A 48 year-old man presented with many years of progressive psychiatric impairment and dementia. Prominent chorea was present on examination. There was a similar history in his father and many relatives in the father's family.
Coronal MRI scan (T1 weighted) of the patient (left) and a normal (right). Note the prominent atrophy of the cortex as well as the atrophy of the caudate nucleus of the patient compared to the normal. Huntington's is a neurodegenerative disease with dominant inheritance. Abnormal movements (especially chorea) and behavioral/psychiatric symptoms are common.
The Electronic Curriculum is copyrighted 1998, Case Western Reserve University School of Medicine.