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Adults with Williams syndrome. Preliminary study of social, emotional and behavioural difficulties.

BACKGROUND: In recent years there has been an upsurge of interest in the study of distinctive patterns of behavioural and psychological characteristics associated with specific, biologically determined, intellectually disabling conditions. This study investigates whether such a profile can be identified in adults with Williams syndrome. METHOD: Parents and other care-givers were interviewed about the social, emotional and behavioural characteristics of 70 adults with Williams syndrome, aged 19 years to 39 years 9 months. RESULTS: The adults were reported to have high rates of behavioural and emotional difficulties, particularly in terms of poor social relationships, over-friendliness and social disinhibition, preoccupations and obsessions, and high levels of anxiety and distractibility. CONCLUSIONS: The findings provide preliminary support for the existence of a specific pattern of behavioural and personality characteristics and associated difficulties in adults with Williams syndrome, which persist from childhood and often require intervention from mental health professionals. Implications for clinical practice are considered.

Adolescent↗

[Dysmorphophobia: past and present of a centuries-old disorders].

Dysmorphophobia is a very interesting psychiatric syndrome that remains controversial, despite it being described more than a century ago. Current knowledge allow us to think of it as a clinical entity in itself, but its relationship with affective disorders and obsessive-compulsive disorder remain unclear. Delusional and non-delusional dysmorphophobia might be two separate disorders or, more likely, variants of the same disorder. Several therapeutic approaches have shown efficacy in the treatment of this condition, like pimozide, serotonergic antidepressants and cognitive-behaviour therapy, but well controlled clinical trials are lacking. It has still to be determined to what extent all this findings about dysmorphophobia in clinical samples do apply to people with body image disorders in the general population.

Antidepressive Agents↗

Compulsive voyeurism and exhibitionism: a clinical response to paroxetine.

The compulsive behaviors seen in sexual paraphilias may be related to those of obsessive-compulsive disorder (OCD). Based primarily upon case reports as well as studies indicating the effectiveness of serotonin reuptake inhibitors in the treatment of sexual paraphilias, it has been speculated that sexual paraphilias lie within the obsessive-compulsive spectrum. There have been no reports of the use of paroxetine in the treatment of sexual paraphilias. This is a report of two patients, the first a voyeur and the second an exhibitionist, both of whom responded to treatment with paroxetine. The discussion addresses the need for further comparative studies investigating the role of the serotonin system in the pathogenesis of sexual paraphilias and OCD as well as the effect of serotonin reuptake inhibitors in comparison to other pharmacologic modalities used in the treatment of paraphilias.

Adult↗

[The neurobiological bases of obsessive-compulsive disorder and Tourette syndrome].

OBJECTIVE: To describe and discuss evidence-based articles on the neurobiology of obsessive-compulsive disorder and Tourette syndrome. SOURCES OF DATA: A review of the most relevant papers on the phenomenology, neuroanatomy, neuroimaging, genetic and immunological aspects of these two disorders was performed. SUMMARY OF THE FINDINGS: Ritualistic behaviors and repetitive thoughts have been extensively studied in the last years. The definitions of obsessive-compulsive disorder and Tourette syndrome emphasize the existence of a continuum of symptoms, with high prevalence in the general population. Neurobiological findings have implicated genetic and immunological factors in the etiology of these two disorders. CONCLUSIONS: Advances in neuroscience triggered genetic and immunological research studies, allowing new perspectives on the treatment of obsessive-compulsive disorder and Tourette syndrome patients.

Diagnosis, Differential↗

Intolerance of uncertainty in obsessive-compulsive disorder.

Pathological doubt, a prominent feature of obsessive-compulsive disorder (OCD), may be related to difficulty tolerating ambiguous or uncertain situations. This is thought to be particularly true of those patients with checking compulsions. Intolerance of uncertainty (IU) has been studied extensively within the domains of worry and generalized anxiety; however, it has received relatively little empirical attention in OCD patients. We administered the Intolerance of Uncertainty Scale [Personality and Individual Differences 17 (1994) 791] to 55 clinic patients with OCD, 43 of whom had checking compulsions, and 14 nonanxious controls. OC checkers showed greater IU than did OC noncheckers and NACs. The latter two groups did not differ from each other. Furthermore, both repeating and checking rituals were associated with IU. Pathological doubt may be understood not only in terms of knowledge-based constructs, but also patients' emotional reaction to feelings of uncertainty. We discuss the implications for increasing tolerance via cognitive-behavioral therapy.

