Encephalitis lethargica
Synonyms: Von Economo encephalitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare brain inflammatory disease characterized by acute or subacute encephalitis with involvement of the midbrain and basal ganglia occurring in children as well as adults. Initial symptoms are pharyngitis and fever, followed by progressive lethargy, sleep disturbances, extrapyramidal symptoms (parkinsonism, chorea, dystonia), neuropsychiatric manifestations (obsessive-compulsive behavior, mutism, catatonia), and ocular features (oculogyric crises). Autoantibodies against human basal ganglia are often positive. Survivors may develop post-encephalitic syndromes, most prominently parkinsonism.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Encephalitis lethargica | Von Economo encephalitis |
| fr | Encéphalite léthargique | Encéphalite de von Economo |
| de | Encephalitis lethargica | Von Economo-Enzephalitis |
| es | Encefalitis letárgica | Encefalitis de Von Economo |
| it | Encefalite letargica | Encefalite di von Economo |
| nl | Encephalitis lethargica | Encefalitis, von Economo-type |
| pt | Encefalite letárgica | |
| pl | Śpiączkowe zapalenie mózgu | Zapalenie mózgu Von Economo |
| cs | Encephalitis lethargica | Von Economova encefalitida · Ekonomova encefalitida |
| uk | Летаргічний енцефаліт | Енцефаліт фон Економо |
| zh | 昏睡性脑炎 | 埃科诺莫脑炎 |
| tr | Ensefalit letarjika | Von Economo ensefaliti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | A85.8 | NTBT | attributed | validated |
| ICD-11 | 1C80 | NTBT | index | validated |
| MONDO | 0019384 | E | validated | |
| UMLS | C0014040 | E | validated | |
| GARD | 6332 | E | validated | |
| MedDRA | 10052369 | 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 (3):
- ORPHA:98255Chronic encephalitis
rare neurological diseases - ORPHA:166490Infectious disease with epilepsy
rare neurological diseases - ORPHA:306666Rare parkinsonian syndrome due to neurodegenerative disease
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet validated | 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
30 annotations · validated 2018-03-24| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001298 | Encephalopathy | |
| HP:0002360 | Sleep abnormality | |
| Frequent (79-30%) | ||
| HP:0012547 | Abnormal involuntary eye movements | |
| HP:0002960 | Autoimmunity | |
| HP:0007146 | Bilateral basal ganglia lesions | |
| HP:0000651 | Diplopia | |
| HP:0100660 | Dyskinesia | |
| HP:0001945 | Fever | |
| HP:0002315 | Headache | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0010702 | Increased circulating immunoglobulin concentration | |
| HP:0001254 | Lethargy | |
| HP:0005986 | Limitation of neck motion | |
| HP:0001268 | Mental deterioration | |
| HP:0003326 | Myalgia | |
| HP:0001300 | Parkinsonism | |
| HP:0025439 | Pharyngitis | |
| HP:0004429 | Recurrent viral infections | |
| HP:0001337 | Tremor | |
| HP:0003484 | Upper limb muscle weakness | |
| Occasional (29-5%) | ||
| HP:0002607 | Bowel incontinence | |
| HP:0001662 | Bradycardia | |
| HP:0001259 | Coma | |
| HP:0002883 | Hyperventilation | |
| HP:0009763 | Limb pain | |
| HP:0000613 | Photophobia | |
| HP:0000709 | Psychosis | |
| HP:0001250 | Seizure | |
| HP:0025258 | Stiff neck | |
| HP:0000020 | Urinary incontinence | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 14570817[PMID]_10563641[PMID]_25129855[PMID]_3801849[PMID]_6126720[PMID]_6039103[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/83600
- This record as JSON: /data/d/83600.json