Hashimoto encephalopathy with angiographic CNS vasculitis

Hashimoto encephalopathy (HE)—also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis—is a rare syndrome loosely associated with Hashimoto thyroiditis. HE mostly affects middle-aged women; is characterized by delirium, seizures, and myoclonus; and is thought to be an immune-mediated disorder. The pathophysiology of HE is not entirely understood. Most cases appear to be due to autoimmune vasculitis or an immune complex deposition that disrupts the cerebral microvasculature. This immunologic phenomenon has been observed on brain biopsy with a lymphocytic infiltration around small arterioles and venules.1,2 Patients with HE appear to respond to corticosteroid therapy or other forms of immunosuppression. The presence of elevated antithyroid antibodies, which is consistent with an active autoimmune process, is essential for a diagnosis of HE.
Source: Nature Clinical Practice - Category: Neurology Authors: Tags: Other cerebrovascular disease/ Stroke, Autoimmune diseases, Vasculitis Cases Source Type: research