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Disease Profile
Hashimoto encephalopathy
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
All ages
ICD-10
G04.8
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Hashimoto's encephalopathy; Steroid-responsive encephalopathy associated with autoimmune thyroiditis
Categories
Immune System Diseases; Nervous System Diseases
Summary
Hashimoto
Symptoms
Symptoms may include:[1][2][3]
- Confusion
- Memory loss
- Behavior changes
- Hallucinations or delusions
- Muscle weakness
Seizures
HE usually occurs in adults from 40-50 years of age but it can also occur in children. Some people have episodes of symptoms that come and go over time. Other people may have symptoms that slowly get worse. In some cases, without treatment, coma can occur. Most people with HE will respond to treatment and get better.
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
100% of people have these symptoms | ||
Acute encephalopathy | 0006846 | |
80%-99% of people have these symptoms | ||
Cerebral vasculitis | 0005318 | |
Hashimoto thyroiditis | 0000872 | |
30%-79% of people have these symptoms | ||
Abnormality of the cerebral white matter | 0002500 | |
Confusion |
Disorientation
Easily confused
Mental disorientation
[ more ] |
0001289 |
Goiter |
Enlarged thyroid gland in neck
|
0000853 |
Hyponatremia |
Low blood sodium levels
|
0002902 |
Hypothyroidism |
Underactive thyroid
|
0000821 |
Paralysis |
Inability to move
|
0003470 |
5%-29% of people have these symptoms | ||
Abnormal autonomic nervous system physiology | 0012332 | |
Anxiety |
Excessive, persistent worry and fear
|
0000739 |
Depressivity |
Depression
|
0000716 |
Fever | 0001945 | |
Focal-onset seizure |
Seizure affecting one half of brain
|
0007359 |
Generalized-onset seizure | 0002197 | |
Headache |
Headaches
|
0002315 |
Decreased immune function
|
0002721 | |
Leukocytosis |
Elevated white blood count
High white blood count
Increased blood leukocyte number
[ more ] |
0001974 |
Myoclonus | 0001336 | |
Nausea and vomiting | 0002017 | |
Psychosis | 0000709 | |
Status epilepticus |
Repeated seizures without recovery between them
|
0002133 |
Low platelet count
|
0001873 | |
1%-4% of people have these symptoms | ||
Limited neck flexion |
Limited neck flexibility
|
0005991 |
Diagnosis
Treatment
Specialists involved in the care of someone with HE may include:
Neurologist Endocrinologist Psychiatrist /Psychologist
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
Autoimmune Encephalitis Alliance
920 Urban Avenue
Durham, NC 27701
E-mail: https://aealliance.org/contact/
Website: https://aealliance.org/ -
Encephalitis Society
32 Castlegate
Malton, North Yorkshire YO17 7DT
United Kingdom
Telephone: +44 (0)1653 699599
E-mail: [email protected]
Website: https://www.encephalitis.info/
Social media: Twitter: @encephalitis Facebook: /encephalitissociety -
International Autoimmune Encephalitis Society
Telephone: 352-527-2470
E-mail: [email protected]
Website: https://autoimmune-encephalitis.org/
Social Networking Websites
- Visit the HESA Hashimoto's Encephalopathy SREAT Alliance group on Facebook.
- The International Autoimmune Encephalitis Society Facebook group offers educational support through this forum.
Organizations Providing General Support
-
American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: [email protected]
Website: https://www.aarda.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- The International Autoimmune Encephalitis Society offers patients and families detailed information on diagnosis and treatment.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Hashimoto encephalopathy. Click on the link to view a sample search on this topic.
References
- Rubin DI. Hashimoto encephalopathy. UpToDate. Waltham, MA: UpToDate; Updated Mar 11, 2019; https://www.uptodate.com/contents/hashimoto-encephalopathy.
- Mattozzi S, Sabater L, Escudero D, Ariño H, Armangue T, Simabukuro M, Iizuka T, Hara M, Saiz A, Sotgiu S, Dalmau J, Graus F. Hashimoto encephalopathy in the 21st century. Neurology. Jan 14, 2020; 94(2):e217-e224. https://pubmed.ncbi.nlm.nih.gov/31882532/.
- DeBiase JM, Avasthi D. Hashimoto's Encephalopathy: A Case Report and Literature Review of an Encephalopathy With Many Names. Cureus. Aug 7, 2020; 12(8):e9601. https://pubmed.ncbi.nlm.nih.gov/32923205/.
- Zhou JY, Xu B, Lopes J, Blamoun J, Li L. Hashimoto encephalopathy: literature review. Acta Neurol Scand. Mar 2017; 135(3):285-290. https://pubmed.ncbi.nlm.nih.gov/27324276/.
- Laycock K, Chaudhuri A, Fuller C, Khatami Z, Nkonge F, Stojanovic N. A novel assessment and treatment approach to patients with Hashimoto's encephalopathy. Endocrinol Diabetes Metab Case Rep. Apr 27, 2018; 2018:17-0117. https://pubmed.ncbi.nlm.nih.gov/29732162/.
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