AXONAL DEGENERATION OF THE PERIPHERAL-NERVES AND POSTGANGLIONIC ANHIDROSIS IN A PATIENT WITH MULTIPLE-SCLEROSIS
AXONAL DEGENERATION OF THE PERIPHERAL-NERVES AND POSTGANGLIONIC ANHIDROSIS IN A PATIENT WITH MULTIPLE-SCLEROSIS
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DOI：
10.1620/tjem.162.279
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发表时间：
1990-11-01
影响因子：
2.2
通讯作者：
ISHII, T
中科院分区：
文献类型：
--
作者：
SAITO, H;KOBAYASHI, K;ISHII, T
一位36岁的女性，自24岁起，有多次不同程度的视力丧失和脊柱症状和体征，被诊断为多发性硬化症(MS)。脑脊液髓鞘碱性蛋白升高。神经学和电生理检查提示周围神经受累。发病约六年后进行腓肠神经活检，发现有髓纤维和无髓纤维均严重丢失。随后，组胺皮肤反应在下肢出现缺陷。运动和瞳孔功能测试显示中枢和节后交感神经系统均有缺陷。虽然我们无法发现周围神经损害的致病因素，但我们的患者似乎是第一个记录的与周围躯体和自主神经系统轴突变性相关的多发性硬化病例。
A 36-year-old woman had, since the age of 24, numerous episodes of visual loss and spinal symptoms and signs at various levels, and was diagnosed as multiple sclerosis(MS). CSF myelin-basic-protein was increased. Neurological and electrophysiological investigations suggested the peripheral nerve involvement. Sural nerve biopsy performed about six years after the onset, revealed severe loss of both myelinated and unmyelinated fibers. Subsequently, histamine skin reaction was defective in the lower limbs. Tests on sudomotor and pupillary functions indicated deficits of both central and postganglionic sympathetic systems. Though we could not detect causative factors for the peripheral nerve lesions, our patient appears to be the first documented case of MS associated with axonal degeneration of the peripheral somatic and autonomic nervous systems.