A86
Abstracts
268 Attention deficit hyperactivity disorder: Study of motor control, attention, memory and experience with nootropics N N ZAVADENKO, S N YU Neurology Department, Paediatric Faculty, Medical University, Moscow, Russia
Russian State
Section Xl: Metabolism 039 Hormonal changes in girls with juvenile neuronal ceroid lipofuscinosis L E ABERG A TIITINEN, T AUTTI, P SANTAVU~RI Helsinki,
Attention deficit hyperactivity disorder (ADHD) is for certain years the most common disorder of childhood. According to the data of our neurological and psychological study, performed in several Moscow elementary schools, in the group of 537 schoolchildren (300 boys, 237 girls) aged from 7 to 10 years, ADHD was diagnosed in 7.6% of all children. It was more prevalent in boys, than girls (11.2% DS 2.5% respectively). The developmental delay of some higher cerebral functions, including motor control, attention and memory, is characteristic of children with ADHD. We performed a comparative study of gross and fine motorics, attention, reaction time measurements, acoustic-verbal and visual memory in 180 children with ADHD (151 boys and 29 girls), who met DSM-IV diagnostic criteria (American Psychiatric Association, 1994), and normal children aged from 6 to 10 years of age. The following methods proved of diagnostic value for distinguishing children with ADHD from normal ones: scored examination of subtle signs (gross and fine motorics) by Den&la, the Bourdon-Vos test for continuous attention, Wechsler Intelligence Test for Children Coding subtest for selective attention, the Stroop test for divided attention, choice reaction tasks using Vienna reaction timer device, Luria-90 battery for acousticverbal and visual memory. These functions were investigated also in children with ADHD before and after 1 month treatment with nootropic drugs (open controlled study): peptidergic nootropics cerebrolysin (42 patients, 0.1 ml/kg daily intramuscularly) and semax (20 patients, 12 pg/kg daily intranasally), GABA derivatives piracetam (27 patients, 48mg/kg daily orally) and phenibut (20 patients, 12.5 mg/kg daily orally), instenon (22 patients, 1.5-2 tablets daily). The comparative group of 18 patients with ADHD received thioridazine (lmg/ kg daily orally) and the control group of 31 children with ADHD was given low doses of multivitamins for a month. The comparisons of the patient’s individual characteristics before and after the treatment showed definite improvement of behaviour, motor control, attention and memory measures in 50% or more of children with ADHD after nootropics therapy compared with overall improvement in only 22% of children after thioridazine treatment and 10% in the control group. No significant side-effects were observed during nootropics therapy. Taking into account ADHD underlying mechanisms, the use of nootropic agents, aimed to improve the deficient cognitive and motor functions by their multimodal action, can be especially important in childhood, when intensive processes of the central nervous system development and maturation occur.
Fhand
Acne, obesity, and hirsutism are common findings in girls with juvenile neuronal ceroid lipofuscinosis (JNCL). Recently, valproate, a commonly used anti-epileptic drug in JNCL patients, was found to cause hyperandrogenism and polycystic ovaries. We therefore wanted to find out if hormonal changes are associated with the valproate therapy, or perhaps, with the JNCL itself. A total of 16 girls with JNCL entered the study. Their mean age was 16.8 years (range 11-27). A questionnaire was sent to parents of all patients. Extensive laboratory tests were done on all, and in addition, 1.5T MRI of the lower abdomen was performed on 11 patients. The mean age of the menarche was 11 years (range 915). Three patients had had an ovariectomy, and three patients had monthly injections of medroxy-progesteroneacetate to avoid menstrual bleeding. As regards the rest of the patients, four had irregular menstruation, and seven had regular menstruation with the mean ovarian cycle of 26 days (range 23-28); 11/16 patients had acne, and lo/16 had hirsutism. The body mass index varied from 16 to 37 (mean 25). In the laboratory tests, hyperandrogenism was observed in 10 patients. High prolactin values were found in four patients. Using MRI, polycystic ovaries were found in two patients. Conclusions: (1) The menarche in girls with JNCL seems to be earlier than in the normal female population. (2) The mean ovarian cycle is shorter than in the normal female population. (3) High prolactin values were found in four patients. All of these patients had therapy with risperidone, previously associated with high prolactin values. (4) Hyperandrogenism was found in 60% of the patients. Only half of these patients had current therapy with valproate. Thus, it is possible, that the neuronal ceroid lipofuscinosis disorder, by an unknown mechanism, causes hyperandrogenism. (5) Polycystic ovaries were found in two patients. This is within the normal range, as prevalence of polycystic ovaries in the normal healthy female population in different studies is around 20%.
256 IgA Deficiency and hectic fever in a patient with spinocerebellar Friedreich’s ataxia I ASTADICKO, H BENKHADRA, P LEROY, M MASSIN, J-P MISSON Paediatric Neurology, University Department of Paediatrics, CHR Citadel/e, University of Li.Gge, Belgium
Spinocerebellar Friedreich’s ataxia includes characteristically cerebellar ataxia, peripheral neuropathy and pes cavus, cardiomyopathy and tendency to diabetes mellitus. This disorder is linked to a GAA expansion on the