HYPOGLYCEMIA IN SPINAL MUSCULAR-ATROPHY

Repeated episodes of hypoglycaemia were observed in two girls with spi nal muscular atrophy. During a 12 h fast blood glucose fell to 3.4 and 2.7 mmol/L, respectively. One girl developed hypoglycaemia and ketonu ria. Reduced gluconeogenesis was probably the cause of hypoglycaemia i n these patients who had a muscle mass of about 10% of bodyweight (nor mal 30-40%). Hypoglycaemia must be suspected and treated when patients with severe muscle wasting due to chronic neuromuscular disorders are admitted comatose. In our experience this condition is often regarded as respiratory insufficiency.