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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 signs and symptoms of Hashimoto's encephalitis?

What causes Hashimoto's encephalitis?

Is Hashimoto's encephalitis inherited?

How might Hashimoto's encephalitis be treated?

What is Hashimoto's encephalitis?

Hashimoto's encephalitis (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.[1]
Last updated: 9/18/2014

What are the signs and symptoms of Hashimoto's encephalitis?

The symptoms of Hashimoto's encephalitis can vary among affected people. They most often include sudden or subacute onset of confusion with alteration of consciousness. Some affected people have multiple, recurrent episodes of neurological deficits with cognitive dysfunction. Others experience a more progressive course characterized by slowly progressive cognitive impairment with dementia, confusion, hallucinations, or sleepiness. In some cases, rapid deterioration to coma can occur.[1]

In addition to confusion and mental status changes, symptoms may include seizures and myoclonus (muscle jerking) or tremor. Psychosis, including visual hallucinations and paranoid delusions, has also been reported.[1]
Last updated: 9/18/2014

What causes Hashimoto's encephalitis?

The exact cause of Hashimoto's encephalitis (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.[1]
Last updated: 9/18/2014

Is Hashimoto's encephalitis inherited?

We are aware of only one instance when more than one person in the same family had Hashimoto's encephalitis (HE).[2] To our knowledge, no other cases of familial HE have been reported; HE typically occurs in people with no family history of the condition (sporadically). HE can occur in association with other autoimmune disorders, so HE may develop due to an interaction between genes that predispose a person (susceptibility genes) and environmental triggers.[2]
Last updated: 9/18/2014

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. 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.[3] Recurrences typically also respond to steroid treatment.[4]

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.[4][3] Due to the rarity of these cases, data regarding treatment effectiveness is lacking.
Last updated: 5/6/2013

References
  1. Devon I Rubin. Hashimoto's encephalopathy. UpToDate. Waltham, MA: UpToDate; September, 2014; Accessed 9/18/2014.
  2. Yuceyar N. Thyrotoxic autoimmune encephalopathy in a female patient: only partial response to typical immunosuppressant treatment and remission after thyroidectomy. Clin Neurol Neurosurg. June, 2007; 109(5):458-462. Accessed 9/18/2014.
  3. Rubin D. Hashimoto's encephalopathy. In: Basow. UpToDate. Waltham, MA: UpToDate; 2013;
  4. Marshall GA, Doyle JJ. J Neuropsychiatry Clin Neurosci. 2006; http://neuro.psychiatryonline.org/article.aspx?articleID=102037&resultClick=3. Accessed 4/16/2010.


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.