SREAT
MCID: HSH004
MIFTS: 36

Hashimoto Encephalopathy (SREAT)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Hashimoto Encephalopathy

MalaCards integrated aliases for Hashimoto Encephalopathy:

Name: Hashimoto Encephalopathy 53
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis 53 59
Hashimoto's Encephalopathy 53
Hashimoto Encephalitis 59
Sreat 59

Characteristics:

Orphanet epidemiological data:

59
steroid-responsive encephalopathy associated with autoimmune thyroiditis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare neurological diseases


Summaries for Hashimoto Encephalopathy

NIH Rare Diseases : 53 Hashimoto encephalopathy (HE) is a condition characterized by onset of confusion with altered level of consciousness; seizures; and jerking of muscles (myoclonus). Psychosis, including visual hallucinations and paranoid delusions, has also been reported. The exact cause of HE is not known, but may involve an autoimmune or inflammatory abnormality. It is associated with Hashimoto's thyroiditis, but the nature of the relationship between the two conditions is unclear. Most people with HE respond well to corticosteroid therapy or other immunosuppressive therapies, and symptoms typically improve or resolve over a few months.

MalaCards based summary : Hashimoto Encephalopathy, also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis, is related to thyroiditis and limbic encephalitis. An important gene associated with Hashimoto Encephalopathy is ENO1 (Enolase 1), and among its related pathways/superpathways is Thyroxine (Thyroid Hormone) Production. The drugs Hematinics and Epoetin alfa have been mentioned in the context of this disorder. Affiliated tissues include thyroid, brain and testes, and related phenotypes are depressivity and hypothyroidism

Wikipedia : 76 Hashimoto''s encephalopathy, also known as steroid responsive encephalopathy associated with autoimmune... more...

Related Diseases for Hashimoto Encephalopathy

Diseases related to Hashimoto Encephalopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 59)
# Related Disease Score Top Affiliating Genes
1 thyroiditis 30.0 TPO TSHR
2 limbic encephalitis 29.5 ENO1 TPO
3 hyperthyroidism 29.5 TPO TSHR
4 graves' disease 29.5 TPO TSHR
5 hashimoto thyroiditis 29.4 TPO TSHR
6 encephalopathy 10.7
7 depression 10.3
8 ataxia and polyneuropathy, adult-onset 10.1
9 status epilepticus 10.1
10 schizophrenia 10.0
11 dementia 10.0
12 epilepsy 10.0
13 vasculitis 10.0
14 encephalitis 10.0
15 myoclonus 10.0
16 tic disorder 10.0
17 dystonia 10.0
18 neonatal hypothyroidism 9.9 TPO TSHR
19 hypothyroidism 9.9 TPO TSHR
20 endemic goiter 9.9 TPO TSHR
21 goiter 9.9 TPO TSHR
22 graves disease 1 9.9 TPO TSHR
23 nontoxic goiter 9.9 TPO TSHR
24 myxedema 9.9 TPO TSHR
25 toxic diffuse goiter 9.9 TPO TSHR
26 hyperthyroxinemia 9.9 TPO TSHR
27 subacute thyroiditis 9.9 TPO TSHR
28 nodular goiter 9.9 TPO TSHR
29 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9 TPO TSHR
30 autoimmune disease of endocrine system 9.9 TPO TSHR
31 multinodular goiter 9.9 TPO TSHR
32 thyroid gland disease 9.9 TPO TSHR
33 follicular adenoma 9.9 TPO TSHR
34 papillary carcinoma 9.9 TPO TSHR
35 immune system disease 9.9 TPO TSHR
36 autoimmune disease 9.9 ENO1 TPO
37 differentiated thyroid carcinoma 9.9 TPO TSHR
38 congenital hypothyroidism 9.8 TPO TSHR
39 creutzfeldt-jakob disease 9.8
40 multiple system atrophy 1 9.8
41 down syndrome 9.8
42 kearns-sayre syndrome 9.8
43 aceruloplasminemia 9.8
44 neuropathy, congenital hypomyelinating or amyelinating, autosomal recessive 9.8
45 charcot-marie-tooth disease 9.8
46 amyotrophic neuralgia 9.8
47 meningoencephalitis 9.8
48 sensory peripheral neuropathy 9.8
49 myocarditis 9.8
50 neuropathy 9.8

Graphical network of the top 20 diseases related to Hashimoto Encephalopathy:



