Case Presentation: A 30-year-old man presented with progressively worsening weakness of both shoulder girdle muscles associated with wasting for a period of 1 year. The patient had initially noticed muscle aches followed by easy fatigability in using both arms and also difficulty in climbing stairs. There was no history to suggest involvement of the distal muscles of the extremities or pharyngeal, neck, trunk, or ocular muscles. He indicated that he had gained 4 kg during a 1-year period and had periorbital puffiness. Physical examination revealed sinus bradycardia (pulse, 52/min), dry skin, a dull expressionless face with puffy eyelids, and no evidence of goiter (Fig. 1). Neurologic examination disclosed proximal weakness of both shoulders in all movements in association with atrophy of muscles but without evidence of percussion myotonia over the deltoid muscles (Fig. 2). He had grade 4 strength (on a scale of 1 to 5) in both lower limbs proximally in conjunction with normal strength distally. Deep tendon jerks were elicitable with delayed relaxation. The rest of the systemic examination showed normal findings.
展开▼
