Skeletal dysplasia and myelopathy in congenital disorder of glycosylation type IA.

Schade van Westrum, Steven M; Nederkoorn, Paul J; Schuurman, P Richard; Vulsma, Tom; Duran, Marinus; Poll-The, Bwee Tien · J Pediatr · 2006

case_report · Level V

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Abstract

We report on a boy with a congenital disorder of glycosylation (CDG) Ia and a severe narrowing of the spinal canal caused by atlantoaxial subluxation with anterior displacement of C1. C1-laminectomy improved the progressive paresis. Progressive paresis caused by spinal cord compression is a hitherto unrecognized complication in patients with CDG-Ia.

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