Bálint S de Vries1, Laura M M Rustemeijer1, Anneke J van der Kooi2, Joost Raaphorst2,3, Carin D Schröder4, Tanja C W Nijboer4,5, Jeroen Hendrikse6, Jan H Veldink1, Leonard H van den Berg1, Michael A van Es1. 1. a Department of Neurology , Brain Center Rudolf Magnus, University Medical Center Utrecht , Utrecht , The Netherlands. 2. b Department of Neurology , Academic Medical Center, University of Amsterdam , Amsterdam , The Netherlands. 3. c Department of Neurology , Radboud University Medical Center , Nijmegen , The Netherlands. 4. d Brain Center Rudolf Magnus and Center of Excellence for Rehabilitation Medicine , University Medical Center Utrecht and De Hoogstraat Rehabilitation , Utrecht , The Netherlands. 5. e Department of Experimental Psychology , Helmholtz Institute, Utrecht University , Utrecht , The Netherlands , and. 6. f Department of Radiology , University Medical Center Utrecht , Utrecht , The Netherlands.
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.
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 PLSpatients 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%) PLSpatients 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.
Authors: Eoin Finegan; Stacey Li Hi Shing; Rangariroyashe H Chipika; Kai Ming Chang; Mary Clare McKenna; Mark A Doherty; Jennifer C Hengeveld; Alice Vajda; Niall Pender; Colette Donaghy; Siobhan Hutchinson; Russell L McLaughlin; Orla Hardiman; Peter Bede Journal: Brain Imaging Behav Date: 2021-01-07 Impact factor: 3.978
Authors: Veronica Hirsch-Reinshagen; Omar A Alfaify; Ging-Yuek R Hsiung; Cyril Pottier; Matt Baker; Ralph B Perkerson; Rosa Rademakers; Hanna Briemberg; Dean J Foti; Ian R Mackenzie Journal: Amyotroph Lateral Scler Frontotemporal Degener Date: 2019-06-27 Impact factor: 4.092
Authors: Eoin Finegan; Rangariroyashe H Chipika; Stacey Li Hi Shing; Mark A Doherty; Jennifer C Hengeveld; Alice Vajda; Colette Donaghy; Russell L McLaughlin; Niall Pender; Orla Hardiman; Peter Bede Journal: J Neurol Date: 2019-07-19 Impact factor: 4.849
Authors: Eoin Finegan; Stacey Li Hi Shing; Rangariroyashe H Chipika; Mark A Doherty; Jennifer C Hengeveld; Alice Vajda; Colette Donaghy; Niall Pender; Russell L McLaughlin; Orla Hardiman; Peter Bede Journal: Neuroimage Clin Date: 2019-11-12 Impact factor: 4.881