Amyotrophic Lateral Sclerosis and the Frontotemporal Dementias

Description

• Chapter authors are internationally respected authorities in the field
• Full colour illustrations and photomicrographs are used throughout the book to illustrate key points
• The book is thoroughly referenced throughout

Over the past ten years, there has been an increasing recognition that syndromes of frontotemporal dysfunction (FTD) are a common occurrence in patients with amyotrophic lateral sclerosis (ALS). Such syndromes may be present in as many as 60% of patients with ALS. Conversely, the occurrence of motor neuron dysfunction in patients with clinically pure frontotemporal dementia is increasingly recognized. This suggests that to some extent there are overlapping syndromes in which both ALS and FTD occur within the same individual.
This volume summarizes the advances in our understanding of these two disorders, as well as the potential relationship between the two. Key topics include advances in our ability to clinically describe the frontotemporal syndromes, preclinical detection, neuroimaging, and genetics. The exploding field of new markers in neuropathology is examined, as is the role of new genetic mutations in DNA/RNA transport systems. This book is the essential reference text for this topic, and will be of interest to neurologists and neurological trainees with a clinical or research interest in the FTDs or ALS, neuropsychologists, neuropathologists, and researchers.

Readership: This book is aimed at neurologists and neurological trainees, and will also be of interest to neuropsychologists, neuropathologists, and researchers.

Table of Contents

1: Ralph M. Garruto: Lessons from the study of natural experiments of hyperendemic foci of neurodegeneration
2: Jeffrey Rosenfeld: The motor neuron diseases
3: Shigeki Kuzuhara and Yasumasa Kokubo: Amyotrophic lateral sclerosis-Pakinsonism-Dementia complex in the Kii peninsula of Japan (Muro disease): a review on recent research and new concept
4: Alexandre Henri-Bhargava and Morris Freedman: The frontotemporal dementias: an overview
5: J. B. Orange and A. E. Hillis: Language profiles in amyotrophic lateral sclerosis
6: Hiroo Ichikawa, Sotaro Hieda, Hideki Ohno, Kenji Ishihara, and Mitsuru Kawamura: Language impairment in amyotrophic lateral sclerosis from an historical review: kana and kanji versus alphabetical languages
7: Mitsuru Kawamura and Hiroo Ichikawa: Amyotrophic lateral sclerosis with dementia: neuropsychological aspects
8: Sharon Abrahams and Christopher Kipps: Social cognition in frontotemporal dementia and amyotrophic lateral sclerosis
9: Laura H. Goldstein: Behavioural change in amyotrophic lateral sclerosis
10: Jennifer Murphy, Fizaa Ahmed, and Catherine Lomen-Hoerth: Cognition in amyotrophic lateral sclerosis
11: Vincenzo Silani, Barbara Poletti, and Stefano Zago: Frontotemporal syndromes of primary lateral sclerosis
12: Jeremy M. Shefner: Electrophysiological evaluation of lower motor neuron loss in amyotrophic lateral sclerosis
13: Jan Kassubek and Albert C. Ludolph: Multimodality approach to neuroimaging in amyotrophic lateral sclerosis/frontotemporal dementia
14: Sandra E. Black and Yana Yunusova: Using neuroimaging to understand brain-behaviour relationships in the context of motor neuron disease
15: S. Kalra: Amyotrophic lateral sclerosis and the frontotemporal dementias: using neuroimaging to quantitate disease progression
16: Tiffany W. Chow: Differentiating normal from pathological atrophy: when is frontotemporal atrophy normal?
17: Robert Bowser, James Connor, and Martin Turner: Cerebrospinal fluid-based biomarkers for amyotrophic lateral sclerosis
18: Nigel J. Cairns: Neuropathology of frontotemporal lobar degeneration
19: J. Robin Highley and Paul G. Ince: The neuropathology of the motor neurone diseases
20: Bryan J. Traynor and Stuart Pickering-Brown: Genetics of frontotemporal dementia
21: Ashley Jones and Ammar Al-Chalabi: Genetics of the MNDs
22: Kathryn Volkening and Michael J. Strong: Perturbed RNA metabolism in amyotrophic lateral sclerosis
23: Michael J. Strong, Wencheng Yang, May Gohar, and Wendy L. Strong: Alterations in tau metabolism in amyotrophic lateral sclerosis with cognitive impairment
24: Emanuele Buratti: Transactive response DNA-binding protein of 43 kDa
25: Jennifer Gass and Leonard Petrucelli: Progranulin

Author Information

Edited by Michael J. Strong, Dean, Schulich School of Medicine, The University of Western Ontario, Canada

Dr. Michael Strong is Dean of the Schulich School of Medicine and Dentistry at the University of Western Ontario, a Distinguished University Professor and the Arthur J. Hudson Chair in ALS Research. Dr Strong is a scientist at the Robarts Research Institute, and has also served as co-chair of the Canadian ALS Research Consortium and is a former member of the Board of Directors of the ALS Society of Canada. His basic research focuses on the study of alterations in neurofilament metabolism in degenerating motor neurons in ALS and how perturbations in RNA metabolism contribute to this process. His clinical research has been central to identifying the frontotemporal syndromes of ALS.