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. 2018 Jan 5;13(1):e0190818.
doi: 10.1371/journal.pone.0190818. eCollection 2018.

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

Diana Caine  1   2   3 , Akin Nihat  1   3 , Philippa Crabb  2 , Peter Rudge  1   3 , Lisa Cipolotti  2 , John Collinge  1   3 , Simon Mead  1   3
Affiliations

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

Diana Caine et al. PLoS One. .

Abstract

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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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

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