SREAT
MCID: HSH004
MIFTS: 29

Hashimoto Encephalopathy (SREAT)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Hashimoto Encephalopathy

MalaCards integrated aliases for Hashimoto Encephalopathy:

Name: Hashimoto Encephalopathy 20
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis 20 58
Hashimoto's Encephalopathy 20
Hashimoto Encephalitis 58
Sreat 58

Characteristics:

Orphanet epidemiological data:

58
steroid-responsive encephalopathy associated with autoimmune thyroiditis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare neurological diseases


Summaries for Hashimoto Encephalopathy

GARD : 20 Hashimoto encephalopathy (HE) affects the brain and how the brain works. Symptoms of HE may include behavior changes, confusion, cognitive difficulty, and seizures. Psychosis, including visual hallucinations and paranoid delusions, has also been reported. HE occurs mainly in adults and affects females more than males. The exact cause of HE is not known but may involve an abnormal immune or inflammatory response. HE is associated with Hashimoto's thyroiditis, but the nature of the relationship between the two conditions is unclear. Diagnosis of HE is based on the symptoms, clinical exam, and laboratory testing. More common conditions may need to be excluded first. Treatment is focused on managing the symptoms. Most people with HE respond well to corticosteroid therapy or other immunosuppressive therapies. With treatment, 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 encephalopathy and thyroiditis. An important gene associated with Hashimoto Encephalopathy is ENO1 (Enolase 1). Affiliated tissues include thyroid, brain and temporal lobe, and related phenotypes are acute encephalopathy and hashimoto thyroiditis

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 168)
# Related Disease Score Top Affiliating Genes
1 encephalopathy 10.9
2 thyroiditis 10.6
3 hashimoto thyroiditis 10.6
4 hypothyroidism 10.4
5 vasculitis 10.4
6 myoclonus 10.4
7 tremor 10.4
8 ocular motor apraxia 10.3
9 status epilepticus 10.3
10 ataxia and polyneuropathy, adult-onset 10.3
11 myxedema 10.3
12 goiter 10.2
13 nervous system disease 10.2
14 specific language disorder 10.2
15 seizure disorder 10.2
16 pathologic nystagmus 10.2
17 late-onset retinal degeneration 10.1
18 spinocerebellar degeneration 10.1
19 limbic encephalitis 10.1
20 parkinsonism 10.1
21 encephalitis 10.1
22 autoimmune encephalitis 10.1
23 amnestic disorder 10.1
24 thyroid gland disease 10.1
25 speech disorder 10.1
26 dysphagia 10.1
27 autoimmune disease 10.0
28 creutzfeldt-jakob disease 10.0
29 aphasia 10.0
30 meningoencephalitis 10.0
31 graves' disease 10.0
32 dementia 10.0
33 epilepsy 10.0
34 dystonia 10.0
35 hyperthyroidism 10.0
36 subacute delirium 10.0
37 major affective disorder 1 10.0
38 chorea, childhood-onset, with psychomotor retardation 10.0
39 agraphia 10.0
40 choreatic disease 10.0
41 motor neuron disease 10.0
42 dysgraphia 10.0
43 acute disseminated encephalomyelitis 10.0
44 viral encephalitis 10.0
45 thyroid carcinoma 10.0
46 mental depression 9.9
47 episodic kinesigenic dyskinesia 1 9.9
48 multiple system atrophy 1 9.9
49 progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 1 9.9
50 pernicious anemia 9.9

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:

