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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.
 
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

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
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
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

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
Pinedo-Torres I, Paz-Ibarra JL
Medwave 2018 Oct 22;18(6):e7298. doi: 10.5867/medwave.2018.06.7298. PMID: 30451215
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
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170
Castillo P, Woodruff B, Caselli R, Vernino S, Lucchinetti C, Swanson J, Noseworthy J, Aksamit A, Carter J, Sirven J, Hunder G, Fatourechi V, Mokri B, Drubach D, Pittock S, Lennon V, Boeve B
Arch Neurol 2006 Feb;63(2):197-202. doi: 10.1001/archneur.63.2.197. PMID: 16476807

Diagnosis

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
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
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
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
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Therapy

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
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
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
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
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Prognosis

Toft A, Meisingset TW, Sæther SG
Tidsskr Nor Laegeforen 2020 May 26;140(8) Epub 2020 May 25 doi: 10.4045/tidsskr.19.0612. PMID: 32463185
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
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
Bien CG, Vincent A
Handb Clin Neurol 2013;111:521-31. doi: 10.1016/B978-0-444-52891-9.00055-5. PMID: 23622200
Mocellin R, Walterfang M, Velakoulis D
CNS Drugs 2007;21(10):799-811. doi: 10.2165/00023210-200721100-00002. PMID: 17850170

Clinical prediction guides

Sharma B, Paul M, Bagaria AK
J Assoc Physicians India 2023 Sep;71(9):39-44. doi: 10.59556/japi.71.0312. PMID: 38700300
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
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

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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