Pathological laughter and crying in neurological disorders: recognition and treatment.

Pathological laughter and crying is a disabling symptom complex associated with damage to various central nervous system pathways that control the reflex motor component of emotional expression. Many underlying conditions-including neurodegenerative diseases, CNS inflammation, vascular lesions and traumatic brain injury-can be associated with disinhibition of emotional reflex control. This suggests a disruption of anatomical and functional networks, rather than any specific unifying pathological process. There is a wide differential diagnosis, including depression, dementia and other forms of behavioural disturbance. Diagnostic criteria and rating scales can help with clinical assessments and facilitate clinical trials. There is now good-quality evidence for a combination of dextromethorphan and quinidine, with weaker evidence for tricyclic and selective serotonin reuptake inhibitor antidepressants. Pathological laughter and crying is disabling and underdiagnosed but potentially treatable, and its wider recognition is important.

Excessive Laughter-like Vocalizations, Microcephaly, and Translational Outcomes in the Ube3a Deletion Rat Model of Angelman Syndrome.

Angelman syndrome (AS) is a rare genetic neurodevelopmental disorder characterized by intellectual disabilities, motor and balance deficits, impaired communication, and a happy, excitable demeanor with frequent laughter. We sought to elucidate a preclinical outcome measure in male and female rats that addressed communication abnormalities of AS and other neurodevelopmental disorders in which communication is atypical and/or lack of speech is a core feature. We discovered, and herein report for the first time, excessive laughter-like 50 kHz ultrasonic emissions in the Ube3amat-/pat+ rat model of AS, which suggests an excitable, playful demeanor and elevated positive affect, similar to the demeanor of individuals with AS. Also in line with the AS phenotype, Ube3amat-/pat+ rats demonstrated aberrant social interactions with a novel partner, distinctive gait abnormalities, impaired cognition, an underlying LTP deficit, and profound reductions in brain volume. These unique, robust phenotypes provide advantages compared with currently available mouse models and will be highly valuable as outcome measures in the evaluation of therapies for AS.SIGNIFICANCE STATEMENT Angelman syndrome (AS) is a severe neurogenetic disorder for which there is no cure, despite decades of research using mouse models. This study used a recently developed rat model of AS to delineate disease-relevant outcome measures to facilitate therapeutic development. We found the rat to be a strong model of AS, offering several advantages over mouse models by exhibiting numerous AS-relevant phenotypes, including overabundant laughter-like vocalizations, reduced hippocampal LTP, and volumetric anomalies across the brain. These findings are unconfounded by detrimental motor abilities and background strain, issues plaguing mouse models. This rat model represents an important advancement in the field of AS, and the outcome metrics reported herein will be central to the therapeutic pipeline.

Neuropsiquiatría del síndrome de Susac: a propósito de un caso / Neuropsychiatry of Susac Syndrome: a Case Report

RESUMEN El síndrome de Susac es una entidad clínica poco frecuente, posiblemente mediada por un proceso autoinmune; la tríada clásica se compone de retinopatía, disminución en la agudeza auditiva y síntomas neuropsiquiátricos (encefalopatía). Hay pocos casos descritos con sintomatología neuropsiquiátrica como la sintomatología principal. Presentamos un caso de síndrome de Susac, que corresponde a una mujer de 34 arios, con predominio de sintomatologia neuropsiquiátrica, caracterizada por un síndrome de Klüver-Bucy parcial, un síndrome apático, risa y llanto patológico y alteraciones cognitivas de predominio atencional; dichos síntomas mejoraron cualitativamente con el uso de terapia inmunológica. Este caso revela la importancia de las manifestaciones neuropsiquiátricas como presentación clínica en pacientes con entidades neurológicas.

