Preface

Chapter 8 **Tiny microRNAs Fine-Tune Amyotrophic Lateral Sclerosis**

Chapter 9 **Selective Vulnerability of Neuronal Subtypes in ALS: A Fertile**

Chapter 10 **Neuronal Intermediate Filaments in Amyotrophic Lateral**

Chapter 11 **Trophic Factors in the Therapeutic Challenge Against ALS:**

Chapter 12 **Molecular Taxonomy: Use of Transcriptional Profiles to Identify**

Chapter 13 **Amyotrophic Lateral Sclerosis: Role of the Canonical Wnt/Beta-**

**Current Research Directions 213** Anna Sobuś and Bogusław Machaliński

**Different ALS Subtypes 229**

Spampinato and Sebastiano Cavallaro

**Catenin Pathway and PPAR Gamma 257** Yves Lecarpentier and Alexandre Vallée

Utpal Bhadra, Anisha Pal, Jagamohan Chhatai and Manika Pal

**Ground for the Identification of Therapeutic Targets 165** Cylia Rochat, Nathalie Bernard-Marissal and Bernard L. Schneider

Philippe Codron, Julien Cassereau, Joël Eyer and Franck Letournel

Giovanna Morello, Francesca Luisa Conforti, Antonio Gianmaria

**Regulation 135**

**Sclerosis 195**

Bhadra

**VI** Contents

Amyotrophic Lateral Sclerosis (ALS) causes 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 the degree of neurodegeneration. ALS is also related to mitochondrial dys‐ functions, increased oxidative stress and atypical protein assemblies and continues being a serious health problem leading to death in a few years' time in most of the cases. Before death, patients suffer from weakness or paralysis, muscle atrophy, fasciculation, dysphagia, dysarthria, and other complications.

The introductory chapter presents, summarised, information about the progress made in ALS and published by InTech.

The information will be grouped by topics and countries in two graphics.

The number of publications written about ALS increased remarkably over the past four years. With new information consistently being presented regarding this disease, it is our hope that the information in this book is a representation of novel research to benefit the readership community and our patients. In this book, readers will find a compilation of state-of-the-art reviews about pathogenesis, aetiology, therapeutic, investigations, the mo‐ lecular basis of disease progression and clinical manifestations, and the genetics familial ALS, as well as novel diagnostic criteria in the field of imagenology.

Update on Amyotrophic Lateral Sclerosis is comprised of 13 chapters from some of the world's top central nervous system researchers and neurologists to provide a timely review of the most recent developments in ALS, covering historic aspects, experimental animal models, genetics, pathogenesis, clinical aspects and imagenology among others. Contribu‐ tors from Belgium, France, Japan, India, Italy, Mexico, Russia, South Africa, and Switzerland have collaborated enthusiastically and efficiently, dedicating their time to create this readerfriendly yet comprehensive work which includes many explanatory figures, tables and pho‐ tos to enhance legibility and make the book clinically useful.

It is important to highlight that a lot of effort has been made to ensure the relevance of this book. After the initial review process, all chapters were peer reviewed at least two more times before final acceptance for publication. This was done in effort to ensure that the most novel information was provided to readers and researchers. We all attempted to bring in valuable updated information for all issues/topics mentioned in this book.

First of all, we would like to thanks InTech Open Access Publisher that unconditionally sup‐ ported us in editing this book. Our families who supported us during this process. My Mom, Dad and my first daughter Zayra Susana, from heaven, who continue to inspire me.

My daughters Lorna Maria (32 years old) and Fatima Susana Adolfina (7 years old) who encouraged me all the time to continue moving forward with persistence. My son Thabo Humberto Jorge (8 years old) who pushed me to play physics games with him which helped me to relax and to find new ideas. My whole family contributed to this project in one way or another and they deserve our deep gratitude. I also want to thank the families, relatives, and friends of all contributors for their support. Special thanks to Walter Sisulu University (WSU), named in honour of an icon of the South Africa liberation struggle and close com‐ rade of Nelson Mandela, the late Walter Max Ulyate Sisulu. Many thanks to Dr. EN Cishe: Acting Director Research Development of WSU, Dr. WW Chita: Dean of the Faculty of Health Sciences (WSU), Prof. A Awotedu: Head of Department of General Medicine and Therapeutic, Dr. M Mdledle: Acting Governor General Director: Clinical Governance of Nel‐ son Mandela Central Hospital and Mrs. NP Makwedeni: Chief Executive Officer of Nelson Mandela Central Hospital for the best understanding and support. At the end, I extend my deepest sense of appreciation for the support received by Dr. Roberto Morales Ojeda: Minis‐ ter of Public Health of Cuba and Dr. Jorge Delgado Bustillo: Deputy Head of the National Unit for International Cooperation in Health.

#### **Prof. H Foyaca Sibat. MD, PhD, MSc,**

First and Second Degree Specialist in Neurology Full Scientist Research Head of Department of Neurology Nelson Mandela Central Hospital, Faculty of Health Sciences, WSU Mthatha, South Africa

**Dr. Lurdes de Fatima Ibañez Valdés. MD, MSc, Family Physician**

Aggregate Scientist Research Department of Neurology, Nelson Mandela Central Hospital. Walter Sisulu University. Mthatha, South Africa.
