Sanderson Alan B, Arnold W David, Elsheikh Bakri, Kissel John T
Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, 7th Floor, Columbus, Ohio, 43210, USA.
Muscle Nerve. 2015 Mar;51(3):358-62. doi: 10.1002/mus.24326. Epub 2015 Jan 9.
Isolated peripheral motor dysfunction due to lower motor neuron or peripheral nerve disorders presents an interesting challenge to clinicians because of the diverse differential diagnosis with a broad range of prognoses.
We retrospectively reviewed clinical data of adults who presented over 12 years with muscle weakness, atrophy, or fasciculations, but without hyperreflexia or sensory involvement.
In 119 patients, 52% had a motor neuron disease (MND), 13% had immune neuropathies, 11% had genetic neuronopathies, 10% had residual or post-polio syndrome, 5% had benign fasciculation, 1% had an infectious etiology, and 8% were not given a final diagnosis. Only MND patients had cognitive dysfunction or frontal release signs. Bulbar and respiratory symptoms virtually excluded consideration of immune neuropathy.
Only half of the patients were diagnosed with MND. A significant minority have treatable conditions. Cognitive involvement, frontal release signs, and bulbar or respiratory symptoms are strongly suggestive of MND.
由于下运动神经元或周围神经疾病导致的孤立性周围运动功能障碍，给临床医生带来了有趣的挑战，因为其鉴别诊断多样，预后范围广泛。
我们回顾性分析了12年来出现肌肉无力、萎缩或肌束震颤，但无反射亢进或感觉受累的成年患者的临床资料。
119例患者中，52%患有运动神经元病（MND），13%患有免疫性神经病，11%患有遗传性神经元病，10%患有残留或小儿麻痹后遗症，5%患有良性肌束震颤，1%有感染性病因，8%未得到最终诊断。只有MND患者有认知功能障碍或额叶释放征。延髓和呼吸症状几乎排除了免疫性神经病的考虑。
只有一半的患者被诊断为MND。少数患者有可治疗的疾病。认知受累、额叶释放征以及延髓或呼吸症状强烈提示MND。
