SREAT
MCID: HSH004
MIFTS: 42

Hashimoto Encephalopathy (SREAT)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Hashimoto Encephalopathy

MalaCards integrated aliases for Hashimoto Encephalopathy:

Name: Hashimoto Encephalopathy 54
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis 54 60
Hashimoto's Encephalopathy 54
Hashimoto Encephalitis 60
Sreat 60

Characteristics:

Orphanet epidemiological data:

60
steroid-responsive encephalopathy associated with autoimmune thyroiditis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 60  
Rare neurological diseases


Summaries for Hashimoto Encephalopathy

NIH Rare Diseases : 54 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 hypothyroidism. An important gene associated with Hashimoto Encephalopathy is ENO1 (Enolase 1), and among its related pathways/superpathways is Thyroxine (Thyroid Hormone) Production. The drugs Epoetin alfa and Hematinics have been mentioned in the context of this disorder. Affiliated tissues include thyroid, testes and b cells, and related phenotypes are acute encephalopathy and hashimoto thyroiditis

Wikipedia : 77 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 94)
# Related Disease Score Top Affiliating Genes
1 thyroiditis 30.0 TPO TSHR
2 hypothyroidism 29.8 TPO TSHR
3 graves' disease 29.6 TPO TSHR
4 limbic encephalitis 29.6 ENO1 TPO
5 hyperthyroidism 29.5 TPO TSHR
6 encephalopathy 10.8
7 status epilepticus 10.2
8 ataxia and polyneuropathy, adult-onset 10.1
9 schizophrenia 10.1
10 dementia 10.1
11 epilepsy 10.1
12 vasculitis 10.1
13 encephalitis 10.1
14 myoclonus 10.1
15 tremor 10.1
16 tic disorder 10.0
17 dystonia 10.0
18 acute disseminated encephalomyelitis 10.0
19 creutzfeldt-jakob disease 9.9
20 multiple system atrophy 1 9.9
21 down syndrome 9.9
22 ocular motor apraxia 9.9
23 kearns-sayre syndrome 9.9
24 aceruloplasminemia 9.9
25 neuropathy, congenital hypomyelinating, 1, autosomal recessive 9.9
26 charcot-marie-tooth disease 9.9
27 amyotrophic neuralgia 9.9
28 meningoencephalitis 9.9
29 neurosarcoidosis 9.9
30 sensory peripheral neuropathy 9.9
31 myocarditis 9.9
32 neuropathy 9.9
33 progressive myoclonus epilepsy 9.9
34 narcolepsy 9.9
35 aids dementia complex 9.9
36 autoimmune encephalitis 9.9
37 lymphomatous thyroiditis 9.9
38 myoclonus epilepsy 9.9
39 seizure disorder 9.9
40 neonatal hypothyroidism 9.9 TPO TSHR
41 endemic goiter 9.9 TPO TSHR
42 goiter 9.9 TPO TSHR
43 graves disease 1 9.9 TPO TSHR
44 nontoxic goiter 9.9 TPO TSHR
45 myxedema 9.9 TPO TSHR
46 toxic diffuse goiter 9.9 TPO TSHR
47 hyperthyroxinemia 9.9 TPO TSHR
48 subacute thyroiditis 9.9 TPO TSHR
49 nodular goiter 9.8 TPO TSHR
50 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.8 TPO TSHR

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:

60 33 (show all 29)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 acute encephalopathy 60 33 obligate (100%) Obligate (100%) HP:0006846
2 hashimoto thyroiditis 60 33 hallmark (90%) Very frequent (99-80%) HP:0000872
3 cerebral vasculitis 60 33 hallmark (90%) Very frequent (99-80%) HP:0005318
4 hypothyroidism 60 33 frequent (33%) Frequent (79-30%) HP:0000821
5 goiter 60 33 frequent (33%) Frequent (79-30%) HP:0000853
6 paralysis 60 33 frequent (33%) Frequent (79-30%) HP:0003470
7 hyponatremia 60 33 frequent (33%) Frequent (79-30%) HP:0002902
8 confusion 60 33 frequent (33%) Frequent (79-30%) HP:0001289
9 abnormality of the cerebral white matter 60 33 frequent (33%) Frequent (79-30%) HP:0002500
10 depressivity 60 33 occasional (7.5%) Occasional (29-5%) HP:0000716
11 nausea and vomiting 60 33 occasional (7.5%) Occasional (29-5%) HP:0002017
12 fever 60 33 occasional (7.5%) Occasional (29-5%) HP:0001945
13 immunodeficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0002721
14 anxiety 60 33 occasional (7.5%) Occasional (29-5%) HP:0000739
15 thrombocytopenia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001873
16 psychosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000709
17 status epilepticus 60 33 occasional (7.5%) Occasional (29-5%) HP:0002133
18 leukocytosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001974
19 headache 60 33 occasional (7.5%) Occasional (29-5%) HP:0002315
20 myoclonus 33 occasional (7.5%) HP:0001336
21 abnormal autonomic nervous system physiology 33 occasional (7.5%) HP:0012332
22 focal-onset seizure 33 occasional (7.5%) HP:0007359
23 generalized-onset seizure 33 occasional (7.5%) HP:0002197
24 limited neck flexion 60 33 very rare (1%) Very rare (<4-1%) HP:0005991
25 dysautonomia 60 Occasional (29-5%)
26 generalized seizures 60 Occasional (29-5%)
27 focal seizures 60 Occasional (29-5%)
28 cerebral edema 60 Excluded (0%)
29 involuntary jerking movements 60 Occasional (29-5%)