58 31 (show all 27)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 acute encephalopathy 58 31 obligate (100%) Obligate (100%) HP:0006846
2 hashimoto thyroiditis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000872
3 cerebral vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0005318
4 hypothyroidism 58 31 frequent (33%) Frequent (79-30%) HP:0000821
5 hyponatremia 58 31 frequent (33%) Frequent (79-30%) HP:0002902
6 goiter 58 31 frequent (33%) Frequent (79-30%) HP:0000853
7 abnormality of the cerebral white matter 58 31 frequent (33%) Frequent (79-30%) HP:0002500
8 confusion 58 31 frequent (33%) Frequent (79-30%) HP:0001289
9 paralysis 58 31 frequent (33%) Frequent (79-30%) HP:0003470
10 nausea and vomiting 58 31 occasional (7.5%) Occasional (29-5%) HP:0002017
11 depressivity 58 31 occasional (7.5%) Occasional (29-5%) HP:0000716
12 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
13 immunodeficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0002721
14 anxiety 58 31 occasional (7.5%) Occasional (29-5%) HP:0000739
15 thrombocytopenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001873
16 headache 58 31 occasional (7.5%) Occasional (29-5%) HP:0002315
17 psychosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000709
18 status epilepticus 58 31 occasional (7.5%) Occasional (29-5%) HP:0002133
19 leukocytosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001974
20 focal-onset seizure 58 31 occasional (7.5%) Occasional (29-5%) HP:0007359
21 generalized-onset seizure 58 31 occasional (7.5%) Occasional (29-5%) HP:0002197
22 myoclonus 31 occasional (7.5%) HP:0001336
23 abnormal autonomic nervous system physiology 31 occasional (7.5%) HP:0012332
24 limited neck flexion 58 31 very rare (1%) Very rare (<4-1%) HP:0005991
25 dysautonomia 58 Occasional (29-5%)
26 involuntary jerking movements 58 Occasional (29-5%)
27 cerebral edema 58 Excluded (0%)

Drugs & Therapeutics for Hashimoto Encephalopathy

Search Clinical Trials , NIH Clinical Center for Hashimoto Encephalopathy

Genetic Tests for Hashimoto Encephalopathy

Anatomical Context for Hashimoto Encephalopathy

MalaCards organs/tissues related to Hashimoto Encephalopathy:

40
Thyroid, Brain, Temporal Lobe

Publications for Hashimoto Encephalopathy

Articles related to Hashimoto Encephalopathy:

