Gorson K C, Ropper A H, Adelman L S, Raynor E M, Saper C B
Division of Neurology, St. Elizabeth's Medical Center, Boston, Massachusetts 02135, USA.
Muscle Nerve. 1999 Feb;22(2):266-70. doi: 10.1002/(sici)1097-4598(199902)22:2<266::aid-mus17>3.0.co;2-v.
Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process.
慢性免疫性和炎性运动神经病可能类似于运动神经元病，如果不存在传导阻滞或GM1抗体，鉴别可能会特别困难。除了少数与副蛋白血症相关的病例外，这种轴索性慢性运动神经病的病理特征尚未明确。我们描述了一名患有慢性运动轴索性神经病、免疫电泳正常且无GM1抗体患者的临床、电生理和病理表现。尸检时，除了染色质溶解的运动神经元外，脊髓正常。所有腹侧神经根均明显变细。在采样的10条混合神经和众多脊神经根中，5条显示有神经束膜、血管周围淋巴细胞浸润区域。运动神经根存在严重的轴突丢失，在混合神经中不那么明显，感觉神经和神经根基本未受影响。我们的研究结果表明，在某些情况下，无副蛋白血症或GM1抗体的慢性运动轴索性神经病可能由炎症过程引起。
