Chopra J S, Dhand U K, Mehta S, Bakshi V, Rana S, Mehta J
Brain. 1986 Apr;109 ( Pt 2):307-23. doi: 10.1093/brain/109.2.307.
Forty-three children (aged 7 to 62 months) with protein calorie malnutrition (PCM) were studied; 13 had mild to moderate PCM and 30 severe PCM. A reduction of motor nerve conduction velocity and abnormalities of sensory conduction were present in both groups. The abnormality of motor nerve conduction was directly related to the severity of PCM and the presence of hypotonia and/or hyporeflexia. Sural nerve biopsies from both groups were studied for myelinated fibre density, fibre size spectrum, relationship of internodal length with diameter and qualitative light microscopic changes. The biopsies from children with mild to moderate PCM were characterized by a normal developmental change in myelinated fibres with an increasing proportion of medium and large size fibres, a transition from a unimodal to a bimodal distribution and an appropriate relationship of internodal length to fibre diameter. Evidence of mild segmental demyelination was observed in only one patient of this group. In contrast, in the biopsies from children with severe PCM, the normal developmental pattern for myelinated fibre size distribution was impaired with a persistence of small myelinated fibres, and there was a failure of internodal segments on large fibres to elongate with increase in age and significant segmental demyelination in about 50 per cent of cases. Retarded myelination and segmental demyelination probably form the morphological basis for impaired peripheral nerve function in PCM. Short internodes on large diameter fibres may also contribute to this effect.
对43名患有蛋白质热量营养不良(PCM)的儿童(年龄在7至62个月之间)进行了研究;其中13名患有轻度至中度PCM,30名患有重度PCM。两组均存在运动神经传导速度降低和感觉传导异常。运动神经传导异常与PCM的严重程度以及肌张力减退和/或反射减弱的存在直接相关。对两组的腓肠神经活检进行了研究,观察有髓纤维密度、纤维大小谱、结间长度与直径的关系以及定性光镜变化。轻度至中度PCM儿童的活检表现为有髓纤维正常的发育变化,中、大尺寸纤维比例增加,从单峰分布向双峰分布转变,结间长度与纤维直径关系合适。该组仅1例患者观察到轻度节段性脱髓鞘证据。相比之下,重度PCM儿童的活检显示,有髓纤维大小分布的正常发育模式受损,小的有髓纤维持续存在,大纤维的结间段未随年龄增长而延长,约50%的病例存在明显的节段性脱髓鞘。髓鞘形成延迟和节段性脱髓鞘可能是PCM患者周围神经功能受损的形态学基础。大直径纤维上的短结间段也可能导致这种情况。