Abstract
Pathological crying and laughing (PCL) has significant quality-of-life implications in amyotrophic lateral sclerosis (ALS); it can provoke restrictive life-style modifications and lead to social isolation. Despite its high prevalence and quality of life implications, it remains surprisingly understudied. Divergent pathophysiological models have been proposed, centered on corticobulbar tract degeneration, prefrontal cortex pathology, sensory deafferentation, and impaired cerebellar gate-control mechanisms. Quantitative MRI techniques and symptom-specific clinical instruments offer unprecedented opportunities to elucidate the anatomical underpinnings of PCL pathogenesis. Emerging neuroimaging studies of ALS support the role of cortico–pontine–cerebellar network dysfunction in context-inappropriate emotional responses. The characterization of PCL-associated pathophysiological processes is indispensable for the development of effective pharmacological therapies.
Highlights
The terms “pathological crying and laughing,” “pseudobulbar affect,” “emotional lability,” and “involuntary emotional expression disorder” are often used interchangeably depending on geographical location and year of publication
This association is still well-recognized [13, 18], the term pseudobulbar affect” (PBA) may be misleading; new evidence suggests that corticobulbar tract dysfunction alone is neither necessary, nor sufficient to cause Pathological crying and laughing (PCL) [19,20,21,22,23]
“Emotional Lability” (EL) was described by Pierre-Marie as early as 1892, a term still commonly used in the literature [24]
Summary
The terms “pathological crying and laughing,” “pseudobulbar affect,” “emotional lability,” and “involuntary emotional expression disorder” are often used interchangeably depending on geographical location and year of publication. While there is a paucity of studies comparing PCL prevalence across the spectrum of MND phenotypes, a recent study confirmed the relationship between clinical upper motor neuron dysfunction and PCL prevalence; PCL was most commonly identified in PLS and UMN-predominant patients (39%), followed by typical ALS (29%) and lower motor neuron (LMN) predominant groups (10%). Consistent with this pattern, there was a single case of PCL in a group of 12 patients with progressive
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