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Objective: To describe the epidemiological and clinical characteristics of Venezuelan pediatric patients with multiple sclerosis (MS)., Methods: Database records from the National Program for MS were searched for patients with an established diagnosis of MS whose first symptoms appeared before age 18., Results: The national database held records of 1.710 patients; 3.8% had onset of the first symptoms before age 18. 46.7% were boys, yielding an F:M ratio of 1.13:1. Many children had a disease onset characterized by motor impairment (30.7%), brainstem/cerebellum and spinal cord affectation (27.6%), headache (26%). Less frequent symptoms were sensory symptoms (8%) and optic neuritis (7%)., Discussion: Pediatric MS patients in Venezuela represent a significant proportion of all MS cases. The clinical pattern is characterized by motor symptoms at onset, and predominantly monosymptomatic presentation with a relapsing-remitting pattern. This is the first systematic attempt to estimate the prevalence of pediatric MS in Venezuela.

Aicardi syndrome is characterized by agenesis of the corpus callosum, infantile spasms and chorioretinal lacunae. The evolution of this disorder is variable, with a severe outcome over the first five years of age. The purpose of this report was to demonstrate the spectrum of the clinical phenotype and the course of this disorder in four Venezuelan patients. All patients met the major criteria, had severe psychomotor impairment and early onset seizures. There were microphtalmia in two of the patients. Three patients (75%) showed coloboma, interhemispheric cyst and periventricular heterotopias. The first patient, with longer follow-up, is currently aged 22. They all exhibited a typical asymmetric pattern on the electroencephalogram. These cases illustrate the variable clinical expression and severity of the Aicardi syndrome. A diagnosis of this disorder should be considered in girls with developmental delay, particularly, when there are accompanying recurrent seizures occurring in early childhood.

A total of 1,535 4-12 year-old children were screened with the Conners' rating scales, followed by diagnostic confirmation by the diagnostic interview schedule for children-IV-parent version. The prevalence of ADHD was estimated to be 10.03%, and only 3.9% of children had received medication for the treatment of ADHD symptoms. Prevalence rates and demographic profile of Venezuelan children with ADHD are very similar to those found in samples from other countries. Authorities need to develop public health policies to correctly identify and treat affected subjects. Furthermore, clinicians must actively search for children with ADHD in order to provide the best-available treatment.

The study aims to determine the prevalence of autism spectrum disorders (ASDs) for children receiving services in Maracaibo County, Venezuela. Children aged 3-9 with diagnosis of any ASD were recruited. We ascertained area, referral process, and definitions of ASD for each patient. A total of 430 children were identified, and 76.5 percent were boys. Prevalences were 1.7 per 1000 for all ASD, 1.1 per 1000 for autism, and 0.6 per 1000 for PDD-NOS and Asperger syndrome combined. These prevalences are lower than current reports in the literature. Differences in case-finding methods, diagnostic criteria, and lack of awareness in the general population may have influenced the number of cases identified. An ASD prevalence of 1.7 per 1000 should alert the health and education authorities to the need to reassess the services available for children with these disorders and their families.

Results from studies comparing boys and girls diagnosed as having Attention Deficit-Hyperactivity Disorder (ADHD) have been non conclusive. In general, the results of such studies report boys as being more hyperactive and presenting more conduct problems, and girls as having more cognitive and learning problems. The aim of this study was to collect information about the characterization of the disorder depending on the gender. 169 children (123 males, 46 females), between 4 and 13 years of age with ADHD were studied. The assessment battery included Conners' rating scales-Revised for parents and teachers, short forms of the Wechsler Intelligence Scale for Children-Third Edition (WISC-III) and Wechsler Preschool and Primary Scale of Intelligence-Revised (WPPSI-R), academic achievement measures, developmental history and the Diagnostic Interview Schedule for Children-IV Version-Parents (DISC-IV). The results indicated the lack of significant differences between genders for the studied variables, ADHD boys and girls scored alike in the various behavioral and cognitive measures. The results presented describe homogeneity in the symptoms, demographic characteristics and neuropsychological functioning for children of both genders; suggesting a syndrome with the same criteria and independent of the gender.

Populations: Whole blood samples from 74 unrelated healthy individuals were collected. The donors' sample included Venezuelan mestizos from various regions of the country, but mostly from the resident population of Caracas City. A Venezuelan mestizo is the offspring of a mating between a native Venezuelan and a person born in Europe, mainly in Spain.

Introduction: Neuronal ceroid lipofuscinosis (NCL), represents a group of inherited neurodegenerative disorders. Based on the age of the patient at onset, clinical course and ultrastructural morphology it has been identified three clinical types for the pediatric group: 1) Infantile NCL (INCL); 2) Late infantile NCL (LINCL); and 3) Juvenile NCL (JNCL). Other variants or atypical forms represent around 20% of the NCL in different populations. Genetic advances have made possible a better characterization, diagnostic and classification of these disorders., Case Reports: We present the clinical, neurophysiological, neuroradiological, and morphological data from 6 patients with NCL, who were assessed at the pediatric neurology department of the Hospital Universitario de Maracaibo during a ten years period (1993 2003). All 6 cases corresponded with the late infantile form. Age of onset ranged form 2 to 5 years. For most of the patients initial symptoms included seizures, psychomotor delay, accompanied by macular degeneration and optic atrophy. The EEG was characterized by high voltage spikes elicited by low frequency photic stimulation, in 5 cases. Neuroimaging findings were characteristic of the late infantile form of the NCL. In three patients a decreased intensity of signal was seen in the thalami and putamen on T2-weighted images. The ultrastructural examination of the samples obtained through a biopsy showed curvilinear bodies in all patients., Conclusion: There is not epidemiological data of the NCL in Venezuela; it is presumed the presence of clinical forms and variants in the pediatric group. This first study could contribute to the knowledge and a better research of this group of disorders in our population.

