Amy Brodtmann1. 1. National Stroke Research Institute, Level 1, Neurosciences Building, Austin Health, Heidelberg 3084, Australia. amyb@alphalink.com.au.
Abstract
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.
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.