The Electromyographic, Physiopathogenetic Evaluation of Amyotrophic Lateral Sclerosis
In our present state of knowledge, we lack a specific definition and clinical classification of idiopathic ALS. Its borders vanish among different forms of spinal muscular atrophy (SMA) or lateral sclerosis; moreover its differentiation from hereditary ALS, phenocopies and exogenous similar syndromes is hardly possible except by the identification of the respective etiologies. A pathognomonic marker of primary sporadic ALS seems to be represented by the increase in c. s. f. of thiamine/thiamine monophosphate (T/TMP). On the other hand a goal-directed electromyographic investigation complete with histochemical, neurobiological and radiological examinations can allow a precise study of the single motor neuron in its whole life-span during the course of the disease. If such a longitudinal investigation could be carried out in patients affected by different types of motor neuron pathology, specific aspects of the neurobiological processes occurring in ALS could be better defined and their relationship with the physiopathogenesis of the disease could probably be discussed.
KeywordsAmyotrophic Lateral Sclerosis Motor Neuron Motor Unit Spinal Muscular Atrophy Motor Neuron Disease
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