An 18-year-old male presented with a two-year history of bilateral hand tremulousness. Neurological examination revealed weakness in both hands, but predominantly on the right. With arms outstretched, distal mini-polymyoclonus occurred11. Ong JJY, Wang FSJ, Chan YC. Teaching neuroimages: a case of Hirayama disease presenting with polymyoclonus. Neurology. 2015;85:e156-7. https://doi.org/10.1212/WNL.0000000000002144
https://doi.org/10.1212/WNL.000000000000...
. The rest of the neurological examination was unremarkable. Motor and sensory nerve conduction studies were normal. Electromyography showed fibrillations and fasciculations at rest and signs of mild chronic reinnervation in C8-T1 muscles bilaterally. Spine MRI with dynamic flexion sequences22. Lehman VT, Luetmer PH, Sorenson EJ, Carter RE, Gupta V, Fletcher GP et al. Cervical spine MR imaging findings of patients with Hirayama disease in North America: a multisite study. AJNR Am J Neuroradiol. 2013;34(2):451-6. https://doi.org/10.3174/ajnr.A3277
https://doi.org/10.3174/ajnr.A3277...
revealed cervical cord atrophy, forward shifting of the posterior wall of the cervical dural sac, and contrast-enhancement of an enlarged posterior epidural compartment (Figure). This constellation of features suggests Hirayama disease.
Neutral cervical spine MRI sagittal images (A and B). In A, a T1-weighted image shows C6-T1 cord atrophy (arrowheads) and in B a T2-weighted image demonstrates slight detachment of the posterior dura mater (arrow). Flexion cervical spine MRI sagittal images (C and D). In C, a T2-weighted image reveals forward displacement of the posterior wall of the dural sac with some impression over the cord (arrow). In D, a contrast-enhanced T1-weighted image shows engorgement of the posterior epidural venous plexus, with enhancement (arrow).
References
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1Ong JJY, Wang FSJ, Chan YC. Teaching neuroimages: a case of Hirayama disease presenting with polymyoclonus. Neurology. 2015;85:e156-7. https://doi.org/10.1212/WNL.0000000000002144
» https://doi.org/10.1212/WNL.0000000000002144 -
2Lehman VT, Luetmer PH, Sorenson EJ, Carter RE, Gupta V, Fletcher GP et al. Cervical spine MR imaging findings of patients with Hirayama disease in North America: a multisite study. AJNR Am J Neuroradiol. 2013;34(2):451-6. https://doi.org/10.3174/ajnr.A3277
» https://doi.org/10.3174/ajnr.A3277
Publication Dates
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Publication in this collection
Oct 2017
History
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Received
09 Jan 2017 -
Accepted
18 July 2017