Autoimmune limbic encephalitis

Summary

Autoimmune limbic encephalitis (MONDO:0850097) is a disease. A subtype of limbic encephalitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 24

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.25	Europe	Validated
Point prevalence	1-9 / 100 000	1.7	Europe	Validated

Signs & symptoms

Clinical features (HPO)

24 HPO clinical features (Orphanet curated; top 24 by frequency):

HPO ID	Term	Frequency
HP:0000708	Atypical behavior	Frequent (30-79%)
HP:0000709	Psychosis	Frequent (30-79%)
HP:0000716	Depression	Frequent (30-79%)
HP:0000737	Irritability	Frequent (30-79%)
HP:0000739	Anxiety	Frequent (30-79%)
HP:0001250	Seizure	Frequent (30-79%)
HP:0001254	Lethargy	Frequent (30-79%)
HP:0001268	Mental deterioration	Frequent (30-79%)
HP:0001945	Fever	Frequent (30-79%)
HP:0002315	Headache	Frequent (30-79%)
HP:0002922	Increased CSF protein concentration	Frequent (30-79%)
HP:0010843	EEG with focal slow activity	Frequent (30-79%)
HP:0012229	CSF pleocytosis	Frequent (30-79%)
HP:0012658	Abnormal brain FDG positron emission tomography	Frequent (30-79%)
HP:0033687	Short term memory impairment	Frequent (30-79%)
HP:5000016	Anti-Hu antibody positivity	Frequent (30-79%)
HP:5000020	Anti-LGI1 antibody	Frequent (30-79%)
HP:6000397	CSF oligoclonal immunoglobulin G bands	Frequent (30-79%)
HP:6001115	EEG with temporal slowing	Frequent (30-79%)
HP:0010532	Paroxysmal vertigo	Occasional (5-29%)
HP:5000005	Anti-CASPR2	Occasional (5-29%)
HP:5000010	Anti-GABA(B)R antibody	Occasional (5-29%)
HP:0002383	Infectious encephalitis	Excluded (0%)
HP:0100836	Malignant neoplasm of the central nervous system	Excluded (0%)

Identifiers

Disease identifiers

Field	Value
Canonical name	autoimmune limbic encephalitis
Mondo ID	MONDO:0850097
Orphanet	623615
ICD-11	1254443511
UMLS	C2930824
MedGen	419645
GARD	0022492
Is cancer (heuristic)	no

Disease family

This is a subtype of limbic encephalitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › encephalomyelitis › encephalitis › limbic encephalitis › autoimmune limbic encephalitis

Related subtypes (5): paraneoplastic limbic encephalitis, posttransplant acute limbic encephalitis, non-herpetic acute limbic encephalitis, limbic encephalitis with caspr2 antibodies, limbic encephalitis with DPP6 antibodies

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.