[Neuropsychiatric abnormalities and continuous spikes and waves during slow sleep syndrome: a case report]. / Symptômes psychiatriques et syndrome des pointes-ondes continues du sommeil: à propos d'une observation.

UNLABELLED: Continuous spikes and waves during slow wave sleep syndrome (CSWS) is a seldom form of epilepsy which may manifest by neurocognitive and/or psychiatric abnormalities, with or without clinical seizures. CASE REPORT: A 5-year-old child was presented with a language disorder and behaviour abnormalities. A standard electroencephalograph (EEG) showed left tempororolandic spikes waves without any electrical discharges. Cerebral imaging excluded an underlying expansive disease. A continuous EEG recording revealed a CSWS syndrome by showing continuous and diffuse spike waves during slow wave sleep. Thanks to antiepileptic medications and orthophonic therapy, evolution was positive. CONCLUSION: In complex cases of language and behavioural disorders, it is necessary to perform an EEG even in the absence of convulsive seizures, to diagnose a CSWS syndrome. Given the etiological and treatment difficulties concerning the CSWS, a treatment with antiepileptic seems necessary. EEG normalisation under antiepileptic medications usually leads to significant, although partial, neuropsychological improvement. Furthermore, orthophonic therapy seems useful as a complement to pharmacological treatment.

[Cardiac consequences of adolescent anorexia nervosa]. / Conséquences cardiaques de l'anorexie mentale de l'adolescence.

Cardiac complications are common in adolescent anorexia nervosa and are the cause of a third of deaths. Some workers have reported prolongation of the QT interval and cases of sudden death in these patients. The aim of this study was two-fold: to assess the cardiac complications of anorexic adolescents and to determine the outcome after renutrition in the hospital setting. This was a prospective study of 48 consecutive cases (45 girls) with an average age of 14 +/- 2 years, admitted to the paedopsychiatric unit and fulfilling the DSM-IV criteria of anorexia nervosa. The digitised ECG, Holter ECG and echocardiography were recorded before and after renutrition. Anorexia nervosa was severe with a body mass index < 14 in 2/3 of cases. Over 2/3 of patients had bradycardia with a heart rate < 50/min in half the cases but normal chronotropic function on Holter monitoring. Prolongation of the QTc interval was demonstrated (QTc > 440 ms in 11/44 cases). Echocardiographic abnormalities, in particular left ventricular dysfunction (24/46) and pericardial effusion (12/46) were reversible after renutrition. There were no clinical or biological predictive factors for the occurrence of cardiac complications on admission. The authors confirm that cardiac complications of anorexia nervosa are common, usually benign and always reversible after renutrition in hospital. Therefore, most electrical abnormalities normalise with the heart rate and echocardiographic abnormalities with improvement of conditions of load.

[Disorder of gender identity in a child with asymmetric gonadal dysgenesis with complex karyotype abnormality]. / Trouble de l'identité de genre chez une enfant porteuse d'une dysgénésie gonadique asymétrique avec anomalie caryotypique complexe.

BACKGROUND: Gender identity is influenced by genetic and hormonal factors and also by the sex of rearing. CASE REPORT: A child was born with ambiguous genitalia. Male sex assignment was made. Hypospadias and left inguinal hernia were present. The hernial sac contained an ovarian tissue. Plasma testosterone, 17-OH progesterone, cortisone levels were normal as were basal FSH and LH levels. There was a vagina but no uterus. The karyotype showed 45X/46XY mosaicism. The testis in the right scrotum was removed and vulvoplasty was performed at the age of 10 months; the patient was than raised as a female. Several courses of reconstructive genital surgery were necessary between 1 and 8 years of age. The girl suffered from trichotillomania and sleep disorders from the age of 4 years and learning difficulties and social phobia from the age of 8. When 11.5 years-old, she had clinical features of Turner's syndrome, was depressive but appeared to prefer male playmates. CONCLUSION: Two mechanisms may explain alterations in gender identity in this patient with asymmetric gonadal dysgenesis; 1) early and prolonged androgen exposure as seen in patients with congenital adrenal hyperplasia; 2) insufficient counselling with parents regarding questions of gender identity.

Preliminary observations of the significance of monitoring tricyclic antidepressant plasma levels in the pediatric patient.

Plasma concentrations of clomipramine and desmethylclomipramine were monitored together with clinical effects over a period of 1--9 months in children and young adolescents (5--19 years of age) receiving clomipramine because of depressive features or enuresis. Data indicate that in the child, clomipramine is rapidly absorbed while its disposition rate is age dependent, as indicated by the significantly lower level:dose ratio (p less than 0.01) observed in the younger groups (5--10; 10--15 years). The incidence of side effects was clearly related to clomipramine plasma concentrations, whereas no relationship could be observed for the desmethyl metabolite. In the case of enuresis, a therapeutic effect was observed at clomipramine concentrations of 20--60 ng/ml, while lower or higher levels were associated with lack of therapeutic effect or adverse effects. No relationship was found between oral daily doses and clomipramine or desmethylclomipramine plasma concentrations. The data point to the usefulness of monitoring drug plasma levels in children undergoing treatment with tricyclic antidepressive drugs.