[Dropped head sign and tongue atrophy in systemic sclerosis-associated myopathy: a case report]

We report a 70-year-old man with a 5-year history of Raynaud's phenomenon and slow progression of weight loss, easy fatigability, muscle weakness, skin sclerosis, and dropped head sign. The patient was assumed to have motor neuron disease by his attending physician, and was referred to our hospital. Physical examination showed skin sclerosis on the fingers, hands, forearms, face, and neck. Neurological examination showed advanced systemic muscle atrophy and weakness, especially in the neck, tongue, and proximal limb muscles. Fasciculations were not observed in these involved muscles. Deep tendon reflexes were hypoactive and pathological reflexes were negative. Sensory disturbance was absent. Laboratory tests showed moderately elevated serum creatine kinase level, and increased serum antinuclear antibody titer (1:5120 with a nucleolar pattern). Needle electromyography showed a typical myogenic pattern in proximal muscles. The patient was diagnosed as having systemic sclerosis (SSc) with SSc-associated myopathy. Severe systemic muscle involvements, including dropped head sign and tongue atrophy, are rare manifestations in patients with SSc-associated myopathy. Our observations suggest that it is essential to pay attention to the dermatological findings of SSc in differential diagnosis of patients with muscular atrophy.