Diseases related to Hashimoto Encephalopathy

Symptoms & Phenotypes for Hashimoto Encephalopathy

Human phenotypes related to Hashimoto Encephalopathy:

59 32 (show all 29)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 depressivity 59 32 occasional (7.5%) Occasional (29-5%) HP:0000716
2 hypothyroidism 59 32 frequent (33%) Frequent (79-30%) HP:0000821
3 nausea and vomiting 59 32 occasional (7.5%) Occasional (29-5%) HP:0002017
4 fever 59 32 occasional (7.5%) Occasional (29-5%) HP:0001945
5 immunodeficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0002721
6 anxiety 59 32 occasional (7.5%) Occasional (29-5%) HP:0000739
7 hashimoto thyroiditis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000872
8 thrombocytopenia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001873
9 goiter 59 32 frequent (33%) Frequent (79-30%) HP:0000853
10 psychosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000709
11 status epilepticus 59 32 occasional (7.5%) Occasional (29-5%) HP:0002133
12 paralysis 59 32 frequent (33%) Frequent (79-30%) HP:0003470
13 leukocytosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001974
14 hyponatremia 59 32 frequent (33%) Frequent (79-30%) HP:0002902
15 headache 59 32 occasional (7.5%) Occasional (29-5%) HP:0002315
16 confusion 59 32 frequent (33%) Frequent (79-30%) HP:0001289
17 cerebral vasculitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0005318
18 abnormality of the cerebral white matter 59 32 frequent (33%) Frequent (79-30%) HP:0002500
19 acute encephalopathy 59 32 obligate (100%) Obligate (100%) HP:0006846
20 limited neck flexion 59 32 very rare (1%) Very rare (<4-1%) HP:0005991
21 dysautonomia 59 Occasional (29-5%)
22 myoclonus 32 occasional (7.5%) HP:0001336
23 generalized seizures 59 Occasional (29-5%)
24 focal seizures 59 Occasional (29-5%)
25 cerebral edema 59 Excluded (0%)
26 involuntary jerking movements 59 Occasional (29-5%)
27 abnormal autonomic nervous system physiology 32 occasional (7.5%) HP:0012332
28 focal-onset seizure 32 occasional (7.5%) HP:0007359
29 generalized-onset seizure 32 occasional (7.5%) HP:0002197

Drugs & Therapeutics for Hashimoto Encephalopathy

Drugs for Hashimoto Encephalopathy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 52)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Hematinics Phase 4
2 Epoetin alfa Phase 4 113427-24-0
3
Aldesleukin Approved Phase 1, Phase 2 85898-30-2, 110942-02-4
4
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
5
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
6
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
7
rituximab Approved Phase 2 174722-31-7 10201696
8
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
9
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
10 Antiviral Agents Phase 1, Phase 2
11 Anti-Retroviral Agents Phase 1, Phase 2
12 Anti-HIV Agents Phase 1, Phase 2
13 Anti-Infective Agents Phase 1, Phase 2
14 Methylprednisolone acetate Phase 2
15 Rho(D) Immune Globulin Phase 2
16 Antibodies Phase 2,Not Applicable
17 Prednisolone acetate Phase 2
18 Immunologic Factors Phase 2,Not Applicable
19 gamma-Globulins Phase 2
20 Immunoglobulins Phase 2,Not Applicable
21 Immunoglobulins, Intravenous Phase 2
22 Antirheumatic Agents Phase 2
23 Pharmaceutical Solutions Phase 2
24 Autoantibodies Phase 2
25
Cyclophosphamide Approved, Investigational 6055-19-2, 50-18-0 2907
26
Heparin Approved, Investigational Not Applicable 9005-49-6 772 46507594
27
Dexamethasone acetate Approved, Investigational, Vet_approved Not Applicable 1177-87-3
28
Dexamethasone Approved, Investigational, Vet_approved Not Applicable 50-02-2 5743
29
Vigabatrin Approved Not Applicable 60643-86-9, 68506-86-5 5665
30 Aspartic Acid
31 N-Methylaspartate
32 Antineoplastic Agents, Hormonal
33 Alkylating Agents
34 Hormones, Hormone Substitutes, and Hormone Antagonists ,Not Applicable
35 Antiemetics
36 Anti-Inflammatory Agents
37 Protective Agents
38 Neuroprotective Agents
39 Immunosuppressive Agents
40 Peripheral Nervous System Agents
41 Hormone Antagonists ,Not Applicable
42 glucocorticoids ,Not Applicable
43 Gastrointestinal Agents
44 Hormones ,Not Applicable
45 Antineoplastic Agents, Alkylating
46 Autonomic Agents
47 calcium heparin Not Applicable
48 Anticoagulants Not Applicable
49 BB 1101 Not Applicable
50 Neurotransmitter Agents Not Applicable

