Bettcher B M, Gelfand J M, Irani S R, Neuhaus J, Forner S, Hess C P, Geschwind M D
Department of Neurology, Memory and Aging Center, UCSF, San Francisco, CA, USA.
Eur J Neurol. 2014 Oct;21(10):1301-10. doi: 10.1111/ene.12482. Epub 2014 Jul 1.
Autoimmune encephalopathies (AEs) are a heterogeneous group of neurological disorders that affect cognition. Although memory difficulties are commonly endorsed, few reports of AEs inclusively assess all cognitive domains in detail. Our aim was to perform an unbiased cognitive evaluation of AE patients with voltage-gated potassium channel complex antibodies (VGKCC -Abs) in order to delineate cognitive strengths and weaknesses.
Serial VGKCC -Ab AE subjects (n = 12) were assessed with a comprehensive evaluation of memory, executive functions, visuospatial skills and language. Clinical magnetic resonance imaging (MRI) (n = 10/12) was evaluated. Five subjects had serial cognitive testing available, permitting descriptive analysis of change.
Subjects demonstrated mild to moderate impairment in memory (mean Z = -1.9) and executive functions (mean Z = -1.5), with variable impairments in language and sparing of visuospatial skills. MRI findings showed T2 hyperintensities in medial temporal lobe (10/10) and basal ganglia (2/10). Serial cognitive examination revealed heterogeneity in cognitive function; whereas most patients improved in one or more domains, residual impairments were observed in some patients.
This study augments previous neuropsychological analyses in VGKCC -Ab AE by identifying not only memory and executive function deficits but also language impairments, with preservation of visuospatial functioning. The study further highlights the importance of domain-specific testing to parse out the complex cognitive phenotypes of VGKCC -Ab AE.
自身免疫性脑病（AE）是一组影响认知的异质性神经系统疾病。尽管记忆障碍很常见，但很少有关于AE的报告全面详细地评估所有认知领域。我们的目的是对电压门控钾通道复合物抗体（VGKCC -Abs）相关的AE患者进行无偏倚的认知评估，以明确认知优势和劣势。
对连续的VGKCC -Ab AE受试者（n = 12）进行记忆、执行功能、视觉空间技能和语言的综合评估。对临床磁共振成像（MRI）（n = 10/12）进行评估。5名受试者有连续的认知测试数据，可进行变化的描述性分析。
受试者在记忆（平均Z = -1.9）和执行功能（平均Z = -1.5）方面表现出轻度至中度损害，语言功能存在不同程度的损害，视觉空间技能未受影响。MRI结果显示内侧颞叶（10/10）和基底神经节（2/10）有T2高信号。连续的认知检查显示认知功能存在异质性；虽然大多数患者在一个或多个领域有所改善，但部分患者仍存在残留损害。
本研究不仅发现了记忆和执行功能缺陷，还发现了语言障碍，而视觉空间功能得以保留，从而扩展了先前对VGKCC -Ab AE的神经心理学分析。该研究进一步强调了针对特定领域进行测试对于剖析VGKCC -Ab AE复杂认知表型的重要性。
