Taylor & Francis Group
Browse
DOCUMENT
Supplementary Table I.docx (24 kB)
DOCUMENT
Supplementary material for PLS case series and overview of the literature.docx (75.4 kB)
1/0
2 files

A case series of PLS patients with frontotemporal dementia and overview of the literature

dataset
posted on 2017-07-28, 07:45 authored by Bálint S. de Vries, Laura M.M. Rustemeijer, Anneke J. van der Kooi, Joost Raaphorst, Carin D. Schröder, Tanja C.W. Nijboer, Jeroen Hendrikse, Jan H. Veldink, Leonard H. van den Berg, Michael A. van Es

Objective: Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen (±15%). However, cognitive and behavioural changes are generally not considered to be a part of PLS. Methods: To report the clinical findings and frequency of PLS patients that developed FTD in a referral-based cohort and provide an overview of the literature. Results: In our cohort six out of 181 (3.3%) PLS patients developed FTD. In the literature a few cases of PLS with FTD have been reported and only a limited number of small studies have investigated cognition in PLS. However, when these studies are summarised a pattern emerges with FTD diagnoses in ±2% and frontotemporal impairment in 22% of patients. Conclusions: These findings suggest that PLS is part of the FTD-MND continuum and would favour viewing it as a subtype of ALS. It is, however, not a restricted (isolated UMN involvement) phenotype.

History

Usage metrics

    AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION

    Licence

    Exports

    RefWorks
    BibTeX
    Ref. manager
    Endnote
    DataCite
    NLM
    DC