1/4. Hereditary progressive dystonia with diurnal fluctuation (Segawa's syndrome)--an unusual case.A young girl with hereditary progressive dystonia with diurnal fluctuation or Segawa's syndrome, completely handicapped and confined to a wheelchair between the age of 5 and 9, revealed an unusually slow response to levodopa. The ability to walk returned only after 12 to 14 months of treatment. Apart from peculiarities of behaviour due to longstanding immobility and ensuing parental overprotection, there were no psychological or mental abnormalities. Other organic diseases were ruled out. A series of regular follow-ups over the course of 7 years was performed. While residual and irregular day-to-day variation of functional capacities almost disappeared after conversion to a preparation with a decarboxylase inhibitor, some mild neurological abnormalities and a slight choreic hyperkinesia persisted with doses providing functional results. The patient today leads an almost normal teenage life and has performed well in school.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/4. Phenylketonuria and anorexia nervosa.A girl with phenylketonuria and mild mental retardation developed a behaviour disorder and anorexia nervosa at the age of 14. The anorexic symptoms disappeared at the age of 20, but the behaviour disorder worsened, leading to compulsory detention in a psychiatric hospital. serum phenylalanine levels may have influenced the course of the disorder.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
3/4. anorexia nervosa at normal body weight!--The abnormal normal weight control syndrome.Disgust with "fatness" and a consequent preoccupation with body weight, coupled with an inability to reduce it to or sustain it at the desired low level, characterizes the abnormal normal weight control syndrome. Individuals remain sexually active in a biological sense and often also socially. Indeed their sexual behaviour may be as impulse ridden as is their eating behaviour, which often comprises phases of massive bingeing coupled with vomiting and/or purgation. The syndrome is unlike frank anorexia nervosa in that the latter involves a regression to a position of phobic avoidance of normal body weight and consequent low body weight control with inhibition of both biological and social sexual activity. In abnormal normal weight control there is a strong and sometimes desperate hedonistic and extrovert element that will often not be denied so long as body weight does not get too low. Individuals nevertheless feel desperately "out of control" and insecure beneath their bravura. The syndrome is much more common in females than in males. There is a clinical overlap with anorexia nervosa and obesity in many cases as the disorder evolves. depression, stealing, drug dependence (including alcohol) and acute self-poisoning and self-mutilation are common complications. Clinic cases probably only represent the tip of the iceberg of the much more widespread morbidity within the general population. Like anorexia nervosa and for the same reasons the disorder is probably more common than it used to be.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
4/4. Long-term octreotide treatment reduced hyperinsulinemia, excess body weight and skin lesions in severe obesity with acanthosis nigricans.A boy affected by severe obesity (kg 117, body mass index 37 kg/m2) and acanthosis nigricans, was treated with octreotide for 150 days (50 micrograms x three daily subcutaneous administrations). Before treatment the patient showed an exaggerated insulin (IRI) and c-peptide (CPR) response to a standard meal with a lowering in after-meal CPR/IRI molar ratio. During octreotide treatment both IRI and CPR response was reduced but CPR/IRI molar ratio rised after meal indicating an increase in hepatic insulin removal. body weight and acanthosis nigricans were sharply reduced during treatment and the reduction was still maintained six months after the cessation of therapy. Furthermore, IRI and CPR response, as well as the behaviour of CPR/IRI molar ratio, remained within normal range. In conclusion long-term octreotide treatment has been able to correct hyperinsulinemia and to reduce body weight and acanthosis nigricans.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |