Single photon emission computed tomographic blood flow and magnetic resonance volume imaging of basal ganglia in Huntington's disease

G. J. Harris, E. H. Aylward, C. E. Peyser, G. D. Pearlson, J. Brandt, J. V. Roberts-Twillie, P. E. Barta and S. E. Folstein
Department of Psychiatry, Tufts University, New England Medical Center, Boston, Massachusetts, USA.

OBJECTIVE: To examine basal ganglia dysfunction and atrophy in patients with mild to moderate Huntington's disease, with correlation of imaging measures with clinical and neuropsychological measures. DESIGN: Survey study in patients with Huntington's disease and matched controls, with imaging measures being evaluated by investigators unaware of the diagnosis. SETTING: Baltimore Huntington's Disease Project, The Johns Hopkins Hospital, Baltimore, Md. PATIENTS AND OTHER PARTICIPANTS: Subjects included 10 patients with mild to moderate Huntington's disease and nine healthy age-matched control subjects. MAIN OUTCOME MEASURES: Imaging measures included single photon emission computed tomographic regional cerebral blood flow in caudate, putamen, and thalamus, and magnetic resonance imaging measures of caudate and putamen volumes and bicaudate ratios. Patients underwent neurologic and mental status examinations and neuropsychological tests. RESULTS: The measure with the greatest difference between patients and control subjects was mean putamen volume, reduced 54.3% in patients, with no overlap between groups (P<.001). Of the cerebral blood flow measures, caudate showed the greatest difference (21.5% decrease; P<.001). Quantitative neurologic indexes of disease severity correlated with both putamen measures (P<.03), while Mini-Mental State Examination scores correlated with caudate volume (P<.02). Bicaudate ratio correlated with both clinical measures and was the best index of neurologic deterioration (r=.95; P<.001), while global atrophy (measured by cerebrospinal fluid percentage) was the best correlate of several neuropsychological tests, such as the Trail Making Test (r=93; P<.001). CONCLUSIONS: Volumetric measurement of putamen best discriminated patients with Huntington's disease from healthy subjects. Measures of caudate atrophy or single photon emission computed tomographic measures performed less well. Neurologic decline correlated best with subcortical atrophy measured by the bicaudate ratio, but neuropsychological performance best corresponded to cerebrospinal fluid percentage, a measure of global atrophy.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Thalamic Atrophy in Huntington's Disease Co-varies with Cognitive Performance: A Morphometric MRI Analysis
Kassubek et al.
Cereb Cortex 2005;15:846-853.
ABSTRACT | FULL TEXT  

In vivo evidence of cerebellar atrophy and cerebral white matter loss in Huntington disease
Fennema-Notestine et al.
Neurology 2004;63:989-995.
ABSTRACT | FULL TEXT  

Magnetic Resonance Imaging and Magnetic Resonance Spectroscopy in Dementias
Hsu et al.
J Geriatr Psychiatry Neurol 2001;14:145-166.
ABSTRACT  

Subcortical volumes in girls with Tourette syndrome: Support for a gender effect
Zimmerman et al.
Neurology 2000;54:2224-2229.
ABSTRACT | FULL TEXT  

Decreased striatal monoaminergic terminals in Huntington disease
Bohnen et al.
Neurology 2000;54:1753-1759.
ABSTRACT | FULL TEXT  

Bradykinesia in early Huntington's disease
Sanchez-Pernaute et al.
Neurology 2000;54:119-119.
ABSTRACT | FULL TEXT  

Reduced basal ganglia blood flow and volume in pre-symptomatic, gene-tested persons at-risk for Huntington's disease
Harris et al.
Brain 1999;122:1667-1678.
ABSTRACT | FULL TEXT  

Clinical correlation of striatal 1H MRS changes in Huntington's disease
Sanchez-Pernaute et al.
Neurology 1999;53:806-806.
ABSTRACT | FULL TEXT  

New Techniques for Understanding Huntington's Disease
Hurley et al.
J. Neuropsychiatry Clin. Neurosi. 1999;11:173-175.
FULL TEXT