Drugs & Therapeutics for Hashimoto Encephalopathy

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

(show top 50) (show all 51)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Epoetin alfa Phase 4 113427-24-0
2 Hematinics Phase 4
3
Aldesleukin Approved Phase 1, Phase 2 85898-30-2, 110942-02-4
4
rituximab Approved Phase 2 174722-31-7 10201696
5
Methylprednisolone hemisuccinate Approved Phase 2,Not Applicable 2921-57-5
6
Methylprednisolone Approved, Vet_approved Phase 2,Not Applicable 83-43-2 6741
7
Prednisolone phosphate Approved, Vet_approved Phase 2,Not Applicable 302-25-0
8
Prednisolone Approved, Vet_approved Phase 2,Not Applicable 50-24-8 5755
9
Ocrelizumab Approved, Investigational Phase 2 637334-45-3
10
Prednisolone hemisuccinate Experimental Phase 2,Not Applicable 2920-86-7
11 Antiviral Agents Phase 1, Phase 2
12 Anti-Infective Agents Phase 1, Phase 2
13 Anti-Retroviral Agents Phase 1, Phase 2
14 Anti-HIV Agents Phase 1, Phase 2
15 Immunologic Factors Phase 2
16 Antibodies Phase 2
17 Methylprednisolone Acetate Phase 2,Not Applicable
18 Antirheumatic Agents Phase 2
19 Antineoplastic Agents, Immunological Phase 2
20 gamma-Globulins Phase 2
21 Immunoglobulins, Intravenous Phase 2
22 Rho(D) Immune Globulin Phase 2
23 Immunoglobulins Phase 2
24 Prednisolone acetate Phase 2,Not Applicable
25 Pharmaceutical Solutions Phase 2
26 Autoantibodies Phase 2
27
Cyclophosphamide Approved, Investigational 6055-19-2, 50-18-0 2907
28
Heparin Approved, Investigational Not Applicable 9005-49-6 772 46507594
29
Vigabatrin Approved Not Applicable 68506-86-5, 60643-86-9 5665
30 N-Methylaspartate
31 Aspartic Acid
32 Peripheral Nervous System Agents ,Not Applicable
33 Anti-Inflammatory Agents ,Not Applicable
34 Antineoplastic Agents, Alkylating
35 Immunosuppressive Agents
36 Hormones ,Not Applicable
37 Autonomic Agents ,Not Applicable
38 Alkylating Agents
39 Antineoplastic Agents, Hormonal
40 Hormone Antagonists ,Not Applicable
41 Antiemetics ,Not Applicable
42 Neuroprotective Agents ,Not Applicable
43 Gastrointestinal Agents ,Not Applicable
44 Protective Agents ,Not Applicable
45 glucocorticoids ,Not Applicable
46 Hormones, Hormone Substitutes, and Hormone Antagonists ,Not Applicable
47 Anticoagulants Not Applicable
48 calcium heparin Not Applicable
49 Neurotransmitter Agents Not Applicable
50 Anticonvulsants Not Applicable

Interventional clinical trials:

(show all 15)
# 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 Efficacy of Ocrelizumab in Autoimmune Encephalitis Recruiting NCT03835728 Phase 2 Ocrelizumab;Saline
4 IVIG and Rituximab in Antibody-associated Psychosis - SINAPPS2 Recruiting NCT03194815 Phase 2 Intravenous immunoglobulin;Placebo;Rituximab
5 Multicenter and Prospective Clinical Registry Study of Autoimmune Encephalitis in China Unknown status NCT02561260
6 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in Beijing Area Unknown status NCT02443350
7 Multicenter and Prospective Clinical Registry Study of Anti-N-methyl-D-aspartate Receptor Encephalitis in China Unknown status NCT02559089
8 To Explore Cognitive Neural Mechanism of Autoimmune Encephalitis by Using Neuropsychological Tests and Multi-modal MRI Completed NCT03530462 rituximab;cyclophosphamide;Steroids;Intravenous immunoglobulin
9 Sodium Intake in Multiple Sclerosis Completed NCT01846234
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 Mechanisms of Auto-immune Encephalitis Active, not recruiting NCT02905136
14 Neuro-ophthalmology and Autoimmune Encephalitis (NODE) Not yet recruiting NCT03872284
15 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:

42
Thyroid, Testes, B Cells, Brain

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
Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies: A clinical subtype of Hashimoto encephalopathy. ( 28272206 )
2017
7
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Fever and Confusion. ( 29234548 )
2017
8
Cerebellopathy secondary to anti-peroxidase antibody-mediated toxicity. A special case of Hashimoto encephalopathy. ( 28863961 )
2017
9
Improved 123I-Ioflupane Binding After Immunotherapy in Anti-NAE Antibody-Positive Hashimoto Encephalopathy That Clinically Mimicked Multiple System Atrophy. ( 28632692 )
2017
10
Hashimoto encephalopathy with high plasma monoamine metabolite levels: a case report. ( 28435274 )
2017
11
Treatment of Steroid-Resistant Hashimoto Encephalopathy With Misidentification Delusions and Catatonia. ( 28190544 )
2017
12
Risks and Benefits of Rituximab in the Treatment of Hashimoto Encephalopathy in Children: Two Case Reports and a Mini Review. ( 27789116 )
2017
13
Hashimoto encephalopathy: literature review. ( 27324276 )
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
Hashimoto Encephalopathy with an Unusual Presentation of Status Epilepticus Seizures: A Case Report. ( 27957468 )
2016
18
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
19
Hashimoto Encephalopathy Presenting With Stroke-Like Episodes in an Adolescent Female: A Case Report and Literature Review. ( 27033176 )
2016
20
Hashimoto Encephalopathy: A Case Report. ( 27154779 )
2016
21
Clinical spectrum of Hashimoto encephalopathy: report of 5 cases. ( 26243438 )
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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