the clinical features and mechanism of the transience of myoclonus in patients with a transient myoclonic state with asterixis (TMA).<br> Methods We investigated the clinical and eletrophysiological profiles of 6 patients with TMA (age: 84±3 years). During an asymptomatic period, somatosensory evoked potentials (SEPs) were recorded in all 6 patients and motor evoked potentials (MEPs) were examined in 1 patient. SEPs were recorded and jerk-locked back averaging (JLA) was performed in 2 patients while symptomatic. SEPs were also recorded from 8 aged control subjects (age: 68±5 years).<br> Results All TMA patients had mild chronic systemic diseases. During an asymptomatic period, SEP amplitudes were not significantly enlarged in comparison with control subjects, and MEPs were normal. Examination of 2 patients during symptomatic period indicated no enlargement of SEP amplitudes and JLA disclosed a positive spike preceding myoclonic jerks. In one of these patients, the amplitude of the positive spike decreased once myoclonus improved.<br> Conclusion TMA occurred in aged patients with mild chronic systemic diseases. JLA findings and the absence of giant SEPs further support that TMA is a cortical non-reflex myoclonus. In addition, transient hyperexcitability at the primary motor cortex disclosed by JLA correlated well with its transient symptoms.<br>

収録刊行物

• Internal Medicine

  Internal Medicine 50 (20), 2303-2309, 2011

  一般社団法人 日本内科学会

キーワード

• transient myoclonic state with asterixis (TMA)
• generator mechanism of TMA

詳細情報

• CRID: 1390001204872282240
• NII論文ID: 130001087737
• DOI: 10.2169/internalmedicine.50.5590
• ISSN: 13497235
• ISSN: 09182918
• Web Site: http://www.jstage.jst.go.jp/article/internalmedicine/50/20/50_20_2303/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2012260065
• 本文言語コード: en
• データソース種別:
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  • Crossref
  • CiNii Articles
  • KAKEN
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