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## Meet the editors

Humberto Foyaca Sibat, born on May 3, 1948 in Havana City, Cuba, graduated as a Medical Doctor from Havana University in 1971. He has been a first degree specialist in neurology since 1975 and Second Degree Specialist of Neurology since 1984. He has also achieved his PhD, MSc, Full Professor and Full Scientist Researcher. Dr. Foyaca is working as an Associate Professor in the Faculty of Health Sciences at Walter Sisulu University (South

Africa) and Nelson Mandela Central Hospital in Mthatha as a Head of Department of Neurology since 1997. Dr. Foyaca is a member of 15 medical societies. He has presented more than 360 papers in national and international medical conferences. He has been invited to deliver key lectures several times. He has edited six books, and published more than 70 papers in peer-reviewed medical journals. Since 1992, he has been the Editor-in-Chief of the Internet Journal of Neurology.

Dr. Lourdes de Fátima Ibañez-Valdés was born on October 13, 1963 in Havana City, Cuba. She graduated as a Medical Doctor from Havana University in 1998. She is also a specialist in family medicine, has her MSc in neuro-infectology, and is an aggregated scientist researcher at the Cuban Academy of Sciences. Currently she works for Department of Neurology at Walter Sisulu University, Nelson Mandela Academic Central Hospital in Mtha-

tha, Eastern Cape Province, South Africa where she is the Head of Epilepsy and NCC-clinic. She has presented more than 60 papers at national and international medical conferences, published 8 chapters in text books and more than 40 articles in peer-reviewed journals. She is a member of 10 medical societies worldwide.

Contents

**Section 1**

Neuron Disease

*and Hema Biju*

**Section 2**

Studies on ALS *by Junling Wang*

A Brief Update *by Wei Li*

**Preface III**

Last Update on Motor Neuron Disease **1**

**Chapter 1 3**

**Chapter 2 7**

**Chapter 3 27**

**Chapter 4 51**

Muscular Atrophic **73**

**Chapter 5 75**

**Chapter 6 87**

Introductory Chapter: Introduction to Novel Aspects on Motor

*by Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés*

Contribution of Aberrant Astrocytes to Motor Neuron Damage and Death in the SOD1G93A Rat Experimental Model of ALS

*by Gabriel Otero Damianovich, Olga Cristina Parada,* 

*Carmen Isabel Bolatto Pereira and Silvia Olivera-Bravo*

*by Alok Sharma, Hemangi Sane, Nandini Gokulchandran, Prerna Badhe, Amruta Paranjape, Radhika Pradhan, Rohit Das* 

*by Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés*

Novel Information on Amyoyrophic Lateral Sclerosis and Spinal

Novel Aspects on Motor Neuron Disease: The Recent Genetic

Structural and Functional Consequences of the SMA-Linked Missense Mutations of the Survival Motor Neuron Protein:

*Pablo Díaz-Amarilla, Eugenia Eloísa Isasi,* 

Stem Cell Therapy in Motor Neuron Disease

Introduction to Novel Motor Neuron Disease

## Contents


Preface

Motor Neuron Disease (MND) continues to be a progressive loss of structure and function of neurons in the cerebral cortex, brainstem, and the spinal cord. Usually the loss of specific functions precedes the death of affected neurons, and the related clinical features depend on localization and degree of neurodegeneration. Amyotrophic Lateral Sclerosis (ALS) is the more common clinical presentation and MND is also related to mitochondrial dysfunctions, increased oxidative stress and atypical protein assemblies. MND continues to be a serious health problem leading to death in few years' times in most of the patients. Before death, patients suffer from weakness or paralysis, muscle wasting and fasciculation, dysphagia, dysarthria and several complications. There are two forms of this fatal disease: sporadic, with no known genetic component, and familial, which makes up about 5% of all ALS cases. Among the familial cases, approximately 20% are caused by dominantly inherited mutations in the Cu/Zn superoxide dismutase (SOD1) gene, with more than 100 known mutations.

This is the second book that we have edited on ALS/MND. To edit this book, we have provided the best, safest, most confident and novel science information to our readers after following a rigorous process. All chapters were screened and analysed by a strict peer-review process, followed by corrections made by authors and the Academic Editor. Then a second revision was made by authors and editors performed the final review. It is important to highlight that after the peer-review process, all chapters were reviewed twice or even more to be accepted for publication. The chapter written by the Editor was

In this book, the readers will find a compilation of state-of-the-art reviews about aetiology, therapies, investigations, the molecular basis of disease progression and clinical manifestations, and the genetics familial ALS, as well as novel diagnostic criteria.

An update aspect on ALS sourced 5 chapters from some of the world's top central nervous system researchers and neurologists to provide a timely review of the latest developments in MND/ALS, covering experimental animal models, genetics, clinical

Contributors from different countries have collaborated enthusiastically and efficiently to create this reader-friendly and comprehensive work covering the topics with many explanatory figures, tables and photos to enhance legibility and make the book clinically useful. Countless hours have gone into writing these chapters, precious free time to be dedicated to our family, relatives and friends have been sacrificed but at the end, we all

Every effort has been made to check the novel information given in this book but it is important for our readers to scrutinize other information arriving considering it is a dynamic process for learning. We all attempted to include valuable updated information for all issues mentioned in this book. Every effort has been made in the preparation and editing of this book to ensure that the information given is correct, but it is possible that errors have been overlooked. Finally, we would like to highlight that we reviewed all controversial matters and our medical criteria and scientist's opinions have been expressed with modesty, honesty and respect but the reader is advised to refer to other published information from other editorial houses and other reference works to check accuracy.

also screened by another peer-review team.

aspects and treatment options, amongst others.

are very proud of this book.
