Nov 9, 2019

COG-01 Phenotypic variation in 
ALS-FTD and effect on survival

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration
Rebekah M AhmedMatthew C Kiernan


Background: Within the Amyotrophic Lateral Sclerosis (ALS)-Frontotemporal dementia (FTD) spectrum there is considerable heterogeneity in clinical presentation and survival.Objectives: The current study aimed to examine how initial symptoms (motor compared to cognitive) may affect survival, with specific focus on structural cognitive and behavioural differences between ALS-FTD and bvFTD 
cohorts.Methods: Cognitive and behavioural profiles were examined in 98 patients (59 ALS-FTD and 39 bvFTD patients). The initial presentation of ALS-FTD was categorized into either motor or cognitive, based on symptoms combined with carer reports. Survival was calculated from initial symptom onset. Brain atrophy patterns on MRI were examined using a verified visual rating scale.Results: In the ALS-FTD group, 69% were categorized as having an initial cognitive presentation and 31% a motor presentation. Those patients with motor presentation of ALS-FTD experienced a significantly shorter survival of 33 months, compared to 63 months (p<0.007) in those with a cognitive presentation of ALS-FTD. On cognitive testing there were no differences between motor versus cognitive onset ALS-FTD. When compared to bvFTD, ALS-FTD, particularly the cognitive prese...Continue Reading

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Mentioned in this Paper

Imaging Studies
Cerebral Atrophy
Magnetic Resonance Imaging
Pyramidal Cell of Precentral Gyrus of Cerebral Hemisphere
Clinical Trials
Impaired Cognition
Frontotemporal Dementia
Physiological Aspects

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