THE ORTHOPEDIC MANIFESTATIONS OF PELIZAEUS-MERZBACHER DISEASE IN CHILDREN

Pelizaeus-Merzbacher disease (PMD) is a degenerative leukodystrophy of the central nervous system resulting in progressive spasticity and ne urologic deterioration. Seitelberger (13) divided this rare disease in to six types. Five patients with the type I and two patients with the type II form of PMD have been treated at the Children's Hospital of Ea stern Ontario. Our study sought to identify the orthopaedic manifestat ions of PMD and to develop a common orthopaedic profile for these pati ents. All children with types I and II PMD developed spastic quadripar esis, truncal hypotonia, thoracolumbar scoliosis, soft-tissue contract ures of the adductors and hamstrings, osteopenia, bilateral coxa valga , and associated hip dislocation.