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Hashimoto encephalopathy: syndrome or myth?
Ji Y Chong1, Lewis P Rowland, Robert D Utiger
1Department of Neurology, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Archives of Neurology
|February 13, 2003
Summary
Hashimoto encephalopathy, characterized by brain dysfunction and high thyroid antibodies, appears to be a distinct syndrome. Most patients significantly improve with glucocorticoid treatment, suggesting a link beyond mere chance association.
Area of Science:
- Neurology
- Endocrinology
- Immunology
Background:
- Hashimoto encephalopathy is a debated syndrome linking brain dysfunction with elevated antithyroid antibodies.
- Previous associations between encephalopathy, high antithyroid antibodies, and glucocorticoid responsiveness were considered potentially coincidental.
Observation:
- A systematic literature review identified 85 patients with noninfectious encephalopathy and high serum antithyroid antibody concentrations.
- Common clinical features included seizures (66%), psychosis (38%), and abnormal EEG findings (98%).
- Thyroid function abnormalities ranged from hypothyroidism to hyperthyroidism, with subclinical hypothyroidism being most frequent (35%).
Findings:
- The study found a strong association between encephalopathy, high antithyroid antibodies, and positive response to glucocorticoid therapy in 96% of treated patients.
- These findings suggest Hashimoto encephalopathy is an identifiable neurological syndrome.
- Despite the strong association, a direct pathogenic role for antithyroid antibodies in the brain remains unproven, suggesting they may be markers of an underlying autoimmune process.
Implications:
- The results support Hashimoto encephalopathy as a distinct clinical entity, not merely a chance association.
- The findings highlight the importance of considering autoimmune thyroid disease in patients presenting with unexplained encephalopathy.
- Further research is needed to elucidate the exact autoimmune mechanisms involved in Hashimoto encephalopathy and the role of antithyroid antibodies.