Hashimoto encephalopathy

What causes Hashimoto encephalopathy?

The exact cause of Hashimoto encephalopathy (HE) is unknown, but is thought to relate to autoimmune or other autoinflammatory processes. While it is associated with Hashimoto's thyroiditis, the exact nature of the relationship between the two conditions is unclear. It does not appear to be directly related to hypothyroidism or hyperthyroidism.

Last updated on 05-01-20

How is Hashimoto encephalopathy diagnosed?

Diagnosis of Hashimoto encephalopathy can be made when all six of the following criteria have been met:

1. Encephalopathy with seizures, myoclonus, hallucinations, or stroke-like episodes
2. Subclinical or mild overt thyroid disease (usually hypothyroidism)
3. Normal brain MRI or with non-specific abnormalities
4. Presence of serum thyroid (thyroid peroxidase, thyroglobulin) antibodies
5. Absence of well-characterized neuronal antibodies in serum and CSF
6. Reasonable exclusion of alternative causes

A team of leading autoimmune encephalitis researchers have proposed that the term Hashimoto’s encephalopathy only be used when rigorous clinical assessment and comprehensive testing for well characterized neuronal antibodies exclude other potential causes of encephalopathy. The underlying cause of Hashimoto’s encephalopathy is unclear. Experts in the field recommend that cases of Hashimoto’s encephalopathy be classified as probable autoimmune encephalitis.

View the full position paper, entitled A Clinical Approach to Diagnosis of Autoimmune Encephalitis.

For more on diagnosis, visit the following link to the International Autoimmune Encephalitis Society Web site:
https://autoimmune-encephalitis.org/diagnosis

Last updated on 05-01-20

Is Hashimoto encephalopathy inherited?

Hashimoto encephalopathy (HE) typically is not inherited, occurring sporadically in people with no family history HE. It can occur in association with other autoimmune disorders however, so in some cases it may develop due to an interaction between genetic susceptibility and environmental triggers.

Last updated on 05-01-20

How might Hashimoto encephalopathy be treated?

Medical management of Hashimoto encephalopathy (HE) usually involves corticosteroids and treatment of thyroid abnormalities (if present). The optimal dose of oral steroids is not known. Most patients with HE respond to steroid therapy. Symptoms typically improve or resolve over a few months. Decisions regarding the length of steroid treatment and the rate of tapering off steroids are based on the individual's response to treatment. Treatment may last as long as two years in some patients.

People with HE who experience repeated HE relapses, do not respond to steroids, and/or cannot tolerate steroid treatment have been treated with other immunosuppressive medications such as azathioprine and cyclophosphamide. Intravenous immunoglobulin, and plasmapheresis have also been used.

Last updated on 05-01-20

Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
Name: Hashimoto’s Encephalopathy SREAT Alliance HESA Email: hesasocialmedia@gmail.com Url: https://www.hesaonline.info/
Name: Autoimmune Encephalitis Alliance 920 Urban Avenue
Durham, NC, 27701,
Email: https://aealliance.org/contact/ Url: https://aealliance.org/
Name: International Autoimmune Encephalitis Society United States Phone: 352-527-2470 Email: IAES@autoimmune-encephalitis.org Url: https://autoimmune-encephalitis.org/
Name: Encephalitis Society 32 Castlegate Malton, North Yorkshire YO17 7DT
United Kingdom
Phone: +44 (0)1653 699599 Email: support@encephalitis.info Url: http://www.encephalitis.info/ Social media: Twitter: @encephalitis Facebook: /encephalitissociety

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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