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Attention deficit, hyperactivity and conduct disorders: A developmental and differential study
gm), and for whom an extensive data base, including risk factors and medical data, were available. Data collected in the last tollow-up assessment included tests of IQ (WISC), psycholinguistic abilities (ITPA), school performance (TALE), and a parents' rating scale (LPP). Medical risk factors are significantly intercorrelated with cognitive-behavioral development and the academic functioning of these infants with VLBW in their middle childhood. The results of regression analysis suggest that some perinatal and neonatal complications, such as maturity at birth, intraventricular hemorrhage, and respiratory distress syndrome, are good predictors of specific developmental deficits of VLBW infants in a variety of ways. These findings suggest that longterm follow-ups of VLBW infants with severe perinatal and neonatal difficulties are needed to prevent possible developmental delays. 57. MOYA-MOYA DISEASE AND MACROTHROMBOCYTOPENIA F. Mutas, M. Baquero, J.A. Moreno, P. Barbero, F.J. Domfnguez, F. Menor, and N. Pujol, Valencia and Barcelona, Spain A girl was evaluated initially at age 2 years, 10 months due to mental retardation. She had congenital familial macrothrombocytopenia. Her sister died at age 2 months of thrombocytopenia and cardiorespiratory failure. Late motor development was registered. Brunet-Lezine scale development was 45% globally. Examination revealed uncoordinated bilateral movements and excessively brisk deep tendon reflexes. On evolution, she suffered brief episodes of alternating hemiplegia and motor focal seizures. At age 5 years, she died of an uncontrolled febrile illness, with multiple pulmonary atelectasia, hypertension, and seizures. Bone marrow was normal. BERA disclosed neurosensorial hypoacusia. EEG demonstrated poorly differentiated, slow basal rhythm. CT was normal. MRI demonstrated bilateral multifocal ischemic white matter areas and some lesions with hemorrhagic components. Digital substraction angiography demonstrated carotid narrowing and occlusion, located above the origin of the posterior communicating artery, with prominent collateral vessels from deep posterior territories, and indirect signs of jugular thrombosis. To our knowledge, no similar report has been described. Both entities could be related. It should be determined which anomaly is primary: if disturbances in thrombocyte aggregation provoked the arteriopathy, or vascular anomalies are the cause of thrombocytopenia. 58. ATTENTION DEFICIT, HYPERACTIVITY AND CONDUCT DISORDERS: A DEVELOPMENTAL AND DIFFERENTIAL STUDY A. Miranda, F. Mulas, C. Ciscar, and Y.B. Rosello, Valencia, Spain The purpose of this study is to clarify two distinct but related issues in child neuropsychology: to delineate efficient measures for the differential diagnosis of children with inattention/hyperactivity, aggression, and attention deficit disorders, and to describe the significant effects of age and sex on a wide range of symptoms observed in these problems. The sample includes 89 children (60 boys, 29 girls), ages 6-10 years. Twenty-two have received a medical diagnosis of attention deficit-hyperactivity disorder, 22 a psychologic diagnosis of attention deficit disor-
358 PEDIATRIC NEUROLOGY Vol. 8 No. 5
der, and 23 a diagnosis of conduct disorders. Twtmty two comparison subjects, without problems have also participated in this research. All children were evaluated by using the leachers" and the parents' rating scales. The significant effecl ~q age and sex have been discussed, and implications for determi~fing treatment efficiency of these clinical disorders have been addressed. 59. CONGENITAL S T R U C T U R A L DEFECTS OF CNS DURING INFANCY P-G.M. Aleu, G.E. Burches, P.A. Gomez, V.C. Garcia, S.J.L. Fayos, S.V. Martinez, and A.V. Alvarez, Valencia, Spain A retrospective study of clinical and radiologic findings in 25 patients with brain malformations are reported. This series was compiled from children referred to our service over 9 years. Results: Sixty percent were females. Forty-four percent of mothers had had a previous abortion and/or a dead newborn. Twenty percent of the patients were delivered preterm. Mean maternal age was 28 years. Pathologic pregnancy was proved in 44% of patients (i.e., drugs, diabetes, bleeding). Extraneural malformations were recorded in 60% of patients and chromosomal abnormalities in 2 patients. Diagnosis was made prenatally in 5 patients and before the first month of life in 14 patients. The main neonatal neurologic symptoms included depression of unknown origin with hypotonia, hyporeflexia, and feeding difficulties (75%) and seizures (20%). Cranial ultrasonography was abnormal in 15 patients (performed in 16 patients). CT provided the diagnosis in 16 patients (performed in 19 patients). MRI was performed in 11 patients. Recorded malformations included Arnold-Chiari malformation, anencephaly, encephaloccle, myelomeningocele, holoprosencephaly, agenesis of the corpus callosum, cerebellar anomalies, Dandy-Walker syndrome, heterotopias, microgyria, and hydranencephaly. Twenty-eight percent of patients had multiple brain malformations. Follow-up: Nine patients died during the first year of life and 5 were lost to follow-up. Ninety-one percent of the remaining patients were mentally retarded. Eighty-two percent developed spastic or dystonic cerebral palsy. EEG abnormalities were registered in 91% of patients and 63% suffered secondary epilepsy. We want to point out the severe prognosis of CNS malformations: high death rate or cerebral palsy with mental retardation and seizures. MRI provides an accurate anatomic diagnosis. 