Evangelista T, Carvalho M, Conceição I, Pinto A, de Lurdes M, Luís M L
Department of Neurology, Centro de Estudos Egus Montz, Hospital de Santa Maria, Lisbon, Portugal.
J Neurol Sci. 1996 Aug;139 Suppl:95-8. doi: 10.1016/0022-510x(96)00120-7.
We report three patients in whom the initial diagnosis was of possible A myotrophic lateral sclerosis (ALS/MND) according to the 'El Escorial Criteria'. All of them presented with monomelic paresis, atrophy of the paretic muscles and generalised brisk reflexes. The initial electromyograms showed a neurogenic pattern in the limbs with normal sensory and motor conduction velocities. Laboratory evaluation and imagiological investigations were normal in all of them. The previous diagnosis was changed in to demyelinating motor neuropathy with conduction block in 2 patients and tomaculous neuropathy in one after clinical and electromyographic follow-up and nerve biopsy. Patients 1 and 2 were given intravenous immunoglobulin treatment and showed moderate improvement.
我们报告了三名患者，根据“埃尔埃斯科里亚尔标准”，他们最初被诊断为可能患有肌萎缩侧索硬化症（ALS/MND）。他们均表现为单肢轻瘫、瘫痪肌肉萎缩以及全身性反射亢进。最初的肌电图显示四肢呈神经源性模式，感觉和运动传导速度正常。他们所有人的实验室评估和影像学检查均正常。经过临床和肌电图随访以及神经活检后，其中两名患者先前的诊断改为伴有传导阻滞的脱髓鞘性运动神经病，另一名患者诊断为腊肠样神经病。患者1和患者2接受了静脉注射免疫球蛋白治疗，并显示出中度改善。
