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.
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.
Authors: Natalia M Moll; Anna M Rietsch; Smitha Thomas; Amy J Ransohoff; Jar-Chi Lee; Robert Fox; Ansi Chang; Richard M Ransohoff; Elizabeth Fisher Journal: Ann Neurol Date: 2011-11 Impact factor: 10.422
Authors: George K Tofaris; Sarosh R Irani; Binith J Cheeran; Ian W S Baker; Zameel M Cader; Angela Vincent Journal: Neurology Date: 2012-06-27 Impact factor: 9.910
Authors: Michael P Malter; Christoph Helmstaedter; Horst Urbach; Angela Vincent; Christian G Bien Journal: Ann Neurol Date: 2010-04 Impact factor: 10.422
Authors: Katherine L Possin; Victor R Laluz; Oscar Z Alcantar; Bruce L Miller; Joel H Kramer Journal: Neuropsychologia Date: 2010-10-26 Impact factor: 3.139
Authors: Georges Naasan; Sarosh R Irani; Brianne M Bettcher; Michael D Geschwind; Jeffrey M Gelfand Journal: JAMA Neurol Date: 2014-10 Impact factor: 18.302
Authors: Thomas D Miller; Trevor T-J Chong; Anne M Aimola Davies; Michael R Johnson; Sarosh R Irani; Masud Husain; Tammy Wc Ng; Saiju Jacob; Paul Maddison; Christopher Kennard; Penny A Gowland; Clive R Rosenthal Journal: Elife Date: 2020-01-24 Impact factor: 8.140
Authors: Thomas D Miller; Trevor T-J Chong; Anne M Aimola Davies; Tammy W C Ng; Michael R Johnson; Sarosh R Irani; Angela Vincent; Masud Husain; Saiju Jacob; Paul Maddison; Christopher Kennard; Penny A Gowland; Clive R Rosenthal Journal: Brain Date: 2017-05-01 Impact factor: 13.501
Authors: Julia Thompson; Mian Bi; Andrew G Murchison; Mateusz Makuch; Christian G Bien; Kon Chu; Pue Farooque; Jeffrey M Gelfand; Michael D Geschwind; Lawrence J Hirsch; Ernest Somerville; Bethan Lang; Angela Vincent; Maria I Leite; Patrick Waters; Sarosh R Irani Journal: Brain Date: 2018-02-01 Impact factor: 13.501