Escudero Domingo, Guasp Mar, Ariño Helena, Gaig Carles, Martínez-Hernández Eugenia, Dalmau Josep, Graus Francesc
From the Service of Neurology (D.E., M.G., C.G., J.D., F.G.), Hospital Clinic, University of Barcelona; Neuroimmunology Program (H.A., C.G., E.M.-H., J.D., F.G.), Institut d'Investigació Biomèdica August Pi i Sunyer; Institució Catalana de Recerca i Estudis Avançats (J.D.), Barcelona, Spain; and Department of Neurology (J.D.), University of Pennsylvania, Philadelphia.
Neurology. 2017 Oct 3;89(14):1471-1475. doi: 10.1212/WNL.0000000000004541. Epub 2017 Sep 6.
To report the CNS syndromes of patients ≥60 years of age with antibodies against neuronal surface antigens but no evidence of brain MRI and CSF inflammatory changes.
This was a retrospective clinical analysis of patients with antibodies against neuronal surface antigens who fulfilled 3 criteria: age ≥60 years, no inflammatory abnormalities in brain MRI, and no CSF pleocytosis. Antibodies were determined with reported techniques.
Among 155 patients ≥60 years of age with neurologic syndromes related to antibodies against neuronal surface antigens, 35 (22.6%) fulfilled the indicated criteria. The median age of these 35 patients was 68 years (range 60-88 years). Clinical manifestations included faciobrachial dystonic seizures (FBDS) in 11 of 35 (31.4%) patients, all with LGI1 antibodies; a combination of gait instability, brainstem dysfunction, and sleep disorder associated with IgLON5 antibodies in 10 (28.6%); acute confusion, memory loss, and behavioral changes suggesting autoimmune encephalitis (AE) in 9 (25.7%; 2 patients with AMPAR, 2 with NMDAR, 2 with GABAbR, 2 with LGI1, and 1 with CASPR2 antibodies); and rapidly progressive cognitive deterioration in 5 (14.3%; 3 patients with IgLON5 antibodies, 1 with chorea; 1 with DPPX antibody-associated cerebellar ataxia and arm rigidity; and 1 with CASPR2 antibodies).
In patients ≥60 years of age, the correct identification of characteristic CNS syndromes (FBDS, anti-IgLON5 syndrome, AE) should prompt antibody testing even without evidence of inflammation in MRI and CSF studies. Up to 15% of the patients developed rapidly progressive cognitive deterioration, which further complicated the differential diagnosis with a neurodegenerative disorder.
报告年龄≥60岁、存在抗神经元表面抗原抗体但无脑MRI及脑脊液炎症改变证据的患者的中枢神经系统综合征。
这是一项对符合以下3条标准的抗神经元表面抗原抗体患者的回顾性临床分析：年龄≥60岁、脑MRI无炎症异常、脑脊液无细胞数增多。采用已报道的技术检测抗体。
在155例年龄≥60岁、患有与抗神经元表面抗原抗体相关神经系统综合征的患者中，35例（22.6%）符合指定标准。这35例患者的中位年龄为68岁（范围60 - 88岁）。临床表现包括35例患者中的11例（31.4%）出现面臂肌张力障碍性癫痫（FBDS），均有LGI1抗体；10例（28.6%）出现与IgLON5抗体相关的步态不稳、脑干功能障碍和睡眠障碍；9例（25.7%）出现提示自身免疫性脑炎（AE）的急性意识模糊、记忆力减退和行为改变（2例有AMPAR抗体、2例有NMDAR抗体、2例有GABAbR抗体、2例有LGI1抗体、1例有CASPR2抗体）；5例（14.3%）出现快速进展性认知衰退（3例有IgLON5抗体、1例有舞蹈症；1例有DPPX抗体相关的小脑共济失调和手臂僵硬；1例有CASPR2抗体）。
在年龄≥60岁的患者中，即使MRI和脑脊液检查无炎症证据，正确识别特征性中枢神经系统综合征（FBDS、抗IgLON5综合征、AE）也应促使进行抗体检测。高达15%的患者出现快速进展性认知衰退，这使与神经退行性疾病的鉴别诊断更加复杂。
