autoimmune dementia
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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
- 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
- cation channel complex autoantibody
- inflammatory CSF
- elevated CSF protein (>100 mg/dL)
- CSF pleocytosis
Diagnostic procedures
- electroencephalography: improvements with therapy
Radiology
- neuroimaging: improvements with therapy Differential diagnsosis:
- CNS vasculitis
- multiple sclerosis
- anti-NMDA receptor encephalitis
Management
More general terms
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: https://www.ncbi.nlm.nih.gov/pubmed/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