Dropped-head syndrome due to steroid responsive focal myositis: a case report and review of the literature

Isolated severe weakness of the paraspinal musculature, either at the cervical level leading to a "dropped head syndrome" or at the thoracic level leading to a "bent-spine syndrome", is a rare disorder. Etiologically it may be present in a variety of neurological diseases including Parkinson's disease, multiple system atrophy, neuromuscular or motor neuron diseases, as well as non-inflammatory, inflammatory, dystrophic or metabolic myopathies. We present a previously healthy 74-year-old man with a 2-month history of progressive difficulty in lifting his chin off his chest. Magnetic resonance imaging and skeletal muscle biopsy revealed an isolated myositis of the neck extensor and trapezius muscles, which responded well to steroid therapy. This case and other rare reports obtained from a systematic review of the literature indicate that in a subgroup of patients "dropped head syndrome" or "bent spine syndrome" is caused by a myositis, which emphasizes the necessity to obtain a MRI examination, as well as a muscle biopsy to diagnose this potentially treatable disease.