Morena Jonathan, Elsheikh Bakri, Hoyle J Chad
Department of Neurology, The Ohio State University, Columbus, OH, USA.
Neurohospitalist. 2021 Jul;11(3):263-266. doi: 10.1177/1941874420987053. Epub 2021 Jan 19.
MFS has been reported to recur in 10-12% of patients. There may be a genetic component related to HLA-DR2. Anti-GAD antibodies can be present in MFS along with anti-GQ1b. Common EMG/NCS associations consist of a predominantly axonal, sensory polyneuropathy and absent H reflexes. A 32-year-old female with a history of hypothyroidism presented to our institution twice with symptoms of diplopia, lower extremity weakness and distal paresthesias occurring a year apart. She had ophthalmoplegia, reduced reflexes, and ataxia on exam. CSF showed a borderline elevated protein of 47 and white blood cells <3. She was positive for anti-GQ1b both times. Her anti-GAD65 antibody was elevated during both admissions. EMG/NCS on her first admission revealed comparatively reduced sensory nerve action potentials (SNAPs) and a normal blink reflex. Her SNAPs improved on the second admission, however, the EMG was performed only 2 days after the onset of her symptoms, limiting some early findings that may have not matured electrophysiologically. She was treated with IVIG on both occasions with rapid recovery within 5 days. This case highlights the fact that MFS can be recurrent. It also provides further evidence that anti-GAD antibodies may be associated with MFS. Reported findings of the blink reflex in MFS are diverse and further data is needed to determine if certain findings are more predominant than others. Treatment typically consists of IVIG, though steroids may also be considered for recurrence. Prognosis is generally favorable, regardless of treatment.
据报道,10%-12%的患者会出现米勒-费雪综合征(MFS)复发。可能存在与HLA-DR2相关的遗传因素。抗GAD抗体可与抗GQ1b一同出现在MFS患者中。常见的肌电图/神经传导速度检查(EMG/NCS)表现主要为轴索性感觉性多发性神经病,且H反射消失。一名有甲状腺功能减退病史的32岁女性,因复视、下肢无力和远端感觉异常症状,相隔一年两次前来我院就诊。检查发现她有眼肌麻痹、反射减弱和共济失调。脑脊液检查显示蛋白水平临界升高至47,白细胞<3。她两次检查抗GQ1b均为阳性。两次住院期间她的抗GAD65抗体均升高。她首次住院时的EMG/NCS显示感觉神经动作电位(SNAPs)相对降低,眨眼反射正常。第二次住院时她的SNAPs有所改善,然而,EMG是在症状出现后仅2天进行的,限制了一些可能尚未在电生理上成熟的早期发现。她两次均接受静脉注射免疫球蛋白(IVIG)治疗,5天内迅速康复。该病例突出了MFS可复发这一事实。它还进一步证明抗GAD抗体可能与MFS有关。报道的MFS患者眨眼反射的结果各不相同,需要更多数据来确定某些结果是否比其他结果更常见。治疗通常包括IVIG,不过复发时也可考虑使用类固醇。无论治疗如何,预后一般良好。