CASE REPORTS
JOURNAL ARTICLE
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A rare anterior spinal epidural cyst mimicking Hirayama disease.

Muscle & Nerve 2012 March
INTRODUCTION: Hirayama disease is a rare focal motor neuron disorder that manifests as slowly progressive unilateral or bilateral hand weakness and atrophy.

METHODS: The case report of a young man who presented with the phenotype of Hirayama disease indicated an extensive anterior cervical epidural arachnoid cyst.

RESULTS: A 34-year-old man presented with a 5-year history of slowly progressive hand and forearm weakness and atrophy. Nerve conduction studies demonstrated low median and ulnar motor amplitudes, and EMG demonstrated fibrillation potentials and long-duration, high-amplitude motor unit potentials in C6-T4-innervated muscles. MRI demonstrated a longitudinally extensive anterior spinal epidural cyst extending from C2 to L1. The patient had improved hand strength after surgery.

CONCLUSIONS: Anterior cervical epidural spinal cysts should be considered in the differential diagnosis in patients who present with slowly progressive hand weakness.

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