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.
To describe the presentation of Hashimoto encephalopathy in patients with Down syndrome.
Clinical case reports.
General neurology unit.
Two Down syndrome patients diagnosed as having Hashimoto encephalopathy are described.
High-dose oral corticosteroids.
Main Outcome Measures
Neurologic examination, electroencephalography, and blood analysis results.
Both patients responded to treatment, with a slow return to their premorbid level of function.
Hashimoto encephalopathy should be considered in Down syndrome patients with rapidly progressive cognitive decline.