ABSTRACT Susac syndrome is a rare clinical condition, possibly mediated by an autoimmune process; the classic triad is composed of retinopathy, decreased hearing acuity and neuropsychiatric symptoms (encephalopathy). There are few cases reported with neuropsychiatric symptoms as the main manifestation. We present a case of Susac syndrome in a 34-year-old female with a predominance of neuropsychiatric symptoms, characterised by partial Klüver-Bucy syndrome, apathy syndrome, pathological laughter and crying, and cognitive dysfunction predominantly affecting attention, which showed a qualitative improvement with the use of immunological therapy. This case report highlights the importance of neuropsychiatric manifestations as clinical presentation in patients with neurological conditions.

The importance of humour in oncology: a survey of patients undergoing radiotherapy.

Background: Humour has long been considered an important coping tool for patients with cancer, but published quantitative data about its significance are limited. The purpose of our study was to survey patients with cancer undergoing radiotherapy regarding their opinions about the use of humour in their care. Methods: An anonymous 35-item questionnaire evaluating the patient experience, including the value of humour, was developed by an interdisciplinary team of health care providers (hcps) working within the Radiation Medicine program. This anonymous, voluntary, paper-based survey for self-completion required approximately 10 minutes to finish and was administered during the fall of 2018 and the spring of 2019. Results: For the 199 patients who completed the survey [108 women, 89 men (2 respondents did not specify)], median age was 68 years. That group represents approximately 30%-35% of the patients on treatment during the study period. Almost all respondents (86%) indicated that, during their visits to the cancer centre, it was "somewhat important" or "very important" for health care providers (hcps) to use appropriate humour, and 61% of respondents indicated using humour "frequently" or "always" when dealing with their individual cancers. Most respondents (79%) said that humour decreased anxiety, and 86% indicated that laughing was considered "somewhat important" or "very important." Approximately 4% of respondents even listed "sense of humour" as being the most important quality that they looked for in their interactions with their hcps. Conclusions: Cancer patients undergoing radiotherapy clearly view humour as being important for coping and dealing with their disease, and oncology hcps should routinely consider incorporating the use of appropriate humour into the care that they provide.

Pseudobulbar Affect: An Overview.

Pseudobulbar affect (PBA) is a disorder that develops in the context of a brain injury or underlying neurological dysfunction. It is characterized as an affective disorder of emotional expression. PBA manifests as frequent uncontrollable outbursts of laughing or crying, incongruent with the individual's internal emotional state. It can be challenging for clinicians to differentiate PBA from mood disorders, contributing to its high rate of misdiagnosis. This lack of recognition leads to ineffective and insufficient treatment, impacting patients' quality of life. The current article provides an overview of PBA, including its history, prevalence, pathophysiology, diagnostic criteria, clinical implications, and treatment. [Journal of Psychosocial Nursing and Mental Health Services, 58(9), 19-24.].

Humour and laughing in patients with prolonged incurable cancer: an ethnographic study in a comprehensive cancer centre.

PURPOSE: Most people are familiar with the expression 'laughter is the best medicine'. By enhancing cognitive flexibility and strengthening relationships, laughter can be considered a holistic care-approach. Yet, in medical oncology, especially the palliative phase, using humour can be considered inappropriate or taboo. We aimed to explore the acceptability and functions of humour and laughter in patients with prolonged incurable cancer. METHODS: This study was performed in a Dutch Comprehensive Cancer Centre. We included four short conversations with patients, eighteen in-depth patient-interviews and eleven observational fieldnotes in which humour was a major topic of the conversation. We further administered an online questionnaire to thirty-three oncology clinicians about their experiences with humour. Qualitative data were thematically analysed. We specifically distinguished between humour and laughter. RESULTS: Nearly all specialists reported using humour (97%), and all reported sometimes laughing during consultations; 83% experienced a positive effect of laughter. These results were in line with patients' experiences: Patients noted that humour always stayed alive despite medical difficulties. Apart from this human aspect, patients also used humour to broach difficult topics and downplay challenges. Patients and specialists acknowledged that using humour is sometimes inappropriate, partly because they did not always share the same humour. Laughter, in contrast, was regarded as 'lighter' than humour, and could, accordingly, more easily be implemented. Specialists cautioned against patients using laughter to avoid broaching difficult topics. CONCLUSION: Many conversations were full of laughter. Hierarchy as usually experienced between healthcare professionals and patients/relatives seemed to disappear when using laughter. If applied appropriately, adding shared laughter may help optimize shared decision-making.

