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运动神经元疾病的病因学与发病机制研究。1. 山黧豆中毒:确诊病例的临床发现。

Studies on the aetiology and pathogenesis of motor neuron diseases. 1. Lathyrism: clinical findings in established cases.

作者信息

Ludolph A C, Hugon J, Dwivedi M P, Schaumburg H H, Spencer P S

出版信息

Brain. 1987 Feb;110 ( Pt 1):149-65. doi: 10.1093/brain/110.1.149.

Abstract

Lathyrism is a toxic disease of the motor system constantly associated with primary consumption of the seed of Lathyrus sativus (chickling or grass pea). Neurological examination was performed on 38 affected subjects drawn from four regions of the Indian subcontinent endemic for lathyrism. All showed a consistent and largely symmetric pattern of neurological deficit similar to that displayed by 5 Europeans with longstanding lathyrism now living in Israel. Hallmarks of lathyrism include a pyramidal pattern of motor weakness combined with greatly increased tone in the thigh extensors and adductors and in the gastrocnemius muscles so that the more severely affected walk on the balls of their feet with a lurching scissoring gait. Extensor plantar responses are uniformly present in such cases, and the knee and ankle tendon reflexes are exaggerated and often clonic. Hoffmann signs and exaggerated biceps and/or triceps tendon jerks are also found in the most severely affected. Sensory signs are absent, although perverse sensations in the legs are frequently reported at the onset. Walking difficulties commonly begin suddenly but may also appear subacutely or insidiously. Some individuals experience partly reversible symptoms suggestive of a diffuse CNS excitation of somatic, motor and autonomic function.

摘要

山黧豆中毒是一种运动系统的中毒性疾病,一直与主要食用山黧豆(鸡豆或草豌豆)种子有关。对来自印度次大陆四个山黧豆中毒流行地区的38名患者进行了神经学检查。所有患者均表现出一致且基本对称的神经功能缺损模式,类似于目前居住在以色列的5名患有长期山黧豆中毒的欧洲人所表现出的模式。山黧豆中毒的特征包括运动性无力的锥体模式,同时大腿伸肌、内收肌和腓肠肌的肌张力大大增加,因此受影响较严重的患者用脚尖行走,步态呈蹒跚剪刀样。在这种情况下,跖伸反应均呈阳性,膝腱和跟腱反射亢进,且常为阵挛性。在受影响最严重的患者中还发现霍夫曼征以及肱二头肌和/或肱三头肌腱反射亢进。虽然在发病初期经常报告腿部有异常感觉,但无感觉体征。行走困难通常突然开始,但也可能亚急性或隐匿性出现。一些患者会出现部分可逆的症状,提示中枢神经系统对躯体、运动和自主功能存在弥漫性兴奋。

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