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diseases. This is expected to help in prevention through informed genetic counsellingand in designing new modalities of therapy.With regards to diseases of muscle, it has been established that the integrity of thecytoskeletal-extracellular linkage, mediated by the dystrophin glycoprotein complex(DGC), is important to the muscle membrane stability, and loss or abnormality of certaincomponents lead to a disruption of this linkage and cause various forms of MD. Sofar, at least seven different MDs are related to abnormalities of the DGC."Adhalin", derived from the Arabic word "Adhal" for muscle, is one of the DGC whichhas recently been implicated in the pathogenesis of severe childhood autosomal recessivemuscular dystrophy (SCARMD). During the last two decades, (SCARMD) wasidentified as a unique form of MD with high prevalence in populations of Arab descentin Africa and the Middle East. Over the decade 1982-1993, it was found to be commoner(30%) than Duchenne MD (25°/.)) and similar to congenital MD (30%) at KingKhalid University Hospital (KKUH) in Riyadh.The paper reviews the clinical and molecular pathological features of SCARMD seenin Sudan and Saudi Arabia; a newly described form of MD associated with deficiencyof a component of the DGC ( -dystroglycan); and a novel mutation in a Saudi familywith congenital MD due to partial deficiency of another component of the DGC (i.e.merosin or LAMA2). A fifth new variant or congenital MD characterized by arthrogryposismultiplex, cobblestone lissencephaly and merosin (LAMA2)-positive immunohistochemistrywill also be described, as well as, a sixth novel form (Salih, CMD).The latter was first described in a Sudanese family and is characterized by congenitalhypotonia associated with minimal myopathic changes and type-I fibre predominanceon muscle histology. Following achievement of motor developmental milestones, affectedpatients started to show progressive weakness associated with features of leftventricular dilated cardiomyopathy. Repeated muscle biopsies revealed florid dystrophicfeatures with normal expression of the DGC, including merosin (LAMA2).Integrin 7 and -dystroglycan (which has been discovered recently to be deficientin sub-groups of patients who present with congenital MD) were normally expressedin thesepatients.