- J Korean Neurol Assoc. 2002;20(2):199-201.
- A Case of Mucopolysaccharidosis Type I with Spinal Cord Compression
- Sang-Myung Cheon
- Department of Neurology, College of Medicine, Dong-A University
- 척수압박을 동반한 I형 뮤코다당체침착증 1예
- 천상명 , 박민정 차재관
- 동아대학교 의과대학 신경과학교실
- 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"
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