Kannoth Sudheeran, Anandakkuttan Anandkumar, Mathai Annamma, Sasikumar Anuja Nirmala, Nambiar Vivek
Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India; Neuroimmunology Laboratory, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India.
Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara PO, Kochi 682041, Kerala, India.
J Neurol Sci. 2016 Mar 15;362:40-6. doi: 10.1016/j.jns.2016.01.006. Epub 2016 Jan 13.
Immunological causes of atypical parkinsonism/Parkinson plus syndromes are rare.
To study the clinical and laboratory features and treatment outcome of autoimmune atypical parkinsonism.
Retrospective case series. Patients with atypical parkinsonism and positive antibodies were identified retrospectively. Those who received immunotherapy (intravenous methyl prednisolone 1g daily for five days followed by mycophenylate mofetil 2g daily or azathioprine 2-3mg/kg/day) and consented for publication of non-anonymized videos were included.
There were ten cases (nine males, age range 49-75years, disease duration 2months to 13years, follow-up 1-7months) of atypical parkinsonism [probable multiple system atrophy (MSA)-2, possible progressive supranuclear palsy (PSP)-1, probable PSP-3]. Eight had new uncharacterized neuronal antibodies, leucine rich glioma associated protein 1 (LGI1) antibody in one, and the other had another uncharacterized neuronal antibody along with LGI1 antibody. Four had abnormal CSF. There was a prompt, dramatic improvement in terms of Unified Parkinson Disease Rating Scale motor scale and or modified Rankin Scale as well as improvement in eye movement, postural instability, cerebellar, autonomic and non-motor symptoms. Two had reappearance of symptoms on discontinuing steroids and improvement on restarting. One died of infection despite good recovery of encephalopathy and parkinsonism.
Autoimmune atypical parkinsonism is characterized by atypical parkinsonism with neuronal specific antibodies, sometimes associated with abnormal CSF and significant response to immunotherapy.
非典型帕金森病/帕金森叠加综合征的免疫病因较为罕见。
研究自身免疫性非典型帕金森病的临床和实验室特征及治疗结果。
回顾性病例系列研究。对非典型帕金森病且抗体呈阳性的患者进行回顾性识别。纳入接受免疫治疗（静脉注射甲泼尼龙1g/天，共5天，随后口服霉酚酸酯2g/天或硫唑嘌呤2 - 3mg/kg/天）且同意发表非匿名视频的患者。
有10例非典型帕金森病患者（9例男性，年龄范围49 - 75岁，病程2个月至13年，随访1 - 7个月）[可能的多系统萎缩（MSA）- 2例，可能的进行性核上性麻痹（PSP）- 1例，可能的PSP - 3例]。8例有新的未明确特征的神经元抗体，1例有富亮氨酸胶质瘤失活蛋白1（LGI1）抗体，另1例除LGI1抗体外还有另一种未明确特征的神经元抗体。4例脑脊液异常。在统一帕金森病评定量表运动评分、改良Rankin量表以及眼球运动、姿势不稳、小脑、自主神经和非运动症状方面均有迅速、显著改善。2例在停用类固醇后症状复发，重新用药后改善。1例尽管脑病和帕金森症状恢复良好，但死于感染。
自身免疫性非典型帕金森病的特征为伴有神经元特异性抗体的非典型帕金森病，有时伴有脑脊液异常且对免疫治疗有显著反应。
