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Steroid-responsive encephalopathy associated with autoimmune thyroiditis

MedGen UID:
98280
•Concept ID:
C0393639
•
Disease or Syndrome
Synonyms: Hashimoto's encephalitis; Hashimoto's encephalopathy
SNOMED CT: Autoimmune encephalitis (95643007); Hashimoto encephalitis (771271000); Steroid-responsive encephalopathy associated with autoimmune thyroiditis (771271000); SREAT - steroid-responsive encephalopathy associated with autoimmune thyroiditis (771271000); Autoimmune encephalopathy (95643007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019385
Orphanet: ORPHA83601

Definition

A rare acquired neurological disease with characteristics of 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. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSteroid-responsive encephalopathy associated with autoimmune thyroiditis

Professional guidelines

PubMed

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.
Laurent C, Capron J, Quillerou B, Thomas G, Alamowitch S, Fain O, Mekinian A
Autoimmun Rev 2016 Dec;15(12):1129-1133. Epub 2016 Sep 15 doi: 10.1016/j.autrev.2016.09.008. PMID: 27639840
Myorhythmia: phenomenology, etiology, and treatment.
Baizabal-Carvallo JF, Cardoso F, Jankovic J
Mov Disord 2015 Feb;30(2):171-9. Epub 2014 Dec 9 doi: 10.1002/mds.26093. PMID: 25487777
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Recent clinical studies

Etiology

Steroid responsive encephalopathy associated with autoimmune thyroiditis as a cause of acuteencephalopathy.
Maretta M, Lacková A, Wagnerová H, Feketeová E, Gdovinová Z
Vnitr Lek 2023 Summer;69(E-5):25-29. doi: 10.36290/vnl.2023.068. PMID: 37827820
Current knowledge on Hashimoto's encephalopathy: a literature review.
Pinedo-Torres I, Paz-Ibarra JL
Medwave 2018 Oct 22;18(6):e7298. doi: 10.5867/medwave.2018.06.7298. PMID: 30451215
Autoimmune dementia and encephalopathy.
Flanagan EP, Drubach DA, Boeve BF
Handb Clin Neurol 2016;133:247-67. doi: 10.1016/B978-0-444-63432-0.00014-1. PMID: 27112681
Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or Hashimoto's encephalopathy: a case and review.
Lee SW, Donlon S, Caplan JP
Psychosomatics 2011 Mar-Apr;52(2):99-108. doi: 10.1016/j.psym.2010.12.010. PMID: 21397101
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Diagnosis

Hashimoto's Encephalopathy: Case Series and Literature Review.
Chaudhuri J, Mukherjee A, Chakravarty A
Curr Neurol Neurosci Rep 2023 Apr;23(4):167-175. Epub 2023 Feb 28 doi: 10.1007/s11910-023-01255-5. PMID: 36853554Free PMC Article
MRI and steroid-responsive encephalopathy associated with autoimmune thyroiditis: first report of conus medullaris involvement and literature review of the known neuroimaging profiles.
Cipriano L, Miele G, Ugga L, Abbadessa G, Caranci F, Bonavita S
Neurol Sci 2023 May;44(5):1773-1776. Epub 2023 Feb 21 doi: 10.1007/s10072-023-06642-x. PMID: 36809420
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.
Laurent C, Capron J, Quillerou B, Thomas G, Alamowitch S, Fain O, Mekinian A
Autoimmun Rev 2016 Dec;15(12):1129-1133. Epub 2016 Sep 15 doi: 10.1016/j.autrev.2016.09.008. PMID: 27639840
Immune-mediated pediatric epilepsies.
Bien CG, Vincent A
Handb Clin Neurol 2013;111:521-31. doi: 10.1016/B978-0-444-52891-9.00055-5. PMID: 23622200
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Therapy

Steroid responsive encephalopathy associated with autoimmune thyroiditis as a cause of acuteencephalopathy.
Maretta M, Lacková A, Wagnerová H, Feketeová E, Gdovinová Z
Vnitr Lek 2023 Summer;69(E-5):25-29. doi: 10.36290/vnl.2023.068. PMID: 37827820
Hashimoto's Encephalopathy: Case Series and Literature Review.
Chaudhuri J, Mukherjee A, Chakravarty A
Curr Neurol Neurosci Rep 2023 Apr;23(4):167-175. Epub 2023 Feb 28 doi: 10.1007/s11910-023-01255-5. PMID: 36853554Free PMC Article
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.
Laurent C, Capron J, Quillerou B, Thomas G, Alamowitch S, Fain O, Mekinian A
Autoimmun Rev 2016 Dec;15(12):1129-1133. Epub 2016 Sep 15 doi: 10.1016/j.autrev.2016.09.008. PMID: 27639840
Steroid-responsive depression.
Normann C, Frase L, Berger M, Nissen C
BMJ Case Rep 2013 Apr 18;2013 doi: 10.1136/bcr-2013-009101. PMID: 23605832Free PMC Article
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Prognosis

Autoimmune dementia and encephalopathy.
Flanagan EP, Drubach DA, Boeve BF
Handb Clin Neurol 2016;133:247-67. doi: 10.1016/B978-0-444-63432-0.00014-1. PMID: 27112681
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): case report of reversible coma and status epilepticus in an adolescent patient and review of the literature.
Hilberath JM, Schmidt H, Wolf GK
Eur J Pediatr 2014 Oct;173(10):1263-73. Epub 2014 Aug 2 doi: 10.1007/s00431-014-2391-6. PMID: 25084973
Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood.
Patnaik SK, Upreti V, Dhull P
J Pediatr Endocrinol Metab 2014 Jul;27(7-8):737-44. doi: 10.1515/jpem-2013-0435. PMID: 24598831
Immune-mediated pediatric epilepsies.
Bien CG, Vincent A
Handb Clin Neurol 2013;111:521-31. doi: 10.1016/B978-0-444-52891-9.00055-5. PMID: 23622200
Hashimoto's encephalopathy : epidemiology, pathogenesis and management.
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Clinical prediction guides

Patients with a relapsing course of steroid-responsive encephalopathy associated with autoimmune thyroiditis exhibit persistent intrathecal CD4+ T-cell activation.
Pfeuffer S, Ruck T, Rolfes L, Pawlowski M, Pawlitzki M, Wiendl H, Kovac S, Meuth SG
Eur J Neurol 2021 Apr;28(4):1284-1291. Epub 2020 Dec 17 doi: 10.1111/ene.14657. PMID: 33230897
Immune-mediated pediatric epilepsies.
Bien CG, Vincent A
Handb Clin Neurol 2013;111:521-31. doi: 10.1016/B978-0-444-52891-9.00055-5. PMID: 23622200

Recent systematic reviews

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.
Laurent C, Capron J, Quillerou B, Thomas G, Alamowitch S, Fain O, Mekinian A
Autoimmun Rev 2016 Dec;15(12):1129-1133. Epub 2016 Sep 15 doi: 10.1016/j.autrev.2016.09.008. PMID: 27639840

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