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PubMed2005

McLeod syndrome: life-long neuropsychiatric disorder due to a novel mutation of the XK gene.

A 50-year-old man presented with worsening, virtually lifelong, chorea and progressive behavioural disturbance, involving disinhibition and hoarding, over 10 years. Clinical assessment revealed chorea, dysarthria, areflexia, an inappropriately jovial, impulsive manner and neuropsychological evidence of frontosubcortical dysfunction. Investigation results included an elevated creatine kinase, caudate atrophy and hypoperfusion, acanthocytes in the peripheral blood and the McLeod phenotype. DNA studies demonstrated a single-base deletion at position 172 in exon 1 of the XK gene, giving rise to a premature stop codon at position 129 in exon 2.

Acanthocytes↗
PubMedDate not supplied

Hoarding--what does it mean?

Collecting is a normal phenomenon, in contrast with pathological collecting, or hoarding. Is hoarding a different phenomenon, or an extreme aspect existing on the same spectrum of behavior? How may these phenomena be understood when they are part of everyone's repertoire on one hand, but may be symptomatic of a grave disturbance on the other hand. An overview and a discussion of hoarding are presented.

Humans↗
PubMed2003

Estrogen fluctuations, oral contraceptives and borderline personality.

Results from three studies suggest fluctuation in estrogen level may influence the expression of borderline personality disorder (BPD) symptoms. In the first study, 226 women were administered the Personality Assessment Inventory, borderline scales (PAI-BOR; L.C. Morey, The Personality Assessment Inventory, Professional Manual, 1991) and a questionnaire that assessed time in menstrual cycle and use of oral contraceptives, that is synthetic estrogens. BPD symptoms were most common in women using oral contraceptives and during times in the menstrual cycle when estrogen level is rising. In Study 2, 52 women were measured four times across one menstrual cycle and provided salivary samples at each test session. The samples were assayed and estrogen levels were obtained. The principle finding was that variation in estrogen levels predicted the presence of BPD symptoms (r=0.4, p<0.01). This relationship remained significant when a general increase in negative affect was statistically controlled. Study 3 employed a pre-post Oral Contraceptive (OC) design with a control group. It was found that for women with high pre-existing levels of BPD, symptoms became significantly worse after starting pill use (F (3,42)=4.7; p<0.01). Research findings that link the serotonin system and estrogen are reviewed and theoretical and practical implications are discussed.

Adolescent↗
PubMed1996

Obsessive-compulsive disorder.

The prevalence of OCD in a dermatologic practice may be much higher than in the general population. OCDs can be debilitating in one's interpersonal, social, and occupational functioning. The obsessions and compulsions typically begin fairly early in life and may consume prolonged lengths of the patient's time to complete daily rituals of washing, checking, touching, arranging, hoarding, or ruminating. Evidence is mounting for support of a neurobiologic basis in the etiology of OCD. In terms of treatment, the psychopharmacologic agents (clomipramine, fluoxetine, fluvoxamine, sertraline, paroxetine) and behavior therapy alone or in combination with SRIs help a significant majority of patients suffering from this disorder. The OCD spectrum of disorders is varied. Patients presenting to the dermatologist will exhibit an interesting array of symptoms, including those who compulsively hand wash, pick at nails or skin, pull body hair, or display other SIB. Increased awareness of these disorders will enable the dermatologist to identify and treat patients with OCD appropriately.

Antipsychotic Agents↗
PubMed1995

A case-controlled study of repetitive thoughts and behavior in adults with autistic disorder and obsessive-compulsive disorder.

