TY - JOUR
T1 - A case series of PLS patients with frontotemporal dementia and overview of the literature
AU - de Vries, Bálint S.
AU - Rustemeijer, Laura M. M.
AU - van der Kooi, Anneke J.
AU - Raaphorst, Joost
AU - Schröder, Carin D.
AU - Nijboer, Tanja C. W.
AU - Hendrikse, Jeroen
AU - Veldink, Jan H.
AU - van den Berg, Leonard H.
AU - van Es, Michael A.
PY - 2017
Y1 - 2017
N2 - 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. 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. 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. 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
AB - 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. 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. 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. 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
U2 - https://doi.org/10.1080/21678421.2017.1354996
DO - https://doi.org/10.1080/21678421.2017.1354996
M3 - Article
C2 - 28745069
SN - 2167-8421
VL - 18
SP - 534
EP - 548
JO - Amyotrophic lateral sclerosis and frontotemporal degeneration
JF - Amyotrophic lateral sclerosis and frontotemporal degeneration
IS - 7-8
ER -