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.

1. Lessons from the study of natural experiments of hyperendemic foci of neurodegeneration. 2. The motor neuron diseases. 3. Amyotrophic lateral sclerosis-Pakinsonism-Dementia complex in the Kii peninsula of Japan (Muro disease): a review on recent research and new concept. 4. The frontotemporal dementias: an overview. 5. Language profiles in amyotrophic lateral sclerosis. 6. Language impairment in amyotrophic lateral sclerosis from an historical review: kana and kanji versus alphabetical languages. 7. Amyotrophic lateral sclerosis with dementia: neuropsychological aspects. 8. Social cognition in frontotemporal dementia and amyotrophic lateral sclerosis. 9. Behavioural change in amyotrophic lateral sclerosis. 10. Cognition in amyotrophic lateral sclerosis. 11. Frontotemporal syndromes of primary lateral sclerosis. 12. Electrophysiological evaluation of lower motor neuron loss in amyotrophic lateral sclerosis. 13. Multimodality approach to neuroimaging in amyotrophic lateral sclerosis/frontotemporal dementia. 14. Using neuroimaging to understand brain-behaviour relationships in the context of motor neuron disease. 15. Amyotrophic lateral sclerosis and the frontotemporal dementias: using neuroimaging to quantitate disease progression. 16. Differentiating normal from pathological atrophy: when is frontotemporal atrophy normal?. 17. Cerebrospinal fluid-based biomarkers for amyotrophic lateral sclerosis. 18. Neuropathology of frontotemporal lobar degeneration. 19. The neuropathology of the motor neurone diseases. 20. Genetics of frontotemporal dementia. 21. Genetics of the MNDs. 22. Perturbed RNA metabolism in amyotrophic lateral sclerosis. 23. Alterations in tau metabolism in amyotrophic lateral sclerosis with cognitive impairment. 24. Transactive response DNA-binding protein of 43 kDa. 25. Progranulin.

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.