MOTOR-NEURON DISEASE WITH PALLIDO-LUYSIO-NIGRAL ATROPHY

被引：28

作者：

BERGMANN, M ^{[1
]}

KUCHELMEISTER, K ^{[1
]}

MIGHELI, A ^{[1
]}

SCHIFFER, D ^{[1
]}

GULLOTTA, F ^{[1
]}

机构：

[1] UNIV TURIN,NEUROL CLIN,I-10124 TURIN,ITALY

来源：

ACTA NEUROPATHOLOGICA | 1993年 / 86卷 / 01期

关键词：

PALLIDO-LUYSIO-NIGRAL ATROPHY; MOTOR NEURON DISEASE; UBIQUITIN;

D O I：

10.1007/BF00454908

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

A case of motor neuron disease (MND) with pallido-luysio-nigral atrophy (PLNA) is reported. The 45-year-old male patient presented with lower motor neuron symptoms and signs of basal ganglia disturbance. He died after a progressive course of 7 months. Neuropathological examination revealed motor neuron loss at all spinal cord levels with sparing of Onuf's nucleus. Nerve cell loss and gliosis were also present in substantia nigra, globus pallidus, and subthalamic nucleus. The presence of ubiquitin-positive inclusions, a hallmark of most variants of MND, confirms this case as an example of MND. At immunoelectron microscopy the granules were distributed on filamentous material. The combination of clinically apparent PLNA with MND has only been described twice previously. The relationship of this syndrome to other forms of MND and its nosological placement are discussed.

引用

页码：105 / 108

页数：4

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