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INTRODUCTION: Characteristic symptoms of autistic disorder (AD) can be the result of cognitive impairment which can be produced by specific neurological irregularities. Up until now a specific cognitive deficit in autism has not been found, although the majority of people with autism show intellectual impairment, verbal scores lower than manipulative measures and executive dysfunctions. AIMS: A neuropsychological evaluation of children with AD was planned. These children had intellectual abilities in the normal range. They were compared with two other groups, one with pervasive developmental disorder not otherwise specified (PDD-NS), and the other from the general population. SUBJECTS AND METHODS: A battery of neuropsychological tests was carried out on five boys AD, five boys PDD-NS, and five boys of the general population. All of them were between 9 and 15 years old and their intellectual abilities were within the normal range. RESULTS: The children AD obtained verbal scores lower than their visual-perception scores. They also showed good dynamic coordination of movement. Scores in episodic memory tasks where executive strategies are needed were low. CONCLUSION: The characteristics described in the paper do not demonstrate a specific profile of the AD, but they can be useful in diagnoses and in planning treatment.

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Introducción. Los síntomas característicos del trastorno autista (TA) pueden ser el resultado de déficit cognitivos que, a su vez, derivan de alteraciones neurológicas específicas. Hasta el momento no se conoce ningún déficit cognitivo patognómico del autismo, aunque la mayoría de las veces presentan retraso mental, índices verbales inferiores a los manipulativos y disfunciones ejecutivas. Objetivo. Se planteó una valoración neuropsicológica de niños con TA con una inteligencia en el intervalo considerado típico, y se comparó con niños de la población general y con niños con trastorno generalizado del desarrollo no especificados (TGD-NE). Pacientes y métodos. Se aplicó una batería neuropsicológica a cinco niños con TA, a cinco niños con TGD-NE y a cinco niños de la población general. Todos ellos tenían entre 9 y 15 años, y su nivel intelectual se situaba en el intervalo considerado normal. Resultados. Los niños con TA mostraron un rendimiento verbal inferior al visuoperceptivo, buena coordinación dinámica del movimiento y un bajo rendimiento en tareas de memoria episódica que requieren estrategias ejecutivas. Conclusiones. Las características que se dan descrito no suponen un perfil específico del TA, sin ambargo, pueden resultar útiles para complementar el diagnóstico y fundamentar la rehabilitación

Introduction. Characteristic symptoms of autistic disorder (AD) can be the result of cognitive impairment which can be produced by specific neurological irregularities. Up until now a specific cognitive deficit in autism has not been found, although the majority of people with autism show intellectual impairment, verbal scores lower than manipulative measures and executive dysfunctions. Aims. A neuropsychological evaluation of children with AD was planned. These children had intellectual abilities in the normal range. They were compared with two other groups, one with pervasive developmental disorder not otherwise specified (PDD-NS), and the other from the general population. Subjects and methods. A battery of neuropsychological tests was carried out on five boys AD, five boys PDD-NS, and five boys of the general population. All of them were between 9 and 15 years old and their intellectual abilities were within the normal range. Results. The children AD obtained verbal scores lower than their visual-perception scores. They also showed good dynamic coordination of movement. Scores in episodic memory tasks where executive strategies are needed were low. Conclusion. The characteristics described in the paper do not demostrate a specific profile of the AD, but they can be useful in diagnoses and in planning treatment

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Introducción: La disfunción serotoninérgica ha sido implicada en la fisiopatología del autismo. Los ISRS han mostrado eficacia en la mejoría de algunos síntomas en niños con autismo. Objetivos: Evaluar, en un estudio piloto, la eficacia y seguridad de la fluoxetina en niños con autismo. Método: Se realizó un estudio abierto de un año con fluoxetina en 12 niños (entre 3 y 13 años) con Trastorno Generalizado del Desarrollo. Pa ra la medida de la severidad y la mejoría de los síntomas se utilizó el ICG de severidad. La mejoría individual de los síntomas fue evaluada tanto por los padres como por los terapeutas. La fluoxetina fue dosificada en todos los pacientes desde 1,2 ml/día hasta alcanzar una dosis final de 3,6 o 5 ml/día en cuatro semanas. La tolerancia fue evaluada mediante la recogida de los efectos adversos espontáneos. Resultados: 11 niños completaron el estudio. Los niños experimentaron una mejoría moderada o marcada (puntuación final de ICG de mejoría de 3 a 5). Mejora ron la comunicación y la capacidad de atención. Disminuyeron también los rituales, las estereotipias y los comportamientos repetitivos. Los efectos adversos más frecuentes fueron la impulsividad e inquietud, también aparecieron trastornos del sueño y pérdida de apetito. Seis niños necesitaron a lo largo del estudio medicación concomitante con carbamacepina y uno levopromacina. Conclusiones: Estos resultados señalan que la fluoxetina a dosis de 5 ml/día (20 mg/d) pueden ayudar a mejora r algunos síntomas del Trastorno Generalizado del Desarrollo y permitir aumentar la efectividad de otros abordajes terapéuticos que se realizan con estos pacientes. Más estudios son necesarios para confirmar estos resultados. (AU)

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INTRODUCTION: Serotonergic dysfunction have been implicated in the pathophysiology of the autism. SRIs have shown efficacy in improving some symptoms in children with Autism. OBJECTIVES: To evaluate, in a pilot study, the efficacy and safety of fluoxetine in very young children with autism. METHOD: 1 year open-label trial was made with fluoxetine on 12 patients (3 to 13 years old) with Pervasive Developmental Disorder. CGI-severity was used to assess the severity and the improvement of symptoms. Individual symptoms improvement was assessed by both parents and therapists. Fluoxetine dose was titrated in all patients from 1.2 ml/day to reach a final doses of 3,6 or 5 ml/day in four weeks. Tolerance was assessed by collecting spontaneous adverse events. RESULTS: 11 children completed the study. Children experienced a moderate or marked improvement (final score in CGI 3 to 5). Communication and attention skills were improved. There were also a decrease of rituals, stereotypies and repetitive behaviours. Most common adverse events were increase of impulsivity and restlessness, other events were sleep disturbances and lost of appetite. 6 children needed concomitant medication with carbamacepine and 1 with levopromacine along the study. CONCLUSIONS: These results support that Fluoxetine at doses of 5 ml/day (20 mg/day) could help to improve some symptoms in Pervasive Developmental Disordersand allow to increase the effectiveness of therapies in these patients. More studies are needed to confirm these results.

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A multicentric study of 15 cases of Rett syndrome selected with the diagnostic criteria according HAG-BERG et al: female sex, normal pre and perinatal period, normal psychomotor development through the first months of life, early dementia between 1-3 years of age with autistic behaviour, loss of acquired purposeful hand skill, "washing hands" stereotypies, normal head circumference at birth with later deceleration of head growth and truncal ataxia with gait apraxia. Waking EEG showed unspecific abnormalities while sleep recording demonstrated extremely frequent multifocal spike and sharp waves mainly over the rolandic region and generalized, and also pseudo-periodic suppression of background activity. In 3 cases the EMG showed a peripheral axonal neuropathy. Only in one case we found hyperammonemia. Karyotypic studies performed in 12 cases demonstrated non specific fragile sites. CT scan was normal in almost all cases. The QD was extremely low.

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