Behaviour, physiology and experience of pathological laughing and crying in amyotrophic lateral sclerosis

Abstract

Pathological laughing and crying is a disorder of emotional expression seen in a number of neurological diseases. The aetiology is poorly understood, but clinical descriptions suggest a disorder of emotion regulation. The goals of this study were: (i) to characterize the subjective, behavioural and physiological emotional reactions that occur during episodes of pathological laughing and crying; (ii) to compare responses during these episodes to those that occur when emotions are elicited under standard conditions (watching sad and amusing emotional films, being startled); and (iii) to examine the ability of patients with this disorder to regulate their emotions under standardized conditions. Twenty-one patients with pathological laughing and crying due to amyotrophic lateral sclerosis and 14 with amyotrophic lateral sclerosis but no pathological laughing and crying were studied. Emotional measures included self-reported emotional experience, video recordings of facial reactivity and peripheral physiological responses (skin conductance, heart rate and somatic activity). Nineteen of the 21 patients with histories of pathological laughing and crying had at least one episode in the laboratory that they agreed constituted pathological laughing or crying (a total of 56 episodes were documented). Compared with viewing sad and amusing films, the episodes were associated with greater facial and physiological activation. Contrary to many clinical descriptions, episodes were often induced by contextually appropriate stimuli and associated with strong experiences of emotion that were consistent with the display. When instructed to regulate their facial responses to emotion-eliciting films, patients with pathological laughing and crying showed impairments compared with patients who did not have a history of this disorder. These findings support the idea that pathological laughing and crying represents activation of all channels of emotional responding (i.e. behavioural, physiological and subjective). Furthermore, they support previously advanced theories that, rather than being associated with general emotional hyperreactivity, this disorder may be due to dysfunction in frontal neural systems that support voluntary regulation of emotion.

Pathological Laughter and Crying: A Case Series and Proposal for a New Classification

“…Our patient with bulbar ALS evolved from negative motor DLC to mixed DLC. We and others 11,30 have observed complex DLC in ALS (negative motor or mixed), possibly due to progressive involvement of additional brain areas and serotonergic neurons. 31 The differentiation of positive and negative DLC forms is one of the novel features of our classification.…”

Pathological Laughter and Crying: A Case Series and Proposal for a New Classification

“…Our patient with bulbar ALS evolved from negative motor DLC to mixed DLC. We and others 11,30 have observed complex DLC in ALS (negative motor or mixed), possibly due to progressive involvement of additional brain areas and serotonergic neurons. 31 The differentiation of positive and negative DLC forms is one of the novel features of our classification.…”

Pseudobulbar affective disorder, emotion and the brain

“…These ideas find support in recent diffusion tensor imaging studies of the brain in ALS and primary lateral sclerosis 10. These have shown reduced fractional anisotropy in the corticospinal and callosal white matter pathways, with increased mean diffusivity in white matter pathways underlying frontotemporal cortex, in transverse pontine fibres and in the middle cerebellar peduncle.…”

Definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in amyotrophic lateral sclerosis (ALS): Consensus from ALS and Motor Neuron Disease Scientific Department of the Brazilian Academy of Neurology

“…18 In addition to this pattern of abnormalities, evidence from laboratory evaluations has documented that patients with ALS also exhibit abnormal processing of emotional stimuli. 5,6 Contrary to prior views, episodes of uncontrollable laughter and crying were most frequently associated with experience of emotional distress, supporting the idea that they were associated with enhanced activation of all channels of emotional response (emotional hyperactivity due to dysfunction of neural systems that control voluntary regulation of emotion). In most cases, the triggers for episodes of laughter and crying were the same as the thoughts and stimuli that could induce crying and laughter in anyone, but they led to rapidly developing high intensity, uncontrollable bursts.…”

“…In most cases, the triggers for episodes of laughter and crying were the same as the thoughts and stimuli that could induce crying and laughter in anyone, but they led to rapidly developing high intensity, uncontrollable bursts. 5 6 Laughter episodes had a greater tendency to occur without an obvious precipitant. Of note, considering the classic distinction between PLC and EI, most episodes described by Olney are consistent with EI rather than true PLC.…”

