Background: Hashimoto encephalopathy is a potentially fatal condition associated with a presentation of myoclonus, altered conscious state, strokelike episodes, rapid cognitive decline, and neuropsychiatric symptoms. Both congenital hypothyroidism and acquired hypothyroidism are common in patients with Down syndrome.
Objective: To describe the presentation of Hashimoto encephalopathy in patients with Down syndrome.
Design: Clinical case reports.
Setting: General neurology unit. Patients Two Down syndrome patients diagnosed as having Hashimoto encephalopathy are described. Intervention High-dose oral corticosteroids.
Main outcome measures: Neurologic examination, electroencephalography, and blood analysis results.
Results: Both patients responded to treatment, with a slow return to their premorbid level of function.
Conclusion: Hashimoto encephalopathy should be considered in Down syndrome patients with rapidly progressive cognitive decline.