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Neck extensor muscle weakness causing dropped head is most commonly associated with myasthenia gravis, ALS, and paraspinous myopathy.1–3 Less often, myotonic dystrophy, facioscapulohumeral muscular dystrophy, hypothyroidism, chronic inflammatory demyelinating polyneuropathy, polymyositis/dermatomyositis, and inclusion body myositis are associated with dropped head.1 We describe a patient with isolated dropped head and no limb weakness due to adult-onset nemaline myopathy that improved with a posterior cervical spinal fusion. A 72-year-old man presented with a 10-year history of a progressive head drop. He denied limb weakness, diplopia, or imbalance. There was no family history of neuromuscular disorders. His physical examination revealed isolated neck extensor weakness with normal limb muscle strength (figure, A). Deep tendon reflexes and sensation were normal. Creatine kinase, thyroid-stimulating hormone, and acetylcholine receptor antibodies were normal or negative. Cervical spine MRI demonstrated multilevel degenerative changes. Sensory and motor …
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Katirji et al. (2005) studied this question.
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