Effects of mirthful laughter on pain tolerance: A randomized controlled investigation.

INTRODUCTION: Chronic pain is a debilitating condition that affects many people. Currently, there is no single treatment known to cure or assure relief from chronic pain. Accordingly, the management of patients' discomfort is an integral part of treating chronic pain. Such treatment, however, is not effective for many patients. We investigated whether mirthful laughter provided by comic relief can influence pain tolerance and muscle soreness in young healthy participants. METHODS: Forty participants underwent a randomized controlled cross-over designed experiment. Each participant was exposed to a comedy video eliciting mirthful laughter and an uninteresting documentary. Delayed onset muscle soreness was induced in one leg at a time by eccentric exercise. Pain tolerance was tested using blunt force application and assessed subjectively using a visual analog scale. RESULTS: Watching the comedy video elicited a significantly greater irregular breathing pattern compared with watching the documentary video (p < 0.001). After watching the comedy, the participants' positive affect was increased (Δ2 ±â€¯1) while it was largely decreased (Δ-11 ±â€¯2) after watching the documentary video (p < 0.001). Pain tolerance was decreased by 17 ±â€¯5 N after viewing the documentary video (p < 0.001), but did not change significantly after watching the comedy. CONCLUSIONS: Thirty minutes of watching a comedy eliciting laughter favorably influenced pain tolerance in healthy humans. CLINICAL TRIAL NO.: #NCT02896075.

The Humor in Therapy: the Healing Power of Laughter.

Although humour is a key element of human communication, and, for this reason, it is also present in therapeutic contexts, its use in this sense still remains largely untapped. The purpose of this article is to increase curiosity and broaden the reflection on the use of humour in the psychotherapeutic relationship. The first part is dedicated to a short review of the main theories on the origins of laughter. The second part will examine those studies reporting a beneficial effect of humour on physical well-being, while the third part will review those studies describing how humour can help improve psychological well-being. The fourth part will further explore the clinical effects of humour in the therapeutic relationship. Both the positive and negative effects of humour in the therapeutic relationship will be discussed. In addition, some brief examples are included.

Pharmaceutical interventions for emotionalism after stroke.