Adult↗

Thyroid hormone concentrations in depressed and nondepressed adolescents: group differences and behavioral relations.

OBJECTIVE: To examine thyroid hormone concentrations and the influence of these hormones on mood and problem behaviors in adolescents with depression. METHOD: The sample included 21 depressed adolescents and 20 matched control adolescents. Blood was drawn to measure thyroid-stimulating hormone (TSH), free thyroxine (FT4), thyroxine (T4), and triiodothyronine (T3). Major depression (MD), attention deficit (AD), and obsessive-compulsive (OC) symptom scores were abstracted from the Diagnostic Interview Schedule for Children. Total behavior problem scores from the Youth Self-Report also were obtained. RESULTS: Paired analysis revealed there were no significant group or gender differences or group by gender interactions for TSH, T4, or T3. For FT4, however, there were significant group differences (p = .008) showing lower concentrations in depressed adolescents than control subjects, suggesting that the former might be functionally hypothyroid. Although there were no significant correlations of TSH with any of the psychological measures obtained, in the depressed group correlations were negative (although not always significant) with FT4 and total behavior problems (r = -.40, p = .09), as well as with symptom scores of MD (r = -.25, p = .288), OC (r = -.56, p = .011), and AD behaviors (r = -.57, p = .008). Higher numbers of symptom scores of OC and AD were related to lower concentrations of FT4. CONCLUSIONS: FT4 concentrations were lower in depressed adolescents. These findings suggest a relationship between negative behaviors and dysfunction of the hypothalamic-pituitary-thyroid axis in adolescents with depression.

Adolescent↗

Behavioral and neuroendocrine responses to metaCPP in anorexia nervosa.

In response to the partial serotonin agonist meta-chlorophenylpiperazine (metaCPP), patients with obsessive-compulsive disorder have been reported to exhibit an increase in obsessive symptoms and a diminished release of prolactin and/or cortisol compared to controls. We examined the response to metaCPP of 10 patients with anorexia nervosa, before and after weight gain, and of eight healthy controls. Prior to weight gain, the patients exhibited a greater behavioral response to metaCPP than the controls and diminished prolactin and cortisol responses. Only the prolactin abnormality persisted after weight gain. These observations suggest that, in patients with anorexia nervosa, weight loss is associated with the development of neurobiological disturbances which are in some ways similar to those of obsessive-compulsive disorder.

Adult↗

Positive and negative schizotypy in a student sample: neurocognitive and clinical correlates.

Positive and negative schizotypy may represent discrete factors or dimensions. To determine if distinct neurocognitive profiles are associated with these dimensions or factors, we classified university students on the basis of positive and negative schizotypal symptoms and conducted separate analyses. Following prior work in the neuropsychiatric literature, we predicted that subtle prefrontal deficits would be selectively associated with negative schizotypal personality features in a nonclinical student sample. We also investigated the relationship between positive/negative schizotypy and associated clinical states or personality dimensions including antisocial personality disorder, obsessive-compulsive personality traits, generalized and social anxiety, empathy, and impulsivity. Classification of subjects into positive and negative schizotypy groups revealed distinct neurocognitive and clinical profiles. We observed a positive relation between measures of temporolimbic dysfunction, impulsivity, antisocial behavior, and positive schizotypal phenomena. Negative schizotypy was associated with subtle performance deficits on measures of frontal executive function, increased social anxiety, and obsessive-compulsive phenomena. Findings are consistent with the contention that positive and negative schizotypy represent discrete factors.

Adolescent↗

[Exposing obsessive-compulsive patients: often only 3 simple questions are enough. The family physician as "facilitator of self-disclosure"].

For numerous patients with obsessive-compulsive disorder, the family doctor is the first contact person. Many of these patients have concealed their problem for years. This paper summarizes the forms of the disorder and their indirect manifestations in the doctor's office, discusses the reasons for concealment on the part of the patient and/or his relatives, and describes the family doctor's help in three areas: aiding the "birth of revelation"; initiating pharmacotherapy and supporting behavior therapy-related self-help for patient and relatives; motivating patient and relatives to seek expert psychiatric and psychotherapeutic aid. In the second part of the paper, the family doctor is given model explanations designed as answers to common questions asked by sufferers from obsessive-compulsive disorder.

Behavior Therapy↗

Obsessive-compulsive syndromes in older adolescents.

OBJECTIVE: This report presents data on the prevalence of both obsessive-compulsive disorder (OCD) and subthreshold obsessive-compulsive syndrome (sOCS) in a representative sample of older male adolescents living in Piedmont, Italy. METHOD: A total of 1,883 recruits to the Italian Defence Force were interviewed using the OC portion of the Diagnostic Interview Schedule. A diagnosis of sOCS was made for individuals who had obsessive and/or compulsive symptoms but who failed to meet one of the severity criteria of impairment. RESULTS: The point and lifetime prevalence rates of OCD were 2.0% and 2.6%, respectively. In total, 12.3% of the subjects received a current diagnosis of sOCS. No differences were found when examining prevalence rates according to area of residence or occupational status of the subjects. CONCLUSION: Our findings suggest that OCD is as common in older male adolescents as it is in adults, and that sOCS, being commonly endorsed in a general adolescent population, may represent a normal phenomenon in older adolescents.

Adolescent↗

Cognitive therapy with obsessive-compulsive disorder.

BACKGROUND: People with obsessive-compulsive disorders (OCD) are widely treated with a combination of medication and behavioural techniques. The success rate is 50-85%, but both relapse and drop-out rates appear high. The use of cognitive therapy (CT) for the treatment of OCD has been suggested. The empirical evidence supporting the use of CT for OCD is examined. METHOD: A manual and computer (Medline) literature search was performed. RESULTS: Fifteen empirical studies were found: ten non-controlled, and five controlled. CONCLUSIONS: There are few controlled CT studies, and these show little evidence of improvement when CT is added to existing therapeutic techniques.

Adult↗

Obsessive compulsive disorder in children and adolescents.

During 1992, further progress has been made in determining the incidence of obsessive compulsive disorder and change in symptoms over time. Advances have been made in determining what category of mood disorder obsessive compulsive disorders belong to; contributions have been made in the theoretic understanding of the cause of obsessive compulsive disorder, and why it may be associated with trichotillomania and Tourette's syndrome. Finally, the treatment of children with both medication and behavior therapy has been explored.

Adolescent↗

Obsessive-compulsive symptoms and the family.

A questionnaire study of 98 family members of individuals with obsessive-compulsive symptoms revealed that 60% of the family members were involved to some extent in rituals with the affected family member. Nearly all family members reported at least some degree of interference in their lives. Information was also gathered about the sort of rituals in which members were involved, how they responded to the demands of the affected relative to engage in the rituals, their beliefs and knowledge about compliance, and the degree to which the rituals interfered in their lives. The study highlights the possible need for support, advice, and education for family members of persons with obsessive-compulsive symptoms.

Adolescent↗

Recollections of parent-child relationships in OCD out-patients compared to depressed out-patients and healthy controls.

The present study examined the relationship between parental bonding in 26 psychiatric out-patients with obsessive-compulsive disorder (OCD) compared to 34 out-patients with major depressive disorder (MDD) and 41 healthy controls. The results indicated significantly lower levels of parental care and higher levels of maternal overprotection for the patients with a current principal diagnosis of depression, compared to healthy controls. In multiple regression analyses, the presence of depressive disorder was related to all Parental Bonding Inventory (PBI) subscales. When the presence of current depressive disorder was controlled for statistically, the diagnosis of OCD was not associated with abnormal patterns of parental bonding on the PBI.

Adult↗

Association between maternal smoking and increased symptom severity in Tourette's syndrome.

