Rebekah M Ahmed1, Emma M Devenney2, Cherie Strikwerda-Brown2, John R Hodges2, Olivier Piguet2, Matthew C Kiernan2. 1. From the Memory and Cognition Clinic, Department of Clinical Neurosciences (R.M.A., M.C.K.), Royal Prince Alfred Hospital; Central Sydney Medical School and Brain & Mind Centre (R.M.A., E.M.D., J.R.H., M.C.K.) and School of Psychology and Brain & Mind Centre (C.S.-B., O.P.), The University of Sydney; and ARC Centre of Excellence of Cognition and its Disorders (C.S.-B., O.P.), Sydney, Australia. Rebekah.ahmed@sydney.edu.au. 2. From the Memory and Cognition Clinic, Department of Clinical Neurosciences (R.M.A., M.C.K.), Royal Prince Alfred Hospital; Central Sydney Medical School and Brain & Mind Centre (R.M.A., E.M.D., J.R.H., M.C.K.) and School of Psychology and Brain & Mind Centre (C.S.-B., O.P.), The University of Sydney; and ARC Centre of Excellence of Cognition and its Disorders (C.S.-B., O.P.), Sydney, Australia.
Abstract
OBJECTIVE: To determine if survival and cognitive profile is affected by initial presentation in amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) (motor vs cognitive), we compared survival patterns in ALS-FTD based on initial phenotypic presentation and their cognitive profile compared to behavioral variant FTD (bvFTD). METHODS: Cognitive/behavioral profiles were examined in 98 patients (59 ALS-FTD and 39 bvFTD). The initial presentation of ALS-FTD was categorized into either motor or cognitive. Survival was calculated from initial symptom onset. MRI brain atrophy patterns were examined using a validated visual rating scale. RESULTS: In the ALS-FTD group, 41 (69%) patients were categorized as having an initial cognitive presentation and 18 (31%) a motor presentation. Patients with motor presentation experienced a significantly shorter median survival of 2.7 years compared to 4.4 years (p < 0.001) in those with a cognitive presentation. No differences between motor vs cognitive onset ALS-FTD were found on cognitive testing. When compared to bvFTD, ALS-FTD-cognitive presentation was characterized by reduced language function (p < 0.001), verbal fluency (p = 0.001), and naming (p = 0.007). Both motor and cognitive onset ALS-FTD showed reduced emotion processing (p = 0.01) and exhibited greater motor cortex and dorsal lateral prefrontal cortex atrophy than bvFTD. Increased motor cortex atrophy was associated with 1.5-fold reduction in survival. CONCLUSIONS: Initial motor presentation in ALS-FTD leads to faster progression than in those with a cognitive presentation, despite similar overall cognitive deficits. These findings suggest that disease progression in ALS-FTD may be critically linked to physiologic and motor changes.
OBJECTIVE: To determine if survival and cognitive profile is affected by initial presentation in amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) (motor vs cognitive), we compared survival patterns in ALS-FTD based on initial phenotypic presentation and their cognitive profile compared to behavioral variant FTD (bvFTD). METHODS: Cognitive/behavioral profiles were examined in 98 patients (59 ALS-FTD and 39 bvFTD). The initial presentation of ALS-FTD was categorized into either motor or cognitive. Survival was calculated from initial symptom onset. MRI brain atrophy patterns were examined using a validated visual rating scale. RESULTS: In the ALS-FTD group, 41 (69%) patients were categorized as having an initial cognitive presentation and 18 (31%) a motor presentation. Patients with motor presentation experienced a significantly shorter median survival of 2.7 years compared to 4.4 years (p < 0.001) in those with a cognitive presentation. No differences between motor vs cognitive onset ALS-FTD were found on cognitive testing. When compared to bvFTD, ALS-FTD-cognitive presentation was characterized by reduced language function (p < 0.001), verbal fluency (p = 0.001), and naming (p = 0.007). Both motor and cognitive onset ALS-FTD showed reduced emotion processing (p = 0.01) and exhibited greater motor cortex and dorsal lateral prefrontal cortex atrophy than bvFTD. Increased motor cortex atrophy was associated with 1.5-fold reduction in survival. CONCLUSIONS: Initial motor presentation in ALS-FTD leads to faster progression than in those with a cognitive presentation, despite similar overall cognitive deficits. These findings suggest that disease progression in ALS-FTD may be critically linked to physiologic and motor changes.
Authors: Smriti Agarwal; Elizabeth Highton-Williamson; Jashelle Caga; James Howells; Thanuja Dharmadasa; José M Matamala; Yan Ma; Kazumoto Shibuya; John R Hodges; Rebekah M Ahmed; Steve Vucic; Matthew C Kiernan Journal: Sci Rep Date: 2021-01-26 Impact factor: 4.996
Authors: Hulya Ulugut; Simone Stek; Lianne E E Wagemans; Roos J Jutten; Maria Antoinette Keulen; Femke H Bouwman; Niels D Prins; Afina W Lemstra; Welmoed Krudop; Charlotte E Teunissen; Bart N M van Berckel; Rik Ossenkoppele; Frederik Barkhof; Wiesje M van der Flier; Philip Scheltens; Yolande A L Pijnenburg Journal: J Neurol Date: 2021-07-03 Impact factor: 4.849
Authors: Georgia Peakman; Lucy L Russell; Rhian S Convery; Jennifer M Nicholas; John C Van Swieten; Lize C Jiskoot; Fermin Moreno; Raquel Sanchez-Valle; Robert Laforce; Caroline Graff; Mario Masellis; Maria Carmela Tartaglia; James B Rowe; Barbara Borroni; Elizabeth Finger; Matthis Synofzik; Daniela Galimberti; Rik Vandenberghe; Alexandre de Mendonça; Chris R Butler; Alex Gerhard; Simon Ducharme; Isabelle Le Ber; Fabrizio Tagliavini; Isabel Santana; Florence Pasquier; Johannes Levin; Adrian Danek; Markus Otto; Sandro Sorbi; Jonathan D Rohrer Journal: J Neurol Neurosurg Psychiatry Date: 2021-08-05 Impact factor: 10.154