OBJECTIVE: The purpose of this study was to investigate the types of repetitive thoughts and behavior demonstrated by adults with autistic disorder and compare them with those of age- and sex-matched adults with obsessive-compulsive disorder. METHOD: Fifty consecutive patients admitted to the Yale Adult Pervasive Developmental Disorders (Autism) Clinic with a primary diagnosis of autistic disorder (DSM-III-R and DSM-IV) completed the symptom checklist of the Yale-Brown Obsessive Compulsive Scale. Types of current obsessions and compulsions were evaluated. The comparison group consisted of 50 age- and sex-matched adults with obsessive-compulsive disorder (without tics) (DSM-III-R and DSM-IV). RESULTS: Direct discriminant function analysis showed that the patients with autistic disorder could be distinguished from those with obsessive-compulsive disorder on the basis of the types of current repetitive thoughts and behavior that they demonstrated. Compared to the obsessive-compulsive group, the autistic patients were significantly less likely to experience thoughts with aggressive, contamination, sexual, religious, symmetry, and somatic content. Repetitive ordering; hoarding; telling or asking (trend); touching, tapping, or rubbing; and self-damaging or self-mutilating behavior occurred significantly more frequently in the autistic patients, whereas cleaning, checking, and counting behavior was less common in the autistic group than in the patients with obsessive-compulsive disorder. In addition, a specific subset of seven obsessive-compulsive variables from the Yale-Brown Obsessive Compulsive Scale symptom checklist was identified that reliably predicted membership in the autistic group. CONCLUSIONS: These results suggest that the repetitive thoughts and behavior characteristics of autism differ significantly from the obsessive-compulsive symptoms displayed by patients with obsessive-compulsive disorder. Future studies are warranted to assess the treatment response and neurobiological underpinnings of repetitive thoughts and behavior in patients with autism and obsessive-compulsive disorder.

Adolescent↗
PubMed2003

Obsessions and compulsions in anorexia nervosa subtypes.

OBJECTIVE: Obsession and compulsions in anorexia nervosa (AN) patients are often confused with the preoccupations and rituals that are characteristic of obsessive-compulsive disorder (OCD). We examined the type and frequency of characteristic OCD obsessions and compulsions in a large sample of AN patients. METHOD: In personal interviews with 324 AN patients, we assessed lifetime histories of eating disorder symptomatology and obsessive-compulsive behaviors with valid semistructured interviews. Checklist category sums on the Yale-Brown Obsessive Compulsive Scale were compared between AN and OCD subjects using generalized estimating equations. RESULTS: Lifetime obsessions and compulsions occurred in 68% of the AN restricting type and in 79.1% of the AN binge/purge type. The AN subgroups did not differ from OCD controls in frequency of obsessions in the symmetry and somatic categories or in the compulsion categories of ordering and hoarding. In all other categories, the AN subgroups had a significantly lower frequency compared with the OCD controls. DISCUSSION: Some common phenotype characteristics shared by most AN and OCD patients suggest these disorders may share common brain behavioral pathways. However, the lack of complete overlap indicates they most likely have different loci of pathology within those pathways.

Adult↗
PubMed2003

Neural correlates of anxiety associated with obsessive-compulsive symptom dimensions in normal volunteers.

BACKGROUND: The neural correlates of anxiety associated with obsessive-compulsive symptomlike provocation in normal volunteers are unknown. METHODS: Ten healthy volunteers participated in four functional magnetic resonance experiments. Subjects were scanned while viewing alternating blocks of emotional (normally aversive, washing-relevant, checking-relevant, or hoarding-relevant pictures) and neutral pictures, and imagining scenarios related to the content of each picture type. Nonparametric brain mapping analyses were used. RESULTS: In response to the provocative pictures in all experiments, increases in subjective anxiety and activation in bilateral ventral prefrontal, limbic, dorsal prefrontal, and visual regions were demonstrated. Anxiety related to different symptom dimensions was associated with different patterns of activation: provocation of washing-relevant anxiety predominantly activated dorsal and ventral prefrontal regions; checking-relevant anxiety predominantly activated dorsal prefrontal regions; and hoarding-relevant anxiety predominantly activated ventral prefrontal regions and the left amygdala. CONCLUSIONS: Our findings support a dimensional model of obsessive-compulsive disorder (OCD) whereby 1) the brain systems implicated in the mediation of anxiety in response to symptom-related material in normal subjects are similar to those identified in OCD patients during symptom provocation, and 2) anxiety associated with different symptom dimensions is associated with differential patterns of activation of these neural systems. Further investigation of the neural basis of OCD symptom dimensions is required.

