Analysis of terms used for the diagnosis and classification of amyotrophic lateral sclerosis and motor neuron disease

Rutter-Locher, Zoe, Turner, Martin R, Leigh, Nigel and Al-Chalabi, Ammar (2016) Analysis of terms used for the diagnosis and classification of amyotrophic lateral sclerosis and motor neuron disease. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17 (7-8). pp. 600-604. ISSN 2167-8421

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Abstract

There is no test for amyotrophic lateral sclerosis (ALS) and so attempts have been made to produce standardized diagnostic criteria based on clinical and electrophysiological findings, e.g. El Escorial. However, the phenotypic classification of the subtypes of ALS is also based on clinical features leading to conflation of diagnosis and phenotype. We used a five-question online survey with ALS specialists to explore the range of descriptors and how they are used. Of 101 specialists approached, 72 completed the survey. The most frequently used labels were ‘ALS’, ‘PLS’ and ‘familial’. Labels other than the El Escorial categories were mainly used as clinical descriptors (83%). Approximately 50% of respondents recorded that the El Escorial criteria had no useful role in patient discussion or in the diagnostic process. Only 31% of respondents rated their current classification system above the median for being logical. A more rational system explicitly distinguishing diagnostic and phenotypic criteria is essential

Item Type: Article
Keywords: Classification of ALS/MND; diagnosis of ALS/MND; phenotype of ALS/MND
Schools and Departments: Brighton and Sussex Medical School > Neuroscience
Subjects: R Medicine > RC Internal medicine > RC0321 Neurosciences. Biological psychiatry. Neuropsychiatry > RC0346 Neurology. Diseases of the nervous system Including speech disorders
Depositing User: Patricia Butler
Date Deposited: 06 Jan 2017 12:27
Last Modified: 25 Jul 2017 15:08
URI: http://sro.sussex.ac.uk/id/eprint/66049
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