- Devon I Rubin, MD
Devon I Rubin, MD
- Professor of Neurology
- Mayo Clinic College of Medicine
- Section Editors
- Michael J Aminoff, MD, DSc
Michael J Aminoff, MD, DSc
- Editor-in-Chief — Neurology
- Section Editor — Medical Neurology
- Professor of Neurology
- University of California, San Francisco School of Medicine
- Douglas S Ross, MD
Douglas S Ross, MD
- Section Editor — Thyroid Disease
- Professor of Medicine
- Harvard Medical School
Hashimoto encephalopathy (HE) is an uncommon syndrome associated with Hashimoto thyroiditis. Originally described in 1966, it remains a somewhat controversial disorder . HE is most often characterized by a subacute onset of confusion with altered level of consciousness, seizures, and myoclonus. In contrast to the cognitive dysfunction associated with hypothyroidism or hyperthyroidism, HE is believed to be an immune-mediated disorder rather than representing the direct effect of an altered thyroid state on the central nervous system.
Although there is some debate regarding the appropriateness of the term Hashimoto encephalopathy, it remains the most widely used term to describe this syndrome. Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and a more general term, nonvasculitic autoimmune meningoencephalitis, are also used to describe this condition.
Hashimoto encephalopathy will be reviewed here. Hashimoto thyroiditis and other neurologic complications of thyroid disease are discussed separately. (See "Pathogenesis of Hashimoto's thyroiditis (chronic autoimmune thyroiditis)" and "Myxedema coma" and "Neurologic manifestations of hypothyroidism" and "Neurologic manifestations of hyperthyroidism and Graves' disease".)
The mechanism of Hashimoto encephalopathy (HE) is unknown. It does not appear to be directly related to hypothyroidism or hyperthyroidism, as the majority of reported patients are euthyroid at the time of presentation. A toxic effect of increased thyrotropin-releasing hormone on the central nervous system has been proposed, as some patients appear to improve with thyroid supplementation despite being euthyroid [2-4]. However, these cases appear to be the exception rather than the rule.
The bulk of evidence points to an autoimmune vasculitis or other inflammatory process, perhaps associated with immune complex deposition, and possibly disrupting the cerebral microvasculature [2,5]:
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- Yu HJ, Lee J, Seo DW, Lee M. Clinical manifestations and treatment response of steroid in pediatric Hashimoto encephalopathy. J Child Neurol 2014; 29:938.
- RELATED DISORDERS
- Nonvasculitic autoimmune inflammatory meningoencephalitis
- Acute disseminated encephalomyelitis
- CLINICAL FEATURES
- LABORATORY FEATURES
- Antithyroid antibodies
- Thyroid hormone levels
- Cerebrospinal fluid
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS