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A 17-year-old boy developed bilateral hand tremulousness, weakness, and decreased muscle bulk over 6 months. Bilateral extremity wasting with sparing of brachioradialis muscles (figure 1) was evident. With arms outstretched, distal mini-polymyoclonus (video 1) occurred. Neurophysiology suggests a pure motor neuropathy (chronic denervative changes in C7-T1 roots). MRI spine (dynamic flexion sequences)1 (figure 2) revealed cervical cord atrophy, dural sac anterior shifting, and contrast-enhancing epidural compartment. The constellation of features suggests Hirayama disease,2 postulated to be secondary to chronic segmental cord ischemia from repeated neck flexion over a tight dural sac. The polymyoclonus should not be mistaken for tremors.
Footnotes
Republished from Neurology 2015;85(21):e156-e157. doi: 10.1212/WNL.0000000000002144
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- © 2015 American Academy of Neurology
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