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[Pathological laughing and crying].

The classic literature on pathological laughter and crying emphasizes the difference between incontinence and lability of affect. Pathological laughter and crying as key symptoms of affect incontinence are viewed as the effects of disinhibition of motor synergisms without congruent affect, which is the crucial difference to affect lability. The interpretation as a disinhibitory phenomenon is supported by clinical and electromyographic observations, which found a lack of modulation of intensity in pathological laughter and crying. In 1924, Wilson postulated a supranuclear pontobulbar center for affective synergisms that is controlled by cortex and thalamus. Accordingly, Kleist viewed a combined lesion of thalamic or brainstem structures and corticofugal motor projection systems as the pathoanatomic basis of affect incontinence. Recent work reported the frequent occurrence of affect incontinence with stroke and dementia of the Alzheimer type and thus disagrees with the classical theory. However, the methods used cannot rule out a confounding between affect lability and incontinence.

Brain↗

Paroxetine versus citalopram treatment of pathological crying after brain injury.

Selective serotonin reuptake inhibitors (SSRIs) are the treatment of choice for pathological crying after brain injury, independent of accompanying depression. In a series of 26 consecutive patients with acquired brain damage and episodes of involuntary crying, the efficacy and tolerability of paroxetine and citalopram were compared. The severity of pathological crying or laughing was rated based on clinical interviews with symptom provocation. The first 13 patients were treated with paroxetine and another 13 patients received citalopram in single daily doses of 10 to 40 mg. Rapid onset (within 1-3 days) and highly significant (p < 0.001) improvements of emotionalism were observed after both paroxetine and citalopram. There were no efficacy differences, despite the longer symptom duration in the citalopram group. The only adverse effect after paroxetine was nausea, which was reversible, in two patients, and nausea with vomiting in another two patients, who were switched to citalopram. Citalopram was tolerated without adverse effects.

Adult↗

Molecular and phenotypic mapping of the short arm of chromosome 5: sublocalization of the critical region for the cri-du-chat syndrome.

Forty-nine individuals have been identified with deletions or translocations involving the short arm of chromosome 5. While most display the classical phenotype of the cri-du-chat syndrome, several of the patients do not have the syndrome or have only a subset of the clinical features. Somatic cell hybrids containing the deleted chromosome 5 were derived from each patient. Each somatic cell hybrid was analyzed at the DNA level using 136 chromosome 5p-specific DNA fragments. It was possible to unambiguously order most of the chromosomal breakpoints present in the somatic cell hybrids based on the hybridization patterns of Southern blots. Further comparisons between the deletions present in the patients and their clinical features identified several chromosomal regions that were involved in specific clinical features. A critical chromosomal region involved the high-pitched cry mapped to 5p15.3, while the chromosomal region involved in the remaining features of the cri-du-chat syndrome mapped to a small region within 5p15.2. Deletions that did not include these two chromosomal regions presented varying clinical phenotypes from severe mental retardation and microcephaly to a clinically normal phenotype. These results demonstrate the need for careful characterization of a 5p deletion in prenatal cases before clinical predictions are made.

Animals↗

Lamotrigine treatment for post-stroke pathological laughing and crying.

Pathologic laughing and crying (PLC) is a common distressing and socially disabling condition in stroke patients. Antidepressants, particularly selective serotonin reuptake inhibitors (SSRIs), have been increasingly recognized as the treatment of choice for pathologic crying (PC). However, little is known about etiologies and other treatment options for various clinical manifestations of PLC. This case report illustrates the beneficial effect of lamotrigine, a novel antiepileptic drug with antidepressant and mood-stabilizing properties in post-stroke PLC. A 60-year-old woman developed PLC after an ischemic stroke affecting the left frontal and temporal lobes. She was treated with lamotrigine initially at the dose of 50 mg a day, which was gradually increased to 100 mg a day over a 4-week period. There was a significant and rapid recovery in both laughing and crying components of PCL with lamotrigine treatment. The symptoms of pathologic laughing have shown a better response to lamotrigine than PC. Controlled investigations are needed to evaluate the beneficial as well as the differential effects of lamotrigine on PLC.

Crying↗

Caregiving and early infant crying in a danish community.

Maternal caregiving and fussing/crying in Danish infants at 3, 6, and 12 weeks were examined using self-report scales and 24-hour behavior diaries. Mothers reported practices commonly associated with responsive caregiving: frequent feeding, prompt response to infant cries, and considerable time holding the infant. Fuss/cry durations peaked in the first 2 months, were highest in evenings, and decreased approximately 50% by 12 weeks. Fussing was the majority behavior, and 9.2% of the infants fussed and cried more than 3 hours per day. In contrast with other Western studies, 24-hour fuss/cry durations were lower, and fussing accounted for up to 80% of total distress. Danish caregiving practices may partially explain the lower durations of infant distress and the lower ratio of cry to fuss. However, some infants fuss/cry a great deal despite sensitive care, which may reflect individual differences in infant maturation of behavior regulation.

Caregivers↗

Corneal abrasion in infancy as a cause of inconsolable crying.

Inconsolable crying is a disturbing symptom in young infants. The following is a case report of an infant who presented with inconsolable crying caused by a corneal abrasion. Corneal abrasion should be considered when inconsolable crying appears in an otherwise asymptomatic infant.

Acute Disease↗

Persistent crying as predominant manifestation of sepsis in infants and newborns.

Acute episodes of unexplained crying in infants may be due to serious and even life-threatening conditions. We present six infants in whom excessive crying was the predominant initial manifestation of sepsis for a period of time that ranged from 2 to 10 hours, before other symptoms or signs became evident. This led to a diagnostic delay in two patients who were considered initially to have infant colic. Sepsis should be considered in the differential diagnosis of acute unexplained crying in infants.

Colic↗

Mirtazapine treatment for pathological laughing and crying after stroke.

Selective serotonin reuptake inhibitors (SSRIs) have been recognized as the treatment of choice for pathological laughing and crying (PLC), which is a common, distressing condition that follows stroke. There have been few reports about other treatment options for PLC. Here, the authors report rapid responses to mirtazapine in two patients with poststroke PLC who failed to respond to SSRIs or bupropion. In the first case, a 63-year-old woman with severe long-standing crying spells that had persisted for 3 months responded well to low-dose mirtazapine within a few days; she could not tolerate citalopram or sertraline. In the second case, both the laughing and crying spells of a 64-year-old woman were improved within a few days of mirtazapine administration, after they had not responded to bupropion. This is one of the first reports to suggest that mirtazapine may be an alternative to SSRIs for treating poststroke PLC.

Antidepressive Agents↗

A comparison of the patterns of mother-baby interaction for a group of crying, irritable babies and a group of more amenable babies.

Ten babies aged 9-14 months, with a previous history of excessive crying behaviour and sleeplessness were matched with similar more contented babies. The twenty mother-baby pairs were observed individually in a 30-minute play session to test the hypothesis that prolonged crying behaviour of babies over several months would have an aversive effect on their mothers to the extent that even after this behaviour had ceased the mothers would interact with the babies less and these pairs would be less responsive to each other's overtures than the non-crying mother-baby pairs. Significant differences were seen between the two groups, 'the criers' group of pairs interacting less (P less than 0.01) and were less responsive to their partners (P less than 0.01); the most marked difference being the percentage of overtures made by the 'cryer' babies which were not responded to by their mothers (P = 0.001).

Child Behavior↗

Asymmetric crying facies with microcephaly and mental retardation. An autosomal dominant syndrome with variable expressivity.

An infant boy with asymmetric crying facies, microcephaly, developmental retardation and failure to thrive is reported. His two siblings died in the newborn period because of complex congenital heart defects. The mother and the maternal grandmother have asymmetric crying facies, microcephaly and normal intelligence. A maternal aunt has severe physical and mental retardation, facial asymmetry, microcephaly, and cleft palate. This family allows an expansion of the spectrum of malformations associated with asymmetric crying facies and suggests autosomal dominant inheritance with variable expressivity.

Abnormalities, Multiple↗

Marfan and cri du chat syndromes in an 18-month-old child: evidence of phenotype interaction.

We report on an 18-month-old girl who has both the cri du chat and Marfan syndromes. She was born at term to a 29-year-old woman with the clinical diagnosis of Marfan syndrome. An evaluation for developmental delay at 2 months of age showed a karyotype of 46,XX,del(5)(15.1), consistent with cri du chat syndrome. At age 18 months she was tall (90 cm, > 95th centile), with an decreased upper segment:lower segment ratio (1.0), and microcephalic (OFC 42.5 cm, < 5th centile). Facial features were typical of cri du chat syndrome. The palm, middle finger and foot lengths were at or above the 95th centile for age. She was hypotonic, and her developmental level was approximately 8-10 months. Echocardiography showed redundant mitral valve tissue, mild mitral insufficiency, dilated aortic sinuses, and a small muscular VSD. We would have anticipated that a patient with an autosomal deletion who also had Marfan syndrome would have had growth failure. However, in this patient the skeletal features of Marfan syndrome (increased body length, decreased upper segment:lower segment ratio, and increased palm, finger, and foot length) predominate.

Chromosome Deletion↗

Anaesthetic considerations for the patient with cri du chat syndrome.

Cri du chat syndrome is an inherited disease affecting multiple organ systems. Most characteristic is the anatomical abnormality of the larynx resulting in a cat-like cry. Issues important in developing an anaesthetic plan include: anatomical abnormalities of the airway, congenital heart disease, hypotonia, mental retardation, and temperature maintenance. We report the case of a 33-month-old patient with cri du chat syndrome undergoing patent ductus arteriosus (PDA) ligation and discuss the anaesthetic issues.

Anesthesia, General↗

Maternal sensitivity behavior and infant crying, fussing and contented behavior: the effects of mother's experienced social support.

Maternal sensitivity behavior, mother's experienced difficulties, experienced social support, and the amount of infant's crying, fussing and contented behavior were studied when the infants were 3 and 12 months of age. Fifty-seven Finnish low-risk mothers' sensitivity behavior was assessed by the Parent-Child Early Relational Assessment method. Infant's crying behavior was assessed by the Baby's Day Record and by the Crying Patterns Questionnaire. The Social Support Questionnaires (SSQ 1 and 2) were used to evaluate mother's experienced difficulties and support. The infants of More Sensitive (MS) mothers were more contented than those with Less Sensitive (LS) mothers. The MS mothers experienced fewer difficulties with their infants and experienced more support from their best friend and their husband than LS mothers. In addition, some gender differences were found concerning infant behavior.

Crying↗

The neuropsychological profile of cri du chat syndrome without significant learning disability.

The clinical picture typically associated with cri du chat syndrome is one of profound intellectual impairment, severe motor delay and increased morbidity. In contrast to this picture, the present case report describes the cognitive and behavioural profile of a young female with cri du chat syndrome who displays no evidence of severe learning disability. Cognitive performance indicated good verbal skills with specific strengths on those tasks that require the ability to store and retrieve verbal information in comparison to poor non-verbal, spatial skills and weaknesses on those tasks that require multi-step manipulation of spatial stimuli and the ability to form whole percepts from fragmentary parts. The finding underlies the importance of assessing the wide range of cognitive potential of individuals with cri du chat syndrome.

Child↗

Fathers' experience of childbirth and its relation to crying in his infant.

As part of a research project exploring ways through which we can understand the crying infant and its family, this study focuses on the experiences of fathers during labour and delivery of their infant. In a previous part of the project it was shown that fathers' negative experiences during the childbirth were correlated with the amount of crying in the infant during the first months after birth. The aim of the present study was to explore and interpret the experiences that fathers reported in an interview when the infant was between six months and one year of age. A hundred and nine fathers were interviewed. The interviews, which took place in the families' homes and with both parents present, were carried out in dialogue form with open-ended questions. The results reveal that complications during the delivery were significantly correlated with the amount of crying in the infant. Feelings of helplessness, of guilt and that staff behaviour had been negative were more common in the group of fathers who experienced the delivery as a negative event. 'Locus of control' seems to be the most relevant concept.

Adult↗

Cri-du-chat syndrome: dental considerations and report of case.

Cri-du-chat syndrome is a severe disease resulting from a deletion of the short arm of chromosome number 5. The basic medical disorder includes dysmorphic facies, mental retardation, and a striking catlike cry in infancy. Because of significant oral anomalies and difficulty in behavior management, the syndrome is of particular interest to the dental practitioner. A case with many of the typical medical findings and significant dental considerations is reported. An aggressive preventive dentistry program is essential. A thorough understanding of the medical and developmental problems of the patient with cri-du-chat syndrome ensures safe and effective dental care.

Child↗

Developmental and behavioural characteristics of cri du chat syndrome.

Developmental and behavioural characteristics were assessed in 27 children with cri du chat syndrome using the Society for the Study of Behavioural Phenotypes questionnaire, which gave information on prenatal and perinatal conditions, neurological problems, and developmental and behavioural difficulties. The findings suggest that the behavioural profile of children with cri du chat syndrome incorporates self injurious behaviour, repetitive movements, hypersensitivity to sound, clumsiness, and obsessive attachments to objects. In terms of a developmental profile, children with cri du chat syndrome were able to communicate their needs, socially interact with others, and have some degree of mobility.

Activities of Daily Living↗

Randomised controlled trial of sucrose by mouth for the relief of infant crying after immunisation.

OBJECTIVES: To evaluate the effect of sucrose solution given by mouth on infant crying times and measures of distress in the immunisation clinic. DESIGN: Randomised, double blind, placebo controlled trial of sucrose solution 75% wt/vol v sterile water as a control. SETTING: The immunisation clinic of the Women's and Children's Hospital, Adelaide. PATIENTS: A total of 107 healthy infants attending for 2, 4, or 6 month immunisations with polio by mouth (Sabin), intramuscular diphtheria, tetanus, and pertussis (DTP), and intramuscular Haemophilus influenzae type b were randomised to receive 2 ml 75% sucrose solution or sterile water by mouth before the two injections. METHODS: The duration of infant crying was recorded during and immediately after two intramuscular immunisations and infant distress was assessed by a visual analogue scale (Oucher scores) independently by a nurse and a parent. RESULTS: The administration of 2 ml 75% sucrose solution by mouth reduced the infant crying time and Oucher distress scores after immunisation with DTP/H influenzae type b. CONCLUSIONS: Infant immunisation by intramuscular injection is a distressing procedure for infants and parents. Sucrose solution at a high concentration reduces infant distress and is safe and clinically useful in this setting.

Administration, Oral↗