MRI findings in Hirayama's disease:: flexion-induced cervical myelopathy or intrinsic motor neuron disease?

被引:91
作者
Schröder, R
Keller, E
Flacke, S
Schmidt, S
Pohl, C
Klockgether, T
Schlegel, U
机构
[1] Univ Bonn, Neurol Klin & Poliklin, D-53105 Bonn, Germany
[2] Univ Bonn, Radiol Klin, D-53105 Bonn, Germany
关键词
Hirayama's disease; juvenile spinal muscular atrophy; upper limbs;
D O I
10.1007/s004150050514
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Hirayama's disease is a benign juvenile form of focal amyotrophy affecting the upper limbs. Previous studies have suggested that the disorder is a neck flexion induced cervical myelopathy. We report clinical and magnetic resonance imaging findings in nine patients with Hirayama's disease. Cervical imaging of seven patients revealed spinal cord changes consisting of focal atrophy and foci of signal alterations. On neck flexion a forward movement and mild reduction in the anteroposterior diameter of the lower cervical cord against the vertebral bodies was noted in affected individuals as well as in five normal controls. In contrast to earlier reports, none of our patients showed complete obliteration of the posterior subarachnoid space. Measurement of the anteroposterior spinal cord diameter in each vertebral segment (C4-C7) revealed no significant differences in the degree of spinal cord flattening between the two groups. Furthermore, two of our patients had significant degenerative changes in the cervical spine (disc herniation, retrospondylosis) contralateral to the clinically affected side. These degenerative changes resulted in a marked cord compression on neck flexion but were not associated with ipsilateral clinical abnormalities or spinal cord alterations. Our results argue against a flexion-induced cervical myelopathy and support the view that Hirayama's disease is an intrinsic motor neuron disease.
引用
收藏
页码:1069 / 1074
页数:6
相关论文
共 24 条
[1]  
BIONDI A, 1989, AM J NEURORADIOL, V10, P263
[2]   JUVENILE DISTAL SPINAL MUSCULAR-ATROPHY OF UPPER EXTREMITIES IN CHINESE MALES - A SINGLE FIBER ELECTROMYOGRAPHIC STUDY OF ARMS AND LEGS [J].
CHAN, YW ;
KAY, R ;
SCHWARTZ, MS .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1991, 54 (02) :165-166
[3]  
Chen CJ, 1998, AM J NEURORADIOL, V19, P365
[4]   CHRONIC ASYMMETRICAL SPINAL MUSCULAR-ATROPHY [J].
HARDING, AE ;
BRADBURY, PG ;
MURRAY, NMF .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 1983, 59 (01) :69-83
[5]   COMPUTED TOMOGRAPHIC MYELOGRAPHY CHARACTERISTICS OF SPINAL-CORD ATROPHY IN JUVENILE MUSCULAR-ATROPHY OF THE UPPER EXTREMITY [J].
HIRABUKI, N ;
MITOMO, M ;
MIURA, T ;
HASHIMOTO, T ;
KAWAI, R ;
KOZUKA, T .
EUROPEAN JOURNAL OF RADIOLOGY, 1991, 13 (03) :215-219
[6]   FOCAL CERVICAL POLIOPATHY CAUSING JUVENILE MUSCULAR-ATROPHY OF DISTAL UPPER EXTREMITY - A PATHOLOGICAL-STUDY [J].
HIRAYAMA, K ;
TOMONAGA, M ;
KITANO, K ;
YAMADA, T ;
KOJIMA, S ;
ARAI, K .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1987, 50 (03) :285-290
[7]  
Hirayama K, 1991, HDB CLIN NEUROLOGY, V15, P107
[8]  
Hirayama K, 1959, J Psychiatr Neurol, V61, P2190
[9]   CERVICAL FLEXION MYELOPATHY - A TIGHT DURAL CANAL MECHANISM - CASE-REPORT [J].
IWASAKI, Y ;
TASHIRO, K ;
KIKUCHI, S ;
KITAGAWA, M ;
ISU, T ;
ABE, H .
JOURNAL OF NEUROSURGERY, 1987, 66 (06) :935-937
[10]   LACK OF CERVICAL PARASPINAL MUSCLE INVOLVEMENT IN JUVENILE DISTAL SPINAL MUSCULAR-ATROPHY - AN ELECTROMYOGRAPHIC STUDY ON 15 CASES [J].
KAO, KP ;
LIN, KP ;
CHERN, CM ;
WU, ZA ;
TSAI, CP ;
LIAO, KK .
JOURNAL OF NEUROLOGY, 1993, 240 (05) :284-286