Steroid-responsive encephalopathy associated with autoimmune thyroiditis

disease
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Also known as Hashimoto's encephalitisHashimoto's encephalopathySREATsteroid-responsive encephalopathy associated with thyroid disease

Summary

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (MONDO:0019385) is a disease. A subtype of postinfectious encephalitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: <1 / 1 000 000 (Europe)
  • Phenotypes (HPO): 25

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Point prevalence<1 / 1 000 000EuropeNot yet validated

Signs & symptoms

Clinical features (HPO)

25 HPO clinical features (Orphanet curated; top 25 by frequency):

HPO IDTermFrequency
HP:0006846Acute encephalopathyObligate (100%)
HP:0005318Cerebral vasculitisVery frequent (80-99%)
HP:0000872Hashimoto thyroiditisVery frequent (80-99%)
HP:0000821HypothyroidismFrequent (30-79%)
HP:0000853GoiterFrequent (30-79%)
HP:0001289ConfusionFrequent (30-79%)
HP:0002500Abnormal cerebral white matter morphologyFrequent (30-79%)
HP:0002902HyponatremiaFrequent (30-79%)
HP:0003470ParalysisFrequent (30-79%)
HP:0000709PsychosisOccasional (5-29%)
HP:0000716DepressionOccasional (5-29%)
HP:0000739AnxietyOccasional (5-29%)
HP:0001873ThrombocytopeniaOccasional (5-29%)
HP:0001945FeverOccasional (5-29%)
HP:0001974LeukocytosisOccasional (5-29%)
HP:0002017Nausea and vomitingOccasional (5-29%)
HP:0002133Status epilepticusOccasional (5-29%)
HP:0002197Generalized-onset seizureOccasional (5-29%)
HP:0002315HeadacheOccasional (5-29%)
HP:0002721ImmunodeficiencyOccasional (5-29%)
HP:0007359Focal-onset seizureOccasional (5-29%)
HP:0009102Anterior open-bite malocclusionOccasional (5-29%)
HP:0012332Abnormal autonomic nervous system physiologyOccasional (5-29%)
HP:0002181Cerebral edemaExcluded (0%)
HP:0005991Limited neck flexionVery rare (<1-4%)

Identifiers

Disease identifiers

FieldValue
Canonical namesteroid-responsive encephalopathy associated with autoimmune thyroiditis
Mondo IDMONDO:0019385
MeSHC535841
Orphanet83601
UMLSC0393639
MedGen98280
GARD0008570
Is cancer (heuristic)no

Also known as: Hashimoto’s encephalitis · Hashimoto’s encephalopathy · SREAT · steroid-responsive encephalopathy associated with thyroid disease

Disease family

This is a subtype of postinfectious encephalitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › nervous system disordercentral nervous system disorderencephalomyelitisencephalitisinfectious encephalitispostinfectious encephalitissteroid-responsive encephalopathy associated with autoimmune thyroiditis

Related subtypes (6): limbic encephalitis with LGI1 antibodies, Rasmussen subacute encephalitis, Bickerstaff brainstem encephalitis, acute disseminated encephalomyelitis, encephalitis lethargica, rubella encephalitis

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.

No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.