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

journal contribution
posted on 2023-06-09, 04:35 authored by Zoe Rutter-Locher, Martin R Turner, Nigel LeighNigel Leigh, Ammar Al-Chalabi
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

History

Publication status

  • Published

Journal

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

ISSN

2167-8421

Publisher

Taylor & Francis

Issue

7-8

Volume

17

Page range

600-604

Department affiliated with

  • BSMS Neuroscience Publications

Full text available

  • No

Peer reviewed?

  • Yes

Legacy Posted Date

2017-01-06

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