Chopra J S, Sharma A
Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
J Neurol Sci. 1992 Jul;110(1-2):8-20. doi: 10.1016/0022-510x(92)90003-4.
Protein-energy malnutrition (PEM), a natural ramification of poverty, continues to be a perennial source of concern to a large segment of the world population. The developing nervous system of a child is specially vulnerable to deprivations in nurture. Peripheral nerve and muscle derangements are clinically evident by weakness, hypotonia and hyporeflexia in accordance with severity and duration of PEM. Motor and sensory nerve conduction studies exhibit significant abnormalities and often furnish useful and ominous correlation with grades of PEM. The human sural nerve histology in cases of severe PEM is characterized by persistence of small myelinated fibres, striking failure of internodal elongation and significant segmental demyelination. Young rhesus monkeys are ideal experimental PEM models and they show myopathic EMG changes amenable to rehabilitation. Muscle pathology comprises obliteration of cross-striations, streaming of Z bands, increased interfibrillary spaces, mitochondriomegaly and small-for-age fibres. Radioisotope assays reveal anomalous incorporation into various nerve and muscle constituents. Central nervous system, specially the neuropsychological functions are affected in a lasting manner. Learning deficits, behavioural problems and manual indexterity are most obtrusive features.
蛋白质-能量营养不良(PEM)是贫困的自然衍生结果,仍然是世界上很大一部分人口长期关注的问题。儿童发育中的神经系统特别容易受到营养缺乏的影响。根据PEM的严重程度和持续时间,周围神经和肌肉紊乱在临床上表现为虚弱、肌张力减退和反射减弱。运动和感觉神经传导研究显示出明显异常,并且常常与PEM的分级呈现出有用且不祥的相关性。严重PEM病例中的人类腓肠神经组织学特征为小的有髓纤维持续存在、节间伸长显著失败以及明显的节段性脱髓鞘。幼年恒河猴是理想的实验性PEM模型,它们表现出适合康复的肌病性肌电图变化。肌肉病理学包括横纹消失、Z带流、肌原纤维间间隙增加、线粒体肿大和年龄相关的小纤维。放射性同位素测定显示在各种神经和肌肉成分中的异常掺入。中枢神经系统,特别是神经心理功能受到持久影响。学习缺陷、行为问题和手部灵活性是最突出的特征。