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

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

doi:10.1080/21678421.2017.1354996

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

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

Research output: Contribution to journal › Article › Academic › peer-review

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.

Original language	English
Pages (from-to)	534-548
Number of pages	15
Journal	Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration
Volume	18
Issue number	7-8
DOIs	https://doi.org/10.1080/21678421.2017.1354996
Publication status	Published - 2017

Keywords

Primary lateral sclerosis
cognition
dementia
frontotemporal dementia

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de Vries, B. S., Rustemeijer, L. M. M., van der Kooi, A. J., Raaphorst, J., Schröder, C. D., Nijboer, T. C. W., Hendrikse, J., Veldink, J. H., van den Berg, L. H., & van Es, M. A. (2017). A case series of PLS patients with frontotemporal dementia and overview of the literature. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 18(7-8), 534-548. https://doi.org/10.1080/21678421.2017.1354996

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title = "A case series of PLS patients with frontotemporal dementia and overview of the literature",

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.",

keywords = "Primary lateral sclerosis, cognition, dementia, frontotemporal dementia",

author = "{de Vries}, {B{\'a}lint S} and Rustemeijer, {Laura M M} and {van der Kooi}, {Anneke J.} and Joost Raaphorst and Schr{\"o}der, {Carin D} and Nijboer, {Tanja C W} and Jeroen Hendrikse and Veldink, {Jan H} and {van den Berg}, {Leonard H} and {van Es}, {Michael A}",

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year = "2017",

doi = "10.1080/21678421.2017.1354996",

language = "English",

volume = "18",

pages = "534--548",

journal = "Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration",

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de Vries, BS, Rustemeijer, LMM, van der Kooi, AJ, Raaphorst, J, Schröder, CD, Nijboer, TCW, Hendrikse, J , Veldink, JH , van den Berg, LH & van Es, MA 2017, 'A case series of PLS patients with frontotemporal dementia and overview of the literature', Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, vol. 18, no. 7-8, pp. 534-548. https://doi.org/10.1080/21678421.2017.1354996

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 - 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.

AB - 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.

KW - Primary lateral sclerosis

KW - cognition

KW - dementia

KW - frontotemporal dementia

U2 - 10.1080/21678421.2017.1354996

DO - 10.1080/21678421.2017.1354996

M3 - Article

C2 - 28745069

SN - 2167-8421

VL - 18

SP - 534

EP - 548

JO - Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration

JF - Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration

IS - 7-8

ER -

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

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Keywords

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