Hashimoto’s Encephalopathy (HE) is recognized as a rare, often steroid-responsive neurological disorder characterized by a wide range of neurological and psychiatric symptoms occurring in patients diagnosed with autoimmune thyroiditis, most commonly Hashimoto’s disease. Although frequently associated with high levels of anti-thyroid antibodies, the symptoms are primarily thought to result from an underlying autoimmune or inflammatory process directly affecting the central nervous system itself, rather than solely from systemic thyroid hormone levels. The clinical presentation is highly variable, potentially including cognitive impairment, recurrent seizures, or acute psychosis.
Origin
The term is named after Hashimoto’s thyroiditis, the autoimmune condition of the thyroid gland first documented by Dr. Hakaru Hashimoto in 1912. The addition of “Encephalopathy,” a medical term signifying a disease or disorder of the brain, clearly denotes the central nervous system involvement that is seen in conjunction with the thyroid autoimmunity.
Mechanism
The precise pathological mechanism of HE remains largely unclear, but the leading theory proposes a form of autoimmune vasculitis or a non-specific inflammatory response mediated by autoantibodies that target various brain antigens, rather than solely the standard thyroid peroxidase or thyroglobulin. This sustained autoimmune assault leads to inflammation and cerebral dysfunction, which ultimately causes the diverse array of neurological symptoms observed. The fact that treatment with corticosteroids frequently results in rapid clinical improvement strongly supports the theory of an underlying inflammatory pathogenesis.
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