A Case of Mucopolysaccharidosis Type I with Spinal Cord Compression

J Korean Neurol Assoc > Volume 20(2); 2002 > Article

Journal of the Korean Neurological Association 2002;20(2): 199-201.

척수압박을 동반한 I형 뮤코다당체침착증 1예

천상명 , 박민정 차재관

동아대학교 의과대학 신경과학교실

A Case of Mucopolysaccharidosis Type I with Spinal Cord Compression

Sang-Myung Cheon

Department of Neurology, College of Medicine, Dong-A University

Abstract

"A 21-year-old woman with gargoyloid face and short trunkal stature showed progressive quadriparesis. Cervical spine MRI showed circumferential compression of cervical spinal cord by thickened dura mater. Elevated urinary dermatan sulfate and decreased enzyme activity of -iduronidase revealed that she has mild form of mucopolysaccharidosis type I. Her weakness was improved with cervical laminectomy and duraplasty. In this case, progressing quadriparesis resulted from cervical spinal cord compression by thickened dura mater. Earlier surgical decompression could have been better for the patient.Key Words : Mucopolysaccharidosis, Spinal cord compression"