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Chapter-04 Diseases of Anterior Horn Cell

BOOK TITLE: Neurology

Author
1. Kenkre Rajendra B
ISBN
9788184489477
DOI
10.5005/jp/books/11181_4
Edition
1/e
Publishing Year
2010
Pages
11
Author Affiliations
1. Goa, India
Chapter keywords

Abstract

There has been better understanding of anterior horn cell, anatomically, physiologically and as a relay station between spinal cord motor system and peripheral nervous system. In the future there is increased likelihood or discovery and understanding of autoimmune disease mechanism, attack on the motor neurons and muscles and their related genes. Kugelberg Welander: May be Autosomal recessive or autosomal dominant, fasciculations, are invariably present and thus is an important feature is in differential diagnosis which includes Duchenne muscular dystrophy Becker’s muscular dystrophy. In DMD and BMD there are no fasciculations. Monomelic amyotrophy worldwide distribution most patients are from Japan and India MRI cervical and upper thoracic cord atrophy. EMG: Motor nerve conduction is normal, preserved sural nerve action potentials signs of denervation are seen in most patients. Contralateral limb may also show EMG abnormalities. Acute anterior poliomyelitis muscle affectation is rare in polio. If subsequently one finds brisk reflexes marked wasting and fasciculations of intrinsic muscles of the hand it points to new pathology in ‘old polio ‘such as motor neuron disease. Postradiation motor neuron syndrome occurs months or years after X-RT treatment to cervical lumbosacral spine, atrophy, fasciculations of muscles normal sensation. Lower cranial nerves may be involved ALS dementia complex endemicity western pacific Kii Peninsula of Japan, west new guinea. No gene has been identified. Clinical features severe dementia akinetic rigid variety Parkinsonism facial (mouth) dystonia.

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