Adult↗
PubMed2006

Principal components analysis of obsessive-compulsive disorder symptoms in children and adolescents.

BACKGROUND: Obsessive-compulsive disorder (OCD) has a broadly diverse clinical expression that may reflect etiologic heterogeneity. Several adult studies have identified consistent symptom dimensions of OCD. The purpose of this study was to conduct an exploratory principal components analysis of obsessive-compulsive (OC) symptoms in children and adolescents with OCD to identify improved phenotypes for future studies. METHODS: This study examined lifetime occurrence of OC symptoms included in the 13 symptom categories of the Yale-Brown Obsessive Compulsive Scale (Y-BOCS) and the Children's Yale-Brown Obsessive-Compulsive Scale (CY-BOCS). Principal components analysis with promax rotation was performed on 231 children and adolescents with OCD and compared with results of similar adult studies. RESULTS: A four-factor solution emerged explaining 59.8% of symptom variance characterized by 1) symmetry/ordering/repeating/checking; 2) contamination/cleaning/aggressive/somatic; 3) hoarding; and 4) sexual/religious symptoms. All factors included core symptoms that have been consistently observed in adult studies of OCD. CONCLUSIONS: In children and adolescents, OCD is a multidimensional disorder. Symptom dimensions are predominantly congruent with those described in similar studies of adults with OCD, suggesting fairly consistent covariation of OCD symptoms through the developmental course. Future work is required to understand changes in specific symptom dimensions observed across the life span.

Adolescent↗
PubMed1993

The influence of growth hormone (rhGH) therapy on tooth formation in idiopathic short statured children.

The purpose of this preliminary study was to evaluate tooth formation in children with idiopathic short stature, before and during treatment with recombinant growth hormone (rhGH). Twenty-nine short-statured children ages 6 to 13 years were assigned into two treatment groups, an "experimental" group (n = 18), which received rhGH, and a "control" group (n = 11), which was observed for 1 year before commencing rhGH treatment. Clinical and radiographic records were obtained at the initial, year 1, and year 2 visits. Tooth formation and stature were assessed by calculating Z-scores, appropriate for the age and gender of each child. Delta-Z scores, which measure the change in Z-score over time, were also calculated between annual visits. Height was measured and recorded every 3 months, and Z-score statural norms for age and gender were derived from the 1977 National Center for Health Services national probability sampling. Tooth formation standards were derived from Moorrees et al. A matched control sample for tooth development was derived from untreated children. Tooth formation was initially delayed although the degree of reduction in stature exceeded the initial degree of delay in tooth formation. During this 2-year study, rhGH therapy had a significant influence on acceleration or gain in stature, but did not have a significant influence on tooth formation.

Adolescent↗
PubMed1984

Hoarding in anorexia nervosa.

A case study of hoarding in a woman with anorexia nervosa is reported. Obsessional and borderline personality characteristics, exacerbated by starvation, may be determinants of hoarding in anorexia nervosa.

Adult↗
PubMed2004

Challenges in treating obsessive-compulsive disorder: introduction.

This issue of In Session is devoted to challenges in the treatment of obsessive-compulsive disorder (OCD). The remarkable diversity of symptoms and the comorbidity in OCD pose substantial challenges to practitioners seeking to treat this disorder. After briefly reviewing the clinical features of OCD and its typical treatment, we provide brief descriptions of the six articles this issue comprises. Articles focus on the treatment of OCD with comorbid depression, compulsive hoarding, incompleteness, and repugnant obsessions, and on treatment of children who have OCD. The final article describes pharmacotherapy for OCD. All articles feature case vignettes and provide practitioners with clinical recommendations.

Adult↗
PubMed2003

Psychiatric disorders in Prader-Willi syndrome: epidemiology and management.

