Abstract
Motor neuron disease is characterised by features of both lower and upper motor neuron dysfunction (Swash and Schwartz 1992). Thus, there is atrophy, weakness and fasciculation, with hyperreflexia, extensor responses and, in some cases, spasticity. These clinical features vary in their distribution at different stages of the disease and in many patients the disease seems to commence asymmetrically, often involving only one limb, or predominantly to involve bulbar muscles or limb muscles. Fasciculation or muscle cramp sometimes precedes the development of more florid upper and lower motor neuron features and in some patients fatigue is a prominent symptom. In about 10% of patients sensory symptoms, mainly consisting of paraesthesiae, are part of the clinical syndrome. These major clinical features of the disease are accompanied by characteristic neurophysiological abnormalities and the latter may be used to assess the extent, severity and rate of progression of the disease (Swash and Schwartz 1984; Stalberg and Sanders 1984).
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Schwartz, M.S., Swash, M. (1995). Neurophysiological Changes in Motor Neuron Disease. In: Leigh, P.N., Swash, M. (eds) Motor Neuron Disease. Springer, London. https://doi.org/10.1007/978-1-4471-1871-8_14
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DOI: https://doi.org/10.1007/978-1-4471-1871-8_14
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