[A clinical research of hirayama disease].
OBJECTIVE
To summarize and analyze the clinical features of Hirayama disease.


METHODS
The authors summarized the clinical data of 29 cases of Hirayama disease in recent 9 years.


RESULTS
All of the 29 cases were male and the age of onset was 12 - 25 years. 22 of the 29 patients only had the symptoms of one upper limb and 7 of them had symptoms of both upper limbs. Most of them had symptoms on the right or the symptoms were on more severe in the right than left, while only one case had symmetric symptoms of both upper limbs. The arm appeared as a slope, this was due to partial muscular atrophy. EMG indicated that the impairment of spinal anterior cells were limited to the arm relevant segments and both sides were involved. Cervical MRI scan suggested spinal atrophy at the level of C(5 - 7) vertebral bodies, being asymmetric in most of the cases. The atrophy was more obvious on the side with more severe symptoms. There was one patient whose MRI showed high T(2) signal in the spinal anterior cells of the C(5 - 6) segments. All the cases used cervical supporter and were followed up for 1 - 5 years. All of them had good prognosis.


CONCLUSIONS
Hirayama disease is more common in men with hidden onset before 20 years of age. Most of the patients noted the symptoms of hands gradually about 2 years after a period of rapid growth in height in adolescence. The predominant clinical manifestations are as follows: atrophy of lower cervical cord in cervical MRI, asymmetric spinal atrophy in horizontal impaction and the degree of atrophy consistent with the symptoms of limbs. EMG could indicate bilateral spinal anterior impairment in lower cervical cord. Rare case may present the abnormal signal in the spinal cord, but pyramid signs were absent.
Ding
Yan
Y
Department of Neurology, Xuanwu Hospital of Capital Medical University, Beijing 100053, China.
Wang
Xiang-bo
XB
Li
Cun-jiang
CJ
D000293:Adolescent; D000328:Adult; D002648:Child; D004576:Electromyography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000503:physiopathology; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology