Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

Michael J. Strong Department of Clinical Neurological Sciences, The University of Western Ontario, London, OntarioCorrespondence[email protected]

Gloria M. Grace Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario; Psychological Services, London Health Sciences Centre, London, Ontario, Canada

Morris Freedman Division of Neurology, Behavioural Neurology Program, and Rotman Research Institute, Baycrest Division of Neurology, Department of Medicine, Mt. Sinai Hospital, University Health Network, and University of Toronto, Toronto, Ontario

Cathy Lomen-Hoerth Department of Neurology, UCSF, San Francisco, California

Susan Woolley Forbes Norris MDA/ALS Research Center, San Francisco, California, USA

Laura H. Goldstein MRC Centre for Neurodegeneration Research, King's College London, Institute of Psychiatry, London, UK

Jennifer Murphy Department of Neurology, USCF, San Francisco

Christen Shoesmith Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario

Jeffery Rosenfeld Division of Neurology, UCSF-Fresno, Fresno, California

P. Nigel Leigh MRC Centre for Neurodegeneration Research, King's College London, Institute of Psychiatry, London, UK

Lucie Bruijn Research and Development, The ALS Association, Palm Harbor, Florida, USA

Paul Ince Department of Neuroscience, University of Sheffield, Sheffield, UK

Denise Figlewicz ALS Society of Canada, Toronto, Ontario, Canada

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Abstract

Amyotrophic lateral sclerosis (ALS) is increasingly recognized to be a multisystem disorder which includes both clinical and neuropathological features of a frontotemporal lobar degeneration (FTLD). In order to provide a common framework within which to discuss the characteristics of the cognitive and behavioural syndromes of ALS, and with which to conduct clinical and neuropathological research, an international research workshop on frontotemporal dementia (FTD) and ALS was held in London, Canada in June 2007. The recommendations arising from this research workshop address the requirement for a concise clinical diagnosis of the underlying motor neuron disease (Axis I), defining the cognitive and behavioural dysfunction (Axis II), describing additional non-motor manifestations (Axis III) and identifying the presence of disease modifiers (Axis IV).

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Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

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Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

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