Journal
LANCET NEUROLOGY
Volume 15, Issue 11, Pages 1182-1194Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/S1474-4422(16)30199-5
Keywords
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Funding
- European Union Joint Programme-Neurodegenerative Diseases (JPND) projects
- Irish Health Board, Ireland
- Ministero dell'Istruzione, dell'Universita e della Ricerca, Italy
- ZonMW, Netherlands
- Medical Research Council, UK
- Economic and Social Research Council, UK
- National Institute for Health Research (NIHR) Dementia Biomedical Research Unit at South London and Maudsley NHS Foundation Trust and King's College London
- European Community [259867]
- Motor Neurone Disease Association
- ALS Association
- NIHR
- European Commission
- Italian Ministry of Health (Ricerca Finalizzata)
- University of Turin
- Fondazione Vialli e Mauro per la Ricerca e lo Sport Onlus
- National Health and Medical Research Council of Australia [1037746]
- Health Research Board [H01300]
- Science Foundation Ireland
- Research Motor Neurone
- ALS Therapy Alliance
- National Institute of Neurological Diseases and Stroke through the NorthEast ALS Consortium
- Massachusetts General Hospital
- Harvard Medical School
- Johns Hopkins University School of Medicine
- Muscular Dystrophy Association ALS Division
- Carolinas ALS Research Fund
- Pinstripes Fund
- Carolinas Garden of Hope
- Heineman Medical Research Fund
- Carolinas Healthcare Foundation
- Netherlands Organization for Health Research and Development (Vici scheme)
- Netherlands ALS Foundation
- Prinses Beatrix Spierfonds
- Medical Research Council
- Economic and Social Research Council
- ESRC [ES/L008238/1] Funding Source: UKRI
- Economic and Social Research Council [ES/L008238/1] Funding Source: researchfish
- Medical Research Council [MR/L501529/1] Funding Source: researchfish
- Motor Neurone Disease Association [AlChalabi-Talbot/Apr14/926-794] Funding Source: researchfish
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Amyotrophic lateral sderosis is a progressive adult-onset neurodegenerative disease that primarily affects upper and lower motor neurons, but also frontotemporal and other regions of the brain. The extent to which each neuronal population is affected varies between individuals. The subsequent patterns of disease progression form the basis of diagnostic criteria and phenotypic classification systems, with considerable overlap in the clinical terms used. This overlap can lead to confusion between diagnosis and phenotype. Formal classification systems such as the El Escorial criteria and the International Classification of Diseases are systematic approaches but they omit features that are important in clinical management, such as rate of progression, genetic basis, or functional effect. Therefore, many neurologists use informal classification approaches that might not be systematic, and could include, for example, anatomical descriptions such as flail-arm syndrome. A new strategy is needed to combine the benefits of a systematic approach to classification with the rich and varied phenotypic descriptions used in clinical practice.
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