A 70-year-old man with a history of hypertension and chronic migraine presented with 10 months’ history of progressive generalized muscle weakness and stiffness. He reported a 19-kg unintentional weight loss over the last 7 months. He described worsening exertional dyspnea over the past 3 weeks prior to presentation. He had multiple surgical procedures for joint contractures and bilateral carpal tunnel syndrome in the past 5 years. He had no history of smoking or alcohol consumption. Physical examination revealed generalized muscle wasting except for hypertrophy of bilateral shoulder muscles (Figure 1 A) with proximal muscle weakness of the shoulders. The reflexes and sensation were intact in both upper limbs, and there was no distal weakness.

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