環状20番染色体をもち, 非けいれん性発作重延状態を呈するてんかん患者の1例 : 高次大脳機能障害の評価  [in Japanese] A Case of Ring Chromosome 20 with Non-convulsive Status Epilepticus : Diagnosis of Ictal Higher Cerebral Dysfunction by Means of a Neuropsychological Test Battery  [in Japanese]

Access this Article

Search this Article

Author(s)

    • 笹川 睦男 SASAGAWA Mutsuo
    • 国利雨量要所西新潟中央病院(てんかんセンター) 神経精神科 Department of Neuro-psychiatry, Epilepsy Center Nishi-niigata Chuou Hospital
    • 中島 悦子 NAKAJIMA Etsuko
    • 国利雨量要所西新潟中央病院(てんかんセンター) 神経精神科 Department of Neuro-psychiatry, Epilepsy Center Nishi-niigata Chuou Hospital
    • 吉野 美穂子 YOSHINO Mihoko
    • 国利雨量要所西新潟中央病院(てんかんセンター) 心理療法室 Department of Neuro-psychiatry, Epilepsy Center Nishi-niigata Chuou Hospital

Abstract

環状20番染色体46、XY、r (20) (p13、q13) をもち、非けいれん性発作重延を呈した18歳男性に高次大脳機能検査を施行した。発作症状は表情に生彩を欠き、うつむき加減で瞬目が多く自発語は少ないが、単純な呼名には応答できる。発作の持続は10-50分間であり、1日に3-4回出現した。発作時脳波は、びまん性の高振幅徐波で両側前頭葉に棘波が時に重畳する。EEG-VTR同時記録を用いて発作出現時の意識障害プロフィールを検討した。検討項目は自発語 (情報の内容、流暢性) 、聴理解 (返答、動作) 、復唱、物品呼称、読みなどWAB失語症検査 (Western Aphasia Battery) の下位検査と統語的問題解決能力測定のため計数/計算 (田中ビネー) と数唱 (WAIS-R数順唱) を加えた。発作間歓時のプロフィールと比較すると復唱は健常を保ったが、自発語、聴理解、計数/計算が不良で、ことに計数/計算の高度障害は前頭葉内側面の巻き込みを推測させた。

A male with a ring chromosome 20 presenting with non-convulsive status epile-pticus (NCSE) was diagnosed to have a higher cerebral dysfunction by means of a neuropsychological test battery. He developed complex partial seizures at the age of 9 years, and since the age of 11, he has had daily episodes of NCSE, not responsive to medication. At the age of 18, he was identified as having a ring chromosome 20 [46, XY, r (20) (p13, q13)]. While being monitored by EEG-VCR, he was examined by a neuropsychological test battery, including tests for spontaneous speech, auditory verbal comprehension, repetition, naming on visual confrontation, reading, and one-and two-digit calculation. Comparing the results with those obtained during the interictal states, we identified his poorer ability of spontaneous speech and one- and two-digit calculation during the ictal state. On the other hand, repetition was intact during both the ictal and interictal states. The discrepancy between the poor spontane-ous speech and the good repetition led us to conclude that this ictal state was similar to transcortical motor aphasia. Involvement of the medial frontal lobe, in particular, the supplementary motor areas, was considered to be responsible for the poor calcula-tion ability.

Journal

  • Journal of the Japan Epilepsy Society

    Journal of the Japan Epilepsy Society 18(2), 140-147, 2000-06-30

    JAPAN EPILEPSY SOCIETY

References:  10

Cited by:  1

Codes

  • NII Article ID (NAID)
    10008556292
  • NII NACSIS-CAT ID (NCID)
    AN10043823
  • Text Lang
    JPN
  • Article Type
    Journal Article
  • ISSN
    09120890
  • Data Source
    CJP  CJPref  J-STAGE 
Page Top