Hashimoto encephalopathy and Down syndrome.
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.


METHODS
Clinical case reports.


METHODS
General neurology unit. Patients Two Down syndrome patients diagnosed as having Hashimoto encephalopathy are described. Intervention High-dose oral corticosteroids.


METHODS
Neurologic examination, electroencephalography, and blood analysis results.


RESULTS
Both patients responded to treatment, with a slow return to their premorbid level of function.


CONCLUSIONS
Hashimoto encephalopathy should be considered in Down syndrome patients with rapidly progressive cognitive decline.
National Stroke Research Institute, Level 1, Neurosciences Building, Austin Health, Heidelberg 3084, Australia. amyb@alphalink.com.au.
Amy
A
Brodtmann
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D001921:Brain / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D001928:Brain Diseases, Metabolic / Q000188:drug therapy / Q000235:genetics* / Q000503:physiopathology*; D015897:Comorbidity; D018450:Disease Progression; D004314:Down Syndrome / Q000188:drug therapy / Q000235:genetics* / Q000503:physiopathology*; D004569:Electroencephalography; D005260:Female; D020022:Genetic Predisposition to Disease; D050031:Hashimoto Disease / Q000188:drug therapy / Q000235:genetics* / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D013961:Thyroid Gland / Q000378:metabolism / Q000503:physiopathology; D013963:Thyroid Hormones / Q000378:metabolism; D016896:Treatment Outcome; D055815:Young Adult
D000305:Adrenal Cortex Hormones; D013963:Thyroid Hormones