title: A severe case of Hirayama disease successfully treated by anterior cervical fusion.
abstract: Hirayama disease, or juvenile amyotrophy of distal upper extremity, is a benign, self-limiting cervical myelopathy consisting of selective unilateral weakness of the hand and forearm. The weakness slowly progresses until spontaneous arrest occurs within 5 years of onset. The condition predominantly affects Asian males and is thought to be secondary to spinal cord compression during neck flexion, because of a forward displacement of the posterior dural sac. The authors present what is to their knowledge the first reported case of a Caucasian male with a severe form of Hirayama disease, suffering from weakness of the leg as well as the forearm. An abnormal range of cervical flexion was observed at the C5-6 level. The patient was successfully treated by anterior cervical discectomy and fusion.
authors.affiliation: Neurosurgery Service.
authors.forename: Igor
authors.initials: I
authors.lastname: Paredes
authors.forename: Jesus
authors.initials: J
authors.lastname: Esteban
authors.forename: Ana
authors.initials: A
authors.lastname: Ramos
authors.forename: Pedro
authors.initials: P
authors.lastname: Gonzalez
authors.forename: Juan José
authors.initials: JJ
authors.lastname: Rivas
mesh_terms: D000328:Adult; D002574:Cervical Vertebrae / Q000473:pathology / Q000601:surgery*; D017586:Diskectomy; D006801:Humans; D008297:Male; D013117:Spinal Cord Compression / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D013123:Spinal Fusion / Q000379:methods*; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome