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Encefalite sensível aos esteroides associada a tireoidite autoimune
ORPHA:83601CID-10 · G04.8CID-11 · 8E4A.0DOENÇA RARA

A encefalopatia responsiva a esteróides associada à tireoidite autoimune (SREAT) é uma doença neurológica adquirida rara, caracterizada por encefalopatia associada a anticorpos antitireoidianos elevados, na ausência de outras causas. A apresentação clínica varia desde comprometimento cognitivo menor até estado de mal epiléptico e coma, e frequentemente inclui convulsões, confusão, distúrbios da fala, comprometimento da memória, ataxia e manifestações psiquiátricas.

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Introdução

O que você precisa saber de cara

📋

A encefalopatia responsiva a esteróides associada à tireoidite autoimune (SREAT) é uma doença neurológica adquirida rara, caracterizada por encefalopatia associada a anticorpos antitireoidianos elevados, na ausência de outras causas. A apresentação clínica varia desde comprometimento cognitivo menor até estado de mal epiléptico e coma, e frequentemente inclui convulsões, confusão, distúrbios da fala, comprometimento da memória, ataxia e manifestações psiquiátricas.

Publicações científicas
93 artigos
Último publicado: 2026 Jun

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
All ages
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G04.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
10 sintomas
🩸
Sangue
2 sintomas
📏
Crescimento
2 sintomas
🛡️
Imunológico
1 sintomas
🦷
Dentes
1 sintomas
🫃
Digestivo
1 sintomas

+ 8 sintomas em outras categorias

Características mais comuns

100%prev.
Encefalopatia aguda
90%prev.
Vasculite cerebral
Muito frequente (99-80%)
90%prev.
Tireoidite de Hashimoto
Muito frequente (99-80%)
55%prev.
Confusão
Frequente (79-30%)
55%prev.
Bócio
Frequente (79-30%)
55%prev.
Hipotireoidismo
Frequente (79-30%)
25sintomas
Muito frequente (3)
Frequente (6)
Ocasional (14)
Muito raro (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 25 características clínicas mais associadas, ordenadas por frequência.

Encefalopatia agudaAcute encephalopathy
Muito frequente100%
Vasculite cerebralCerebral vasculitis
Muito frequente (99-80%)90%
Tireoidite de HashimotoHashimoto thyroiditis
Muito frequente (99-80%)90%
ConfusãoConfusion
Frequente (79-30%)55%
BócioGoiter
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico93PubMed
Últimos 10 anos68publicações
Pico202513 papers
Linha do tempo
2026Hoje · 2026🧪 2015Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

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Onde tratar no SUS

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🇧🇷 Atendimento SUS — Encefalite sensível aos esteroides associada a tireoidite autoimune

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Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
69 papers (10 anos)
#1

Steroid-responsive encephalopathy associated with autoimmune thyroiditis.

BMJ case reports2025 Sep 25

Hashimoto encephalopathy or steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition associated with rapidly progressive dementia, stroke-like symptoms, psychosis and positive antithyroid antibodies. In this case report, a woman in her 80s with hypothyroidism and no other medically relevant history presented with a 2-week complaint of progressive hallucinations, short-term memory loss and paranoia. Eventually hospitalised, her hospital course was complicated by severe hospital-acquired delirium, which clouded the clinical picture; however, initial workup revealed an elevated thyroglobulin antibody and thyroid peroxidase, both highly specific for SREAT. A 3-day course of high-dose methylprednisolone (1 g/day) was administered, which revealed marked improvement in patient symptoms. Definitive treatment included a 13-week oral prednisone taper and outpatient follow-up with neurology and primary care. In cases of new onset neuropsychiatric symptoms in geriatric populations, SREAT is a reversible, must-not-miss diagnosis, when treated with systemic steroids.

#2

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Authors' Reply.

Indian journal of pediatrics2025 Apr
#3

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Correspondence.

Indian journal of pediatrics2025 Apr
#4

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT).

Indian journal of pediatrics2025 Apr
#5

Hashimoto's encephalopathy presenting with manic and psychotic features in a 13-year-old girl: a case report.

Frontiers in psychiatry2025

Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare autoimmune neuropsychiatric syndrome with highly variable clinical manifestations. Pediatric cases are particularly underrecognized, contributing to diagnostic delays. We describe a 13-year-old female patient who developed subacute behavioral changes, including withdrawal, emotional lability, and progressive manic and psychotic symptoms, over 1 month. Laboratory evaluation showed markedly elevated antithyroid antibodies with normal thyroid hormone levels, and thyroid ultrasound revealed features consistent with autoimmune thyroiditis. Neuroimaging and cerebrospinal fluid studies were unremarkable. The patient demonstrated rapid and sustained improvement following pulse corticosteroids, intravenous immunoglobulin (IVIG), and low-dose antipsychotics. Her clinical course, combined with serological and imaging findings, supported the diagnosis of HE presenting with manic and psychotic features. This case emphasizes the importance of considering HE in the differential diagnosis of acute and subacute onset psychiatric symptoms in children and highlights the diagnostic utility of thyroid antibody testing in such presentations. Categories: Pediatrics; Psychiatry; Neurology; Endocrinology; Immunology.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC42 artigos no totalmostrando 68

2025

Hashimoto's encephalopathy presenting with manic and psychotic features in a 13-year-old girl: a case report.

Frontiers in psychiatry
2025

Primary Central Nervous System Lymphoma: A Case Report of the Neurological Great Mimicker.

Cureus
2025

Hashimoto's Encephalopathy as a Cause of Persistent Myoclonic Jerks: A Report of a Unique Case.

Cureus
2025

Hashimoto's encephalopathy in psychiatric inpatients: neuropsychiatric morbidity, diagnostic challenges and treatment.

Frontiers in psychiatry
2025

Steroid-responsive encephalopathy associated with autoimmune thyroiditis.

BMJ case reports
2025

Beyond Pediatric Sleep Disorders: A Case of Hypersomnia Revealing Hashimoto's Encephalopathy.

Cureus
2025

Managing Hashimoto's Encephalopathy: A Case Report.

Cureus
2025

Hashimoto's Encephalopathy: Clinical Features, Therapeutic Strategies, and Rehabilitation Approaches.

Biomedicines
2025

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Authors' Reply.

Indian journal of pediatrics
2025

When thyroid met brain: the enigma of steroid responsive encephalopathy associated with autoimmune thyroiditis a case report.

Frontiers in immunology
2025

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Correspondence.

Indian journal of pediatrics
2025

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT).

Indian journal of pediatrics
2024

A Challenging Diagnosis of Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis (SREAT) in a Systemic Lupus Erythematosus (SLE) Patient With Hashimoto Encephalopathy (HE): A Case Report.

Cureus
2024

An Intriguing Case of Delirium: Recognizing Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis.

Cureus
2025

Hashimoto encephalopathy: a literature review and case report with comprehensive neuropsychological evaluation.

The Clinical neuropsychologist
2024

Difficulties in the Diagnostics and Treatment of Hashimoto's Encephalopathy-A Systematic and Critical Review.

International journal of molecular sciences
2024

Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) presenting as subacute psychosis: Case report and literature review.

Clinical case reports
2023

A Prospective Observational Study of Autoimmune Encephalitis in Northwestern India.

The Journal of the Association of Physicians of India
2024

Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis Presenting With Parkinsonism.

Cureus
2023

[Steroid responsive encephalopathy associated with autoimmune thyroiditis as a cause of acuteencephalopathy].

Vnitrni lekarstvi
2023

The use of intravenous immunoglobulin in the treatment of Hashimoto's encephalopathy: case based review.

Frontiers in neurology
2023

Steroid-responsive encephalopathy associated with autoimmune thyroiditis presenting as cortisone sensible psychosis with reversible leukoencephalopathy.

Journal of neuroimmunology
2023

Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis Masquerading Sepsis.

Cureus
2023

Thyroid Disorders and Movement Disorders-A Systematic Review.

Movement disorders clinical practice
2023

Hashimoto's Encephalopathy: Case Series and Literature Review.

Current neurology and neuroscience reports
2023

MRI and steroid-responsive encephalopathy associated with autoimmune thyroiditis: first report of conus medullaris involvement and literature review of the known neuroimaging profiles.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2022

Cerebellar Ataxia in the Setting of Hashimoto's Thyroiditis: A Case Report.

Cureus
2022

Autoimmune Encephalopathy Associated With Anti-thyroid Antibodies: A Case Report.

Cureus
2022

Hashimoto's encephalopathy with cerebellar ataxia as the main symptom: A case report and literature review.

Frontiers in neurology
2022

Treatable Ataxias: How to Find the Needle in the Haystack?

Journal of movement disorders
2022

Two PerPLEXing Cases of Hashimoto's Encephalopathy Unresponsive to Steroid and Intravenous Immunoglobulin Therapy.

Cureus
2022

Lingering Effects: Hashimoto's Encephalopathy.

Cureus
2022

Steroid-responsive encephalopathy associated with autoimmune thyroiditis presenting with a postpartum psychosis.

BMJ case reports
2022

Hashimoto's Encephalopathy Masquerading as Rapidly Progressive Dementia and Extrapyramidal Failure.

Journal of neurosciences in rural practice
2021

Amino acid sequence homology between thyroid autoantigens and central nervous system proteins: Implications for the steroid-responsive encephalopathy associated with autoimmune thyroiditis.

Journal of clinical &amp; translational endocrinology
2022

[Hashimoto's encephalopathy-An underdiagnosed disease in psychiatry?].

Neuropsychiatrie : Klinik, Diagnostik, Therapie und Rehabilitation : Organ der Gesellschaft Osterreichischer Nervenarzte und Psychiater
2021

A Case of Euthyroid Steroid-Responsive Encephalopathy With Subacute Dementia.

Cureus
2021

MRI Findings of Two Patients With Hashimoto Encephalopathy.

Cureus
2021

Brain dysfunction and thyroid antibodies: autoimmune diagnosis and misdiagnosis.

Brain communications
2021

Hashimoto's encephalopathy: A rare cause of refractory status epilepticus.

CNS neuroscience &amp; therapeutics
2021

Patients with a relapsing course of steroid-responsive encephalopathy associated with autoimmune thyroiditis exhibit persistent intrathecal CD4+ T-cell activation.

European journal of neurology
2021

Serum anti-thyroid antibody titers correlate with severity of illness in steroid-responsive encephalopathy associated with autoimmune thyroiditis.

The Australian and New Zealand journal of psychiatry
2020

Attempted Suicide in a Woman With Steroid-responsive Encephalopathy Associated With Autoimmune Thyroiditis: A Case Report.

Journal of psychiatric practice
2020

Hashimoto's Encephalopathy: Back to Square One.

Movement disorders clinical practice
2020

Anti-Thyroid Peroxidase/Anti-Thyroglobulin Antibody-Related Neurologic Disorder Responsive to Steroids Presenting with Pure Acute Onset Chorea.

Tremor and other hyperkinetic movements (New York, N.Y.)
2021

Echolalia: Presentation of Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis.

Indian journal of pediatrics
2020

Hashimoto's encephalopathy in association with retinitis pigmentosa - First reported case.

Journal of family medicine and primary care
2020

A young man with convulsive laughter.

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
2019

Hashimoto's encephalopathy: Follow-up data from neuropsychology, lumbar puncture, and FDG-PET.

Clinical case reports
2019

SREAT presenting as decades of intractable seizures and isolated delusional episodes with clinical, laboratory, and EEG confirmation of treatment response.

SAGE open medical case reports
2020

Relapsing encephalopathy with dancing eyes and jerky limbs.

Parkinsonism &amp; related disorders
2019

Psychotic Symptoms of Hashimoto's Encephalopathy: A Diagnostic Challenge.

Soa--ch'ongsonyon chongsin uihak = Journal of child &amp; adolescent psychiatry
2018

Current knowledge on Hashimoto's encephalopathy: a literature review.

Medwave
2018

Steroid-responsive encephalopathy with a peculiar CSF biomarker profile in an 89-year-old man.

