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Phenotypic variability in ALS-FTD and effect on survival.

Rebekah M Ahmed1, Emma M Devenney2, Cherie Strikwerda-Brown2

  • 1From 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.

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This summary is machine-generated.

Amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) with initial motor symptoms has shorter survival than those with cognitive symptoms. Disease progression in ALS-FTD is linked to physiological and motor changes.

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Area of Science:

  • Neurology
  • Neuroscience
  • Cognitive Science

Background:

  • Amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) is a complex neurodegenerative disorder.
  • Understanding the impact of initial presentation on disease progression is crucial for patient management.

Purpose of the Study:

  • To compare survival patterns and cognitive profiles in ALS-FTD based on initial motor versus cognitive presentation.
  • To differentiate ALS-FTD presentations from behavioral variant FTD (bvFTD).

Main Methods:

  • Evaluated cognitive/behavioral profiles in 98 patients (59 ALS-FTD, 39 bvFTD).
  • Categorized ALS-FTD initial presentation as motor or cognitive.
  • Analyzed survival from symptom onset and examined MRI brain atrophy patterns.

Main Results:

  • Patients with motor onset ALS-FTD had significantly shorter median survival (2.7 years) than cognitive onset (4.4 years).
  • ALS-FTD-cognitive presentation showed reduced language, verbal fluency, and naming compared to bvFTD.
  • Both ALS-FTD groups exhibited greater motor cortex and prefrontal cortex atrophy; motor cortex atrophy correlated with reduced survival.

Conclusions:

  • Initial motor presentation in ALS-FTD is associated with faster disease progression.
  • Disease progression in ALS-FTD appears critically linked to physiological and motor changes, despite similar cognitive deficits.