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

• Published in: Perceptual and motor skills Vol. 72; no. 3_suppl; pp. 1363 - 1374
• Main Authors: Brooker, Alan E., Dougherty, David S., Love, Karl F., Kelly, William M.
• Format: Journal Article
• Language: English
• Published: Los Angeles, CA SAGE Publications 01-06-1991; Perceptual and Motor Skills; Perceptual and Motor Skills, etc
• Subjects: Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia - diagnosis; Dementia - etiology; Follow-Up Studies; Humans; Huntington Disease - complications; Huntington Disease - diagnosis; Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Neurology; Neuropsychological Tests; Psychomotor Disorders - etiology; Tomography, Emission-Computed; Case study; Human; Nervous system diseases; Cognitive disorder; Huntington disease; Degenerative disease; Nuclear magnetic resonance imaging; Neuropsychologia; Dementia
• ISSN: 0031-5125; 1558-688X
• DOI: 10.2466/pms.1991.72.3c.1363

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.