Hirayama disease.
Hirayama disease (juvenile muscular atrophy of distal upper extremity) is a cervical myelopathy. Predominantly affecting male adolescents, it is characterized by progressive muscular weakness and atrophy of distal upper limbs, followed by spontaneous arrest within several years. Although the cause of cervical myelopathy remains unclear, neuropathologic and neuroradiologic findings suggest a forward displacement of the posterior cervical dural sac during neck flexion, causing compression of the cervical cord, and results in atrophic and ischemic changes in the anterior horn. A good understanding of Hirayama disease is essential because early recognition and management can effectively halt the progressive deterioration.
Department of Diagnostic Radiology, Shuang-Ho Hospital, Taipei Medical University, New Taipei City, Taiwan, Republic of China.
Yen-Lin
YL
Huang
Chi-Jen
CJ
Chen
D002574:Cervical Vertebrae / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D059906:Neuroimaging; D011237:Predictive Value of Tests; D012680:Sensitivity and Specificity; D013116:Spinal Cord / Q000473:pathology; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy