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.

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.

Reported maternal childhood maltreatment experiences, amygdala activation and functional connectivity to infant cry.

Maternal childhood maltreatment experiences (CMEs) may influence responses to infants and affect child outcomes. We examined associations between CME and mothers' neural responses and functional connectivity to infant distress. We hypothesized that mothers with greater CME would exhibit higher amygdala reactivity and amygdala-supplementary motor area (SMA) functional connectivity to own infant's cries. Postpartum mothers (N = 57) assessed for CME completed an functional magnetic resonance imaging task with cry and white-noise stimuli. Amygdala region-of-interest and psychophysiological interaction analyses were performed. Our models tested associations of CME with activation and connectivity during task conditions (own/other and cry/noise). Exploratory analyses with parenting behaviors were performed. Mothers with higher CME exhibited higher amygdala activation to own baby's cries vs other stimuli (F1,392 = 6.9, P < 0.01, N = 57) and higher differential connectivity to cry vs noise between amygdala and SMA (F1,165 = 22.3, P < 0.001). Exploratory analyses revealed positive associations between both amygdala activation and connectivity and maternal non-intrusiveness (Ps < 0.05). Increased amygdala activation to own infant's cry and higher amygdala-SMA functional connectivity suggest motor responses to baby's distress. These findings were associated with less intrusive maternal behaviors. Follow-up studies might replicate these findings, add more granular parenting assessments and explore how cue processing leads to a motivated maternal approach in clinical populations.

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.].

Differential responses to infant faces in relation to maternal substance use: An exploratory study.

BACKGROUND: Maternal substance use and addiction has been associated with negative consequences for parenting and may increase addiction vulnerability in the developing child. Neuroimaging research suggests that substance use may decrease the reward of caring for infants and heighten stress reactivity to affective infant cues. METHODS: Thirty-two substance-using mothers and twenty-two non-substance-using mothers were presented with emotional face and cry stimuli generated from their own and a demographically matched unknown infant during fMRI scanning. Between-group differences in neural activity during task performance were assessed using whole-brain, mixed-effects models corrected for multiple comparisons (voxel-level p < 0.001, pFWE<0.05). RESULTS: Relative to non-substance-using mothers, substance-using mothers exhibited greater activation when viewing their own infant's face as compared to an unknown infant's face across multiple brain regions, including superior medial frontal, inferior parietal, and middle temporal regions. Substance-using mothers also had a decreased response to sad infant faces in the ventral striatum relative to the non-substance-using mothers. Neural responses to own vs. unknown infant cries did not significantly differ between substance-using and non-substance-using mothers. CONCLUSIONS: Findings suggest overlapping cortical and subcortical brain regions implicated in responding to infant faces, with activation differences related to infant familiarity, emotional expression, and maternal substance use. While prior work has focused on attenuated neural responses to infant cues, greater attention is needed toward understanding the increased reactivity to affective infant cues observed in substance-using mothers.

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.

A walk on the dark side: TMS over the right inferior frontal gyrus (rIFG) disrupts behavioral responses to infant stimuli.

Infant signals, including infant sounds and facial expressions, play a critical role in eliciting parental proximity and care. Processing of infant signals in the adulthood brain is likely to recruit emotional empathy neural circuits, including the inferior frontal gyrus (IFG). Here, we used transcranial magnetic stimulation (TMS) to test the role of right IFG (rIFG) in behavioral responses to infant signals. Specifically, a group of nulliparous women were asked to perform a handgrip dynamometer task and an Approach-Avoidance Task (AAT) after receiving TMS over the right IFG or over a control site (vertex). Suppressing activity in the rIFG affected the modulation of handgrip force in response to infant crying. Moreover, the AAT showed that participants tend to avoid the sad infant face after Vertex stimulation, and this bias was counteracted by rIFG stimulation. Our results suggest a causal role of rIFG in sensitive responding towards sad infants and point to the rIFG as a critical node in the neural network underlying the innate releasing mechanism for feelings of love, affection and caring of sad infants.

Manual therapy for unsettled, distressed and excessively crying infants: a systematic review and meta-analyses.

OBJECTIVE: To conduct a systematic review and meta-analyses to assess the effect of manual therapy interventions for healthy but unsettled, distressed and excessively crying infants and to provide information to help clinicians and parents inform decisions about care. METHODS: We reviewed published peer-reviewed primary research articles in the last 26 years from nine databases (Medline Ovid, Embase, Web of Science, Physiotherapy Evidence Database, Osteopathic Medicine Digital Repository , Cochrane (all databases), Index of Chiropractic Literature, Open Access Theses and Dissertations and Cumulative Index to Nursing and Allied Health Literature). Our inclusion criteria were: manual therapy (by regulated or registered professionals) of unsettled, distressed and excessively crying infants who were otherwise healthy and treated in a primary care setting. Outcomes of interest were: crying, feeding, sleep, parent-child relations, parent experience/satisfaction and parent-reported global change. RESULTS: Nineteen studies were selected for full review: seven randomised controlled trials, seven case series, three cohort studies, one service evaluation study and one qualitative study.We found moderate strength evidence for the effectiveness of manual therapy on: reduction in crying time (favourable: -1.27 hours per day (95% CI -2.19 to -0.36)), sleep (inconclusive), parent-child relations (inconclusive) and global improvement (no effect). The risk of reported adverse events was low: seven non-serious events per 1000 infants exposed to manual therapy (n=1308) and 110 per 1000 in those not exposed. CONCLUSIONS: Some small benefits were found, but whether these are meaningful to parents remains unclear as does the mechanisms of action. Manual therapy appears relatively safe. PROSPERO REGISTRATION NUMBER: CRD42016037353.

"A sweet smile": the modulatory role of emotion in how extrinsic factors influence taste evaluation.

It has recently been demonstrated that the reported tastes/flavours of food/beverages can be modulated by means of external visual and auditory stimuli such as typeface, shapes, and music. The present study was designed to assess the role of the emotional valence of the product-extrinsic stimuli in such crossmodal modulations of taste. Participants evaluated samples of mixed fruit juice whilst simultaneously being presented with auditory or visual stimuli having either positive or negative valence. The soundtracks had either been harmonised with consonant (positive valence) or dissonant (negative valence) musical intervals. The visual stimuli consisted of images of emotional faces from the International Affective Picture System (IAPS) with valence ratings matched to the soundtracks. Each juice sample was rated on two computer-based scales: One anchored with the words sour and sweet, while the other scale required hedonic ratings. Those participants who tasted the juice sample while presented with the positively-valenced stimuli rated the juice as tasting sweeter compared to negatively-valenced stimuli, regardless of whether the stimuli were visual or auditory. These results suggest that the emotional valence of food-extrinsic stimuli can play a role in shaping food flavour evaluation and liking.

Evidence for a Caregiving Instinct: Rapid Differentiation of Infant from Adult Vocalizations Using Magnetoencephalography.

Crying is the most salient vocal signal of distress. The cries of a newborn infant alert adult listeners and often elicit caregiving behavior. For the parent, rapid responding to an infant in distress is an adaptive behavior, functioning to ensure offspring survival. The ability to react rapidly requires quick recognition and evaluation of stimuli followed by a co-ordinated motor response. Previous neuroimaging research has demonstrated early specialized activity in response to infant faces. Using magnetoencephalography, we found similarly early (100-200 ms) differences in neural responses to infant and adult cry vocalizations in auditory, emotional, and motor cortical brain regions. We propose that this early differential activity may help to rapidly identify infant cries and engage affective and motor neural circuitry to promote adaptive behavioral responding, before conscious awareness. These differences were observed in adults who were not parents, perhaps indicative of a universal brain-based "caregiving instinct."

Dextromethorphan/quinidine for the treatment of pseudobulbar affect.

OBJECTIVE: To evaluate the role of dextromethorphan/quinidine (DM/Q; Nuedexta™) in the treatment of pseudobulbar affect (PBA). DATA SOURCES: A literature search of MEDLINE/PubMed (January 1966-June 2013) was conducted using search terms pseudobulbar affect, pathological laughing and/or crying, emotional lability, dextromethorphan, and quinidine. STUDY SELECTION AND DATA EXTRACTION: English language clinical trials and case reports evaluating the safety and efficacy of DM/Q in PBA were included for review. Bibliographies of all relevant articles were reviewed for additional citations. DATA SYNTHESIS: PBA, a poorly understood disorder, is characterized by involuntary crying and/or laughing. In the past, antidepressants and antiepileptics have been used off-label with mixed results. Four clinical trials have evaluated the use of DM/Q for the treatment of PBA. Although the therapeutic outcomes with DM/Q have been positive, interpretation of the published evidence is limited by small sample size and short treatment duration. CONCLUSIONS: Based on the data available, DM/Q may be a viable, short-term treatment alternative for PBA. Long-term safety and efficacy data are lacking.

Responding to a crying infant - you do not learn it overnight: a phenomenological study.

OBJECTIVE: to examine the views and practices of first-time and experienced mothers in response to infant crying during the first 12 weeks post birth. DESIGN: longitudinal, qualitative study using an interpretive, phenomenological approach. SETTING: postnatal hospital and home settings in Switzerland. PARTICIPANTS: maximum variation sampling of 15 new mothers of diverse parity and educational background who had given birth to a full-term healthy neonate. METHODS: participant observations in the postnatal ward and two narrative interviews at participants' homes at 6-8 and 12-14 weeks post partum. Data analysis used interpretive approaches of case analysis, thematic analysis and exemplars. FINDINGS: first-time mothers showed some soothing skills from the beginning, but fine-tuned their practices of handling the crying infant and managing their own reactions. With growing experience mothers acquired a differentiated understanding of the crying's reason and urgency and used more successful soothing techniques. At the same time they learned to assess and mitigate their own stress reactions by self-soothing and adopting realistic expectations of normal infant behaviour. Experienced mothers knew the infant's frequent crying would diminish after a while whereas first-time mothers coped without this positive expectation. KEY CONCLUSIONS: with increasing child-care experience mothers' skills and attitudes towards crying changed, leading to a calmer and less escalating response to their crying infant. IMPLICATIONS FOR PRACTICE: inexperienced mothers need information on neonatal crying behaviour and on parents' stress response. They should be taught how to recognise and respond to the new-born's signals, and how to cope with their own stress. Postnatal care should provide novice mothers to learn from experienced role models.

Os efeitos do Toque Terapêutico na Síndrome Pré-Menstrual / The effects of Therapeutic Touch on Premenstrual Syndrome

O estudo teve por finalidade verificar a eficácia do Toque Terapêutico (TT ou Método Krieger-Kunz) sobre os sintomas da Síndrome Pré-Menstrual (SPM) que acometem muitas mulheres.Tratou-se de um estudo piloto e a amostra foi composta por vinte graduandas de enfermagem da Universidade de São Paulo que atenderam aos critérios de inclusão do projeto e se submeteram a uma sessão semanal de TT por três meses. Foi realizada uma análise das proporções com o teste t-pareado relacionada às variáveis, que corresponderam aos sintomas do Índice de Blatt e Kupperman, coletadas antes e depois das intervenções com a terapia complementar. O resultado encontrado foi p=0,000, o que indica uma diferença significativa antes e depois da intervenção, demonstrando a eficácia do TT na diminuição dos sintomas da SPM com mudanças significativas entre o momento zero e o momento final. Neste estudo a intervenção com o Toque Terapêutico trouxe alívio à Síndrome PRé-Menstrual.(AU)

This study aimed to verify the effectiveness of Therapeutic Touch (TT of Krieger-Kunz Method) on the symptoms of premenstrual syndrome (PMS) that affects many women. It was a pilot study and the sample was composed of twenty nursing students from USP who met the inclusion criteria of the project and underwent a weekly session of TT for three months. The researches conducted an analysis which the paired t-test related to the variables that corresponded to the symptoms of the Blatt and Kupperman Index, and they were collected before and after the interventions which the complementary therapy. The result found was p=0,000; this indicates a significant difference before and after the intervention, and it demonstrates the effectiveness of TT in reducing the symptoms of PMS with significant changes between the zero time and the final moment. The intervention with Therapeutic Touch provided relief on premenstrual syndrome in this study.(AU)

The effect of a familiar scent on the behavioral and physiological pain responses in neonates.

There are adverse physiologic effects of pain in neonates, and effective pain management must be an essential aspect of neonatal care. In this study we assessed the effect of a nonmaternal familiar scent on the neonatal pain responses. This study included 135 neonates randomly assigned to one of three groups. During arterial puncture, one group was exposed to a vanillin scent on a gauze pad held next to their nose. They were familiarized with it the night before blood sampling by a scented gauze pad placed in the incubator next to their head for an average duration of 8.65 hours. The second group was not familiarized with the scent but was exposed to it during the procedure. The third group was neither familiarized nor exposed to the scent. The duration of crying in the familiar scent group was significantly lower than in the two other groups. Comparison of the physiologic parameters showed less variation in oxygen saturation level during arterial puncture in the familiar scent group. In this study, a familiar scent could reduce crying and oxygen consumption during arterial puncture.

Associations between first-time expectant women's representations of attachment and their physiological reactivity to infant cry.

Associations among 53 primiparous women's Adult Attachment Interview classifications (secure-autonomous vs. insecure-dismissing) and physiological and self-reported responses to infant crying were explored. Heart rate, skin conductance levels, and respiratory sinus arrhythmia (RSA) were recorded continuously. In response to the cry, secure-autonomous women demonstrated RSA declines, consistent with approach-oriented responses. Insecure-dismissing women displayed RSA and electrodermal increases, consistent with behavioral inhibition. Furthermore, insecure-dismissing women rated the cries as more aversive than secure-autonomous women. Nine months postpartum, secure-autonomous women, who prenatally manifested an approach-oriented response to the unfamiliar cry stimulus, were observed as more sensitive when responding to their own distressed infant, whereas women classified prenatally as insecure-dismissing were observed as less sensitive with their own infants.

Patients with unresponsive wakefulness syndrome respond to the pain cries of other people.

OBJECTIVE: Recent publications show that using imagery instructions, brain activation patterns indicating consciousness can be found in approximately 10% of patients with unresponsive wakefulness syndrome (UWS; previously called vegetative state). It is possible, however, that patients who cannot follow instructions (because of limited memory/attention capacities, for example) are nevertheless conscious and retain emotional abilities to feel pain and pleasure. The aim of this study was to assess residual affective consciousness in a specific network of brain structures, the so-called pain matrix (PM) of the brain. METHODS: We examined 44 carefully diagnosed UWS patients at 2 imaging centers. fMRI was used to investigate the brain hemodynamic responses to (a) imagery instructions, and (b) pain cries as opposed to neutral human vocalizations. RESULTS: In line with the data of other groups, consistent responses to imagery instructions were obtained in 5 patients. In contrast, the PM was activated by pain cries in 24 patients. The PM consists of a sensory subsystem, which underlies pain sensation, and an affective subsystem, which underlies the characteristic aversive emotional tone of pain. The former was activated in 34% of patients, the latter in 30% of patients. CONCLUSION: Although there is debate about whether patients with UWS can perceive their own pain, our data indicate that many of them respond to the signals of pain in others. One can speculate that "affective consciousness" can remain even in patients with very severe brain damage who have no capacity for cognition.