Dementia and Motor Neuron Disease PDF
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00-prelims-cpp 13/6/06 1:07 pm Page i Dementia and Motor Neuron Disease 00-prelims-cpp 13/6/06 1:07 pm Page iii Dementia and Motor Neuron Disease Edited by Michael J Strong MD FRCPC Professor and Co-Chair Department of Clinical Neurological Sciences University of Western Ontario and Scientist, Cell Biology Research Group Robarts Research Institute London, ON Canada Foreword written by Andrew Kertesz MD Professor of Neurology University of Western Ontario St Joseph’s Hospital London, ON Canada CRC Press Taylor & Francis Group 6000 Broken Sound Parkway NW, Suite 300 Boca Raton, FL 33487-2742 © 2006 by Taylor & Francis Group, LLC CRC Press is an imprint of Taylor & Francis Group, an Informa business No claim to original U.S. Government works Version Date: 20130322 International Standard Book Number-13: 978-0-203-09055-8 (eBook - PDF) This book contains information obtained from authentic and highly regarded sources. While all reasonable efforts have been made to publish reliable data and information, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. The publishers wish to make clear that any views or opinions expressed in this book by individual editors, authors or contributors are personal to them and do not necessarily reflect the views/opinions of the publishers. The information or guid- ance contained in this book is intended for use by medical, scientific or health-care professionals and is provided strictly as a supplement to the medical or other professional’s own judgement, their knowledge of the patient’s medical history, relevant manufacturer’s instructions and the appropriate best practice guidelines. Because of the rapid advances in medical science, any information or advice on dosages, pro- cedures or diagnoses should be independently verified. The reader is strongly urged to consult the drug companies’ printed instructions, and their websites, before administering any of the drugs recommended in this book. This book does not indicate whether a particular treatment is appropriate or suitable for a particular individual. Ultimately it is the sole responsibility of the medical professional to make his or her own professional judgements, so as to advise and treat patients appropriately. The authors and publishers have also attempted to trace the copyright holders of all material reproduced in this publication and apologize to copyright holders if permission to publish in this form has not been obtained. If any copyright material has not been acknowledged please write and let us know so we may rectify in any future reprint. Except as permitted under U.S. Copyright Law, no part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers. For permission to photocopy or use material electronically from this work, please access www.copyright.com (http://www.copyright. com/) or contact the Copyright Clearance Center, Inc. (CCC), 222 Rosewood Drive, Danvers, MA 01923, 978-750-8400. CCC is a not-for- profit organization that provides licenses and registration for a variety of users. For organizations that have been granted a photocopy license by the CCC, a separate system of payment has been arranged. Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Visit the Taylor & Francis Web site at http://www.taylorandfrancis.com and the CRC Press Web site at http://www.crcpress.com 00-prelims-cpp 13/6/06 1:07 pm Page v Contents List of contributors.................................................................................................................................... vii Foreword Andrew Kertesz........................................................................................................................ xi Preface ...................................................................................................................................................... xiii Acknowledgments ................................................................................................................................. xiv 1. Frontotemporal dementia in ALS: lessons from history Arthur J Hudson.................................................................................................................................... 1 2. Frontotemporal dementia – current concepts Kirk C Wilhelmsen................................................................................................................................. 9 3. Clinical phenomenology and treatment of frontotemporal dementia Morris Freedman.................................................................................................................................... 23 4. The clinical and pathological spectrum of ALS Alice Brockington, Paul Ince and Pamela J Shaw................................................................................. 31 5. Identification and categorization of frontotemporal impairment in ALS Jennifer Murphy, Roland Henry and Cathy Lomen-Hoerth................................................................. 59 6. The spectrum of altered cognition in amyotrophic lateral sclerosis Stanley H Appel, George M Ringholz and Paul E Schulz................................................................... 67 7. The spectrum of cognitive dysfunction in ALS/MND in the Japanese population Makoto Tanaka and Koichi Okamoto..................................................................................................... 73 8. Primary lateral sclerosis: cognitive, language, and cerebral hemodynamic findings Gloria M Grace, Joseph B Orange, Matthew J Murphy, Ting-Yim Lee, Ann Rowe, Karen Findlater and Michael J Strong................................................................................................... 87 9. The anatomic basis of symptoms in frontotemporal dementia Christopher M Kipps.............................................................................................................................. 