Hirayama disease; an atypical clinical manifestation of a cervical myelopathy with typical MRI features

Description

A young man was evaluated because of progressive weakness in both hands since 2 years. He played sport on a high level and experienced difficulties in catching the ball. Flexion of the neck caused paresthesias and an electric shock like sensation in both arms. He experienced muscle cramps during cold.

Neurological examination revealed almost symmetrical atrophy of the interosseous, thenar and hypothenar muscles without fasciculations and with weakness of these muscles and the distal extensor muscles, sparing the brachioradialis. There was no weakness of the proximal muscles of both arms. The reflexes were normal. There were no signs of a pyramidal syndrome. The examination of the legs, cranial nerves and sensibility was unremarkable.

The paresthesias during flexion of the neck suggested a cervical myelopathy, but the distribution of the muscle weakness suggested a neuromuscular disorder within the differential diagnosis, an anterior horn cell disease, multifocal motor neuropathy (weakness...

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