A Case of Mucopolysaccharidosis Type I with Spinal Cord Compression.
- Author:
Sang Myung CHEON
1
;
Min Jeong PARK
;
Jae Kwan CHA
Author Information
1. Department of Neurology, College of Medicine, Dong-A University, Korea.
- Publication Type:Case Report
- Keywords:
Mucopolysaccharidosis;
Spinal cord compression
- MeSH:
Decompression, Surgical;
Dermatan Sulfate;
Dura Mater;
Female;
Humans;
Laminectomy;
Magnetic Resonance Imaging;
Mucopolysaccharidoses*;
Mucopolysaccharidosis I*;
Quadriplegia;
Spinal Cord Compression*;
Spinal Cord*;
Spine;
Young Adult
- From:Journal of the Korean Neurological Association
2002;20(2):199-201
- CountryRepublic of Korea
- Language:Korean
-
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.
