Journal of neurology
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Journal of neurology · Oct 2004
Comparative StudyCombined MR spectroscopic imaging and diffusion tensor MRI visualizes corticospinal tract degeneration in amyotrophic lateral sclerosis.
Motor neuron damage and cortical spinal tract (CST) degeneration are pathological features of amyotrophic lateral sclerosis (ALS). We combined whole-brain diffusion tensor imaging (DTI) and three-dimensional magnetic resonance spectroscopic imaging (MRSI) to study the CST at different locations. Eight ALS patients were compared with normal controls. ⋯ NAA/Cr ratios were also decreased in ALS patients compared with normal controls, with significant differences in the SWM and PV but not in PIC. Combined whole-brain DTI and MRSI can detect axonal degeneration in ALS. Measurements of FA obtained in the SWM, CS, PV and PIC, and NAA/Cr ratios in the SWM and PV yield the most robust results.
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Journal of neurology · Sep 2004
ReviewOptimising MS disease-modifying therapies: antibodies in perspective.
A proportion of people with multiple sclerosis (MS) treated with interferon (IFN) a develop neutralising anti-IFN beta antibodies (NABs). The immunogenicity of the available commercial compounds relates to the genetic structure of the IFN beta molecule, its mode of production, glycosylation status, aggregate formation, commercial formulation, potency, dose, frequency and, possibly, route of administration. At present, it is not possible to predict who will develop NABs usually appear within the first 2 years of starting therapy. ⋯ One could argue that when comparing the 'true' clinical efficacy of different IFN beta products, the comparisons should be limited to the cohorts that remain NAB-negative. As a corollary, the therapeutic efficacy of IFN beta could be maximised if patients who tolerate higher-dose preparations could be prevented from developing persistent NABs. Strategies employed to prevent or reverse the development of NABs with other biological compounds (e. g. insulin, factor VIII, IFN beta, recombinant human erythropoietin) include improvements in the manufacturing process, immunosuppression, induction of tolerance and deimmunisation, and these should be considered in relation to biological DMT therapy as part of future clinical studies.
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Journal of neurology · Aug 2004
Comparative StudyCognitive changes in patients with Huntington's disease (HD) and asymptomatic carriers of the HD mutation--a longitudinal follow-up study.
Objective information about the onset and progression of cognitive impairment in Huntington's disease (HD) is very important in the light of appropriate outcome measures when conducting clinical trials. Therefore, we evaluated the progression of cognitive functions in HD patients and asymptomatic carriers of the HD mutation (AC) over a 2.5-year period. We also sought to detect the earliest markers of cognitive impairment in AC. ⋯ Tasks measuring mainly attention, object and space perception and executive functions adequately assess the progression of HD disease. Other cognitive functions do not significantly deteriorate. Furthermore, problems in attention, working memory, verbal learning, verbal long-term memory and learning of random associations are the earliest cognitive manifestations in AC.
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Journal of neurology · Aug 2004
Comparative StudyIdiopathic restless legs syndrome: abnormalities in central somatosensory processing.
Neurophysiological studies have shown an impairment of temperature perception in secondary and idiopathic restless legs syndrome (RLS). It is unclear whether these deficits are caused by peripheral nerve fibre damage or by central impairment of somatosensory processing. The aim of the present study was (1) to determine the frequency of thermal hypaesthesia in a large population of secondary and idiopathic RLS patients; (2) to differentiate between a peripheral and central disturbance of somatosensory processing and (3) to correlate these findings with the clinical manifestation of the disease. ⋯ Impairment of temperature perception is present in a high percentage of RLS patients. In secondary RLS the sensory deficits are at least in part caused by small fibre neuropathy. In idiopathic RLS a functional impairment of central somatosensory processing is present.
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Journal of neurology · Jul 2004
Case ReportsDiffusion-weighted MRI of spinal cord infarction--high resolution imaging and time course of diffusion abnormality.
Infarction is a rare cause of spinal cord dysfunction. Whereas diffusion-weighted (DW) MRI has been established as a highly sensitive technique for assessing acute cerebral ischemia, its role in spinal cord infarction remains to be determined. The purpose of this study is to present the signal characteristics of acute spinal cord ischemia using DWMRI within the first two days and after one week. ⋯ Restricted diffusion in the course of spinal cord ischemic infarction can be demonstrated using DW-MRI. Whereas a diffusion abnormality can be found after few hours, it does not last for longer than one week. At this time, the establishment of the diagnosis has to rely mainly on T2-weighted images with additional post contrast T1-weighted images being useful.