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Shy-Drager syndrome

Shy-Drager syndrome is a rare, progressively degenerative disease of the autonomic nervous system. It is named after Dr Milton Shy and Dr Glenn Drager, who identified this syndrome in 1960.

It was officially deprecated as a disease entity in 1996, and existing cases were redefined as multiple system atrophy with autonomic phenomena. The name "Shy-Drager syndrome" is still used occasionally for multiple system atrophy when the primary symptoms are autonomic failure.

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Parkinson's Disease: Methods and protocols
From Neurological Research, 12/1/01 by Agner, Celso

Parkinson's Disease: Methods and Protocols Editor. Maral Mouradian Publisher: Humana Press, Totowa, NJ, 309 pp, 2001 ISBN: 0-89603-761-4

Over the past three decades, research on the pathogenesis of Parkinson's disease has developed significantly. Novel treatment techniques, as well as new animal models of Parkinson's disease were developed over the past few years, particularly based on animal and clinical research. A thorough explanation of the current side-effects or complications of treatment is required. This book approaches this much needed topic from the experimental point of view.

Several authors contribute four sections and twenty-one chapters in this book. The first section explains the basic genetics of Parkinson's disease, particularly relating to point mutations of a-synuclein and the autosomal recessive juvenile Parkinson's disease. These particular concepts aim towards the possible initial spontaneous or induced mutations of genes that will ultimately control the precision of fine movements by patients. It also addresses the potential for experimental research with animal models of the disease.

The second section deals with the current protocols on molecular pathogenetic studies In particular, the chapters in this part are devoted to mutations in a-synuclein and the interaction between this protein and amyloid, which would, in part, explain the nature of diseases like multiple system atrophy (former Shy-Drager syndrome), Lewy body dementia, and others, in which Parkinson's is just one of the features presented. Moreover, the section addresses the controversial issue of dopamine-induced apoptosis, a main cause for clinical deterioration in some patients, even with attempted change in the medication profile. The last part of this section addresses the role of nitric oxide and of active components of oxygen on the development of lesions in the substantia nigra, which is also directly related to the progression of the disease observed in many populations.

The molecular aspects of basal ganglia function is addressed in section three. Transcription mechanisms of dopamine receptor genes, immunochemistry of dopamine transporters, and quantification of tyrosine hydroxylase mRNA is also approached from the experimental point of view. In many chapters, the information goes beyond the protocol format. The figures are opportune and readily explain the topic.

In the last section, newer molecular therapies are consistently explained. The role of genetic therapy, as well as of cell implants (including stem cell implant) is not just a perspective but also a current reality. Neurotransplantation is a popular topic and is the trend in management of neurological disorders, as drugs may frequently accelerate the disease.

This book is a well-written material that should be a reference in neurology, neuroscience, and neurosurgical libraries. The future of management of Parkinson's and other degenerative disorders of the nervous system relies on the kind of research exposed in this book.

Celso Agner, MS, MD

Copyright Forefront Publishing Group Dec 2001
Provided by ProQuest Information and Learning Company. All rights Reserved

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