BACKGROUND: Antidepressants may be useful in the treatment of abnormal crying associated with stroke. This is an update of a Cochrane Review first published in 2004 and last updated in 2010. OBJECTIVES: To determine whether pharmaceutical treatment reduces the frequency of emotional displays in people with emotionalism after stroke. SEARCH METHODS: We searched the trial register of Cochrane Stroke (last searched May 2018). In addition, we searched the Cochrane Central Register of Controlled Trials (CENTRAL; to May 2018), MEDLINE (1966 to 14 May 2018), Embase (1980 to 14 May 2018), CINAHL (1982 to 14 May 2018), PsycINFO (1967 to 14 May 2018), BIOSIS Previews (2002 to 14 May 2018), Web of Science (2002 to 14 May 2018), WHO ICTRP (to 14 May 2018), ClinicalTrials.gov (to 14 May 2018), and ProQuest Dissertations and Theses Database (to 14 May 2018). SELECTION CRITERIA: Randomised controlled trials (RCTs) and quasi-RCTs comparing psychotropic medication to placebo in people with stroke and emotionalism (also known as emotional lability, pathological crying or laughing, emotional incontinence, involuntary emotional expression disorder, and pseudobulbar affect). DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies, assessed risk of bias, extracted data from all included studies, and used GRADE to assess the quality of the body of evidence. We calculated mean difference (MD) or standardised mean difference (SMD) for continuous data and risk ratio (RR) for dichotomous data with 95% confidence intervals (CIs). We assessed heterogeneity using the I2 statistic. The primary emotionalism measures were the proportion of participants achieving at least a 50% reduction in abnormal emotional behaviour at the end of treatment, improved score on Center for Neurologic Study - Lability Scale (CNS-LS), Clinician Interview-Based Impression of Change (CIBIC) or diminished tearfulness. MAIN RESULTS: We included seven trials with a total of 239 participants. Two trials were of cross-over design, and outcome data were not available from the first phase (precross-over) in an appropriate format for inclusion as a parallel randomised controlled trial (RCT). Thus, the results of the review are based on five trials with 213 participants. Treatment effects were observed on the following primary endpoints of emotionalism: There is very low quality of evidence from one small RCT that antidepressants increased the number of people who had 50% reduction in emotionalism (RR 16.50, 95% CI 1.07 to 253.40; 19 participants) and low quality evidence from one RCT of improved scores on Center for Neurologic Study - Lability Scale (CNS-LS) and Clinician Interview-Based Impression of Change (CIBIC) with antidepressants (RR 1.44, 95% CI 0.95 to 2.19; 28 participants). There was moderate quality evidence from three RCTS that they increased the number of people who had a reduction in tearfulness (RR 2.18, 95% CI 1.29 to 3.71; 164 participants); and low quality evidence from one RCT of improved scores on the Pathological Laughter and Crying Scale (PLCS) (MD 8.40, 95% CI 11.56 to 5.24; 28 participants).Six trials reported adverse events (death) and found no difference between the groups in death (RR 0.59, 95% CI 0.08 to 4.50; 6 RCTs, 172 participants, moderate-quality evidence). AUTHORS' CONCLUSIONS: Antidepressants may reduce the frequency and severity of crying or laughing episodes based on very low quality evidence. Our conclusions must be qualified by several methodological deficiencies in the studies and interpreted with caution despite the effect being very large. The effect does not seem specific to one drug or class of drugs. More reliable data are required before appropriate conclusions can be made about the treatment of post-stroke emotionalism. Future trialists investigating the effect of antidepressants in people with emotionalism after stroke should consider developing and using a standardised method to diagnose emotionalism, determine severity and assess change over time; provide treatment for a sufficient duration and follow-up to better assess rates of relapse or maintenance and include careful assessment and complete reporting of adverse events.

Treatment of Poststroke Pathologic Laughing With Duloxetine: A Case Series.

OBJECTIVES: Pathologic laughing is characterized by episodes of uncontrollable laughter caused by underlying neurologic disturbances, such as stroke. Several types of medication, including selective serotonin reuptake inhibitors, have demonstrated only limited success at treating the condition. Duloxetine, a dual serotonin-norepinephrine reuptake inhibitor, is reportedly effective in treating the symptoms of mood disorders. We herein introduce a prospective consecutive sample of stroke patients with pathologic laughing treated with duloxetine. METHODS: We enrolled patients without a history of psychological illness who exhibited poststroke pathologic laughter. Duloxetine administration was commenced at an initial dose of 30 mg once daily. The dose was increased to 60 mg once daily within 2 weeks for all patients except 2. The effect of the treatment was assessed by means of the Pathological Laughter and Crying Scale. RESULTS: A total of 7 patients were included in the study. Improvements were observed within an average of 10 days after duloxetine administration. Pathological Laughter and Crying Scale score decreased after duloxetine administration in all patients, and 4 patients demonstrated a decrease in score of more than 50%. All patients reported subjective improvement of symptoms, and no adverse effects were observed. CONCLUSIONS: This case series demonstrates that duloxetine could attenuate pathologic laughing exhibited by stroke patients; however, further randomized controlled studies are necessary to validate our findings.

Client laughter in psychodynamic psychotherapy: Not a laughing matter.

