Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series

BackgroundGlycine receptor antibody-associated neuropsychiatric disease is currently known to be dominated by the phenotypes stiff-person syndrome and progressive encephalomyelitis entailing rigidity and myoclonus. In our case series we aim to depict the less-often reported feature of cognitive impa...

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Main Authors: Niels Hansen, Claudia Bartels, Winfried Stöcker, Jens Wiltfang, Dirk Fitzner
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-01-01
Series:Frontiers in Psychiatry
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fpsyt.2021.778684/full
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author Niels Hansen
Claudia Bartels
Winfried Stöcker
Jens Wiltfang
Jens Wiltfang
Jens Wiltfang
Dirk Fitzner
author_facet Niels Hansen
Claudia Bartels
Winfried Stöcker
Jens Wiltfang
Jens Wiltfang
Jens Wiltfang
Dirk Fitzner
author_sort Niels Hansen
collection DOAJ
description BackgroundGlycine receptor antibody-associated neuropsychiatric disease is currently known to be dominated by the phenotypes stiff-person syndrome and progressive encephalomyelitis entailing rigidity and myoclonus. In our case series we aim to depict the less-often reported feature of cognitive impairment associated with glycine receptor antibodies.MethodsWe investigated five patients with cognitive impairment varying from mild cognitive impairment to dementia associated with serum glycine receptor antibodies. Mild and major neurocognitive disorders were diagnosed according to the DSM-5 (fifth edition of the Diagnostic and Statistical Manual of Mental Disorders). Neuropsychology via Consortium to Establish a Registry for Alzheimer's Disease (CERAD) testing results, psychopathology data via the Manual for the Assessment and Documentation of Psychopathology in Psychiatry (AMDP), cerebrospinal fluid analysis and magnetic resonance imaging data were retrospectively analyzed from patient files.ResultsWe identified five patients with cognitive impairment as the main neuropsychiatric feature associated with serum glycine receptor antibodies. One patient also presented akinetic rigidity syndrome. The psychopathology comprised disorders of attention and memory, orientation, formal thought, and affect. In addition to suffering deficits in verbal memory function, figural recall, phonematic fluency, and globally deteriorated cognitive function, these patients presented seriously impaired memory recall in particular. Tau protein and phosphorylated tau protein 181 were elevated in 75% of patients.ConclusionsOur results suggest that axonal neurodegeneration and especially impaired verbal memory recall in addition to deficits in verbal and figural memory characterize patients with progressive cognitive impairment associated with glycine receptor antibodies. This unresolved issue should be clarified by researchers to discover whether axonal degeneration is merely an age-related phenomenon or one related to glycine-receptor autoantibodies in old age. Cognitive impairment as a neuropsychiatric syndrome of glycine-receptor antibody disease is a potential, conceivable, but so far unproven additional feature requiring deeper large-scale investigations and consideration during differential diagnosis in memory clinics.
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spelling doaj.art-a91f5a40e99245dc8f05f715880fe2702022-12-21T19:45:26ZengFrontiers Media S.A.Frontiers in Psychiatry1664-06402022-01-011210.3389/fpsyt.2021.778684778684Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case SeriesNiels Hansen0Claudia Bartels1Winfried Stöcker2Jens Wiltfang3Jens Wiltfang4Jens Wiltfang5Dirk Fitzner6Department of Psychiatry and Psychotherapy, University Medical Center Göttingen, Göttingen, GermanyDepartment of Psychiatry and Psychotherapy, University Medical Center Göttingen, Göttingen, GermanyEuroimmun Reference Laboratory, Lübeck, GermanyDepartment of Psychiatry and Psychotherapy, University Medical Center Göttingen, Göttingen, GermanyGerman Center for Neurodegenerative Diseases (DZNE), Göttingen, GermanyNeurosciences and Signaling Group, Department of Medical Sciences, Institute of Biomedicine (iBiMED), University of Aveiro, Aveiro, PortugalDepartment of Neurology, University Medical Center of Göttingen, Göttingen, GermanyBackgroundGlycine receptor antibody-associated neuropsychiatric disease is currently known to be dominated by the phenotypes stiff-person syndrome and progressive encephalomyelitis entailing rigidity and myoclonus. In our case series we aim to depict the less-often reported feature of cognitive impairment associated with glycine receptor antibodies.MethodsWe investigated five patients with cognitive impairment varying from mild cognitive impairment to dementia associated with serum glycine receptor antibodies. Mild and major neurocognitive disorders were diagnosed according to the DSM-5 (fifth edition of the Diagnostic and Statistical Manual of Mental Disorders). Neuropsychology via Consortium to Establish a Registry for Alzheimer's Disease (CERAD) testing results, psychopathology data via the Manual for the Assessment and Documentation of Psychopathology in Psychiatry (AMDP), cerebrospinal fluid analysis and magnetic resonance imaging data were retrospectively analyzed from patient files.ResultsWe identified five patients with cognitive impairment as the main neuropsychiatric feature associated with serum glycine receptor antibodies. One patient also presented akinetic rigidity syndrome. The psychopathology comprised disorders of attention and memory, orientation, formal thought, and affect. In addition to suffering deficits in verbal memory function, figural recall, phonematic fluency, and globally deteriorated cognitive function, these patients presented seriously impaired memory recall in particular. Tau protein and phosphorylated tau protein 181 were elevated in 75% of patients.ConclusionsOur results suggest that axonal neurodegeneration and especially impaired verbal memory recall in addition to deficits in verbal and figural memory characterize patients with progressive cognitive impairment associated with glycine receptor antibodies. This unresolved issue should be clarified by researchers to discover whether axonal degeneration is merely an age-related phenomenon or one related to glycine-receptor autoantibodies in old age. Cognitive impairment as a neuropsychiatric syndrome of glycine-receptor antibody disease is a potential, conceivable, but so far unproven additional feature requiring deeper large-scale investigations and consideration during differential diagnosis in memory clinics.https://www.frontiersin.org/articles/10.3389/fpsyt.2021.778684/fullcognitive impairmentglycine receptor antibodyneurodegenerationtau proteinmemory recall
spellingShingle Niels Hansen
Claudia Bartels
Winfried Stöcker
Jens Wiltfang
Jens Wiltfang
Jens Wiltfang
Dirk Fitzner
Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series
Frontiers in Psychiatry
cognitive impairment
glycine receptor antibody
neurodegeneration
tau protein
memory recall
title Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series
title_full Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series
title_fullStr Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series
title_full_unstemmed Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series
title_short Impaired Verbal Memory Recall in Patients With Axonal Degeneration and Serum Glycine-Receptor Autoantibodies—Case Series
title_sort impaired verbal memory recall in patients with axonal degeneration and serum glycine receptor autoantibodies case series
topic cognitive impairment
glycine receptor antibody
neurodegeneration
tau protein
memory recall
url https://www.frontiersin.org/articles/10.3389/fpsyt.2021.778684/full
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