Poyraz Mürüvvet, Matur Zeliha, Aysal Fikret, Tüzün Erdem, Hanoğlu Lütfü, Öge A Emre
Department of Neurology, İstanbul Medipol University School of Medicine, İstanbul, Turkey.
Department of Neurology, İstanbul Bilim University School of Medicine, İstanbul, Turkey.
Noro Psikiyatr Ars. 2017 Jun;54(2):183-186. doi: 10.5152/npa.2016.14816. Epub 2016 Mar 28.
Cramp-fasciculation syndrome (CFS) is a rare peripheral nerve hyperexcitability syndrome. There are only a few reports on clinical and serological profile of a CFS cohort that was followed up by a single outpatient clinic.
Clinical, electrophysiological, and serological features of 6 CFS patients (5 men, 1 woman; 27-65 years old) were investigated.
All patients presented with cramps, fasciculations, muscle pain, and autonomic symptoms, and 2 also reported numbness and burning sensation in limbs, suggestive of neuropathic pain. Antibodies to uncharacterized voltage-gated potassium channel (VGKC)-complex proteins were found in 2 patients and to contactin-associated protein-like 2 (CASPR2) in 1 patient. None of the patients had a tumor. Most of the patients revealed prolonged after-discharges following tibial nerve stimulation. Nerve conduction studies and R-R interval variability tests were normal, whereas sympathetic skin responses were increased in amplitude in 3 seronegative patients. Five patients showed favorable response to carbamazepine or pregabalin treatment, whereas 1 VGKC-antibody-positive patient was resistant to carbamazepine and immunosuppressant treatment.
Neuropathic pain and VGKC-complex antibodies may be encountered in CFS patients. Although autonomic symptoms are commonly found in CFS, routine autonomic system tests which are done in electrophysiology laboratories might yield normal results.
痛性肌束颤综合征(CFS)是一种罕见的周围神经兴奋性增高综合征。仅有少数关于由单一门诊随访的CFS队列的临床和血清学特征的报告。
对6例CFS患者(5例男性,1例女性;年龄27 - 65岁)的临床、电生理和血清学特征进行了研究。
所有患者均出现肌肉痉挛、肌束震颤、肌肉疼痛和自主神经症状,2例患者还报告有肢体麻木和烧灼感,提示神经性疼痛。2例患者检测到针对未明确的电压门控钾通道(VGKC)复合蛋白的抗体,1例患者检测到针对接触蛋白相关蛋白样2(CASPR2)的抗体。所有患者均无肿瘤。大多数患者在胫神经刺激后出现延长的后放电。神经传导研究和R - R间期变异性测试均正常,而3例血清阴性患者的交感神经皮肤反应振幅增加。5例患者对卡马西平或普瑞巴林治疗反应良好,而1例VGKC抗体阳性患者对卡马西平和免疫抑制剂治疗耐药。
CFS患者可能出现神经性疼痛和VGKC复合抗体。虽然自主神经症状在CFS中很常见,但在电生理实验室进行的常规自主神经系统测试可能结果正常。