Rapidly progressive dementia–associated N-type voltage-gated calcium channel antibody encephalopathy

Thakolwiboon, Smathorn; Sohn, Gyeongmo; Duarte-Celada, Walter R.; Julayanont, Parunyou

doi:10.1080/08998280.2019.1709117

Cited by 2 publications

(3 citation statements)

References 11 publications

(15 reference statements)

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“…We observed axonal neurodegeneration in 71.4% as evidenced by elevated total tau protein and ptau181 of our NABD patients-substantial neurodegeneration that might suffice to drive a rapid cognitive decline in most NABD patients. Autoantibody-associated cognitive decline is reported to begin with a subacute cognitive impairment onset that soon develops into rapid cognitive decline (2,3,(5)(6)(7). No large cohort studies exist on long-term cognitive decline in NABD.…”

Section: Discussionmentioning

confidence: 99%

“…However, there have been no investigations addressing the progression of cognitive dysfunction in autoantibody-associated dementia compared to Alzheimer's dementia (AD). Recent individual case reports (5)(6)(7) and reviews (1,3) have postulated that rapidly progressing cognitive dysfunction associated with neural autoantibodies are a typical clinical manifestation of NABD. We thus conducted a pilot trial relying on retrospective data to identify whether progressing cognitive dysfunction is more prominent in NABD than in AD.…”

Section: Introductionmentioning

confidence: 99%

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Cerebrospinal Fluid Total Tau Protein Correlates With Longitudinal, Progressing Cognitive Dysfunction in Anti-Neural Autoantibody-Associated Dementia and Alzheimer’s Dementia: A Case–Control Study

et al. 2022

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BackgroundNeural autoantibody-associated dementia (NABD) is an increasing phenomenon in memory clinics with a high impact on later therapy. Biomarkers are lacking that differentiate this type of dementia from neurodegenerative dementia such as Alzheimer’s dementia (AD). Our aim is to analyze neurodegeneration markers and their relationship to progressing cognitive dysfunction in NABD and AD to test for tools differentiating these two forms of dementia prior to neural autoantibody testing.MethodsIn our retrospective, observational study, we investigated 14 patients with dementia and serum and/or cerebrospinal fluid (CSF) neural autoantibodies as well as 14 patients with AD by relying on recent CSF and clinical criteria for AD. Patient files were checked for psychopathology, neuropsychological test performance, autoimmune indicators, CSF, and MRI results.ResultsOur patient groups did not differ in their psychopathology, autoimmune indicators, or MRI profile. The progression of cognitive dysfunction [as measured by the difference in Mini-Mental State Examination (MMSE) scores since disease onset, and the yearly progression rate (MMSE loss/per year)] did not vary significantly between groups. Total tau protein was significantly higher in AD patients than NABD patients revealing no signs of Alzheimer’s disease pathology in their CSF (p < 0.05). Total tau protein levels in CSF correlated with cognitive decline since disease onset (r = 0.38, p < 0.05) and yearly progression rates (r = 0.56, p < 0.005) in all patients.DiscussionOur results suggest that the progression of cognitive dysfunction as defined by MMSE does not seem to be an appropriate biomarker for distinguishing NABD from AD. However, the total tau protein level in CSF might be a relevant molecular biomarker that can indicate disease pathology and/or progression in both known AD and NABD, which is often accompanied by axonal degeneration. Total tau protein may be an additional diagnostic tool with which to differentiate anti-neural-associated dementia from AD if further research confirms these proof-of-concept findings in larger patient cohorts.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Cerebrospinal Fluid Total Tau Protein Correlates With Longitudinal, Progressing Cognitive Dysfunction in Anti-Neural Autoantibody-Associated Dementia and Alzheimer’s Dementia: A Case–Control Study

et al. 2022

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“…Anti-N-type calcium channel autoimmune encephalitis commonly present with psychotic symptoms (paranoia, hallucinations) and cognitive decline. Each reported case responded well to immunosuppressive therapy in the form of either high dose corticosteroids, plasmapheresis, or Intravenous immunoglobulin 3 , 4 , 5 .…”

Section: Discussionmentioning

confidence: 99%

Post-infectious SARS-CoV-2 autoimmune encephalitis associated with N-type calcium channel antibodies

Torrico

Kerr

DeGiorgio

2021

Neuroimmunology Reports

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Rapidly progressive dementia–associated N-type voltage-gated calcium channel antibody encephalopathy

Cited by 2 publications

References 11 publications

Cerebrospinal Fluid Total Tau Protein Correlates With Longitudinal, Progressing Cognitive Dysfunction in Anti-Neural Autoantibody-Associated Dementia and Alzheimer’s Dementia: A Case–Control Study

Cerebrospinal Fluid Total Tau Protein Correlates With Longitudinal, Progressing Cognitive Dysfunction in Anti-Neural Autoantibody-Associated Dementia and Alzheimer’s Dementia: A Case–Control Study

Post-infectious SARS-CoV-2 autoimmune encephalitis associated with N-type calcium channel antibodies

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