(show top 50) (show all 179)
# Title Authors PMID Year
1
Patients with a relapsing course of steroid-responsive encephalopathy associated with autoimmune thyroiditis exhibit persistent intrathecal CD4+ T-cell activation. 61
33230897 2021
2
Continuous EEG Findings in Autoimmune Encephalitis. 61
31800465 2021
3
Echolalia: Presentation of Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis. 61
32607670 2021
4
Hashimoto Encephalopathy With Status Epilepticus. 61
33409091 2020
5
Reader response: Hashimoto encephalopathy in the 21st century. 61
33288668 2020
6
Author response: Hashimoto encephalopathy in the 21st century. 61
33288669 2020
7
Gerstmann's Syndrome in a Patient Double-positive for Antibodies against the N-methyl-D-aspartate Receptor and NH2-terminal of α-enolase. 61
33229813 2020
8
Serum anti-thyroid antibody titers correlate with severity of illness in steroid-responsive encephalopathy associated with autoimmune thyroiditis. 61
33174434 2020
9
Successful treatment of Hashimoto encephalopathy with therapeutic plasma exchange. 61
31863616 2020
10
Attempted Suicide in a Woman With Steroid-responsive Encephalopathy Associated With Autoimmune Thyroiditis: A Case Report. 61
32936588 2020
11
Autoantibody-associated psychiatric syndromes: a systematic literature review resulting in 145 cases. 61
32892761 2020
12
Hashimoto encephalopathy: a case report and a short revision of current literature. 61
32921783 2020
13
Immune Activation and Deficit in Neurotransmitters Synthesis in Treatment Resistant Depression: About a Case of Hashimoto Encephalopathy. 61
32702227 2020
14
Anti-Thyroid Peroxidase and Anti-Thyroglobulin Autoantibodies in the Cerebrospinal Fluid of Patients with Unipolar Depression. 61
32726952 2020
15
Malignant Catatonia as the Presenting Manifestation of Systemic Lupus Erythematosus. 61
32510302 2020
16
Myxedema psychosis: A protocol for a systematic review and a pooled analysis. 61
32590756 2020
17
Evaluation of Diagnostic Criteria for Hashimoto Encephalopathy Among Children and Adolescents. 61
32173161 2020
18
Pediatric autoimmune encephalitis: Recognition and diagnosis. 61
32047077 2020
19
A young man with convulsive laughter. 61
32463185 2020
20
Hashimoto's encephalopathy in association with retinitis pigmentosa - First reported case. 61
32509688 2020
21
[A suspected case of Hashimoto encephalopathy with frequent relapses despite multiple courses of immunotherapy]. 61
32101843 2020
22
Hashimoto encephalopathy in the 21st century. 61
31882532 2020
23
The neuromythology of Hashimoto encephalopathy: The emperor has no clothes. 61
31882524 2020
24
Hashimoto encephalopathy as manifestation of central nervous system chronic graft-versus-host disease after hematopoietic stem cell transplantation. 61
31535441 2019
25
Treatable causes of adult-onset rapid cognitive impairment. 61
31715517 2019
26
Serial brain MRI changes related to autoimmune pathophysiology in Hashimoto encephalopathy with anti-NAE antibodies: A case-series study. 61
31525528 2019
27
Cerebellar Syndrome Associated with Thyroid Disorders. 61
31388971 2019
28
Hashimoto's encephalopathy: a rare cause of delirium. 61
31494584 2019
29
[Recent progress in autoimmune encephalitis and its related disorders]. 61
31341132 2019
30
Hashimoto Encephalopathy in Children. 61
31359963 2019
31
[Cotard-syndrome associated with Hashimoto encephalopathy: a case report]. 61
31378725 2019
32
Autoimmune Encephalitides. 61
30952414 2019
33
[A case of dementia with Lewy bodies and Hashimoto encephalopathy successfully treated with immunotherapy]. 61
30700687 2019
34
Hashimoto Encephalopathy as a Complication of Autoimmune Thyroiditis. 61
30355921 2019
35
SREAT presenting as decades of intractable seizures and isolated delusional episodes with clinical, laboratory, and EEG confirmation of treatment response. 61
31205712 2019
36
The effectiveness of phenobarbital in patients with refractory status epilepticus undergoing therapeutic plasma exchange. 61
30216211 2018
37
Hashimoto Encephalopathy Mimicking Acute Ischemic Stroke: Perfusion-Weighted Magnetic Resonance Imaging. 61
30284769 2018
38
Steroid-responsive encephalopathy with a peculiar CSF biomarker profile in an 89-year-old man. 61
30263128 2018
39
Hashimoto Encephalopathy. 61
30263866 2018
40
A 15-Year-Old Boy With Refractory Status Epilepticus. 61
29961495 2018
41
Hashimoto encephalopathy in pediatric patients: Homogeneity in clinical presentation and heterogeneity in antibody titers. 61
28784301 2018
42
Immune-mediated ataxias. 61
29891069 2018
43
Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Presenting with Pure Cerebellar Ataxia. 61
30191089 2018
44
[Hashimoto encephalopathy as explanation of psychotic symptoms in an adolescent]. 61
29436703 2018
45
[Motor Symptoms of Autoimmune Encephalopathies]. 61
29282342 2017
46
Cerebellopathy secondary to anti-peroxidase antibody-mediated toxicity. A special case of Hashimoto encephalopathy. 61
28863961 2017
47
Improved 123I-Ioflupane Binding After Immunotherapy in Anti-NAE Antibody-Positive Hashimoto Encephalopathy That Clinically Mimicked Multiple System Atrophy. 61
28632692 2017
48
Focal neurological presentation in Hashimoto's encephalopathy mimicking a vascular occlusion of the middle cerebral artery. 61
28710237 2017
49
Treatment of Steroid-Resistant Hashimoto Encephalopathy With Misidentification Delusions and Catatonia. 61
28190544 2017
50
Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies: A clinical subtype of Hashimoto encephalopathy. 61
28272206 2017

Variations for Hashimoto Encephalopathy

Expression for Hashimoto Encephalopathy

Search GEO for disease gene expression data for Hashimoto Encephalopathy.

Pathways for Hashimoto Encephalopathy

GO Terms for Hashimoto Encephalopathy

Sources for Hashimoto Encephalopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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