Van den Berg-Vos R M, Franssen H, Wokke J H, Van Es H W, Van den Berg L H
Department of Neurology, Rudolf Magnus Institute for Neurosciences, University Medical Center Utrecht, The Netherlands.
Ann Neurol. 2000 Dec;48(6):919-26.
As multifocal motor neuropathy (MMN) is a potentially treatable disorder, its differentiation from lower motor neuron disease is important. Evidence of conduction block (CB) is considered one of the relevant criteria for the diagnosis of MMN. Strict criteria for CB may lead to underdiagnosis of MMN, however. Using a standardized examination, we studied the clinical, laboratory, and electrophysiological characteristics of 37 patients presenting with a lower motor neuron disorder and electrophysiological features compatible with segmental demyelination. We propose a set of clinical, laboratory, and electrophysiological criteria for the diagnosis of MMN, which has been verified by follow-up and response to treatment with intravenous immunoglobulins. Based on the clinical, laboratory, and electrodiagnostic features, 21 patients were diagnosed with definite MMN (17 responders), 7 were diagnosed with probable MMN (5 responders), and 9 were diagnosed with possible MMN (1 responder). Age at onset, the number of affected limb regions, and the number of patients with a creatine kinase level greater than 180 U/L were significantly lower in responders than in nonresponders. Elevated anti-GM1 antibodies and definite CB were found significantly more often in responders. The proposed diagnostic criteria may be useful in clinical practice and therapeutic trials.
由于多灶性运动神经病（MMN）是一种潜在可治性疾病，因此将其与下运动神经元疾病相鉴别很重要。传导阻滞（CB）证据被视为MMN诊断的相关标准之一。然而，CB的严格标准可能会导致MMN的诊断不足。我们采用标准化检查，研究了37例表现为下运动神经元疾病且电生理特征符合节段性脱髓鞘的患者的临床、实验室和电生理特征。我们提出了一套用于MMN诊断的临床、实验室和电生理标准，该标准已通过随访以及对静脉注射免疫球蛋白治疗的反应得到验证。根据临床、实验室和电诊断特征，21例患者被诊断为确诊MMN（17例有反应者），7例被诊断为可能MMN（5例有反应者），9例被诊断为可能MMN（1例有反应者）。有反应者的发病年龄、受累肢体区域数量以及肌酸激酶水平大于180 U/L的患者数量均显著低于无反应者。有反应者中抗GM1抗体升高和明确CB的情况明显更常见。所提出的诊断标准可能在临床实践和治疗试验中有用。
