Subacute onset Hirayama disease with extensive myelopathy on MRI in flexed posture

Subacute onset Hirayama disease with extensive myelopathy on MRI in flexed posture Acta Neurol Belg (2017) 117:795–797 DOI 10.1007/s13760-017-0782-y LETTER TO THE EDITOR Subacute onset Hirayama disease with extensive myelopathy on MRI in flexed posture 1 1 1 1,2 • • • Merel Brems Johan Van Goethem Mania De Praeter Barbara Willekens Received: 13 March 2017 / Accepted: 12 April 2017 / Published online: 25 April 2017 Belgian Neurological Society 2017 Introduction basketball at high level. The initial examination showed atrophy and decreased force on finger extension and Our case is an exceptional illustration of the proposed flexion- abduction (MRC 4/5), more pronounced at the ulnar side. induced pathophysiology of Hirayama disease. Evidence, Pain nor sensory disturbances were present. Initial MRI privileging a mechanical pathophysiology, is growing and revealed a T2-hyperintense and atrophic cervical cord diagnostic inclusion criteria are involving MRI-confirmed reaching from C4 to C7, initially suggesting a neuro-in- evidence of the mechanical pathophysiology. The forward flammatory medullopathy (Fig. 1). However, extensive displacement of the posterior wall during neck flexion is workup was negative in that aspect. Motor nerve conduc- considered to be the hallmark and primary pathophysiologic tion studies were normal with needle EMG showing evi- mechanism of this disease [1]. In our case, the subacute onset dence of Acta Neurologica Belgica Springer Journals

Subacute onset Hirayama disease with extensive myelopathy on MRI in flexed posture

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Springer International Publishing
Copyright © 2017 by Belgian Neurological Society
Biomedicine; Neurosciences; Neurology; Neuroradiology; Medicine/Public Health, general
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