Laughter, crying and sadness in ALS

Area of Science:

• Neurology
• Neuroscience
• Psychiatry

Background:

• Pseudobulbar affect (PBA) is common in amyotrophic lateral sclerosis (ALS) but its associations and progression are not well understood.
• Limited data exists on the prevalence and clinical correlates of PBA in ALS patients.

Purpose of the Study:

• To determine the prevalence, associations, and course of PBA in an outpatient ALS cohort.
• To examine the relationship between PBA and depression in patients with ALS.
• To investigate the manifestations of PBA, including crying and laughter, in relation to depression and quality of life.

Main Methods:

• Self-reported measures, including the Center for Neurologic Study-Lability Scale (CNS-LS) for PBA and the Patient Health Questionnaire (PHQ-9) for depression, were collected from consecutive ALS patients.
• Data was gathered using tablet devices in waiting rooms as part of the Knowledge Program.
• Multivariable modeling and exploratory factor analysis were employed to identify predictors of PBA and underlying factors.

Main Results:

• PBA was identified in 28.4% (209/735) of ALS patients, associated with bulbar onset, upper motor neuron dysfunction, cognitive impairment, depression, and lower quality of life.
• Predictors of PBA included lower bulbar/motor scores, female gender, younger age, and shorter disease duration.
• Women with PBA reported more crying, which correlated with depression and poorer quality of life, unlike laughter.

Conclusions:

• PBA, particularly crying-predominant PBA, is more prevalent in women with ALS.
• While CNS-LS and PHQ-9 differentiate PBA and depression, significant overlap exists between depression and crying in PBA.
• Future PBA research should stratify by gender, analyze crying and laughter separately, and adjust for depression.