Loss of "insight" into behavioral changes in ALS: Differences across cognitive profiles.

OBJECTIVE Behavioral impairment occurs in amyotrophic lateral sclerosis (ALS) and ALS-fronto-temporal dementia (ALS-FTD). It has been proposed that ALS patients without FTD retain an awareness of their behavioral impairment while ALS-FTD patients lose this awareness (referred to as retention vs. loss of "insight"). Loss of insight has not yet been studied across the entire ALS-FTD spectrum; our study addresses this gap by including patients with all the ALS cognitive-behavioral profiles. METHODS Eighty-three ALS patients (and their informants) took part in this bicentric study involving two German recruitment sites. Patients and informants completed the Frontal Systems Behavior Scale covering the domains of apathy, disinhibition, and executive dysfunctioning. Patients were classified into five groups according to the Strong and Rascovsky criteria: cognitively unimpaired (ALSni), cognitively impaired without dementia (ALSci), behaviorally impaired (ALSbi), a combination of behaviorally and cognitively impaired (ALScbi), and ALS-FTD. We applied Bayesian two-way ANOVA to test whether there were subgroup differences regarding insight into their behavioral decline. RESULTS All patient subgroups experienced behavioral decline (Bayes factor > 3). Only ALS-FTD patients lost insight into disinhibition and executive dysfunctioning. ALSbi patients exhibited worse insight than ALSni and ALSci patients (Bayes factor > 10). Evidence regarding the ALScbi patients was inconclusive. Higher IQ was associated with worse insight (Bayes factor > 3). CONCLUSIONS Our findings provide solid support for the notion that ALS patients without dementia experience behavioral decline regardless of their cognitive-behavioral profile and retain different levels of insight into this decline. The inverse association of premorbid verbal intelligence with insight was unexpected, leaving room for further investigation.