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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: Hashimoto encephalitis (771271000); Steroid-responsive encephalopathy associated with autoimmune thyroiditis (771271000); SREAT - steroid-responsive encephalopathy associated with autoimmune thyroiditis (771271000)
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

Pempera N, Miedziaszczyk M, Lacka K
Int J Mol Sci 2024 Jun 28;25(13) doi: 10.3390/ijms25137101. PMID: 39000209Free PMC Article
Sharma B, Paul M, Bagaria AK
J Assoc Physicians India 2023 Sep;71(9):39-44. doi: 10.59556/japi.71.0312. PMID: 38700300
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

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
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
Bien CG, Vincent A
Handb Clin Neurol 2013;111:521-31. doi: 10.1016/B978-0-444-52891-9.00055-5. PMID: 23622200
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

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
Carl D, Grüllich C, Hering S, Schabet M
BMC Res Notes 2015 Jul 26;8:316. doi: 10.1186/s13104-015-1283-9. PMID: 26209970Free PMC Article
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

Runge K, Rauer S, Waibel E, Nickel K, Brumberg J, Meyer PT, Urbach H, Prüss H, Domschke K, Endres D, Tebartz van Elst L
J Neuroimmunol 2023 Sep 15;382:578177. Epub 2023 Aug 9 doi: 10.1016/j.jneuroim.2023.578177. PMID: 37579547
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
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

Pempera N, Miedziaszczyk M, Lacka K
Int J Mol Sci 2024 Jun 28;25(13) doi: 10.3390/ijms25137101. PMID: 39000209Free 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

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