Centre de référence pour Maladies NeuroMusculaires et SLA, Hôpital Archet 1, CHU de Nice, route saint Antoine de Ginestière, BP3079, 06202 Nice Cedex 3, France.
J Neurol. 2009 Nov;256(11):1876-80. doi: 10.1007/s00415-009-5217-0. Epub 2009 Jul 30.
A decrease in sensory nerve action potentials (SNAP) amplitude has been recently reported in some patients during the course of multifocal motor neuropathy with conduction blocks (MMNCB). It is not known if those patients have different clinical expression and disability when compared with typical MMNCB. Clinical, biological and electrophysiological assessments were performed in 15 patients fitting the diagnosis criteria of MMNCB, including normal SNAP amplitude at initial examination. Patients presenting with nerve entrapment or associated disease causative of sensory neuropathy were excluded. Median time of follow-up was 3 years (1-17 years). At the last examination, four patients had at least one SNAP amplitude below 50% of normal value. None had clinically objective sensory loss. Clinical and electrophysiological data obtained at the last examination were compared between patients with normal SNAP amplitude and patients with decreased SNAP amplitude. No difference between both population in term of age, sex, disease duration, anti-GM1 antibody titers, CSF data and number of conduction blocks was noted. In contrast, patients with decreased SNAP amplitude had worse overall neuropathy limitation scale (ONLS) scores (7 vs. 2; p = 0.02), a higher number of affected nerves (12.5 vs. 4; p = 0.018), a higher number of affected limb regions (6 vs. 2; p = 0.019) and lower median CMAP amplitude (2 mV vs. 6.5 mV; p = 0.04). They were all dependent on higher doses of IVIg (1.4 g/(kg 4 weeks vs. 0.6; p = 0.018). A reduction in SNAP amplitude during the course of MMNCB is associated with a more severe disease and a more prominent axonal loss. This result needs to be confirmed in a larger cohort.
在一些患有多发性运动神经病伴传导阻滞(MMNCB)的患者中,最近有报道称其感觉神经动作电位(SNAP)幅度降低。与典型 MMNCB 相比,这些患者的临床表现和残疾是否不同尚不清楚。对符合 MMNCB 诊断标准的 15 例患者进行了临床、生物学和电生理评估,包括初次检查时 SNAP 幅度正常。排除了有神经嵌压或与感觉神经病相关的疾病的患者。中位随访时间为 3 年(1-17 年)。最后一次检查时,有 4 例患者至少有一个 SNAP 幅度低于正常值的 50%。这些患者均无临床客观感觉丧失。比较了 SNAP 幅度正常和降低的患者最后一次检查时的临床和电生理数据。在年龄、性别、疾病持续时间、抗 GM1 抗体滴度、CSF 数据和传导阻滞数量方面,两组患者无差异。相反,SNAP 幅度降低的患者总体神经病限制量表(ONLS)评分更高(7 分比 2 分;p = 0.02),受累神经数量更多(12.5 条比 4 条;p = 0.018),受累肢体区域数量更多(6 个比 2 个;p = 0.019),CMAP 幅度中位数更低(2 mV 比 6.5 mV;p = 0.04)。他们都依赖于更高剂量的 IVIg(1.4 g/(kg 4 周比 0.6;p = 0.018)。MMNCB 病程中 SNAP 幅度的降低与更严重的疾病和更明显的轴索丢失有关。这一结果需要在更大的队列中得到证实。