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Updated: Feb 10, 2026

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
Published on: July 29, 2007
Self-Regulation and Executive Functioning as Related to Survival in Motor Neuron Disease: Preliminary Findings
Natasha E Garcia-Willingham1, Abbey R Roach, Edward J Kasarskis
1From the Department of Psychology (Garcia-Willingham, Segerstrom), University of Kentucky, Lexington; Departments of Psychology and Neuropsychology (Roach), Frazier Rehabilitation Institute, Louiseville, Kentucky; and Department of Neurology (Kasarskis), University of Kentucky, Lexington.
Objective:
Disease progression varies widely among patients with motor neuron disease (MND). Patients with MND and coexisting dementia have shorter survival. However, implications of mild cognitive and behavioral difficulties are unclear. The present study examined the relative contribution of executive functioning and self-regulation difficulties on survival over a 6-year period among patients with MND, who scored largely within normal limits on cognitive and behavioral indices.
Methods:
Patients with MND (N = 37, age = 59.97 ± 11.57, 46% female) completed the Wisconsin Card Sorting Task as an executive functioning perseveration index. The Behavior Rating Inventory of Executive Functions (BRIEF-A) was used as a behavioral measure of self-regulation in two subdomains self-regulatory behavior (Behavioral Regulation) and self-regulatory problem-solving (Metacognition). Cox proportional hazard regression analyses were used.
Results:
In total, 23 patients died during follow-up. In Cox proportional hazard regressions adjusted for a priori covariates, each 10-point t-score increment in patient-reported BRIEF-A self-regulatory behavior and problem-solving difficulties increased mortality risk by 94% and 103%, respectively (adjusted HR = 1.94, 95% CI = 1.07-3.52; adjusted HR = 2.03, 95% CI = 1.19-3.48). In sensitivity analyses, patient-reported self-regulatory problem-solving remained significant independent of disease severity and a priori covariates (adjusted HR = 1.68, 95% CI = 1.01-2.78), though the predictive value of self-regulatory behavior was attenuated in adjusted models (HR = 1.67, 95% CI = 0.85-3.27). Caregiver-reported BRIEF-A ratings of patients and Wisconsin Card Sorting Task perseverative errors did not significantly predict survival.
Conclusions:
Preliminary evidence suggests patient-reported self-regulatory problem-solving difficulties indicate poorer prognosis in MND. Further research is needed to uncover mechanisms that negatively impact patient survival.
Insights
Self-regulation difficulties in motor neuron disease (MND) patients predict survival. Patient-reported problem-solving issues significantly increase mortality risk, highlighting a key prognostic indicator.
Area of Science:
- Neurology
- Cognitive Science
- Prognostics
Background:
- Motor neuron disease (MND) progression and survival rates vary significantly among patients.
- While dementia in MND patients is linked to shorter survival, the impact of milder cognitive and behavioral issues remains unclear.
- This study investigates the prognostic value of executive functioning and self-regulation in MND patients with largely normal cognitive indices.
Purpose of the Study:
- To determine the relative contribution of executive functioning and self-regulation difficulties to survival in motor neuron disease (MND) patients over a six-year period.
- To assess the prognostic significance of mild cognitive and behavioral deficits in MND.
- To explore the relationship between specific self-regulation domains and mortality risk.
Main Methods:
- 37 patients with MND completed the Wisconsin Card Sorting Task and the Behavior Rating Inventory of Executive Functions (BRIEF-A) assessing self-regulatory behavior and problem-solving.
- Cox proportional hazard regression analyses were employed to evaluate survival.
- Analyses were adjusted for a priori covariates and disease severity in sensitivity testing.
Main Results:
- A total of 23 deaths occurred during the 6-year follow-up period.
- Patient-reported difficulties in self-regulatory behavior and problem-solving were associated with a significant increase in mortality risk (94% and 103% respectively).
- Self-regulatory problem-solving difficulties remained a significant independent predictor of survival, whereas self-regulatory behavior did not in adjusted models. Caregiver reports and Wisconsin Card Sorting Task performance did not predict survival.
Conclusions:
- Patient-reported self-regulatory problem-solving deficits appear to be a significant indicator of poorer prognosis in motor neuron disease (MND).
- These findings suggest that difficulties in self-regulation, particularly in problem-solving, are crucial for understanding MND patient survival.
- Further research is warranted to elucidate the underlying mechanisms linking these deficits to patient survival in MND.
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