A Spinal Muscular Atrophy With Scapuloperoneal Distribution

Abstract

PROGRESSIVE muscular atrophy with scapuloperoneal distribution is unusual. Kaeser indicated the diverse neuromuscular etiologies in his summary of previously reported cases, and described the first patient with proven anterior horn cell degeneration.¹ In the present paper a sporadic case is reported which differs from earlier cases and illustrates the difficulties that may be encountered in distinguishing disorders of the anterior horn cell from those of the peripheral nerve. Report of a Case A Negro girl was born after an uncomplicated full-term pregnancy, labor, and delivery, weighing over 2.7 kg (6 lb) at birth. She sat alone shortly before 1 year and walked at 14 months. Abnormal gait, with toe-walking, was evident at age 4. At age 6, in another hospital, severe bilateral equinovarus deformities of the feet, marked atrophy of the peroneal muscles, and mild weakness about the shoulder girdle were reported. Other muscles were normal. Laboratory tests