99 10. Neuroimaging in ALS and ALS with frontotemporal dementia Erik P Pioro............................................................................................................................................ 107 11. New approaches to imaging in ALS Sharon Abrahams, Laura Goldstein, Martin Turner, Satomi Maekawa and Nigel Leigh................... 133 12. Neuropathology of frontotemporal lobar degenerations Nigel J Cairns......................................................................................................................................... 147 13. Molecular and cellular neuropathology of cognitive dysfunction in ALS Eileen H Bigio......................................................................................................................................... 167 14. Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam Daniel P Perl.......................................................................................................................................... 177 00-prelims-cpp 13/6/06 1:07 pm Page vi vi CONTENTS 15. Neuropathology of the Japanese variants of FTD/ALS Omi Katsuse, Kenji Ikeda, Kuniaki Tsuchiya, Takashi Togo and Dennis W Dickson.......................... 193 16. The genetics of frontotemporal dementia John Hardy, Parastoo Momeni, Amanda Myers and Bryan J Traynor................................................ 201 17. Frontotemporal dementia and the involvement of tau Stuart Pickering-Brown and Leonard Petrucelli................................................................................... 209 18. Altered tau protein metabolism in amyotrophic lateral sclerosis with cognitive impairment Michael J Strong and Wencheng Yang.................................................................................................. 217 19. Frontotemporal syndromes in the motor neuron diseases Michael J Strong, Gloria M Grace, Morris Freedman, Cathy Lomen-Hoerth, Nigel Leigh, Lucie Bruijn and Paul Ince.................................................................................................................... 229 Index............................................................................................................................................................. 239 00-prelims-cpp 13/6/06 1:07 pm Page vii Contributors Sharon Abrahams PhD DClinPsy Nigel J Cairns PhD MRCPath Senior Lecturer Research Associate Professor Department of Psychology Departments of Neurology, and Pathology and The University of Edinburgh Immunology Edinburgh Director, Alzheimer’s Disease Research Center UK Neuropathology Core Washington University School of Medicine Stanley H Appel MD St Louis, MO Professor and Chairman USA Methodist Neurological Institute Houston, TX Dennis W Dickson MD USA Professor Department of Neuroscience Eileen H Bigio MD Mayo Clinic Jacksonville Professor of Pathology Jacksonville, FL Director, Division of Neuropathology USA Director, Northwestern ADC Neuropathology Care Karen Findlater BSc (PT) Northwestern University Department of Clinical Neurological Sciences Feinberg School of Medicine London Health Sciences Centre Chicago, IL London, ON USA Canada Alice Brockington MBChB MRCP Morris Freedman MD FRCPC Wellcome Research Fellow in Neurology Department of Geriatrics and Internal Medicine University of Sheffield Division of Neurology and Rotman Research Sheffield Institute UK Baycrest and Lucie Bruijn PhD Department of Medicine Science Director and Vice-President Division of Neurology The ALS Association Mount Sinai Hospital, University of Health Palm Harbor, FL Network and University of Toronto USA Toronto, ON Canada 00-prelims-cpp 13/6/06 1:07 pm Page viii viii LIST OF CONTRIBUTORS Laura Goldstein PhD MPhil Paul Ince MD Consultant Clinical Psychologist Professor of Neuropathology Department of Psychology Academic Unit of Pathology Institute of Psychiatry Division of Genomic Medicine King’s College Sheffield University London Sheffield UK UK Gloria M Grace PhD Omi Katsuse MD PhD Clinical Neurological Sciences and Yokohama Maioka Hospital Psychological Services Yokohama London Health Sciences Centre, University Japan Hospital London, ON Christopher M Kipps MBBS FRACP Canada Department of Clinical Neurosciences University of Cambridge and Addenbrooke’s John Hardy PhD Hospital Laboratory of Neurogenetics Cambridge National Institute on Aging UK Bethesda, MD USA Ting-Yim Lee PhD and Imaging Program Reta Lila Weston Institute of Neurological Lawson Health Research Institute and Imaging Studies Research Lab University College London Robarts Research Institute London London, ON UK Canada Roland Henry PhD Nigel Leigh MBBS, PhD Assistant Professor Department of Neurology Department of Radiology Institute of Psychiatry UCSF King’s College San Francisco, CA London USA UK Arthur J Hudson MD FRCPC Cathy Lomen-Hoerth MD PhD Professor Emeritus Department of Neurology Department of Clinical Neurological Sciences University of California San Francisco Faculty of Medicine San Francisco, CA University of Western Ontario USA London, ON Canada Satomi Maekawa Department of Neurology Kenji Ikeda MD PhD Institute of Psychiatry Zikei Hospital King’s College Okayama London Japan UK 00-prelims-cpp 13/6/06 1:07 pm Page ix LIST OF CONTRIBUTORS ix Parastoo Momeni PhD Leonard Petrucelli PhD Laboratory of Neurogenetics Department of Neuroscience National Institute on Aging Mayo Clinic Jacksonville Bethesda, MD Jacksonville, FL USA USA Jennifer Murphy PhD Stuart Pickering-Brown MD PhD ALS Center at UCSF Division of Laboratory and Regenerative Department of Neurology Medicine University of California San Francisco San Francisco, CA Manchester USA UK Matthew J Murphy BSc Erik P Pioro MD PhD FRCPC Imaging Program Director, Center for ALS and Related Disorders Lawson Health Research Institute Department of Neurology London, ON Cleveland Clinic Canada Cleveland, OH USA Amanda Myers PhD Laboratory of