Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
-
Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Dec 2005
Global brain atrophy and corticospinal tract alterations in ALS, as investigated by voxel-based morphometry of 3-D MRI.
In ALS, advanced magnetic resonance imaging (MRI) techniques are increasingly used to investigate the underlying pathology. In this study, the technique of voxel-based morphometry (VBM) was applied to 3-D MRI data in ALS patients to localize regional grey and white matter changes. Twenty-two ALS patients (mean age 58+/-9 years) with clinically definite ALS by revised El Escorial criteria were studied. ⋯ Furthermore, regional white matter alterations were observed along the corticospinal tracts bilaterally and in multiple smaller areas including corpus callosum, cerebellum, frontal and occipital subcortical regions. Besides considerable global atrophy in ALS, the topography of ALS-associated cerebral morphological changes could be mapped using VBM, in particular white matter signal changes along the bilateral corticospinal tracts, but also in extra-motor areas. VBM might be a potential tool to visualize disease progression in future longitudinal studies.
-
Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Jun 2005
Comparative StudyComplications and survival following radiologically and endoscopically-guided gastrostomy in patients with amyotrophic lateral sclerosis.
In the natural progression of amyotrophic lateral sclerosis (ALS), a state of malnutrition often develops, associated with reduced oral intake, caused by difficulties with swallowing, adequate salivation, and/or anorexia. The placement of a feeding tube should be considered at the point where patients demonstrate swallowing difficulty and/or alteration in nutritional status. The goal of this prospective work was to compare, in 50 patients having definite or probable ALS, the complications after endoscopic (PEG), or radiologically-guided gastrostomy (RIG) and to evaluate their effects on survival. ⋯ Kaplan-Meier survival curves from the date of gastrostomy placement were not different in univariate analysis (p=0.85). In multivariate analysis, survival was not different between one group and the other (p=0.28). The major interest of the RIG technique rests on its greater feasibility and on the possibility of utilizing it in ALS patients who have significant ventilatory compromise.
-
Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Dec 2004
Comparative StudyChanges in the management of ALS since the publication of the AAN ALS practice parameter 1999.
To determine if the publication in 1999 of the AAN Practice Parameter on ALS was associated with an improvement in the standard of management of the disease. ⋯ The publication of the AAN Practice Parameter was associated with improvement in the standard of care. Most cases in the database come from specialized ALS centers, and further information on the community care of ALS patients is needed.
-
The ALSFRS-R is an attractive primary outcome measure in clinical trials of ALS because it is validated, easy to administer, minimizes dropout, reduces cost, and correlates with survival. Unlike the other standard outcome measures currently employed, the ALSFRS-R is also a measure of global function.
-
Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Sep 2004
Comparative StudyReduced NAA in motor and non-motor brain regions in amyotrophic lateral sclerosis: a cross-sectional and longitudinal study.
After replication of previous findings we aimed to: 1) determine if previously reported (1)H MRSI differences between ALS patients and control subjects are limited to the motor cortex; and 2) determine the longitudinal metabolic changes corresponding to varying levels of diagnostic certainty. ⋯ NAA ratios are reduced in the motor cortex and outside the motor cortex in ALS, suggesting widespread neuronal injury. Longitudinal changes of NAA are not reliable, suggesting that NAA may not be a useful surrogate marker for treatment trials.