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The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature

BACKGROUND: Akinetic mutism is a key diagnostic feature of prion diseases, however, their rapidly progressive nature makes detailed investigation of the language disorder in a large cohort extremely challenging. This study aims to position prion diseases in the nosology of language disorders and imp...

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Autores principales: Caine, Diana, Nihat, Akin, Crabb, Philippa, Rudge, Peter, Cipolotti, Lisa, Collinge, John, Mead, Simon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5755885/
https://www.ncbi.nlm.nih.gov/pubmed/29304167
http://dx.doi.org/10.1371/journal.pone.0190818
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author Caine, Diana
Nihat, Akin
Crabb, Philippa
Rudge, Peter
Cipolotti, Lisa
Collinge, John
Mead, Simon
author_facet Caine, Diana
Nihat, Akin
Crabb, Philippa
Rudge, Peter
Cipolotti, Lisa
Collinge, John
Mead, Simon
author_sort Caine, Diana
collection PubMed
description BACKGROUND: Akinetic mutism is a key diagnostic feature of prion diseases, however, their rapidly progressive nature makes detailed investigation of the language disorder in a large cohort extremely challenging. This study aims to position prion diseases in the nosology of language disorders and improve early clinical recognition. METHODS: A systematic, prospective investigation of language disorders in a large cohort of patients diagnosed with prion diseases. 568 patients were included as a sub-study of the National Prion Monitoring Cohort. All patients had at least one assessment with the MRC Scale, a milestone-based functional scale with language and non-language components. Forty patients, with early symptoms and able to travel to the study site, were also administered a comprehensive battery of language tests (spontaneous speech, semantics, syntax, repetition, naming, comprehension and lexical retrieval under different conditions). RESULTS: 5/568 (0.9%) patients presented with leading language symptoms. Those with repeated measurements deteriorated at a slower rate in language compared to non-language milestones. Amongst the subgroup of 40 patients who underwent detailed language testing, only three tasks–semantic and phonemic fluency and sentence comprehension–were particularly vulnerable early in the disease. These tasks were highly correlated with performance on non-verbal executive tests. Patients were also impaired on a test of dynamic aphasia. CONCLUSION: These results provide evidence that the language disorder in prion disease is rarely an isolated clinical or cognitive feature. The language abnormality is indicative of a dynamic aphasia in the context of a prominent dysexecutive syndrome, similar to that seen in patients with the degenerative movement disorder progressive supranuclear palsy (PSP).
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spelling pubmed-57558852018-01-26 The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature Caine, Diana Nihat, Akin Crabb, Philippa Rudge, Peter Cipolotti, Lisa Collinge, John Mead, Simon PLoS One Research Article BACKGROUND: Akinetic mutism is a key diagnostic feature of prion diseases, however, their rapidly progressive nature makes detailed investigation of the language disorder in a large cohort extremely challenging. This study aims to position prion diseases in the nosology of language disorders and improve early clinical recognition. METHODS: A systematic, prospective investigation of language disorders in a large cohort of patients diagnosed with prion diseases. 568 patients were included as a sub-study of the National Prion Monitoring Cohort. All patients had at least one assessment with the MRC Scale, a milestone-based functional scale with language and non-language components. Forty patients, with early symptoms and able to travel to the study site, were also administered a comprehensive battery of language tests (spontaneous speech, semantics, syntax, repetition, naming, comprehension and lexical retrieval under different conditions). RESULTS: 5/568 (0.9%) patients presented with leading language symptoms. Those with repeated measurements deteriorated at a slower rate in language compared to non-language milestones. Amongst the subgroup of 40 patients who underwent detailed language testing, only three tasks–semantic and phonemic fluency and sentence comprehension–were particularly vulnerable early in the disease. These tasks were highly correlated with performance on non-verbal executive tests. Patients were also impaired on a test of dynamic aphasia. CONCLUSION: These results provide evidence that the language disorder in prion disease is rarely an isolated clinical or cognitive feature. The language abnormality is indicative of a dynamic aphasia in the context of a prominent dysexecutive syndrome, similar to that seen in patients with the degenerative movement disorder progressive supranuclear palsy (PSP). Public Library of Science 2018-01-05 /pmc/articles/PMC5755885/ /pubmed/29304167 http://dx.doi.org/10.1371/journal.pone.0190818 Text en © 2018 Caine et al http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Article
Caine, Diana
Nihat, Akin
Crabb, Philippa
Rudge, Peter
Cipolotti, Lisa
Collinge, John
Mead, Simon
The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
title The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
title_full The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
title_fullStr The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
title_full_unstemmed The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
title_short The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
title_sort language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5755885/
https://www.ncbi.nlm.nih.gov/pubmed/29304167
http://dx.doi.org/10.1371/journal.pone.0190818
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