X-LINKED RECESSIVE BULBOSPINAL NEURONOPATHY - A CLINICOPATHOLOGICAL STUDY
X-LINKED RECESSIVE BULBOSPINAL NEURONOPATHY - A CLINICOPATHOLOGICAL STUDY
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DOI：
10.1093/brain/112.1.209
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发表时间：
1989-02-01
期刊：
影响因子：
14.5
通讯作者：
TAKAHASHI, A
中科院分区：
文献类型：
--
作者：
SOBUE, G;HASHIZUME, Y;TAKAHASHI, A
对9例x连锁隐性球脊神经病变进行了临床病理研究，对3例尸检进行了形态学观察，并对6例患者进行了腓肠神经活检。下运动神经元和初级感觉神经元均受影响。除第3、第4和第6脑神经外，所有脊髓节段和脑干运动核的下部运动神经元明显减少。初级感觉神经元受影响较轻。对周围神经系统多个层次的初级感觉轴突的定量研究表明，远端加重的轴突病是显著的病理过程。节段性脱髓鞘和再脱髓鞘聚集在单个纤维上，在某些情况下，腓肠神经的g比（轴突直径：总纤维直径）显示出增加的分散。再生的证据不明显。所有检查的神经中无髓鞘纤维保存完好。脊髓Onufrowicz核、中外侧柱和Clarke' s柱内的神经元普遍保存完好。这些观察结果表明，下运动神经病变和原发性感觉神经病变是本病的主要神经学表现。
A clinicopathological study on X-linked recessive bulbospinal neuronopathy ws undertaken on 9 cases, with morphological observations on 3 autopsied cses and sural nerve biopsies from 6 patients. Both lower motor and primary sensory neurons were involved. Lower motor neurons were markedly depleted through all spinal segments and in brainstem motor neuclei except for the third, fourth and sixth cranial nerves. Primary sensory neurons were less severely affected. A quantitative study of primary sensory axons at several levels in the peripheral nervous system suggested that a distally accentuated axonopathy was the salient pathological process. Segmental demyelination and remyelination clustered on individual fibres, and g ratios (axon diameter: total fibre diameter) in the sural nerve showed an increased scatter in some cases. Evidence of regeneration was inconspicuous. Unmyelinated fibres were well preserved throughout all the nerves examined. Neurons in the Onufrowicz nuclei, in the intermediolateral columns nd in Clarke''s columns of the spinal cord were generally well preserved. These observations indicate that a lower motor and primary sensory neuronopathy is major neurological manifestation in this disease.