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一例儿童危重病性多发性神经病:临床及病理表现

A pediatric case of critical illness polyneuropathy: clinical and pathological findings.

作者信息

Ohto Tatsuyuki, Iwasaki Nobuaki, Ohkoshi Norio, Aoki Takeshi, Ichinohe Mika, Tanaka Ryuta, Moriyama Nobuko, Ieshima Atsushi, Kuwajima Katsuko, Matsui Akira

机构信息

Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennohdai, Tsukuba, Ibaraki 305-8575, Japan.

出版信息

Brain Dev. 2005 Oct;27(7):535-8. doi: 10.1016/j.braindev.2004.12.007. Epub 2005 Apr 11.

Abstract

Critical illness polyneuropathy (CIP) is a sensorimotor polyneuropathy recognized in adult intensive care patients with sepsis and multiple organ dysfunction and only a few cases have been reported in children. Here we report a 13-year-old Japanese boy with CIP that developed during the course of encephalopathy. Two months after the onset of encephalopathy, he developed tetraplegia although consciousness had already recovered. Deep tendon reflex was absent. MRI of the brain and spinal cord was normal and no abnormality in the cerebrospinal fluid was detected. Motor and sensory nerve conduction velocities of the lower limbs and somatosensory evoked potential could not be detected. The motor activity subsequently showed gradual recovery, although standing and walking could not be achieved. Sural nerve biopsy performed 3 years after the onset showed severe reduction of the number of myelinated large-diameter fibers, thin myelin in almost all fibers and cluster formation of myelinated small-diameter fibers, indicating primary axonal degeneration with regeneration. We report here for the first time the neuropathological changes in peripheral nerves during the chronic stage of CIP in children.

摘要

危重病性多发性神经病(CIP)是一种在患有脓毒症和多器官功能障碍的成年重症监护患者中被认识到的感觉运动性多发性神经病,而儿童中仅有少数病例报道。在此,我们报告一名13岁日本男孩,其在脑病病程中发生了CIP。脑病发作两个月后,尽管意识已经恢复,但他出现了四肢瘫痪。深腱反射消失。脑和脊髓的MRI正常,脑脊液未检测到异常。下肢的运动和感觉神经传导速度以及体感诱发电位均无法检测到。尽管无法站立和行走,但运动活动随后逐渐恢复。发病3年后进行的腓肠神经活检显示,有髓大直径纤维数量严重减少,几乎所有纤维的髓鞘变薄,有髓小直径纤维形成簇状,提示原发性轴索性变性伴再生。我们首次在此报告儿童CIP慢性期外周神经的神经病理学改变。

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