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Alzheimer disease is a chronic, progressive, and irreversible neurodegenerative disorder and the most common cause of dementia in older adults. It leads to gradual neuronal loss, causing cognitive decline, behavioral changes, and loss of functional independence.Risk Factors and EtiologyThe disease is multifactorial. Age is the strongest risk factor, with prevalence doubling every 5 years after age 65. Genetic factors include mutations in genes such as APP, PSEN1, and PSEN2, which are associated...
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Eye-Tracking Control to Assess Cognitive Functions in Patients with Amyotrophic Lateral Sclerosis
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Published on: October 13, 2016

Cognitive changes predict functional decline in ALS: a population-based longitudinal study.

Marwa Elamin1, Peter Bede, Susan Byrne

  • 1Trinity College Institute of Neuroscience, Trinity College Dublin, Ireland. marwaelamin08@gmail.com

Neurology
|April 5, 2013
PubMed
Summary
This summary is machine-generated.

Cognitive status in amyotrophic lateral sclerosis (ALS) predicts disease progression. Initial executive impairment in ALS patients accelerates motor decline and attrition, while normal cognition indicates slower progression.

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Area of Science:

  • Neuroscience
  • Neurology
  • Clinical Research

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
  • Cognitive impairment is increasingly recognized in ALS patients.
  • The prognostic value of baseline cognitive status in ALS requires further elucidation.

Purpose of the Study:

  • To investigate if cognitive status predicts attrition and motor/cognitive decline in amyotrophic lateral sclerosis (ALS).
  • To identify distinct cognitive subgroups within non-C9orf72-associated ALS.
  • To inform future clinical trial design and models of ALS pathogenesis.

Main Methods:

  • Longitudinal, case-control study of 186 incident ALS patients without C9orf72 expansion.
  • Repeated neuropsychological testing with age-, sex-, and education-matched controls.
  • Analysis to model disease progression and spread.

Main Results:

  • Initial executive impairment in ALS patients correlated with higher attrition rates (disability/death) and faster motor decline, especially bulbar function.
  • Cognitively impaired ALS patients showed faster cognitive decline.
  • Normal baseline cognition was linked to sustained cognitive intactness and slower motor/cognitive progression.

Conclusions:

  • Non-C9orf72 ALS comprises distinct cognitive subgroups with varying disease trajectories.
  • Baseline cognitive status is a significant predictor of disease course in ALS.
  • Findings impact understanding of ALS pathogenesis and clinical trial strategies.