Pathological Laughter and Crying: A Case Series and Proposal for a New Classification

“…Our patient with bulbar ALS evolved from negative motor DLC to mixed DLC. We and others 11,30 have observed complex DLC in ALS (negative motor or mixed), possibly due to progressive involvement of additional brain areas and serotonergic neurons. 31 The differentiation of positive and negative DLC forms is one of the novel features of our classification.…”

Pseudobulbar affective disorder, emotion and the brain

“…These ideas find support in recent diffusion tensor imaging studies of the brain in ALS and primary lateral sclerosis 10. These have shown reduced fractional anisotropy in the corticospinal and callosal white matter pathways, with increased mean diffusivity in white matter pathways underlying frontotemporal cortex, in transverse pontine fibres and in the middle cerebellar peduncle.…”

Definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in amyotrophic lateral sclerosis (ALS): Consensus from ALS and Motor Neuron Disease Scientific Department of the Brazilian Academy of Neurology

“…18 In addition to this pattern of abnormalities, evidence from laboratory evaluations has documented that patients with ALS also exhibit abnormal processing of emotional stimuli. 5,6 Contrary to prior views, episodes of uncontrollable laughter and crying were most frequently associated with experience of emotional distress, supporting the idea that they were associated with enhanced activation of all channels of emotional response (emotional hyperactivity due to dysfunction of neural systems that control voluntary regulation of emotion). In most cases, the triggers for episodes of laughter and crying were the same as the thoughts and stimuli that could induce crying and laughter in anyone, but they led to rapidly developing high intensity, uncontrollable bursts.…”

“…In most cases, the triggers for episodes of laughter and crying were the same as the thoughts and stimuli that could induce crying and laughter in anyone, but they led to rapidly developing high intensity, uncontrollable bursts. 5 6 Laughter episodes had a greater tendency to occur without an obvious precipitant. Of note, considering the classic distinction between PLC and EI, most episodes described by Olney are consistent with EI rather than true PLC.…”

Pathological Laughter and Crying: A Case Series and Proposal for a New Classification

“…Our patient with bulbar ALS evolved from negative motor DLC to mixed DLC. We and others 11,30 have observed complex DLC in ALS (negative motor or mixed), possibly due to progressive involvement of additional brain areas and serotonergic neurons. 31 The differentiation of positive and negative DLC forms is one of the novel features of our classification.…”

Pseudobulbar affective disorder, emotion and the brain

“…These ideas find support in recent diffusion tensor imaging studies of the brain in ALS and primary lateral sclerosis 10. These have shown reduced fractional anisotropy in the corticospinal and callosal white matter pathways, with increased mean diffusivity in white matter pathways underlying frontotemporal cortex, in transverse pontine fibres and in the middle cerebellar peduncle.…”

Definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in amyotrophic lateral sclerosis (ALS): Consensus from ALS and Motor Neuron Disease Scientific Department of the Brazilian Academy of Neurology

“…18 In addition to this pattern of abnormalities, evidence from laboratory evaluations has documented that patients with ALS also exhibit abnormal processing of emotional stimuli. 5,6 Contrary to prior views, episodes of uncontrollable laughter and crying were most frequently associated with experience of emotional distress, supporting the idea that they were associated with enhanced activation of all channels of emotional response (emotional hyperactivity due to dysfunction of neural systems that control voluntary regulation of emotion). In most cases, the triggers for episodes of laughter and crying were the same as the thoughts and stimuli that could induce crying and laughter in anyone, but they led to rapidly developing high intensity, uncontrollable bursts.…”

“…In most cases, the triggers for episodes of laughter and crying were the same as the thoughts and stimuli that could induce crying and laughter in anyone, but they led to rapidly developing high intensity, uncontrollable bursts. 5 6 Laughter episodes had a greater tendency to occur without an obvious precipitant. Of note, considering the classic distinction between PLC and EI, most episodes described by Olney are consistent with EI rather than true PLC.…”