J Korean Neurol Assoc.
2002 Mar;20(2):199-201.
A Case of Mucopolysaccharidosis Type I with Spinal Cord Compression
- Affiliations
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- 1Department of Neurology, College of Medicine, Dong-A University, Korea.
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.
