Basal ganglia volume and clinical correlates in ‘preclinical’ Huntington’s disease

Objective To establish differences in basal ganglia and thalamic volume between preclinical carriers and non-carriers of the Huntington’s disease (HD) gene and to link the volume to motor, cognitive and behavioural characteristics in carriers. Methods Sixteen HD gene carriers without overt clinical...

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Bibliographic Details
Published in:Journal of neurology 2008-11, Vol.255 (11), p.1785-1791
Main Authors: Jurgens, Caroline K., van de Wiel, Lotte, van Es, Ad C. G. M., Grimbergen, Yvette M., Witjes-Ané, Marie-Noëlle W., van der Grond, Jeroen, Middelkoop, Huub A. M., Roos, Raymund A. C.
Format: Article
Language:English
Subjects:
Adult
Basal Ganglia - pathology
Biological and medical sciences
Cognition
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Heterozygote
Humans
Huntington Disease - genetics
Huntington Disease - pathology
Huntingtons disease
Magnetic Resonance Imaging
Male
Medical sciences
Medicine
Medicine & Public Health
Memory
Middle Aged
Motor Activity
Neurology
Neuropsychological Tests
Neuroradiology
Neurosciences
Organ Size
Original Communication
Thalamus - pathology
Trinucleotide Repeats
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Summary:Objective To establish differences in basal ganglia and thalamic volume between preclinical carriers and non-carriers of the Huntington’s disease (HD) gene and to link the volume to motor, cognitive and behavioural characteristics in carriers. Methods Sixteen HD gene carriers without overt clinical motor signs and 14 non-gene carriers underwent clinical evaluation and a MRI scan. Volumes of the caudate nucleus, putamen, gobus pallidus and thalamus were measured using T1-weighted MR images. Motor, cognitive and behavioural functioning was assessed using the Unified Huntington’s Disease Rating Scale (UHDRS), cognitive testing and the Beck Depression Inventory (BDI-II). Results Volumes of the caudate nucleus, putamen and globus pallidus were significantly smaller in carriers than in non-carriers while no differences between groups were found on clinical evaluation. In gene carriers smaller globus pallidus volume was associated with more motor abnormalities. A smaller putamen volume correlated significantly with worse psychomotor function on the Symbol Digit Modalities Task and the Trail Making Test B. Conclusions In line with previous research we demonstrated that basal ganglia abnormalities precede overt disease manifestation of HD. Besides we showed that smaller basal ganglia volumes are related to subtle motor abnormalities and worse psychomotor performance in gene carriers without clinical diagnosis. Motor and psychomotor measures may be suitable clinical markers in future neuroprotective trials when combined with volumetric imaging.
ISSN:0340-5354
1432-1459
DOI:10.1007/s00415-008-0050-4