Motor Neuron Diseases

Motor neuron diseases (MNDs) are characterized clinically by a progressive loss of muscle power brought about by the primary degeneration of lower or upper motor neurons or both. In lower MNDs, the weakness is associated with muscle wasting, fasciculation, hypo-tonia, and a decrease or loss of tendon reflexes. In upper MNDs, the weakness is associated with spasticity, hyperactive reflexes, and Babinski sign. Some diseases are sporadic, and some inherited. Individuals of all ages from birth to late midlife can be affected. The diagnosis depends primarily on the symptoms and signs. An elec-tromyogram (EMG) showing a denervation pattern confirms degeneration of the lower motor neurons.

The three groups of MNDs are: (Table 5.7): (a) ALS, entailing concurrent degeneration of upper and lower motor neurons; (b) spinal muscular atrophies, entailing degeneration of lower motor neurons only; and (c) primary lateral sclerosis and hereditary spastic paraparesis, entailing degeneration of upper motor neurons only.

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Pregnancy And Childbirth

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