Mucopolysaccharidosis type 6

Mucopolysaccharidosis type 6 (MPS 6) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate.

Spinal cord compression

External mechanical compression of the spinal cord.


Total: 2

                       


(per page)
PMID (PMCID)
23258111
 FEMALE Child
Spinal cord compression in Maroteaux-Lamy syndrome: case report and review of the literature with effects of enzyme replacement therapy.
Jurecka A, Opoka-Winiarska V, Jurkiewicz E, Marucha J, Tylki-Szymanska A.
Pediatr Neurosurg. 2012;48(3):191-8.
The purpose of this report is to review the literature regarding spinal cord compression in mucopolysaccharidosis type VI (MPS VI), to discuss the possible impact of enzyme replacement therapy (ERT) and to stress the necessity of timely surgical intervention.
21813307
 MIXED_SAMPLE Child
Spinal cord compression in young children with type VI mucopolysaccharidosis.
Horovitz DD, Magalhaes Tde S, Pena e Costa A, Carelli LE, Souza e Silva D, de Linhares e Riello AP, Llerena JC Jr.
Mol Genet Metab. 2011;104(3):295-300.
Spinal cord compression (SCC) is a known complication of mucopolysaccharidosis type VI (MPS VI) secondary to atlantoaxial subluxation, craniovertebral stenosis, posterior longitudinal ligament hypertrophy, or dural thickening.