• Rebekah M Ahmed, Emma M Devenney, Cherie Strikwerda-Brown, John R Hodges, Olivier Piguet, and Matthew C Kiernan.
• 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.
• Neurology. 2020 May 12; 94 (19): e2005-e2013.

ObjectiveTo 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).MethodsCognitive/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.ResultsIn 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.ConclusionsInitial 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.© 2020 American Academy of Neurology.

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