Autoimmune limbic encephalitis
Synonyms: ALE
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare autoimmune encephalitis involving the mesial temporal lobes and clinically characterized by subacute onset (i. e. rapid progression of less than three months) of short-term memory deficits, seizures or psychiatric symptoms, such as behavioral changes, anxiety, depression, and psychosis. Further diagnostic criteria are bilateral abnormalities restricted to the mesial temporal lobes in brain MRI, cerebrospinal fluid pleocytosis and/or epileptic or slow-wave activity involving the temporal lobes in EEG, and reasonable exclusion of alternative causes. Paraneoplastic or non-paraneoplastic antibodies against neuronal antigens may be found in serum and/or cerebrospinal fluid.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Autoimmune limbic encephalitis | ALE |
| fr | Encéphalite limbique auto-immune | |
| de | Autoimmune limbische Enzephalitis | = en |
| es | Encefalitis límbica autoinmune | |
| it | Encefalite limbica autoimmune | = en |
| nl | Auto-immune limbische encefalitis | = en |
| pt | Encefalite límbica autoimune | = en |
| pl | Autoimmunologiczne zapalenie układu limbicznego mózgu | = en |
| cs | Autoimunitní limbická encefalitida | = en |
| uk | Аутоімунний лімбічний енцефаліт | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G04.8 | NTBT | attributed | validated |
| ICD-11 | 8E4A.0 | NTBT | index | validated |
| MONDO | 0850097 | E | validated | |
| MeSH | C531729 | E | validated | |
| UMLS | C2930824 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:622014Autoimmune encephalitis
rare neurological diseases - ORPHA:36388Paraneoplastic neurologic syndrome
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 1.7 / 100 000 | Europe | Value and class | Validated | 31552550[PMID]_ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.25 / 100 000 | Europe | Value and class | Validated | 31552550[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
24 annotations · validated 2024-10-09| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0012658 | Abnormal brain FDG positron emission tomography | Diagnostic criterion |
| HP:5000016 | Anti-Hu antibody positivity | |
| HP:5000020 | Anti-LGI1 antibody | |
| HP:0000739 | Anxiety | |
| HP:0000708 | Atypical behavior | |
| HP:6000397 | CSF oligoclonal immunoglobulin G bands | |
| HP:0012229 | CSF pleocytosis | Diagnostic criterion |
| HP:0000716 | Depression | |
| HP:0010843 | EEG with focal slow activity | Diagnostic criterion |
| HP:6001115 | EEG with temporal slowing | Diagnostic criterion |
| HP:0001945 | Fever | |
| HP:0002315 | Headache | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0000737 | Irritability | |
| HP:0001254 | Lethargy | |
| HP:0001268 | Mental deterioration | |
| HP:0000709 | Psychosis | |
| HP:0001250 | Seizure | |
| HP:0033687 | Short term memory impairment | |
| Occasional (29-5%) | ||
| HP:5000005 | Anti-CASPR2 | |
| HP:5000010 | Anti-GABA(B)R antibody | |
| HP:0010532 | Paroxysmal vertigo | |
| Excluded (0%) | ||
| HP:0002383 | Infectious encephalitis | Diagnostic criterion |
| HP:0100836 | Malignant neoplasm of the central nervous system | Diagnostic criterion |
Annotation source: 31085562[PMID]_34603897[PMID]_26906964[PMID]_30421340[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/623615
- This record as JSON: /data/d/623615.json