Movement Disorders in Children & Adolescents GP Mathur, Sarla Mathur
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1Movement Disorders in Children and Adolescents
2Movement Disorders in Children and Adolescents
GP Mathur MD (Med), MD (Ped), DCH, FIAP, FIMSA Former Professor and Head Department of Pediatrics GSVM Medical College, Kanpur Sarla Mathur MD, DCH Professor and Head Department of Pediatrics Subharati Medical College, Meerut Former Professor and Head Department of Pediatrics GSVM Medical College, Kanpur
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Movement Disorders in Children and Adolescents
└ 2003, GP Mathur, Sarla Mathur
All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editors and the publisher.
First Edition : 2003
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4to
the fond memory of our revered
parents and in the memory of
our beloved teacher
Prof RS Dayal
5Contributors 7Preface
Neurological disorders of movement in children have been recognized for hundred of years in the classical descriptions of disease by Parkinson, Huntington and Sydenham. The interest in movement disorders of children and adolescents has been more recent. Unlike the situation in adults, several of the movement disorders described in children are characterized by transient states that last for days, weeks, or months with complete (or almost complete) clinical resolution with subsequent nervous system maturation. In otherwise normal infants, choreiform, choreoathetoid and dystonic movements may be apparent for several weeks or months with subsequent resolution and normal development. For some unknown reason, children may be particularly sensitive to medications, producing usually a transient dystonic state. Neuroleptic agents such as haloperidol, pimozide or fluphenazine and antiemetics such as metoclopramide are well known to produce a dyskinetic syndrome with dystonia, oculogyric manifestations, trismus, and rigidity. These effects may follow an initial, often small dose, and are commonly mistaken in the emergency room for tetanus or seizures.
Movements are achieved by contraction and relaxation of skeletal muscles. The motor unit is the final common pathway through which spinal, cerebral and cerebellar control systems act to coordinate movements.
Movements and its control is carried out through the integrated approach of pyramidal system, extrapyramidal system, cerebellum, sensory system, reflex activity and receptors. The extrapyramidal system and particularly the basal ganglia are believed to be the main sites of dysfunction, although the pathophysiology is often poorly understood.
Movement disorders in children and adolescents constitute a wide range of conditions with varied etiology. In these disorders there are abnormal involuntary movements, without loss of muscle strength. The type of movement disorders assists in localization of the pathologic process. On the other hand onset, age and degree of the abnormal motor activity and associated neurologic findings help to classify the disorder and in planning the relevant investigations. Movement disorders are rarely limited to one form. Usually more than one type of movement disorders is seen in an individual.
Because the precise pathologic, pharmacologic, and neurophysiologic, mechanisms of movement disorders are not completely understood, clinical features provide the most reliable basis for their classification and differentiation.
Although many of the movement disorders affecting children and adolescents are rare individually, collectively they represent an important and increasingly recognized presentation of a childhood condition that may respond to specific pharmacologic agents. There is increasing recognition of the molecular and genetic basis of many of these conditions, which has raised hopes that specific substances or gene replacement therapies may be available in the near future
8We are highly indebted to the contributing authors for their invaluable contribution.
We especially wish to express our heartfelt thanks to Dr Sumit Mathur MBBS, DLO for reviewing the manuscript of this book. Our appreciation is extended to Mr Bharat Bhshan Vohra for the great care bestowed in typing the manuscript.
Our publishers, Jaypee Brothers Medical Publishers (P) Ltd. and their dedicated staff, deserve our special “thanks” for the skillful production qualities of the book and for bringing it out in a record time.
GP Mathur
Sarla Mathur