Clinical, electromyographic and cervical magnetic resonance imaging features of Hirayama disease
10.3760/cma.j.issn.1671-8925.2009.08.019
- VernacularTitle:平山病患者的临床电生理及颈磁共振成像研究
- Author:
Shan-Wei FENG
1
;
Yin-Xing LIANG
;
Ying-Yin LIANG
;
Bo-Ning LUO
;
Juan DU
;
Zheng-Shan LIU
;
Cheng ZHANG
Author Information
1. 中山大学
- Keywords:
Hirayama disease;
Muscular atrophy;
Electromyography;
Magnetic resonance imaging;
Cervical vertebrae
- From:
Chinese Journal of Neuromedicine
2009;8(8):828-831
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical, electromyographic and cervical magnetic resonance imaging (MRI) characteristics of Hirayama disease. Methods Fifteen patients with Hirayama disease were observed for special clinical manifestations and underwent electromyographic examination of the bilateral distal upper limb muscles and peripheral nerve conduction velocity. MRI of the neck in neutral and fully flexed positions was performed to identify potential lower cervical cord atrophy and cervical curvature anomalies. Results All the 15 male patients had disease onset during puberty with asymmetric muscular atrophy and weakness of the hands and forearms. Concentric needle electromyography revealed prolonged duration and large amplitude of the motor unit potentials in the compromised distal limb muscles with also increased polyphasic potentials and poor recruitment, involving mainly the C7, C8 and T1 myotomes. In neutral neck position, MRI identified lower cervical cord atrophy in 9 patients, occurring mainly at C5, C6 levels;in fully flexed position, all patients showed forward displacement and flattening of the lower cervical cord, occurring mostly at C6 level. Conclusion Hirayama disease occurs mainly in puberty in young male patients, whose clinical features and electromyographic examination often indicate localized anterior horn anomalies in the lower cervical cord. MRI of the neck in neutral and fully flexed position can provide valuable assistance in the diagnosis of this disease.
