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: Igor Paredes, Jesus Esteban, Ana Ramos, Pedro Gonzalez, Juan José Rivas
• Journal: Journal of neurosurgery. Spine (J Neurosurg Spine) Vol. 20 Issue 2 Pg. 191-5 (Feb 2014) ISSN: 1547-5646 [Electronic] United States
• PMID: 24286527 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Cervical Vertebrae (pathology, surgery)
• Diskectomy
• Humans
• Male
• Spinal Cord Compression (etiology, pathology, surgery)
• Spinal Fusion (methods)
• Spinal Muscular Atrophies of Childhood (complications, pathology, surgery)
• Treatment Outcome