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Arch Neurol. 2009 May;66(5):663-6. doi: 10.1001/archneurol.2009.45.

Hashimoto encephalopathy and Down syndrome.

Author information

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.

PMID:
19433669
DOI:
10.1001/archneurol.2009.45
[Indexed for MEDLINE]

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