CHILDHOOD NEUROMUSCULAR DISORDERS - A DECADES EXPERIENCE IN SAUDI-ARABIA

The study describes the pattern of childhood neuromuscular disorders s een in a decade (1982-1992) at King Khalid University Hospital, Riyadh , Saudi Arabia. Similar data are scanty outside Europe and North Ameri ca, and lacking in Saudi Arabia. Eighty-four children (less than or eq ual to 16 years) were assigned to an entity of neuromuscular disease f ollowing review of the clinical, biochemical and neurophysiological da ta, and after re-examination of the histological and histochemical fea tures of the muscle biopsies. Of the 84 ascertained cases, 40 (48%) ha d different forms of muscular dystrophy (MD), 26 (31%) had one of the various types of spinal muscular atrophy (SMA) and two (2.4%) heredita ry motor and sensory neuropathy type I. The rest were miscellaneous ca ses including one (1.2%) with dermatomyositis. Of the dystrophies, sev ere childhood autosomal recessive muscular dystrophy (SCARMD) was more prevalent (30%) than Duchenne type (25%), conforming with observation s from North African countries known to have a high incidence of consa nguineous marriages. Family history of other cases of SCARMD included three males and three females, one of whom died at 15 years, and consa nguinity was evident in 63%. Congenital MD, inherited in an autosomal recessive pattern, was also common (30%). A history of consanguinity w as present in 55%. Of the 26 cases of SMA, type I (Werdnig-Hoffman dis ease) was the most prevalent (69%). Consanguinity was ascertained in 6 5% of SMA families and histories revealed another 14 affected siblings . Autosomal recessive forms seem to constitute the bulk of neuromuscul ar disorders in Saudi Arabia.