Although people with intellectual disabilities are at increased risk for psychiatric disorders, the type and rate of these problems differ between those with different causes for their retardation. In this paper, we review behavioural and psychiatric problems in persons with Prader-Willi syndrome, a disorder caused by a paternally derived deletion at chromosome 15(q11-q13) in about 70% of affected patients, and by maternal uniparental disomy in the majority of the remaining patients. In addition to the syndrome's characteristic hyperphagia and food seeking, individuals with Prader-Willi syndrome also have increased risks of nonfood, compulsive behaviours. These include skin picking, which is highly prevalent, as well as more variable rates of hoarding, redoing and concerns with symmetry, exactness, cleanliness, ordering and arranging. Relative to others with mental retardation, persons with Prader-Willi syndrome are at a marked increased risk for developing full-blown, obsessive-compulsive disorder. In addition, many people with Prader-Willi syndrome show increased rates of tantrums, oppositionality and aggression. Recent findings suggest that they also have an increased risk of psychotic disorder or affective illness with a psychotic component, especially young adult patients and those with the maternal uniparental disomy as opposed to paternal deletion. Dietary approaches include a reduced-calorie diet and increased physical activity, as well as close supervision around food and keeping food locked away. To date, neither CNS stimulants nor anorectic agents have been effective in treating hyperphagia, in part because hyperphagia in Prader-Willi syndrome is attributed to decreased satiation as opposed to increased hunger. Treatment for compulsivity and maladaptive behaviours include: behavioural programming; a structured, predictable routine; extra help with transitions; family support; and pharmacotherapy. Although formal drug studies have yet to be conducted, SSRIs have been effective in reducing skin picking, compulsivity and aggressive episodes in some individuals with Prader-Willi syndrome. Atypical antipsychotics have also proven helpful in persons with psychotic features or extreme aggression and impulsivity. Largely on the basis of case studies, the risks and benefits of these and other drugs in Prader-Willi syndrome are reviewed. Drug trials that move beyond case studies and that assess the relative efficacy of behavioural treatments alone or in combination with pharmacotherapy are sorely needed.

Antipsychotic Agents↗
PubMed2006

Symptom dimensions in obsessive-compulsive disorder: prediction of cognitive-behavior therapy outcome.

OBJECTIVE: A significant number of patients with obsessive-compulsive disorder (OCD) fail to benefit sufficiently from treatments. This study aimed to evaluate whether certain OCD symptom dimensions were associated with cognitive-behavioral therapy (CBT) outcome. METHOD: Symptoms of 104 CBT-treated in-patients with OCD were assessed with the clinician-rated Yale-Brown Obsessive-Compulsive Scale symptom checklist. Logistic regression analyses examined outcome predictors. RESULTS: The most frequent OCD symptoms were aggressive and contamination obsessions, and compulsive checking and cleaning. Patients with hoarding symptoms at baseline (n = 19) were significantly less likely to become treatment responders as compared to patients without these symptoms. Patients with sexual and religious obsessions tended to respond less frequently, although this failed to reach statistical significance (P = 0.07). Regression analyses revealed that higher scores on the hoarding dimension were predictive of non-response, even after controlling for possible confounding variables. CONCLUSION: Our results strongly indicate that in-patients with obsessive-compulsive hoarding respond poorly to CBT.

Adult↗
PubMed2005

Development and validation of the Schedule of Compulsions, Obsessions, and Pathological Impulses (SCOPI).

This article describes a factor analytically derived, self-report instrument-the Schedule of Compulsions, Obsessions, and Pathological Impulses (SCOPI)-using data from college students, adults, psychiatric outpatients, and patients with obsessive-compulsive disorder (OCD). The five SCOPI scales all are internally consistent (with coefficient alphas of .80 and higher) and are strongly stable across a 2-month interval (with retest correlations ranging from .79 to .82). They also show good convergent and adequate discriminant validity (a) when correlated with other OCD measures and (b) in analyses of self-ratings versus spouse ratings. Additional analyses indicate that three scales-Obsessive Checking, Obsessive Cleanliness, and Compulsive Rituals-assess core symptoms of OCD. The two remaining scales-Hoarding and Pathological Impulses-appear to tap different (but related) constructs.

Adult↗