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Neurological disorders associated with cassava diet: a review of putative etiological mechanisms.

机译:木薯饮食相关的神经系统疾病:推测的病因机制综述。

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摘要

Tropical ataxic neuropathy (TAN) and epidemic spastic paraparesis (konzo) are two neurological disorders associated with the consumption of cassava (Manihot esculenta) in several African countries. TAN is characterized by sensory polyneuropathy, sensory ataxia, bilateral optic atrophy and bilateral sensori-neural deafness. It occurs in elderly individuals subsisting on a monotonous cassava diet with minimal protein supplementation. Konzo is a syndrome of symmetrical spastic paraparesis with a predilection for children and young women and invariably associated with consumption of inadequately processed bitter cassava roots with minimal protein supplementation. Despite numerous epidemiological, clinical and biochemical studies aimed at elucidating the etiological mechanisms of these disorders, their etiologies remain unknown, and there is no known treatment. The diseases continue to be prevalent in endemic areas, causing significant disability and increased mortality. A fresh appraisal of the putative etiologic mechanisms proposed for these intriguing and enigmatic syndromes is presented in this paper. Evidences against a causal role for cyanide intoxication are discussed, and evidences implicating thiamine deficiency as a unifying etiological mechanism for these neurological syndromes are presented. It is concluded that urgent research is needed to evaluate thiamine status and implement a therapeutic trial of thiamine in these debilitating neurological disorders.
机译:热带共济失调性神经病(TAN)和流行性痉挛性轻瘫(konzo)是一些非洲国家与食用木薯(Manihot esculenta)相关的两种神经系统疾病。 TAN的特征是感觉性多神经病,感觉性共济失调,双侧视神经萎缩和双侧感觉神经性耳聋。它发生在依靠单调木薯饮食而蛋白质补充最少的老年人中。 Konzo是对称性痉挛性轻瘫的综合症,易患儿童和年轻妇女,并且总是与食用未充分加工的苦味木薯根以及最少补充蛋白质有关。尽管进行了大量旨在阐明这些疾病的病因机制的流行病学,临床和生化研究,但其病因仍然未知,并且没有已知的治疗方法。该病继续在流行地区流行,造成严重的残疾并增加了死亡率。本文对这些有趣的和神秘的综合症提出了可能的病因学机制进行了新的评估。讨论了针对氰化物中毒的因果作用的证据,并提出了暗示硫胺素缺乏作为这些神经系统综合征的统一病因机制的证据。结论是需要紧急研究以评估硫胺素的状态并在这些使人衰弱的神经系统疾病中进行硫胺素的治疗性试验。

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