Neuropsychological, Neurological, and Mri Correlates of Dementia in Advanced Huntington's Disease: A Single Case Study

Abstract

Affected Huntington's disease patients present with a progressive dementia that can be detected with a variety of neuropsychological procedures. Neuropsychological findings include impaired mental flexibility and concentration, deterioration of verbal and procedural memory, diminished nonverbal memory, and slowing of both fine and gross motor functions. Magnetic resonance imaging (MRI) offers unique advantages in depicting morphologic changes associated with Huntington's disease. Frontotemporal atrophy and, in particular, atrophy of the corpus striatum are characteristically observed. Given the ease of obtaining coronal images and the improved differentiation of gray matter and white matter, MRI can provide better identification of these findings than traditional imaging methods such as computed tomography (CT). Finally, the presence of steadily progressive neuropsychological deterioration in conjunction with characteristic atrophy observed on MRI can be combined with diminished metabolic activity of the corpus striatum as observed on positron emission tomography (PET) for added diagnostic specificity.