Amyotrophic Lateral Sclerosis and the Frontotemporal Dementias

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.

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.