We studied 814 client laughter events nested within 330 sessions nested within 33 clients nested within 16 therapists at one community clinic in which doctoral student therapists provided psychodynamic psychotherapy to adult community clients. Each laughter event in Sessions 1 to 5 and 16 to 20 was rated for cheerfulness, politeness, reflectiveness, contemptuousness, and nervousness. Across all clients, there was an average of about one laughter even per session. The average laughter event lasted 3.5 seconds, and was characterized primarily by politeness and reflectiveness. Overall amount of client laughter and the characteristics of client laughter did not change across sessions. Most of the variance in the laughter characteristics was at the session level, with less variance attributable to clients and therapists. When client attachment avoidance was high, laughter was less cheerful and more contemptuous. When client attachment anxiety was high, laughter was more nervous. Sessions with more reflective laughter were evaluated more positively by clients, and therapists whose clients had more reflective laughter had more positive client session evaluations. Furthermore, within a therapist's caseload, clients with the most nervous and contemptuous laughter evaluated sessions most positively. Implications are discussed. (PsycINFO Database Record

Laughter catches attention!

In social interactions, emotionally salient and sudden changes in vocal expressions attract attention. However, only a few studies examined how emotion and attention interact in voice processing. We investigated neutral, happy (laughs) and angry (growls) vocalizations in a modified oddball task. Participants silently counted the targets in each block and rated the valence and arousal of the vocalizations. A combined event-related potential and time-frequency analysis focused on the P3 and pre-stimulus alpha power to capture attention effects in response to unexpected events. Whereas an early differentiation between emotionally salient and neutral vocalizations was reflected in the P3a response, the P3b was selectively enhanced for happy voices. The P3b modulation was predicted by pre-stimulus frontal alpha desynchronization, and by the perceived pleasantness of the targets. These findings indicate that vocal emotions may be differently processed based on task relevance and valence. Increased anticipation and attention to positive vocal cues (laughter) may reflect their high social relevance.

Laughter, Humor, and Cancer: Delicate Moments and Poignant Interactional Circumstances.

Conversation analysis is employed to examine transcribed excerpts drawn from a subsample of 75 naturally occurring and video recorded interviews between cancer patients and 30 doctors. Close examination is provided of how cancer patients initiate, and doctors respond, to laughter and humor during oncology interviews. Interactions demonstrate that communication about the disease "cancer" shares qualities similar to other medical areas (e.g., primary care): the tendency for patients to initiate laughter or humor to address troubling and challenging circumstances; and that during moments when patients address personal matters, doctors are not invited and do not reciprocate with shared laughter and humor. Prominent in talk about cancer are various precarious circumstances, awkward and delicate moments mirroring the lived experiences of cancer patients (e.g., when patients attempt to minimize fears, justify that they are well when threatened with sickness, claim normality in the midst of chronic conditions, and take stances that weight loss and gain are not problematic). These examples provide a compelling case that routine cancer care involves many poignant situations managed through laughter and humor. Implications are raised for how quality care might be improved through grounded understandings of laughter, humor, and cancer.

Student life - When laughter is the best medicine - and when it is not.

As you progress through your placements, you will witness the extremes of the human condition.

Seriously clowning: Medical clowning interaction with children undergoing invasive examinations in hospitals.

This qualitative study examined the subjective experience of children undergoing an invasive examination in the hospital when accompanied by a medical clown. In-depth semi-structured interviews were conducted with nine such children and nine of their accompanying parents. The children were patients in two outpatient departments (Pediatric Gastroenterology and a Center for the Sexually Abused) in a hospital in Israel. Interviews were coded thematically using an Atlas.ti software program. Analysis of the interviews indicated that the intervention of the clown positively changed the children's perceptions of the hospital, of experiencing the examination, and of their life narrative. Medical clowns thus appear to be a central, meaningful, and therapeutic source for children undergoing invasive examinations in hospital, as well as for their parents. Therefore, it may be advisable to incorporate medical clowns as an integral part of medical teams performing invasive procedures and to include the clowns in all stages of the hospital visit.