Oxford medical case reports
2018

Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Presenting with Pure Cerebellar Ataxia.

Tremor and other hyperkinetic movements (New York, N.Y.)
2017

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Fever and Confusion.

Case reports in neurological medicine
2017

Cerebellopathy secondary to anti-peroxidase antibody-mediated toxicity. A special case of Hashimoto encephalopathy.

Journal of neuroimmunology
2018

Hashimoto encephalopathy in pediatric patients: Homogeneity in clinical presentation and heterogeneity in antibody titers.

Brain &amp; development
2017

Steroid-Responsive Chronic Schizophreniform Syndrome in the Context of Mildly Increased Antithyroid Peroxidase Antibodies.

Frontiers in psychiatry
2017

Nonconvulsive Status Epilepticus Resembling Clinical Absence with Atypical EEG Pattern.

Case reports in neurological medicine
2016

Prominent Bilateral Hand Tremor in Hashimoto's Encephalopathy: A Video Demonstration.

Tremor and other hyperkinetic movements (New York, N.Y.)
2016

Delirious Mania Associated with Autoimmune Gastrothyroidal Syndrome of a Mid-Life Female: The Role of Hashimoto Encephalopathy and a 3-Year Follow-Up including Serum Autoantibody Levels.

Case reports in psychiatry
2016

Autoimmune Schizophrenia? Psychiatric Manifestations of Hashimoto's Encephalitis.

Cureus
2016

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.

Autoimmunity reviews
2016

Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) presenting as major depression.

BMC psychiatry
2016

Autoimmune dementia and encephalopathy.

Handbook of clinical neurology
2015

Ocular flutter and ataxia without cognitive impairment associated with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT).

Journal of the neurological sciences
2015

Steroid responsive encephalopathy associated with autoimmune thyroiditis following ipilimumab therapy: a case report.

BMC research notes

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Steroid-responsive encephalopathy associated with autoimmune thyroiditis.
    BMJ case reports· 2025· PMID 40998544mais citado
  2. Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Authors' Reply.
    Indian journal of pediatrics· 2025· PMID 39976750mais citado
  3. Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Correspondence.
    Indian journal of pediatrics· 2025· PMID 39951036mais citado
  4. Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT).
    Indian journal of pediatrics· 2025· PMID 39821166mais citado
  5. Hashimoto's encephalopathy presenting with manic and psychotic features in a 13-year-old girl: a case report.
    Frontiers in psychiatry· 2025· PMID 41625620mais citado
  6. Super refractory status epilepticus as an atypical presentation of Hashimoto's encephalopathy.
    Epilepsy Behav Rep· 2026· PMID 41908686recente
  7. Primary Central Nervous System Lymphoma: A Case Report of the Neurological Great Mimicker.
    Cureus· 2025· PMID 41446477recente
  8. Hashimoto's Encephalopathy as a Cause of Persistent Myoclonic Jerks: A Report of a Unique Case.
    Cureus· 2025· PMID 41267728recente
  9. Hashimoto's encephalopathy in psychiatric inpatients: neuropsychiatric morbidity, diagnostic challenges and treatment.
    Front Psychiatry· 2025· PMID 41000339recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:83601(Orphanet)
  2. MONDO:0019385(MONDO)
  3. GARD:8570(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q4532132(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Encefalite sensível aos esteroides associada a tireoidite autoimune
Compêndio · Raras BR

Encefalite sensível aos esteroides associada a tireoidite autoimune

ORPHA:83601 · MONDO:0019385
Prevalência
<1 / 1 000 000
Herança
Not applicable
CID-10
G04.8 · Outras encefalites, mielites e encefalomielites
CID-11
Início
All ages
Prevalência
0.0 (Europe)
MedGen
UMLS
C0393639
Repurposing
5 candidatos
diftalonecyclooxygenase inhibitor
methylprednisoloneglucocorticoid receptor agonist
methylprednisolone-aceponateanti-inflammatory agent|glucocorticoid receptor agonist
+2 outros
EuropePMC
Wikidata
Wikipedia
Papers 10a
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