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. 2014 Oct;21(10):1301-10.
doi: 10.1111/ene.12482. Epub 2014 Jul 1.

More than memory impairment in voltage-gated potassium channel complex encephalopathy

B M Bettcher  1 , J M Gelfand, S R Irani, J Neuhaus, S Forner, C P Hess, M D Geschwind
Affiliations

More than memory impairment in voltage-gated potassium channel complex encephalopathy

B M Bettcher et al. Eur J Neurol. 2014 Oct.

Abstract

Background and purpose: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: LGI1; autoimmune; cognitive.

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Conflict of interest statement

COI:

Dr. Gelfand has provided expert witness medical-legal consultation related to CNS inflammatory-demyelinating diseases. Dr. Irani is a coapplicant on a patent for LGI1 and CASPR2 antibody discovery and receives related royalties. Dr. Hess receives research support from General Electric. Dr. Geschwind served as a consultant for MedaCorp, The Council of Advisors, Guidepoint Global, and Neurophage.

Figures

Figure 1
Figure 1. Proportion (%) of VGKCc-Ab patients impaired on neuropsychological tests, grouped by cognitive domain
Displays the proportion of patients whose individual tests were greater than 1.5 standard deviations below the normative sample. Verbal memory and executive function tests were the most commonly affected. Language tests were variably impaired, and visuospatial domains were less commonly affected.
See this image and copyright information in PMC

References

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