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 Effect of Erythropoietin in Refractory Autoimmune Encephalitis Patients Unknown status NCT03004209 Phase 4 Erythropoietin
2 IL-2 in Refractory Autoimmune Encephalitis Unknown status NCT02714959 Phase 1, Phase 2 Proleukin
3 IVIG and Rituximab in Antibody-associated Psychosis - SINAPPS2 Recruiting NCT03194815 Phase 2 Intravenous immunoglobulin;Placebo;Rituximab
4 Multicenter and Prospective Clinical Registry Study of Autoimmune Encephalitis in China Unknown status NCT02561260
5 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in Beijing Area Unknown status NCT02443350
6 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in China Unknown status NCT02559089
7 To Explore Cognitive Neural Mechanism of Autoimmune Encephalitis by Using Neuropsychological Tests and Multi-modal MRI Completed NCT03530462 rituximab;cyclophosphamide;Steroids;Intravenous immunoglobulin
8 Sodium Intake in Multiple Sclerosis Completed NCT01846234
9 Mechanisms of Auto-immune Encephalitis Recruiting NCT02905136
10 Immunotherapy in Autoimmune Encephalitis Recruiting NCT03542279 Not Applicable IVIG, High-dose glucocorticoid
11 Effect of Vigabatrin in Refractory Autoimmune Encephalitis Patients Recruiting NCT03003143 Not Applicable Vigabatrin 500 MG
12 Antibodies Causing Epilepsy Syndromes: The ACES Study. Recruiting NCT02802475
13 Phenotypic and Functional Study of 4BL B Cells in Multiple Sclerosis (MS) Not yet recruiting NCT03796611

Search NIH Clinical Center for Hashimoto Encephalopathy

Genetic Tests for Hashimoto Encephalopathy

Anatomical Context for Hashimoto Encephalopathy

MalaCards organs/tissues related to Hashimoto Encephalopathy:

41
Thyroid, Brain, Testes, T Cells

Publications for Hashimoto Encephalopathy

Articles related to Hashimoto Encephalopathy:

