A Case of Incontinentia Pigmenti with Destructive Encephalopathy

J Korean Neurol Assoc > Volume 16(5); 1998 > Article

Journal of the Korean Neurological Association 1998;16(5): 739-742.

Destructive?Encephalopathy를 보인 Incontinentia Pigmenti?1예

한진규, 최재철 ? 박민규 ? 박건우? 은백린* ? 정지태* ? 이대희

고려대학교 의과대학 신경과학교실, 소아과학교실*

A Case of Incontinentia Pigmenti with Destructive Encephalopathy

Jin-Kyu Han,M.D., Jae-Cheol Choi, M.D., Min-Kyu Park,M.D., Kun-Woo Park,M.D., Baik-Lin Eun,M.D.*, Ji-Tae Chung,M.D.*, Dae-Hie Lee.,M.D.

Department of Neurology, Pediatrics*, Korea University, College of Medicine

Abstract

Spinal arteriovenous malformation ( AVM ) can be a rare cause of relapsing myelopathy. We recently have the opportunity to study a 20-year-old male patient having spinal epidural AVM confirmed by surgical biopsy. To our knowledge, a case of relapsing and remitting course of spinal epidural AVM has not been reported in Korea. A 20-year-old male was admitted because of sudden paraparesis and disturbance of all sensory modalities below the T5 dermatome, 5 times during 2 years. Cerebrospinal fluid profiles did not reveal any pathologic findings. Until the 4th attack, MR findings were non diagnostic. At the 5th attack, thoracic spine MR revealed epidural hematoma compressing the spinal cord. Diagnostic and therapeutic decompressive surgical procedure was done. On surgical field, solitary and tortuous feeding vessels with hematoma were seen in epidural space. Microscopically, the tissue was compatible with arteriovenous malformation. Key words : Relapsing myelopathy - Spinal epidural AV malformation - Spinal epidural hematoma