Open Access Medical Books



Edited by Alvaro G. Estévez .

264 pages .
Open Access .
ISBN 978-953-51-1195-5 .

The research in amyotrophic lateral sclerosis (ALS) has dramatically increased in the last 20 years, greatly improving our understanding of the pathological mechanisms involved in the disease. The number of hypothesis on the pathogenic causes of ALS and the mechanisms of motor neuron death keeps growing. Twenty years ago there was a general agreement that ALS was a motor neuron disease. All other symptoms were secondary to the primary cause that was the death of upper and lower motor neurons. Today there are several hypotheses suggesting that dysfunction in other cell types, such as astrocytes, is the primary cause of the disease that leads to motor neuron death. Twenty years ago there were not animal models for ALS, now there are multiple rodent and non-mammalian models of the disease.
The more accepted hypotheses for the causes of ALS are reviewed in the first chapter by Rossi and coauthors. This chapter reviews the potential pathogenic mechanism involved in the etiology of ALS providing short description of each mechanism.
The chapter by Shin and Lee reviews the cellular mechanisms found to potentially participate in the degeneration of motor neurons in ALS. This chapter provides a cellular approach to the proposed mechanisms of motor neuron death in ALS, supporting the hypothesis that multiple molecular pathways could be involve in motor neuron death in different patients.
Diamanti and collaborators provide further support for the hypothesis of multiple molecular mechanisms in ALS by reviewing correlations between genotype and disease presentation.
Using studies of Italian ALS patients, the authors provide evidence showing the correlation between different phenotypic presentations of ALS with known genetic causes of the disease.
The chapter by Bowerman and collaborators reviews up to day evidence of the role of neuroinflammation
in the pathology of ALS. The role of astrocytes and other inflammatory cells is reviewed in detail. The authors also discuss in depth the possible inflammatory mediators and molecular mechanism involved in the induction of motor neuron death.
Franco and collaborators review the highly controversial participation of oxidative stress in the pathology of ALS. The chapter focuses on the oxidative mechanisms proposed to explain the gain-of-function of ALS-linked mutant SOD.
Ferraiuolo and coauthors provide a new interpretations of the ALS literature based on the recent discovery that a plethora of new genetic mutations affecting a variety of genes are linked to ALS. The review provides an unique perspective on the strengths and weaknesses of classic and new animal models of the disease based on performance and similarities to the human disease.
The chapter by Aggarwal describes studies in at risk presymtomatic individuals carrying mutant SOD. The research uses MUNE to determine motor neurons status and the therapeutic benefits of riluzole. The methods described in this chapter could provide and early diagnosis in patients carrying mutations linked to ALS allowing presymtomatic treatments.
Bocci and collaborators found by Macro-EMG, that there is a possible compensatory overbranching in ALS motor neurons, which provides a possible explanation for the adult onset of the disease.
Abe and collaborators provided a review of the last advances of eye-gaze systems, which allows ALS patient to communicate after almost other voluntary mobility is lost. The technology reported here is relatively inexpensive and for those patients unable to communicate by other means could represent a significant improvement on quality of life.

Alvaro G. Estévez, Ph.D.
Burnett School of Biomedical Sciences,
College of Medicine,
University of Central Florida,


 1 Pathophysiology of Amyotrophic Lateral Sclerosis 1 Fabian H. Rossi, Maria Clara Franco and Alvaro G. Estevez

 2 Multiple Routes of Motor Neuron Degeneration in ALS 35 Jin Hee Shin and Jae Keun Lee

 3 Genetics of ALS and Correlations Between Genotype and Phenotype in ALS — A Focus on Italian Population 73 L. Diamanti, S. Gagliardi, C. Cereda and M. Ceroni

 4 The Neuroinflammation in the Physiopathology of Amyotrophic Lateral Sclerosis 97 Melissa Bowerman, Thierry Vincent, Frédérique Scamps, William Camu and Cédric Raoul

 5 Superoxide Dismutase and Oxidative Stress in Amyotrophic Lateral Sclerosis 141 María Clara Franco, Cassandra N. Dennys, Fabian H. Rossi and Alvaro G. Estévez

 6 The Use of Human Samples to Study Familial and Sporadic Amyotrophic Lateral Sclerosis: New Frontiers and Challenges 159 Laura Ferraiuolo, Kathrin Meyer and Brian Kaspar

 7 The Role of the Statistical Method of Motor Unit Number Estimation (MUNE) to Assess the Potential Therapeutic Benefits of Riluzole on Patients with Pre-symptomatic Familial Amyotrophic Lateral Sclerosis 183 Arun Aggarwal

 8 Changes in Motor Unit Loss and Axonal Regeneration Rate in Sporadic and Familiar Amyotrophic Lateral Sclerosis (ALS) — Possible Different Pathogenetic Mechanisms? 227
Tommaso Bocci, Elisa Giorli, Lucia Briscese, Silvia Tognazzi, Fabio Giannini and Ferdinando Sartucci

 9 Eye-Gaze Input System Suitable for Use under Natural Light and Its Applications Toward a Support for ALS Patients 243 Abe Kiyohiko, Ohi Shoichi and Ohyama Minoru .

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Published by: Unknown - Monday, September 16, 2013


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