Steroid-responsive encephalopathy associated with autoimmune thyroiditis
Synonyms: Hashimoto encephalitis · SREAT
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare, acquired, neurological disease characterized by encephalopathy associated with elevated antithyroid antibodies, in the absence of other causes. Clinical presentation varies from minor cognitive impairment to status epilepticus and coma, and frequently includes seizures, confusion, speech disorder, memory impairment, ataxia and psychiatric manifestations.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Steroid-responsive encephalopathy associated with autoimmune thyroiditis | Hashimoto encephalitis · SREAT |
| fr | Encéphalopathie sensible aux stéroïdes associée à une thyroïdite auto-immune | Encéphalite de Hashimoto · SREAT |
| de | Steroid-responsive Enzephalopathie mit assoziierter Autoimmun-Thyroiditis | Hashimoto-Enzephalitis · SREAT |
| es | Encefalopatía sensible a esteroides asociada a tiroiditis autoinmune | Encefalitis de Hashimoto · SREAT |
| it | Encefalopatia sensibile agli steroidi associata alla tiroidite autoimmune | Encefalite di Hashimoto · SREAT |
| nl | Steroïdresponsieve encefalopathie geassocieerd met auto-immuunthyreoïditis | Hashimoto-encefalitis · SREAT |
| pt | Encefalite sensível aos esteróides associada a tiroidite autoimune | Encefalite de Hashimoto |
| pl | Encefalopatia wrażliwa na steroidy z towarzyszącym autoimmunologicznym zapaleniem tarczycy | SREAT · Zapalenie mózgu Hashimoto |
| cs | Encefalopatie asociovaná s autoimunitní thyroiditidou odpovídající na steroidy | Hashimotova encefalitida · SREAT |
| uk | Стероїдозалежна енцефалопатія, повязана з аутоімунним тиреоїдитом | Енцефаліт Хашимото · SREAT |
| zh | 自身免疫性甲状腺炎相关激素敏感性脑炎 | 桥本脑炎 |
| tr | Otoimmün tiroidit-ilişkili steroide duyarlı ensefalopati | SREAT · Hashimoto ensefaliti |
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 | 0019385 | E | validated | |
| MeSH | C535841 | E | validated | |
| UMLS | C0393639 | E | validated | |
| GARD | 8570 | E | validated | |
| MedDRA | 10069432 | 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 (4):
- ORPHA:247242Acquired ataxia
rare neurological diseases - ORPHA:622014Autoimmune encephalitis
rare neurological diseases - ORPHA:177101Rare adult hypothyroidism
rare endocrine diseases - ORPHA:98033Rare neurologic disease with psychiatric involvement
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
25 annotations · validated 2016-11-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0006846 | Acute encephalopathy | |
| Very frequent (99-80%) | ||
| HP:0005318 | Cerebral vasculitis | |
| HP:0000872 | Hashimoto thyroiditis | |
| Frequent (79-30%) | ||
| HP:0002500 | Abnormal cerebral white matter morphology | |
| HP:0001289 | Confusion | |
| HP:0000853 | Goiter | |
| HP:0002902 | Hyponatremia | |
| HP:0000821 | Hypothyroidism | |
| HP:0003470 | Paralysis | |
| Occasional (29-5%) | ||
| HP:0012332 | Abnormal autonomic nervous system physiology | |
| HP:0009102 | Anterior open-bite malocclusion | |
| HP:0000739 | Anxiety | |
| HP:0000716 | Depression | |
| HP:0001945 | Fever | |
| HP:0007359 | Focal-onset seizure | |
| HP:0002197 | Generalized-onset seizure | |
| HP:0002315 | Headache | |
| HP:0002721 | Immunodeficiency | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0002017 | Nausea and vomiting | |
| HP:0000709 | Psychosis | |
| HP:0002133 | Status epilepticus | |
| HP:0001873 | Thrombocytopenia | |
| Very rare (<4-1%) | ||
| HP:0005991 | Limited neck flexion | |
| Excluded (0%) | ||
| HP:0002181 | Cerebral edema | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 25540320[PMID]_11158076[PMID]_11835965[PMID]_21511782[PMID]_12949305[PMID]_16476807[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/83601
- This record as JSON: /data/d/83601.json