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autoimmune dementia
Epidemiology:
- rare (< 5%)
Pathology:
- coexisting organ-specific autoimmunity
Clinical manifestations:
may present as progressive dementia without delirium
- subacute onset over a few weeks or months
- fluctuating course
- tremor
- behavior & personality changes
- seizures occur early
response to treatment
- most improve in the first week of treatment.
- improvement in almost all cognitive domains, most notably learning & memory
- relapse with discontinuation of immunotherapy
Laboratory:
CSF analysis
- seropostivity for
- cation channel complex autoantibody
- neuronal voltage-gated K+ channel Ab in CSF >
- Ca+2 channel Ab in CSF or neuronal acetylcholine receptor Ab in CSF
- LGI1 Ab in CSF
- inflammatory CSF
- elevated CSF protein (>100 mg/dL)
- CSF pleocytosis
Special laboratory:
- electroencephalography: improvements with therapy
Radiology:
- neuroimaging: improvements with therapy Differential diagnsosis:
- CNS vasculitis
- multiple sclerosis
- anti-NMDA receptor encephalitis
Management:
immunotherapy
- methylprednisolone, prednisone or dexamethasone
- intravenous immune globulin
- plasma exchange
General
dementia; Alzheimer's disease & related dementias (ADRD)
autoimmune disease
References
- Flanagan EP, McKeon A, Lennon VA et al
Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response.
Mayo Clin Proc. 2020 Oct; 85(10): 881
PMID: 20884824 PMCID: PMC2947960 Free PMC article
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2947960/
- Cox D
The dementia that can be cured.
The Guardian. 2020 Oct 25
https://www.theguardian.com/science/2020/oct/25/the-dementia-that-can-be-cured