Abstract:
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.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Jurgens CK,van de Wiel L,van Es AC,Grimbergen YM,Witjes-Ané MN,van der Grond J,Middelkoop HA,Roos RAdoi
10.1007/s00415-008-0050-4subject
Has Abstractpub_date
2008-11-01 00:00:00pages
1785-91issue
11eissn
0340-5354issn
1432-1459journal_volume
255pub_type
杂志文章abstract::Horizontal eye-movement responses of four patients with Alzheimer-type dementia were recorded using the infrared oculographic technique. Abnormally frequent saccadic intrusions occurred during the fixation and slow, smooth pursuit tasks in all four patients. Saccadic intrusions have previously been reported in the lit...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313742
更新日期:1983-01-01 00:00:00
abstract:BACKGROUND:The aim of study was to demonstrate that the first three injections of botulinum neurotoxin type A (BoNT/A) appear to be less effective in botulinum toxin naïve patients with idiopathic cervical dystonia (CD) with mild symptoms and low severity scores (TSUI-scores) at onset of BoNT/A-therapy compared to pati...
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pub_type: 临床试验,杂志文章
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journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-009-5215-2
更新日期:2009-10-01 00:00:00
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pub_type: 杂志文章
doi:10.1007/s00415-006-0358-x
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pub_type: 指南,杂志文章,实务指引
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journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-10278-8
更新日期:2020-12-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
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更新日期:2010-09-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313696
更新日期:1983-01-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8098-z
更新日期:2016-06-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6006-0
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pub_type: 杂志文章
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更新日期:1981-01-01 00:00:00
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pub_type: 杂志文章
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0905-z
更新日期:2002-12-01 00:00:00
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