Neurogenetics George M Ringholz MD PhD National Institute on Aging Emory University School of Medicine Bethesda, MD Atlanta, GA USA USA Koichi Okamoto MD PhD Ann Rowe RN Professor and Chair Department of Clinical Neurological Sciences Department of Neurology London Health Sciences Centre Gunma University Graduate School of London, ON Medicine Canada Gunma Japan Paul E Schulz MD Baylor College of Medicine and Joseph B Orange PhD Michael E DeBakey VAMedical Center Associate Professor Houston, TX School of Communication Sciences and USA Disorders University of Western Ontario Pamela J Shaw MBBS MD FRCP London, ON Professor of Neurology Canada University of Sheffield Sheffield Daniel P Perl MD UK Professor of Pathology (Neuropathology) Mount Sinai School of Medicine New York, NY USA 00-prelims-cpp 13/6/06 1:07 pm Page x x LIST OF CONTRIBUTORS Michael J Strong MD FRCPC Kuniaki Tsuchiya MD PhD Professor and Co-Chair Department of Laboratory Medicine and Department of Clinical Neurological Sciencies Pathology University of Western Ontario Tokyo Metropolitan Matsuzawa Hospital and Tokyo Scientist, Cell Biology Research Group Japan Robarts Research Institute London, ON Martin Turner MAMRCP PhD Canada Department of Neurology The Radcliffe Infirmary Makoto Tanaka MD PhD Oxford Associate Professor UK Department of Neurology Gunma University Graduate School of Kirk C Wilhelmsen MD PhD Medicine Departments of Genetics and Neurology Clinical Professor of Neurology Carolina Center for Genome Sciences Gunma University Hospital Bowles Center for Alcohol Studies Gunma University of North Carolina Japan Chapel Hill, NC USA Takashi Togo MD PhD Yokohama City University of Medicine Wencheng Yang MD Yokohama Department of Clinical Neurological Sciences Japan University of Western Ontario and Robarts Research Institute Bryan J Traynor MD MRCPI London, ON Section on Developmental and Genetic Canada Epidemiology National Institute of Mental Health Bethesda, MD USA 00-prelims-cpp 13/6/06 1:07 pm Page xi Foreword This book reflects the growing interest in the in a large number of cases. In fact it appears in strength and nature of the association of amyo- the majority of FTD autopsies even without clin- trophic lateral sclerosis (ALS) and frontotem- ical ALS. The former entity of dementia lacking poral dementia (FTD) and it is a timely summary distinctive histology (DLDH) underlying many of the burgeoning research in the field. The FTD cases is rapidly disappearing, since most of papers were presented in the First International them turn out to have these distinctive enough Research Workshop on Frontotemporal Demen- ubiquitin positive, tau and synuclein negative tia in ALS in London, Ontario, a year ago and inclusions. ALS researchers emboldened by the the volume appears relatively quickly after the FTD groups describing ALS patients in 7–10 % of conference; a tribute to the editor and organizers their cohorts systematically found FTD and non- and the authors of course who contributed specific cognitive impairment in a fair portion of promptly. their populations. ALS patients are ill, anxious Sporadic reports of this association appeared and depressed, not an ideal group to do system- even before the last 45 years but the remarkable atic cognitive testing. Yet the numbers affected number of endemic ALS and dementia was dis- are large, even though the extent and nature of covered in Guam as Arthur Hudson summarizes deficit is variable, depending on what is being it in his introductory chapter. The topic is tested. reviewed in further detail by Daniel Perl. Patho- The terminology is not getting simpler. Many logists knew of course that the upper motor authors attempt to rename entities, sometimes to neurone is involved in the cortex since Charcot, clarify, sometimes to innovate. What was MND- but ALS was not considered to affect the mind. type Inclusion (MNDI) becomes FTD-U. FTD- Arthur Hudson wrote a much quoted article in MND becomes MND-D. ALS with cognitive Brain in 1981 about what was known of the asso- impairment is ALSci and with behavioral ciation of ALS Parkinsonism and dementia up impairment ALSbi, also mentioned as ALS-FTD until then, but it was not until the Japanese and with Alzheimer’s like clinical pattern ALS- authors, particularly Mitsuyama and others D. Originally called Pick’s disease, the cases published several clinical series that the extent without Pick bodies became FLD, then FTD then of this association began to be appreciated. The FTLD, then again frontal and temporal variant nature of the cognitive impairment was not of FTD, behavioral variant (FTD-bv) and seman- really specified until Neary et al described FTD tic dementia and so on, seemingly a never- and MND and Caselli progressive aphasia and ending process of labeling and relabeling. MND, and both the behaviour and the language Unfortunately, attempts to standardize termi- component of FTD/Pick complex became defi- nology have not been successful, although con- nitely associated with ALS, beyond other cogni- sensus conferences are numerous. The FTD tive impairment detected on neuropsychological meeting four years ago in London, Ontario, examination. summarized the democratic vote of the partici- Once you look, you begin to find, and both the pants for each term in usage, but there seems to ALS and FTD researchers unearthed a spectrum be no appetite for discipline in following them. of abnormalities. Now the search is on in several This is most unfortunate and hinders the recog- directions. Since the availability of ubiquitin nition of the understanding and relationship of staining, pathologists found MND type inclu- the clinical patterns and pathological variants, sions, previously considered typical in the spinal making it impossible for an outsider to follow cord of ALS patients, in frontal association cortex the literature without a comprehensive glossary.
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