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涉及双侧脑半球的儿童持续性部分性癫痫

Epilepsia partialis continua of childhood involving bilateral brain hemispheres.

作者信息

Takahashi Y, Kubota H, Fujiwara T, Yagi K, Seino M

机构信息

National Epilepsy Center, Shizuoka Higashi Hospital, Shizuoka, Japan.

出版信息

Acta Neurol Scand. 1997 Dec;96(6):345-52. doi: 10.1111/j.1600-0404.1997.tb00296.x.

DOI:10.1111/j.1600-0404.1997.tb00296.x
PMID:9449470
Abstract

OBJECTIVES

Epilepsia partialis continua (Kojewnikow's syndrome) can be classified into 2 groups. The 1st group had stable neurological deficit, and the 2nd group had slowly progressive neurological deficit. The latter usually manifests not tetraplegia, but hemiplegia. We describe 3 patients with epilepsia partialis continua, rapid neurological and mental deterioration, resulting in tetraplegia and serious mental deficits within 2 to 3 years from the onset.

RESULTS

Their interictal EEGs showed progressive findings of deterioration, which resulted in an inactive pattern or a pattern reminiscent of suppression-burst within several years. Their cranial CTs revealed rapid progressive atrophy of both hemispheres. Various screening tests failed to confirm a metabolic disease, a degenerative disease, or an infectious disease in the central nervous system.

CONCLUSION

Frequent status epilepticus might contribute to the bilateral brain involvement and the serious neurological and mental outcomes in young patients with epileptia partialis continua.

摘要

目的

持续性部分性癫痫(科热夫尼科夫综合征)可分为两组。第一组有稳定的神经功能缺损,第二组有缓慢进展的神经功能缺损。后者通常表现为偏瘫而非四肢瘫。我们描述了3例持续性部分性癫痫患者,其神经和精神状况迅速恶化,在发病后2至3年内导致四肢瘫和严重的精神缺损。

结果

他们的发作间期脑电图显示出进行性恶化的表现,几年内导致脑电图呈静止模式或类似爆发抑制的模式。他们的头颅CT显示双侧半球迅速进行性萎缩。各种筛查试验未能证实中枢神经系统存在代谢性疾病、退行性疾病或感染性疾病。

结论

频繁的癫痫持续状态可能导致持续性部分性癫痫年轻患者双侧脑受累以及严重的神经和精神后果。

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