ORPHA:2806
Subacute sclerosing leukoencephalitis
Synonyms: Dawson encephalitis · SSPE · Subacute inclusion body encephalitis · Subacute sclerosing panencephalitis · Van Bogaert disease · Van Bogaert encephalitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare infectious disease characterized by slowly progressive brain disorder caused by a mutant measles virus, typically affecting children and young adults. The condition leads to cognitive decline, myoclonus, vision loss, and eventually a vegetative state.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Subacute sclerosing leukoencephalitis | Dawson encephalitis · SSPE · Subacute inclusion body encephalitis · Subacute sclerosing panencephalitis · Van Bogaert disease · Van Bogaert encephalitis |
| fr | Leucoencéphalite sclérosante subaiguë | Encéphalite de Dawson · Encéphalite de Van Bogaert · Encéphalite morbilleuse à inclusions · Maladie de Van Bogaert · Panencéphalite sclérosante subaiguë |
| de | Leukoenzephalitis, sklerosierende subakute | Dawson-Enzephalitis · Einschlußkörperchen-Enzephalitis, subakute · Subakute sklerosierende Panenzephalitis (SSPE) · Van Bogaert-Enzephalitis · Van-Bogaert-Krankheit |
| es | Leucoencefalitis esclerosante subaguda | Encefalitis con cuerpos de inclusión subaguda · Encefalitis de Dawson · Encefalitis de Van Bogaert · Enfermedad de Van Bogaert · Panencefalitis esclerosante subaguda (PEES) |
| it | Leucoencefalite sclerosante subacuta | Malattia di Van Bogaert · Panencefalite sclerosante subacuta |
| nl | Subacute scleroserende leuko-encefalitis | Dawson-encefalitis · SSPE · Subacute inclusielichaampjes-encefalitis · Subacute scleroserende panencefalitis · Van Bogaert-encefalitis · Ziekte van Van Bogaert |
| pt | Leucoencefalite esclerosante sub-aguda | Doença de Bosin · Doença de Van Bogaert · Encefalite de Dawson · Encefalite de corpos de inclusão · Encefalite de corpos de inclusão, sub-aguda · Leucoencefalite esclerosante · Panencefalite esclerosante sub-aguda · SSPE |
| pl | Podostre stwardniające zapalenie mózgu | Choroba Van Bogaerta · Podostre wtrętowe zapalenie mózgu · SSPE · Subacute sclerosing panencephalitis · Zapalenie mózgu Dawsona · Zapalenie mózgu Van Bogaerta |
| cs | Subakutní sklerotizující leukoencefalitida | Dawsonova nemoc · SSPE · Subakutní sklerózující panencefalitida · Van Bogaertova nemoc · Van Bogaertova encefalitida · Subakutní encefalitida s inkluzními tělísky |
| uk | Підгострий склерозуючий лейкоенцефаліт | Енцефаліт Доусона · SSPE · Підгострий енцефаліт з тілами включення · Підгострий склерозуючий паненцефаліт · Хвороба Ван-Богарта · Енцефаліт Ван-Богарта |
| zh | 亚急性硬化脑白质炎 | 亚急性包涵体脑炎 |
| tr | Subakut sklerozan lökoensefalit | SSPE · Dawson ensefaliti · Van Bogaert hastalığı · Van Bogaert ensefaliti · Subakut sklerozan panensefalit · Subakut inklüzyon vücut ensefaliti |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 260470 | E | validated | |
| ICD-10 | A81.1 | NTBT | attributed | validated |
| ICD-11 | 8A45.01 | E | specific | validated |
| MONDO | 0009835 | E | validated | |
| MeSH | D013344 | E | validated | |
| UMLS | C0038522 | E | validated | |
| GARD | 7708 | E | validated | |
| MedDRA | 10042297 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:98255Chronic encephalitis
rare neurological diseases - ORPHA:166490Infectious disease with epilepsy
rare neurological diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | United States | Value and class | Validated | [EXPERT]_22277189[PMID] |
| Annual incidence | 1-9 / 100 000 | 2 / 100 000 | India | Value and class | Validated | [EXPERT] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
36 annotations · validated 2024-11-20| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000708 | Atypical behavior | |
| HP:0001268 | Mental deterioration | |
| HP:0001336 | Myoclonus | |
| Frequent (79-30%) | ||
| HP:0012332 | Abnormal autonomic nervous system physiology | |
| HP:0012672 | Akinetic mutism | |
| HP:0430088 | Anti-measles antibody positivity | Diagnostic criterion |
| HP:0012444 | Brain atrophy | |
| HP:0012424 | Chorioretinitis | |
| HP:0000746 | Delusion | |
| HP:0000726 | Dementia | |
| HP:0000716 | Depression | |
| HP:0001332 | Dystonia | |
| HP:0010856 | EEG with periodic complexes | Diagnostic criterion |
| HP:0010856 | EEG with periodic complexes | |
| HP:0001288 | Gait disturbance | |
| HP:0000738 | Hallucinations | |
| HP:0000737 | Irritability | |
| HP:0001254 | Lethargy | |
| HP:0002371 | Loss of speech | |
| HP:0033044 | Motor regression | |
| HP:0030891 | Periventricular white matter hyperintensities | |
| HP:0001250 | Seizure | |
| HP:0002360 | Sleep abnormality | |
| HP:0031358 | Vegetative state | |
| Occasional (29-5%) | ||
| HP:0012709 | Abnormal brain choline/creatine ratio by MRS | |
| HP:0002071 | Abnormality of extrapyramidal motor function | |
| HP:0001251 | Ataxia | |
| HP:6000397 | CSF oligoclonal immunoglobulin G bands | |
| HP:0012229 | CSF pleocytosis | |
| HP:0100660 | Dyskinesia | |
| HP:0001085 | Papilledema | |
| HP:0012708 | Reduced brain N-acetyl aspartate level by MRS | |
| HP:0000573 | Retinal hemorrhage | |
| HP:0001257 | Spasticity | |
| HP:0002119 | Ventriculomegaly | |
| HP:0000572 | Visual loss | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 32809508[PMID]_31237061[PMID]_30680706[PMID]_38282559[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2806
- This record as JSON: /data/d/2806.json