“ Autosomal dominant inherited neurodegenerative disease associated with choreoathetosis and dementia. The average age of onset is 40 years...
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Autosomal dominant inherited neurodegenerative disease associated with choreoathetosis and dementia.
The average age of onset is 40 years.
MRI
• Heart or Box-shaped enlargement of the frontal horns (due to atrophy of the caudate resulting in ex vacuo dilatation of the frontal horns).
• There may be iron deposition in the basal ganglia, which is low on T2.
• A juvenile form is associated with high T2 signal in the caudate and the putamen.
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