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甲状腺功能减退性多发性神经病。两例患者的临床、电生理及神经活检结果

Hypothyroid polyneuropathy. Clinical, electrophysiological and nerve biopsy findings in two cases.

作者信息

Pollard J D, McLeod J G, Honnibal T G, Verheijden M A

出版信息

J Neurol Sci. 1982 Mar;53(3):461-71. doi: 10.1016/0022-510x(82)90243-x.

Abstract

Two patients in whom polyneuropathy was associated with hypothyroidism have been studied clinically and electrophysiologically. Sural nerve biopsy was performed on both patients and the nerve studied by light and electron microscopy. Both patients had symptoms of paraesthesiae and muscle pain and there was distal weakness, sensory impairment and incoordination in both upper and lower limbs. Gait was impaired and tendon reflexes were depressed. Electrophysiological studies demonstrated moderate slowing of motor conduction velocity and absent sensory potentials. Microscopic studies of the sural nerves revealed a loss of myelinated fibers of all diameters but particularly those of large diameter. On teased fibre examination, the predominant abnormality was axonal degeneration and electron microscopy showed degenerating fibres, prominent cluster formations, abnormalities of mitochondria and prominent glycogen deposits within Schwann cells. Quantitative study of unmyelinated fibres indicated a relative increase in fibres of small diameter. It is concluded that the polyneuropathy associated with hypothyroidism is due largely to axonal degeneration.

摘要

对两名患有与甲状腺功能减退相关的多发性神经病的患者进行了临床和电生理研究。对两名患者均进行了腓肠神经活检,并通过光学显微镜和电子显微镜对神经进行了研究。两名患者均有感觉异常和肌肉疼痛症状,上下肢均存在远端无力、感觉障碍和共济失调。步态受损,腱反射减弱。电生理研究显示运动传导速度中度减慢,感觉电位消失。腓肠神经的显微镜检查显示各种直径的有髓纤维均有丢失,但尤其是大直径纤维。在单纤维检查中,主要异常是轴突变性,电子显微镜显示纤维变性、明显的簇状形成、线粒体异常以及施万细胞内明显的糖原沉积。对无髓纤维的定量研究表明小直径纤维相对增加。结论是,与甲状腺功能减退相关的多发性神经病主要是由于轴突变性所致。

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