ORPHA:83597
Acute disseminated encephalomyelitis
Synonyms: ADEM · Acute demyelinating encephalomyelitis · Acute disseminated encephalitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A demyelinating disorder of the central nervous system.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute disseminated encephalomyelitis | ADEM · Acute demyelinating encephalomyelitis · Acute disseminated encephalitis |
| fr | Encéphalomyélite aiguë disséminée | Encéphalite aiguë disséminée · Encéphalomyélite démyélinisante aiguë |
| de | Enzephalomyelitis, akute disseminierte | ADEM · Akute demyelinisierende Enzephalomyelitis · Enzephalitis, akute disseminierte |
| es | Encefalomielitis aguda diseminada | ADEM · EMAD · Encefalitis aguda diseminada · Encefalomielitis aguda desmielinizante |
| it | Encefalomielite disseminata acuta | Encefalomielite demielinizzante acuta · ADEM · Encefalite disseminata acuta |
| nl | Acute gedissemineerde encefalomyelitis | ADEM · Acute demyeliniserende encefalomyelitis · Acute gedissemineerde encefalitis |
| pt | Encefalite disseminada aguda | ADEM · Encefalomielite disseminada aguda |
| pl | Ostre rozsiane zapalenie mózgu i rdzemoa kręgowego | ADEM · Ostre rozsiane zapalenie mózgu |
| cs | Akutní diseminovaná encefalomyelitida | ADEM · Akutní diseminovaná encefalitida |
| uk | Гострий розсіяний енцефаломієліт | Гострий розсіяний енцефаліт · ADEM |
| zh | 急性播散性脑脊髓炎 | 急性播散性脑炎 |
| tr | Akut dissemine ensefalomiyelit | ADEM · Akut yaygın ensefalit |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G04.0 | NTBT | attributed | validated |
| ICD-11 | 8A42 | E | specific | validated |
| MONDO | 0019383 | E | validated | |
| MeSH | D004673 | E | validated | |
| UMLS | C0014059 | E | validated | |
| GARD | 8639 | E | validated | |
| MedDRA | 10000709 | 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 (3):
- ORPHA:622014Autoimmune encephalitis
rare neurological diseases - ORPHA:499047Autoimmune/inflammatory optic neuropathy
rare ophthalmic disorders - ORPHA:166490Infectious disease with epilepsy
rare neurological diseases
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.6 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Point prevalence | Unknown | — | Worldwide | Class only | 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.
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HPO phenotypes
53 annotations · validated 2021-02-04| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0001298 | Encephalopathy | Diagnostic criterion |
| Very frequent (99-80%) | ||
| HP:0007305 | CNS demyelination | |
| HP:0007204 | Diffuse white matter abnormalities | |
| HP:0010845 | EEG with generalized slow activity | |
| Frequent (79-30%) | ||
| HP:0002134 | Abnormal basal ganglia morphology | |
| HP:0012747 | Abnormal brainstem MRI signal intensity | |
| HP:0001317 | Abnormal cerebellum morphology | |
| HP:0002518 | Abnormal periventricular white matter morphology | |
| HP:0007256 | Abnormal pyramidal sign | |
| HP:0012696 | Abnormal thalamic MRI signal intensity | |
| HP:0002143 | Abnormality of the spinal cord | |
| HP:0032492 | Anti-myelin oligodendrocyte glycoprotein antibody positivity | |
| HP:0001251 | Ataxia | |
| HP:0000708 | Atypical behavior | |
| HP:0200149 | CSF lymphocytic pleiocytosis | |
| HP:0002181 | Cerebral edema | |
| HP:0031696 | Disseminated viral infection | |
| HP:0001945 | Fever | |
| HP:0002315 | Headache | |
| HP:0100786 | Hypersomnia | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0025373 | Interictal EEG abnormality | |
| HP:0000737 | Irritability | |
| HP:0001268 | Mental deterioration | |
| HP:0012486 | Myelitis | |
| HP:0002018 | Nausea | |
| HP:0001250 | Seizure | |
| Occasional (29-5%) | ||
| HP:0002167 | Abnormality of speech or vocalization | |
| HP:0000718 | Aggressive behavior | |
| HP:0001259 | Coma | |
| HP:0001289 | Confusion | |
| HP:0006824 | Cranial nerve paralysis | |
| HP:0010843 | EEG with focal slow activity | |
| HP:0011193 | EEG with focal spikes | |
| HP:0030857 | Eye movement-induced pain | |
| HP:0001269 | Hemiparesis | |
| HP:0001324 | Muscle weakness | |
| HP:0031179 | Nuchal rigidity | |
| HP:0011151 | Obtundation status | |
| HP:0100653 | Optic neuritis | |
| HP:0004887 | Respiratory failure requiring assisted ventilation | |
| HP:0003474 | Somatic sensory dysfunction | |
| HP:0000572 | Visual loss | |
| HP:0002013 | Vomiting | |
| Very rare (<4-1%) | ||
| HP:0006597 | Diaphragmatic paralysis | |
| HP:0012302 | Herpes simplex encephalitis | |
| HP:0007103 | Hypointensity of cerebral white matter on MRI | |
| HP:0004305 | Involuntary movements | |
| HP:0020088 | Post-vaccination measles | |
| HP:0020089 | Post-vaccination rubella | |
| HP:0031693 | Severe Epstein Barr virus infection | |
| HP:0031695 | Severe parainfluenza infection | |
| HP:0006562 | Viral hepatitis | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 31340401[PMID]_31371120[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/83597
- This record as JSON: /data/d/83597.json