OBJECTIVE: Substantial evidence suggests that both environmental and genetic factors contribute to the development and clinical expression of Tourette's syndrome. Although genetic studies of Tourette's syndrome are common, studies of environmental factors are relatively few and have not identified consistent risk factors across studies. This study examines in a large cohort of subjects (N=180) the relationship between prenatal/perinatal adverse events with Tourette's syndrome severity as determined by tic severity and rates of commonly comorbid disorders such as obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), and self-injurious behavior. METHOD: Tic severity, OCD, ADHD, self-injurious behavior, and exposure to a variety of prenatal/perinatal events were systematically assessed in all subjects enrolled in three genetic studies of Tourette's syndrome. Using linear and logistic regression, a best-fit model was determined for each outcome of interest. RESULTS: Prenatal maternal smoking was strongly correlated with increased tic severity and with the presence of comorbid OCD in these Tourette's syndrome subjects. Other variables, such as paternal age and subject's birth weight, were significantly but less strongly associated with increased symptom severity. The authors found no association between symptom severity and hypoxia, forceps delivery, or hyperemesis during pregnancy, which have been previously identified as risk factors. CONCLUSIONS: This study identifies prenatal maternal smoking as a strong risk factor for increased symptom severity in Tourette's syndrome.

Adolescent↗

Development of the OCDS--revised: a measure of alcohol and drug urges with outpatient substance abuse clients.

Urges to use substances is an important construct in understanding the maintenance of substance use as well as relapse. There is a need to evaluate single instruments measuring global urges to use substances (including alcohol). The Obsessive Compulsive Drinking Scale (OCDS; R. F. Anton, D. H. Moak, & P. Latham, 1995) was revised and tested as a single measure of urges to use substances. Participants were 252 adults entering outpatient substance abuse treatment. Results suggest that the revised scale (OCDS-R) primarily assesses 1 global factor for urges to use substances. In addition, strong support was found for the reliability and concurrent validity of the OCDS-R, although results for its predictive validity were mixed. The OCDS-R is a brief, psychometrically sound self-report instrument that has applicability within clinical and research settings.

Adult↗

The relation among perfectionism, obsessive-compulsive personality disorder and obsessive-compulsive disorder in individuals with eating disorders.

OBJECTIVE: Perfectionism and obsessionality are core features of eating disorders (ED), yet the nature of their relation remains unknown. Understanding the relation between these traits may enhance our ability to identify relevant behavioral endophenotypes for ED. METHOD: Six-hundred seven individuals with anorexia and bulimia nervosa from the International Price Foundation Genetic Study were assessed for perfectionism, obsessive-compulsive personality disorder (OCPD), and obsessive-compulsive disorder (OCD). RESULTS: No differences were found across ED subtypes in the prevalence of OCPD and OCD, nor with the association between OCD and OCPD. Perfectionism scores were highest in individuals with OCPD whether alone or in combination with OCD. CONCLUSION: Perfectionism appears to be more closely associated with obsessive-compulsive personality symptoms rather than OCD. The pairing of perfectionism with OCPD may be a relevant core behavioral feature underlying vulnerability to ED.

Adolescent↗

Behavioral effects of cocaine on a transgenic mouse model of cortical-limbic compulsion.

We previously created a transgenic mouse model of cortical-limbic induced compulsions in which dopamine D1 receptor-expressing (D1+) neurons in restricted regional subsets of the cortex and amygdala express a neuropotentiating cholera toxin (CT) transgene. These 'D1CT' mice engage in complex behavioral abnormalities uniquely resembling human compulsions, such as non-aggressive biting of cagemates during grooming, repeated leaping and episodes of perseverance of any and all normal behaviors. Because both compulsions and cocaine-induced behaviors may represent forms of psychomotor activation that have a shared or overlapping neurological basis, we have examined the behavioral response of these 'compulsive' mice to cocaine. In both control and D1CT mice, cocaine increased the amount of time spent engaged in typical cocaine-dependent stereotypies such as locomotion, sniffing, or gnawing, while the remainder of behaviors within their normally complete behavioral repertoires decreased. Cocaine also decreased, rather than facilitated, the incidence of D1CT transgene-induced compulsion-like behaviors such as repeated leaping and perseverance of any and all normal behaviors. The indistinguishable cocaine responses of D1CT and normal mice, as well as the masking (rather than potentiation) of D1CT mouse compulsion-like behaviors by cocaine, suggests that cortical-limbic induced compulsions are significantly different in their origin or circuitry from cocaine-induced stereotyped behaviors. Specifically, these data suggest that the motor circuits stimulated in compulsions represent only a subset of the parallel circuits stimulated by cocaine. These data are, thus, consistent with the hypothesis that topographically restricted subsets of parallel cortical-striatal-thalamic loops induce different types of compulsive behaviors.

Animals↗

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