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The corticomotoneuronal system is selectively vulnerable in amyotrophic lateral sclerosis (ALS). In the past it has been difficult to evaluate the upper motor neuron component of this system. Recent studies using functional imaging and neurophysiological methods are now available as potential...
The frequency of familial amyotrophic lateral sclerosis (ALS) is usually reported as 510% of all ALS cases. This figure is probably an underestimate, primarily due to inadequate recording of family history in the patients charts, and to the not infrequent occurrence of reduced disease...
Significant changes in the epidemiology of amyotrophic lateral sclerosis (ALS) have been observed over the last few years. Based on reports of studies from the 1970s and early 1980s, the incidence of ALS in Europe has been considered to be constant, with only slight differences in the incidence...
Early diagnosis is particularly relevant in the diagnosis of amyotrophic lateral sclerosis (ALS) as new therapies, which may have a direct impact on the outcome of the disease, are being developed. Many ALS patients go to non-neurological specialists rather than neurologists for their first...
The challenge of identifying patients at the earliest stage of disease has highlighted the role of subtle markers of clinical pathology. Electrophysiological changes usually precede evidence of clinical weakness and have, therefore, been implicated. Specifically, the onset of fasciculations,...
While the early diagnosis of disease is generally desirable, this is not always so. There is reason to be concerned about the reliability of clinical diagnosis of amyotrophic lateral sclerosis (ALS), based on currently available methods, early in the disease process. Applying the standard of...
Advantages of early diagnoses in amyo- trophic lateral sclerosis include validation of symptoms, avoidance of unnecessary procedures, enhanced preparation for disability and medical education. Most importantly, earlier in the course of the diseasewhile there is a greater motor neuron pool...
Patient-specific (endogenous) and population-specific (exogenous) risk factor analysis is identifying novel physical and chemical exposures which might be time-linked to the development of amyotrophic lateral sclerosis (ALS) and other motor neuron diseases. Electric injury in a number of...
In amyotrophic lateral sclerosis (ALS), muscles with preserved strength can have fasciculation potentials (FPs) as the only abnormality. The FPs in strong muscles are predominantly simple and stable, and can often be recruited by a slight voluntary contraction. In weak and atrophic muscles, the...
The 2nd Consensus Conference (Versailles) recommended that an ALS knowledge-base for initial healthcare providers, diagnosing neurologists and confirming neurologists should be defined to include a simplified version of diagnostic criteria less formal than the World Federation of Neurology El...
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