Observational studyNeurology2026
Autoimmune Encephalitis as Treatment-Responsive Cause of Rapidly Progressive Dementia: A Multicenter Prospective Cohort Study.
Observational study in Neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
3 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Prion disease mimicking rapidly progressive Alzheimer disease: case series and systematic review.Journal of neurology · 2026Pooled it
- Diagnosing autoimmune encephalitis in psychiatry: clinical recommendations and unresolved challenges from the GENERATE network.Molecular psychiatry · 2026Review
- Characteristics and prognostic factors of cognitive impairment in autoimmune glial fibrillary acidic protein astrocytopathy.Journal of neurology · 2026Article
Corrections and comments
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Authors and funding
29 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesEarly recognition of patients with rapidly progressive dementia (RPD) attributed to autoimmune encephalitis (AE) is important because rapid initiation of immunotherapy improves outcomes. However, ancillary testing is often time-consuming because of the broad differential diagnosis, highlighting the need for accurate patient selection based on early clinical features. We aim to determine the frequency of AE subtypes in patients with RPD (AE-RPD) and characterize presenting subphenotypes (i.e., symptoms in addition to dementia) compared with other diagnoses.
methodsThis prospective multicenter observational cohort study was conducted from December 2019 to December 2024 across centers in the Netherlands and included adult patients with RPD, defined as dementia beginning within 1 year of symptom onset. Clinical features and ancillary testing data were collected and reviewed by 3 neurologists. Serum and CSF were evaluated for neuronal/glial autoantibodies.
resultsA total of 147 patients were included (46% female; median age 67 years, range 36-86). AE-RPD was the largest diagnostic category (58/147; 39%) and most common treatment-responsive cause of RPD (58/95; 61%). Neurodegenerative diseases (20/147; 14%) and Creutzfeldt-Jakob disease (CJD, 17/147; 12%) accounted for the largest nonresponsive causes of RPD. RPD accompanied by seizures at first presentation was more frequent in AE-RPD compared with other diagnoses (20/58; 34% vs 9/89; 10%; DISCUSSION: In this study, AE was the most common treatment-responsive cause of RPD. Anti-leucine-rich, glioma-inactivated 1 encephalitis and autoimmune GFAP astrocytopathy were the predominant subtypes in AE-RPD.
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