Fava Elena, Kofler Markus, Saltuari Leopold
Department of Neurology, Hochzirl Hospital, Zirl, Austria.
Clin Neurophysiol Pract. 2019 May 31;4:128-132. doi: 10.1016/j.cnp.2019.05.002. eCollection 2019.
Vitamin B12 deficiency is common in adult and elderly patients and is often underdiagnosed because of its polymorphous manifestations. Neurological symptoms of this condition include subacute combined degeneration and polyneuropathy, with possible affection of thin-myelinated A-delta fibers. Cutaneous silent periods (CSPs) may serve to test small-diameter fiber function non-invasively, using routine electrodiagnostic equipment, but to the best of our knowledge have not been studied so far in vitamin B12 deficiency.
We report a 49-year-old male patient suffering from B12 hypovitaminosis due to autoantibodies against gastric parietal cells, who underwent neurophysiological investigation to confirm clinically suspected polyneuropathy during the first month of intramuscular vitamin B12 supplementation. We performed standard electroneurography, needle electromyography in tibialis anterior muscle, quantitative sensory testing, and cutaneous silent periods six months after symptom onset and repeated the electrodiagnostic study 21 months later, after intramuscular vitamin B12 supplementation.
Standard electroneurography demonstrated axonal sensory polyneuropathy. Needle electromyography (EMG) in tibialis anterior muscle was unremarkable. Cutaneous silent periods in tibialis anterior muscle after noxious electrical sural nerve stimulation were delayed, with incomplete EMG suppression concurring with dysfunction of thin-myelinated A-delta fibers. Quantitative sensory testing revealed altered cold and warm perception thresholds in both upper limbs, but normal values in both lower limbs. A follow-up electrodiagnostic study after 21 months intramuscular vitamin B12 supplementation revealed improvement of all neurophysiological findings, including normalization of cutaneous silent periods.
Thin-myelinated A-delta fibers may be affected in B12 hypovitaminosis and may show recovery after intramuscular vitamin B12 supplementation. CSP may serve to diagnose small fiber affection in this medical condition and to monitor their recovery after vitamin supplementation.
CSP testing represents a useful, non-invasive, rapidly available diagnostic and follow-up tool in vitamin B12 deficiency.
维生素B12缺乏在成年和老年患者中很常见,由于其表现多样,常被漏诊。这种情况的神经症状包括亚急性联合变性和多发性神经病,可能累及薄髓鞘Aδ纤维。皮肤静息期(CSP)可使用常规电诊断设备无创检测小直径纤维功能,但据我们所知,目前尚未在维生素B12缺乏症中进行研究。
我们报告了一名49岁男性患者,因抗胃壁细胞自身抗体导致维生素B12缺乏,在肌肉注射维生素B12补充治疗的第一个月接受了神经生理学检查,以确诊临床怀疑的多发性神经病。我们在症状出现6个月后进行了标准神经电图、胫前肌针极肌电图、定量感觉测试和皮肤静息期检测,并在肌肉注射维生素B12补充治疗21个月后重复了电诊断研究。
标准神经电图显示轴索性感觉多发性神经病。胫前肌针极肌电图(EMG)无明显异常。有害电刺激腓肠神经后胫前肌的皮肤静息期延迟,EMG抑制不完全,提示薄髓鞘Aδ纤维功能障碍。定量感觉测试显示双上肢冷、热感觉阈值改变,但双下肢正常。肌肉注射维生素B12补充治疗21个月后的随访电诊断研究显示所有神经生理学结果均有改善,包括皮肤静息期恢复正常。
薄髓鞘Aδ纤维可能在维生素B12缺乏症中受到影响,肌肉注射维生素B12补充治疗后可能恢复。CSP可用于诊断这种疾病中的小纤维受累情况,并监测维生素补充治疗后的恢复情况。
CSP检测是维生素B12缺乏症中一种有用、无创、快速可用的诊断和随访工具。