歯状核赤核・淡蒼球ルイ体萎縮症(DRPLA)患者における体性感覚・視覚脳幹誘発電位の所見 : 他の進行性ミオクローヌスてんかんとの比較  [in Japanese] Differences in Evoked Potential Findings Between DRPLA Patients and Patients with Cortical Reflex Myoclonus  [in Japanese]

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Author(s)

    • 笠井 清登 KASAI Kiyoto
    • 国立精神・神経センター武蔵病院・精神科 Department of Psychiatry, National Center Hospital for Mental, Nerbous and Muscular Discorders, National Center of Neurology and Psychiatry
    • 加藤 昌明 KATO Masaaki
    • 国立精神・神経センター武蔵病院・精神科 Department of Psychiatry, National Center Hospital for Mental, Nerbous and Muscular Discorders, National Center of Neurology and Psychiatry
    • 加藤 武治 KATO Takeji
    • 国立精神・神経センター武蔵病院・臨床検査部 National Center of Neurology and Psychiatry
    • 竹谷 淳 TAKEYA Jun
    • 国立精神・神経センター武蔵病院・精神科 Department of Psychiatry, National Center Hospital for Mental, Nerbous and Muscular Discorders, National Center of Neurology and Psychiatry
    • 関本 正規 SEKIMOTO Masanori
    • 国立精神・神経センター武蔵病院・精神科 Department of Psychiatry, National Center Hospital for Mental, Nerbous and Muscular Discorders, National Center of Neurology and Psychiatry
    • 渡辺 慶一郎 WATANABE Keiichiro
    • 国立精神・神経センター武蔵病院・精神科 Department of Psychiatry, National Center Hospital for Mental, Nerbous and Muscular Discorders, National Center of Neurology and Psychiatry
    • 後藤 雄一 GOTO Yu-ichi
    • 国立精神・神経センター神経研究所・微細構造研究部 Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry
    • 三辺 義雄 MINABE Yoshio
    • 国立精神・神経センター神経研究所・疾患研究第7部 Division of Cortical Dunction Disorder, National Institute of Neurosience, National Center of Neurology and Psychiatry

Abstract

歯状核赤核・淡蒼球ルイ体萎縮症 (DRPLA) 患者を対象として、体性感覚および聴覚脳幹誘発電位 (SEP、ABR) を測定した。対象はDRPLA患者9家系10名で、そのうち9名は遺伝子診断によりCAGリピートの増大が確認されていた。対照群は.DRPLAでない進行性ミオクローヌスてんかん (PME) 患者3名で、そのうち2名は遺伝子診断によりUnverricht-Lundborg病と確認されていた。正中神経刺激による皮質SEPにおいて、DRPLA群ではいずれもP2-N2頂点問振幅値が10μV未満であったのに対し、PME群ではいずれも10μV以上の高振幅SEPを認めた。ABRにおいては、DRPLA群では8名中5名でいずれか一方の耳側刺激時にIII波以上の活動が消失していたのに対し、PME群では2名中2名ともI~V波までの活動を認めた。このように、DRPLAと他のPMEはSEP・ABRの所見が異なっていたことから、DRPLAの臨床診断における誘発電位測定の有用性が示唆された。またこれらの所見は、皮質下・脳幹が主たる責任部位であるDRPLAと、皮質の易興奮性が優勢な他のPMEとの病態生理の違いを反映していると考えられた。

Characteristics of evoked potentials in patients with Dentatorubral-pallidoluysian atrophy (DRPLA) were investigated. Ten patients with DRPLA and 3 patients with other progressive myoclonus epilepsy (PME) participated in the study. Nine patients out of 10 were genetically diagnosed as DRPLA, having 62-75 CAG triplet repeat expansion on chromosome 12p; the other one had not been genetically confirmed but clinically diagnosed as DRPLA and was within the same pedigree as one of the 9 genetically confirmed patients. Two patients with PME out of 3 had been tested for dodecamer repeat expansion in cystatin B gene, and were genetically confirmed as Unverricht-Lundborg disease; the other one was also clinically diagnosed as Unverri-cht-Lundborg disease, but the patient did not have such expansion.Somatosensory evoked potentials (SEP) and auditory brainstem responses (ABR) were recorded. Amplitudes of SEP were determined as peak to peak amplitudes between P2 and N2 deflections.The results of the SEP revealed that none of the DRPLA patients evoked high amplitude SEPs; on the other hand, patients with other PME all evoked high amplitude SEPs.Moreover, brainstem activity in ABR were absent in 5 patients with DRPLA out of 8.These results suggest the difference in pathophysiology between DRPLA, being dominantly affected over brainstem and subcortical regions, and other PME, having cortical hyperexcitability.

Journal

  • Journal of the Japan Epilepsy Society

    Journal of the Japan Epilepsy Society 16(3), 184-192, 1998-10-31

    JAPAN EPILEPSY SOCIETY

References:  31

Cited by:  2

Codes

  • NII Article ID (NAID)
    10008556090
  • NII NACSIS-CAT ID (NCID)
    AN10043823
  • Text Lang
    JPN
  • Article Type
    Journal Article
  • ISSN
    09120890
  • Data Source
    CJP  CJPref  J-STAGE 
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