Abstract:
:There are at least seven clinically indistinguishable but genetically different types of autosomal dominant pure spastic paraplegia (ADPSP). In this study we investigated electrophysiological characteristics in patients with ADPSP linked to chromosome 2p (SPG4). Twelve patients from six different families with ADPSP linked to chromosome 2p and 15 control persons were included. Electromyography (EMG), motor and sensory nerve conduction, and motor evoked potentials using single and paired transcranial magnetic stimulation (PTMS) was performed. From the peripheral nervous system we found signs of motor and sensory axonal neuropathy. Motor evoked potentials disclosed greatly reduced corticospinal tract conduction velocity and amplitude of evoked potentials to the lower extremities indicating that the very marked spasticity predominantly seems to rely on dysfunction of the fast conducting axons of the pyramidal tract. PTMS showed an increased intracortical facilitation (ICF), which may reflect an impaired function of gamma-aminobutyric acid (GABA)-controlled interneuronal circuits in the motor cortex, alternatively an increased glutamatergic transmission or a compensatory recruitment of a larger number of neurones with corticospinal projections.
journal_name
Eur J Neuroljournal_title
European journal of neurologyauthors
Nielsen JE,Jennum P,Fenger K,Sørensen SA,Fuglsang-Frederiksen Adoi
10.1046/j.1468-1331.2001.00249.xsubject
Has Abstractpub_date
2001-07-01 00:00:00pages
335-9issue
4eissn
1351-5101issn
1468-1331pii
ene249journal_volume
8pub_type
杂志文章abstract::The muscle overactivity seen in spasticity results in limb stiffness and muscle spasm, to which there is both a neurogenic and a biomechanical component. Spasticity does not always cause harm and can assist in the rehabilitation process enabling a patient to stand when their limb weakness would not otherwise allow it....
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2002.0090s1048.x
更新日期:2002-05-01 00:00:00
abstract::The Australian Pregnancy Registry, affiliated European Register of Antiepileptic drugs in Pregnancy (EURAP), recruits informed consenting women with epilepsy on treatment with antiepileptic drugs (AEDs), those untreated, and women on AEDs for other indications. Enrolment is considered prospective if it has occurred be...
journal_title:European journal of neurology
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doi:10.1111/j.1468-1331.2006.01359.x
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abstract:BACKGROUND AND PURPOSE:The 'hot cross bun' sign (HCBS), typically seen in the patients with multiple system atrophy, refers to a cruciform hyperintensity in the pons on T2-weighted MRI. Little is known about its pathological basis and prevalence in other degenerative cerebellar diseases and healthy population. We inves...
journal_title:European journal of neurology
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doi:10.1111/j.1468-1331.2008.02524.x
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abstract::Visual recognition memory was assessed in terms of delay duration, memory load and amount of interference(s) in non-demented patients with Parkinson's disease (PD) using an automated delayed non-matching-to-sample (DNMS) task with trial-unique stimuli. Special attention was focused on the different cognitive functions...
journal_title:European journal of neurology
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doi:10.1046/j.1468-1331.2000.t01-1-00107.x
更新日期:2000-09-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Approximately 9000 people in the UK are affected by Huntington's disease (HD). People with HD require ongoing health and social care support. There is a knowledge gap about costs of health and social care use associated with HD in the UK. This paper estimates the economic cost in the UK. METHODS...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.13107
更新日期:2016-10-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Accumulation of iron (Fe) is often detected in brains of people suffering from neurodegenerative diseases. However, no studies have compared the Fe load between these disease entities. The present study investigates by T2*-weighted gradient-echo 7.0 T magnetic resonance imaging (MRI) the Fe conte...
journal_title:European journal of neurology
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journal_title:European journal of neurology
pub_type: 杂志文章
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.1999.640515.x
更新日期:1999-07-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1468-1331.2009.02802.x
更新日期:2010-02-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2011.03496.x
更新日期:2012-01-01 00:00:00
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journal_title:European journal of neurology
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更新日期:2015-01-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2011.03521.x
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journal_title:European journal of neurology
pub_type: 杂志文章
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更新日期:2017-07-01 00:00:00
abstract::In this paper we report about the Danish Year of the Brain 1997 because it may serve as a model for other countries. It was possible in Denmark to organize a very large scale effort which has been well received by the general public and which has significantly improved the image of the neurological disciplines. The re...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.1998.560619.x
更新日期:1998-11-01 00:00:00
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journal_title:European journal of neurology
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:2003-09-01 00:00:00
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更新日期:2017-10-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.13038
更新日期:2016-08-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.1999.660639.x
更新日期:1999-11-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Although the genetic contribution to stroke risk is well known, it remains unclear if young-onset stroke has a stronger genetic contribution than old-onset stroke. This study aims to compare the heritability of ischaemic stroke risk between young and old, using common genetic variants from whole-...
journal_title:European journal of neurology
pub_type: 杂志文章
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更新日期:2020-09-30 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2011.03400.x
更新日期:2011-11-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2008.02331.x
更新日期:2008-12-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.12528
更新日期:2015-01-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2001.00166.x
更新日期:2001-01-01 00:00:00
abstract::The purpose of this study was to investigate the surface electromyogram (EMG) of the lower limbs of hemiplegic children with spastic cerebral palsy during gait. The EMG of seven muscles was analyzed for 17 children (5-12 years old). The EMG of the involved side was decreased for the examined shank muscles and increase...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2005.01047.x
更新日期:2005-09-01 00:00:00
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journal_title:European journal of neurology
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更新日期:2007-02-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Non-Western immigrants to Norway acquire an increased risk of multiple sclerosis (MS) after migration. Ethnicity and the presence of oligoclonal bands (OCBs) in the cerebrospinal fluid (CSF) might influence the disease course. The aim of this study was to investigate differences in disease severi...
journal_title:European journal of neurology
pub_type: 杂志文章
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更新日期:2013-12-01 00:00:00
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journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2006.01565.x
更新日期:2007-01-01 00:00:00