Presentation
Abnormal body movement.
Patient Data
Marked atrophic changes involve both caudate nuclei with compensatory enlargement of both frontal horns giving box shape appearance resulting in decreased frontal horn to intercaudate ratio. Prominent putaminal volume loss is also noted bilaterally.
The basal ganglia show abnormal gliotic changes with high T2 - Flair and low T1 signal intensity.
Mild brain atrophic changes disproportionate to the patient's age.
Case Discussion
Huntington disease is a neurodegenerative disorder with autosomal dominant inheritance. Grossly, the patient has a decreased mentality and develops recurrent attacks of choreoathetosis body movement.
Review of the electroencephalogram and laboratory investigations for this patient was normal. A first-degree relative has had the same condition since childhood. The diagnosis of Huntington disease was given by the characteristic imaging features.
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