60. NEUROFIBROMATOSIS IN CHILDREN: A CLINICAL APPROACH P-G.M. Aleu, G.E. Burches, V.C. Garcia. and A.V. Alvarez, Valencia, Spain We report the preliminary results of a prospective study of neurofibromatosis in children. Inclusion criteria: Presence of two or more of the following features: 5 or more cafr-au-lait spots, 2 or more neurofibromas, axillary freckles, 2 or more Lisch spots, brain hamartomas or optochiasmatic gliomas, and family history. Assessment measures: physical examination with anthropometric measures and vital signs; blood and urine chemistry; cardiologic, ophthalmologic, and hearing study; EEG and psychometric study; and radiologic evaluation (i:e., conventional radiography, CT, and MRI). Results: Twenty cases were compiled from patients referred to our outpatient department throughout 6 years; 40% of them were males. Mean age at diagnosis was 5 years, 4 months (range: 2 months to 13 years) with
358 PEDIATRIC NEUROLOGY Vol. 8 No. 5
der, and 23 a diagnosis of conduct disorders. Twtmty two comparison subjects, without problems have also participated in this research. All children were evaluated by using the leachers" and the parents' rating scales. The significant effecl ~q age and sex have been discussed, and implications for determi~fing treatment efficiency of these clinical disorders have been addressed. 59. CONGENITAL S T R U C T U R A L DEFECTS OF CNS DURING INFANCY P-G.M. Aleu, G.E. Burches, P.A. Gomez, V.C. Garcia, S.J.L. Fayos, S.V. Martinez, and A.V. Alvarez, Valencia, Spain A retrospective study of clinical and radiologic findings in 25 patients with brain malformations are reported. This series was compiled from children referred to our service over 9 years. Results: Sixty percent were females. Forty-four percent of mothers had had a previous abortion and/or a dead newborn. Twenty percent of the patients were delivered preterm. Mean maternal age was 28 years. Pathologic pregnancy was proved in 44% of patients (i.e., drugs, diabetes, bleeding). Extraneural malformations were recorded in 60% of patients and chromosomal abnormalities in 2 patients. Diagnosis was made prenatally in 5 patients and before the first month of life in 14 patients. The main neonatal neurologic symptoms included depression of unknown origin with hypotonia, hyporeflexia, and feeding difficulties (75%) and seizures (20%). Cranial ultrasonography was abnormal in 15 patients (performed in 16 patients). CT provided the diagnosis in 16 patients (performed in 19 patients). MRI was performed in 11 patients. Recorded malformations included Arnold-Chiari malformation, anencephaly, encephaloccle, myelomeningocele, holoprosencephaly, agenesis of the corpus callosum, cerebellar anomalies, Dandy-Walker syndrome, heterotopias, microgyria, and hydranencephaly. Twenty-eight percent of patients had multiple brain malformations. Follow-up: Nine patients died during the first year of life and 5 were lost to follow-up. Ninety-one percent of the remaining patients were mentally retarded. Eighty-two percent developed spastic or dystonic cerebral palsy. EEG abnormalities were registered in 91% of patients and 63% suffered secondary epilepsy. We want to point out the severe prognosis of CNS malformations: high death rate or cerebral palsy with mental retardation and seizures. MRI provides an accurate anatomic diagnosis. 60. NEUROFIBROMATOSIS IN CHILDREN: A CLINICAL APPROACH P-G.M. Aleu, G.E. Burches, V.C. Garcia. and A.V. Alvarez, Valencia, Spain We report the preliminary results of a prospective study of neurofibromatosis in children. Inclusion criteria: Presence of two or more of the following features: 5 or more cafr-au-lait spots, 2 or more neurofibromas, axillary freckles, 2 or more Lisch spots, brain hamartomas or optochiasmatic gliomas, and family history. Assessment measures: physical examination with anthropometric measures and vital signs; blood and urine chemistry; cardiologic, ophthalmologic, and hearing study; EEG and psychometric study; and radiologic evaluation (i:e., conventional radiography, CT, and MRI). Results: Twenty cases were compiled from patients referred to our outpatient department throughout 6 years; 40% of them were males. Mean age at diagnosis was 5 years, 4 months (range: 2 months to 13 years) with