(show top 50) (show all 93)
# Title Authors Year
1
Hashimoto Encephalopathy Mimicking Acute Ischemic Stroke: Perfusion-Weighted Magnetic Resonance Imaging. ( 30284769 )
2018
2
Hashimoto Encephalopathy as a Complication of Autoimmune Thyroiditis. ( 30355921 )
2018
3
Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Presenting with Pure Cerebellar Ataxia. ( 30191089 )
2018
4
Hashimoto Encephalopathy. ( 30263866 )
2018
5
Hashimoto encephalopathy in pediatric patients: Homogeneity in clinical presentation and heterogeneity in antibody titers. ( 28784301 )
2018
6
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Fever and Confusion. ( 29234548 )
2017
7
Hashimoto encephalopathy: literature review. ( 27324276 )
2017
8
Risks and Benefits of Rituximab in the Treatment of Hashimoto Encephalopathy in Children: Two Case Reports and a Mini Review. ( 27789116 )
2017
9
Treatment of Steroid-Resistant Hashimoto Encephalopathy With Misidentification Delusions and Catatonia. ( 28190544 )
2017
10
Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies: A clinical subtype of Hashimoto encephalopathy. ( 28272206 )
2017
11
Hashimoto encephalopathy with high plasma monoamine metabolite levels: a case report. ( 28435274 )
2017
12
Improved 123I-Ioflupane Binding After Immunotherapy in Anti-NAE Antibody-Positive Hashimoto Encephalopathy That Clinically Mimicked Multiple System Atrophy. ( 28632692 )
2017
13
Cerebellopathy secondary to anti-peroxidase antibody-mediated toxicity. A special case of Hashimoto encephalopathy. ( 28863961 )
2017
14
Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) presenting as major depression. ( 27268005 )
2016
15
Hashimoto encephalopathy associated rapid onset narcolepsy type 1. ( 27908588 )
2016
16
Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature. ( 27639840 )
2016
17
Clinical spectrum of Hashimoto encephalopathy: report of 5 cases. ( 26243438 )
2016
18
Hashimoto Encephalopathy Presenting With Stroke-Like Episodes in an Adolescent Female: A Case Report and Literature Review. ( 27033176 )
2016
19
Hashimoto Encephalopathy: A Case Report. ( 27154779 )
2016
20
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. ( 27688922 )
2016
21
Hashimoto Encephalopathy with an Unusual Presentation of Status Epilepticus Seizures: A Case Report. ( 27957468 )
2016
22
Hashimoto encephalopathy or neurosarcoidosis? A case report. ( 25829987 )
2015
23
Steroid responsive encephalopathy associated with autoimmune thyroiditis following ipilimumab therapy: a case report. ( 26209970 )
2015
24
Ocular flutter and ataxia without cognitive impairment associated with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT). ( 26671092 )
2015
25
Faciobrachial dystonic seizures associated with Hashimoto encephalopathy. ( 26060928 )
2015
26
Hashimoto encephalopathy: A study of the clinical profile, radiological and electrophysiological correlation in a Tertiary Care Center in South India. ( 26167010 )
2015
27
Hashimoto encephalopathy with angiographic CNS vasculitis. ( 29443143 )
2014
28
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. ( 25084973 )
2014
29
Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood. ( 24598831 )
2014
30
Clinical manifestations and treatment response of steroid in pediatric Hashimoto encephalopathy. ( 24056154 )
2014
31
Hashimoto encephalopathy associated with hyperthyroidism: A case report. ( 25009611 )
2014
32
Hashimoto Encephalopathy in Case of Progressive Cognitive Impairment; a Case Report. ( 26495368 )
2014
33
Hashimoto encephalopathy presenting as progressive myoclonus epilepsy syndrome. ( 22840275 )
2013
34
The misdiagnosis of steroid-responsive encephalopathy associated with autoimmune thyroiditis as masked depression in an elderly euthyroid woman. ( 23453127 )
2013
35
Spectroscopic correlation and role of Azathioprine in long-term remission in patients of Hashimoto encephalopathy. ( 24101841 )
2013
36
Palatal-Myoclonus as a Presentation of Hashimoto Encephalopathy: an interesting case report. ( 24454425 )
2013
37
Steroid-responsive encephalopathy associated with autoimmune thyroiditis presenting as confusion, dysphasia, and myoclonus. ( 22754574 )
2012
38
A patient with steroid responsive encephalopathy associated with autoimmune thyroiditis. ( 22884222 )
2012
39
Hashimoto encephalopathy causing drug-resistant status epilepticus treated with plasmapheresis. ( 22264710 )
2012
40
99mTc ECD and 99mTc TRODAT-1 SPECT in Hashimoto encephalopathy. ( 22391731 )
2012
41
Paroxysmal kinesigenic dyskinesia as the initial symptom of Hashimoto encephalopathy. ( 22449112 )
2012
42
Psychotic episode during steroid therapy in hashimoto encephalopathy. ( 22450647 )
2012
43
Hashimoto encephalopathy diagnosis after 40 years of a schizophrenia-like disorder. ( 22520854 )
2012
44
Pearls & oy-sters: Hashimoto encephalopathy. ( 22641407 )
2012
45
The relevance of electroencephalogram as a follow-up test in Hashimoto encephalopathy course after corticosteroids therapy. ( 22665074 )
2012
46
Hashimoto encephalopathy: a rare intricate syndrome. ( 23843812 )
2012
47
Recurrent partial seizures with ictal yawning as atypical presentation of Hashimoto's encephalopathy (steroid-responsive encephalopathy associated with autoimmune thyroiditis). ( 22030535 )
2011
48
Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or Hashimoto's encephalopathy: a case and review. ( 21397101 )
2011
49
Steroid-responsive Hashimoto encephalopathy mimicking Creutzfeldt-Jakob disease. ( 21556866 )
2011
50
Progressive impairment of cognition and motor function: Hashimoto encephalopathy. ( 21324872 )
2011

Variations for Hashimoto Encephalopathy

Expression for Hashimoto Encephalopathy

Search GEO for disease gene expression data for Hashimoto Encephalopathy.

Pathways for Hashimoto Encephalopathy

Pathways related to Hashimoto Encephalopathy according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 9.4 TPO TSHR

GO Terms for Hashimoto Encephalopathy

Cellular components related to Hashimoto Encephalopathy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 8.8 ENO1 TPO TSHR

Sources for Hashimoto Encephalopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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