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Genetic and Rare Diseases Information Center (GARD)

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Hashimoto's encephalitis


Other Names for this Disease

  • Hashimoto's encephalopathy
  • Steroid-responsive encephalopathy associated with autoimmune thyroiditis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Hashimoto's encephalitis?

What are the symptoms of Hashimoto's encephalitis?

How might Hashimoto's encephalitis be treated?

What is Hashimoto's encephalitis?

Hashimoto's encephalitis (HE) is a rare autoimmune disease with neurological and psychiatric features.[1][2][3][4] The condition is characterized by high titers of antithyroid antibodies in blood and cerebrospinal fluid (CSF).[1][2][4] Two types of HE have been suggested: a vasculitic type characterized by multiple relapsing-remitting stroke-like episodes and mild cognitive impairment, and a diffuse progressive type characterized by dementia and psychiatric symptoms.[1][3] Patients are mostly women.[1] Treatment includes steroids, plasmapheresis (plasma exchange), or immunosuppressant medications.[1][2][4]
Last updated: 4/16/2010

What are the symptoms of Hashimoto's encephalitis?

The symptoms of Hashimoto's encephalitis are variable among individual patients. The relapsing/remitting vasculitic type typically manifests with encephalopathy and stroke-like episodes. The diffuse progressive type has a more insidious onset and progressive course with occasional fluctuations and is characterized by psychiatric symptoms and dementia. Either type might also present with tremor, myoclonus, seizures, aphasia, stupor (a state in which the patient is unresponsive but can be aroused briefly by a strong stimulus, such as sharp pain), hypersomnolence (excessive sleepiness), gait ataxia, or coma.[3]
Last updated: 4/16/2010

How might Hashimoto's encephalitis be treated?

Medical management of Hashimoto's encephalitis (HE) usually involves corticosteroids and treatment of thyroid abnormalities and seizures (if present). The optimal dose of oral steroids is not known, but in general may range from 50 mg/day to 150 mg/day. Decisions regarding the length of steroid treatment and rate of tapering off steroids are based on the individual's response to treatment. Most people with HE improve or fully recover with steroid therapy, typically within a few months. Some people require prolonged steroid treatment.[5] Recurrences typically respond to steroid treatment.[3]

People with HE who experience repeated HE relapses, do not respond to steroids, and/or cannot tolerate steroid treatment have been treated with immunosuppressive medications (e.g., azathioprine and cyclophosphamide), immunosuppressive and steroid combination therapy, intravenous immunoglobulin, or plasmapheresis.[3][5] Due to the rarity of these cases, data regarding treatment effectiveness is lacking.

Last updated: 5/6/2013

References
  1. Mijajlovic M, Mirkovic M, Dackovic, J et al.. J Neurol Sci. 2010; http://www.ncbi.nlm.nih.gov/pubmed/19846119. Accessed 4/16/2010.
  2. Mocellin R, Walterfang M, Velakoulis D. CNS Drugs. 2007; http://www.ncbi.nlm.nih.gov/pubmed/17850170. Accessed 4/16/2010.
  3. Marshall GA, Doyle JJ. J Neuropsychiatry Clin Neurosci. 2006; http://neuro.psychiatryonline.org/cgi/content/full/18/1/14. Accessed 4/16/2010.
  4. Schiess N, Pardo CA. Ann N Y Acad Sci. 2008; http://www.ncbi.nlm.nih.gov/pubmed/18990131. Accessed 4/16/2010.
  5. Rubin D. Hashimoto's encephalopathy. In: Basow. UpToDate. Waltham, MA: UpToDate; 2013;


Other Names for this Disease
  • Hashimoto's encephalopathy
  • Steroid-responsive encephalopathy associated with autoimmune thyroiditis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.