Neurological Institute, Cleveland Clinic, Cleveland, OH, USA.
Neurology, Louis Stokes Cleveland Veteran's Affairs Medical Center, Cleveland, OH, USA.
J Neurol. 2018 Sep;265(9):2114-2119. doi: 10.1007/s00415-018-8959-8. Epub 2018 Jul 9.
As testing for neuronal antibodies become more readily available, the spectrum of conditions potentially associated with these autoantibodies has been widening. Voltage-gated calcium channel antibodies (VGCC-Ab) are no exception to this trend. The significance of an elevated VGCC-Ab titer beyond its original clinicopathological correlate, Lambert-Eaton myasthenic syndrome (LEMS) remains undetermined. We sought to determine the diagnostic significance of an elevated serum VGCC-Ab titer in a large single-center cohort of 100 patients. The majority of patients (58%) with elevated VGCC-Ab levels lacked an inflammatory or autoimmune etiology of their neurologic diagnosis. Only six cases (6%) of LEMS and two cases (2%) of SCLC (without LEMS) were identified. No significant differences in antibody titers were seen between the autoimmune and non-autoimmune groups. These findings support the notions that: (a) elevated VGCC-Ab titers without clinical correlation must be interpreted with caution, and (b) the clinical and electrodiagnostic criteria for LEMS should remain the mainstay in the diagnosis of LEMS.
随着神经元抗体检测变得更加便捷，与这些自身抗体相关的潜在疾病谱也在不断扩大。电压门控钙通道抗体 (VGCC-Ab) 也不例外。除了最初的临床病理相关性 Lambert-Eaton 肌无力综合征 (LEMS) 之外，升高的 VGCC-Ab 滴度的意义仍未确定。我们试图在一个由 100 名患者组成的大型单中心队列中确定血清 VGCC-Ab 滴度升高的诊断意义。大多数（58%）升高的 VGCC-Ab 水平的患者的神经诊断没有炎症或自身免疫病因。仅发现 6 例 LEMS（6%）和 2 例 SCLC（无 LEMS）（2%）。自身免疫组和非自身免疫组之间的抗体滴度没有显著差异。这些发现支持以下观点：(a) 没有临床相关性的升高的 VGCC-Ab 滴度必须谨慎解释，(b) LEMS 的临床和电诊断标准应仍然是 LEMS 诊断的主要依据。
