LAUSR

A 74-year-old, previously healthy woman was admitted with dropped head and proximal dominant limb weakness of 10 months duration (Picture 1A). Laboratory testing revealed a normal creatine kinase level, euthyroid, normokalemia, a negative result for anti-human immunodeficiency virus antibody without inflammatory findings or M-protein. Electromyography showed myogenic motor unit potentials. A muscle biopsy in the biceps brachii with hematoxylineosin and modified Gomori-trichrome staining demonstrated a variation in the muscle fiber diameter with remarkable macrophagic infiltration in the perimysium (Picture 2A), suggesting an inflammatory process, and scattered fibers with nemaline rods (Picture 2B, arrows), respectively. Sporadic late-onset nemaline myopathy (SLONM) was thus diagnosed. Intravenous methylprednisolone (1,000 mg/day for 3 consecutive days ×2) was administered, resulting in a dramatic improvement in the proximal muscle weakness and the symptoms of dropped head, after 3 weeks (Picture 1B). Dropped head has been reported in association with various neurological diseases, such as neuromuscular, muscular, parkinsonism and motor neuron diseases (1). SLONM should therefore be included in the differential diagnosis when encountering patients with dropped head syndrome (2).