A case series of PLS patients with frontotemporal dementia and overview of the literature. Issue 7 (2nd October 2017)

• Record Type: Journal Article
• Authors: de Vries, Bálint S.; Rustemeijer, Laura M.M.; van der Kooi, Anneke J.; Raaphorst, Joost; Schröder, Carin D.; Nijboer, Tanja C.W.; Hendrikse, Jeroen; Veldink, Jan H.; van den Berg, Leonard H.; van Es, Michael A.
• Is Part Of: Amyotrophic lateral sclerosis and frontotemporal degeneration. Volume 18:Issue 7/8(2017)
• Page Start: 534
• Page End: 548
• Publication Date: 2017-10-02
• Subjects: Primary lateral sclerosis -- cognition -- frontotemporal dementia -- dementia; 616.839
• DOI: 10.1080/21678421.2017.1354996
• Languages: English
• ISSNs: 2167-8421

Abstract

Abstract: 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.