tailieunhanh - Handbook of Clinical Neurology Vol. 82_2

This sentiment was well expressed by Charcot in one of his many teaching sessions on amyotrophic lateral sclerosis (ALS). It holds as true today as it did in 1865 and the search must continue but progress has been incredible in recent years. There has been an exponential increase in the number of publications dealing with ALS and motor neuron diseases in the last 50 years, as evidenced by listings in PubMed and related data bases. The Editors extend their utmost thanks to the internationally renowned experts that have contributed to this volume. They have helped create an in-depth reference on motor neuron diseases that is. | Handbook of Clinical Neurology Vol. 82 3rd series Motor Neuron Disorders and Related Diseases . Eisen PJ. Shaw Editors 2007 Elsevier . All rights reserved Chapter 11 Monomelic amyotrophy of upper or lower limbs M. GOURIE-DEVI Institute of Human Behaviour and Allied Sciences and Department of Clinical Neurophysiology Sir Ganga Ram Hospital New Delhi India . Introduction Monomelic amyotrophy in which neurogenic atrophy is restricted to one limb is a heterogenous disorder involving one upper or lower limb. Insidious onset of atrophy and weakness presumed to be due to anterior horn cell involvement starting in the second or third decade with male preponderance and sporadic occurrence are the characteristic features. Progression is slow and followed by stabilization within a few years resulting in a benign outcome. Cranial nerves pyramidal sensory cerebellar and extrapyramidal systems are not involved. Hirayama et al. 1959 from Japan reported atrophy of a single upper limb and labeled it as juvenile muscular atrophy of unilateral upper extremity. Prabhakar et al. 1981 from India reported atrophy of muscles of one lower limb and described it as wasted leg syndrome. Since either one upper or lower limb is affected Gourie-Devi et al. 1984a 1986 suggested the eponym monomelic amyotrophy MMA as a more appropriate term. The authors further suggested that upper limb MMA may be called brachial monomelic amyotrophy to differentiate it from MMA of a lower limb which may now be called crural monomelic amyotrophy Gourie-Devi and Nalini 2003 . Focal amyotrophy has been described under a variety of descriptive names which refer to the limb involved the site of muscles affected and the benign and non-progressive course of the disease Table . . Monomelic amyotrophy of upper limb More than 300 cases have been reported from Japan Hirayama et al. 1963 Hashimoto et al. 1976 Sobue et al. 1978 Hirayama 2000a . The atrophy was distal and segmental confined to one upper .

• Y học thường thức
• Historical aspects
• neuron diseases
• Notable names
• Western Pacific ALS
• Comparative anatomy
• corticospinal system