Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis, Amyotrophic Lateral Sclerosis, Informa Healthcare

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

2009, Vol. 10, No. 3 , Pages 131-146 (doi:10.1080/17482960802654364)

Michael J. Strong¹ , Gloria M. Grace¹,², Morris Freedman³, Cathy Lomen-Hoerth⁴, Susan Woolley⁵, Laura H. Goldstein⁶, Jennifer Murphy⁷, Christen Shoesmith¹, Jeffery Rosenfeld⁸, P. Nigel Leigh⁶, Lucie Bruijn⁹, Paul Ince¹⁰ and Denise Figlewicz¹¹

¹Department of Clinical Neurological Sciences, The University of Western Ontario, London, Ontario

²Psychological Services, London Health Sciences Centre, London, Ontario, Canada

³Division 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

⁴Department of Neurology, UCSF, San Francisco, California

⁵Forbes Norris MDA/ALS Research Center, San Francisco, California, USA

⁶MRC Centre for Neurodegeneration Research, King's College London, Institute of Psychiatry, London, UK

⁷Department of Neurology, USCF, San Francisco

⁸Division of Neurology, UCSF-Fresno, Fresno, California

⁹Research and Development, The ALS Association, Palm Harbor, Florida, USA

¹⁰Department of Neuroscience, University of Sheffield, Sheffield, UK

¹¹ALS Society of Canada, Toronto, Ontario, Canada

Room C7-120, UH-LHSC, 339 Windermere Road, London, Ontario, Canada, N6A 5A5 519 663 3609 mstrong@uwo.ca

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).