Aims: This study reports the findings of a research project aimed at determining the rate of prevalence of attention deficit hyperactivity disorder (ADHD) in school aged children., Patients and Methods: Different behavioural and cognitive measures were administered to a sample of children between 3 and 13 years of age obtained by means of a multistage sampling procedure that was stratified by socioeconomic and educational level. The sample was made up of 394 children; 33.25% (n = 131) were females and the remaining 66.75% (n = 263) males. The mean age of the sample was 7.64 years old (SD: 2.33). The assessment battery included the Conners Rating Scales adapted for parents and teachers, the abbreviated version of the Wechsler Intelligence Scale for Children (WISC III) and for Preschool children Revised (WPPSI R), several academic achievement scales, development history and DISC IV., Results: The estimated prevalence of ADHD was 10.15%, and a figure of 2.03% was obtained for the hyperactive type, 0.51% for the inattentive subtype, and 7.61% for the combined subtype. Prevalence was higher for males, the ratio being 3:1. Subjects performance in the cognitive and academic measures was within the average interval., Conclusions: The prevalence of ADHD in our population oscillates around the average figures reported in other countries. This study confirms the prevalence of ADHD in the population of Venezuela, with a distribution of subtypes and in terms of gender similar to those reported in the literature.

Introduction: The attention deficit hyperactivity disorder (ADHD) has been defined as a disorder of self control characterized clinically by a short attention span, excessive motor activity and poor impulse control., Objective: The main objective is to describe how methodological difficulties related to the design of epidemiological studies make an important contribution to the group of heterogeneous data which might lead to doubts regarding the validity of ADHD in children. In recent decades the psychiatric definition of this disorder has varied. This has affected the number and combination of clinical signs necessary for diagnosis of ADHD. The variation in the prevalence rates of the disorder reported in different studies is largely due to these changes. Other factors involved are related to the assessment methods, the type of sample (clinical or community), the source of information (parents, teachers and/or children) and sociocultural characteristics., Conclusions: ADHD is a valid disorder. It cannot be considered to be a myth since there is considerable evidence of its existence and clinical characteristics in children in different countries.

Aims: This study reports the findings of research aimed at determining the rate of prevalence of attention deficit hyperactivity disorder (ADHD) in children of school age., Subjects and Method: The epidemiological study was conducted using a community sample extracted by means of multi stage stratified sampling according to socio economic level and schooling and consisted in 1,141 children of both sexes of school age from the city of Maracaibo. The revised Conners scales were used to collect data., Results: The estimated prevalence of ADHD was 7.19% and we also obtained 0.35% for the hyperactive type, 1.14% for the disattentional subtype, and 5.70% for the combined type. Contrary to what was expected, prevalence was higher for females. It was found that 7.45% of the general sample scored higher on the academic problems scales, whereas for the sample identified as having ADHD, academic problems were 50% and the comorbidity between ADHD and academic problems was confirmed., Conclusions: The estimates for prevalence found in this study are consistent with those reported in the literature, which suggests that ADHD is a valid diagnosis for Marabino children.

Introduction: Venezuelan equine encephalitis virus has caused periodic epidemics and epizootics in the American continent since the 1920s. Such events have been profusely documented from the epidemiologic point of view, however, reports concerning the clinical features of this disease are rather scarce., Objective: To analyze the clinical characteristics evidenced by Venezuelan equine encephalitis patients from Zulia state (western Venezuela) studied during the outbreak that occurred in Colombia and Venezuela in 1995. These cases, classified as complicated, were hospitalized at the Hospital Universitario de Maracaibo, state of Zulia, Venezuela., Patients and Methods: The clinical charts of 313 Venezuelan equine encephalitis patients hospitalized during the period January 1st 1995-March 31st 1996 were reviewed. These cases accounted for 2.82% of 11,072 patients that were medically assisted during the outbreak. The following variables were analyzed: age, gender, signs and symptoms, contact history, complications and evolution., Results: Intracranial hypertension signs became eloquent in 55.9% of these patients. Neurologic complications were represented by two cases of cerebellitis, two cases of meningoencephalitis and one case of encephalomyelitis. The mortality rate was 1.7%., Conclusion: Our results corroborate the benign evolutionary profile that is typical of this entity.

Hallervorden Spatz syndrome (HSS), described in 1.922, is a rare progressive disorder recessively inherited involving the basal ganglia. It is manifested clinically by mental deterioration, rigidity, choreoathetosis and spasticity evident during the first two decades of life. Increased concentration of iron in the globus pallidus and substantia nigra has been demonstrated convincingly. the objective of this paper is to describe two venezuelan children with this syndrome. Patient 1 is a 14-year-old boy with progressive impairment of language, mental deterioration and slowing of voluntary movements started at the age of 8 years. Patient 2 is a young girl first examined at 8 years of age because of psychomotor development delay, progressive impairment of gait, rigidity of the limbs, progressive dystonia and mental deterioration. Multiple test were performed, including metabolic studies, CT scan and magnetic resonance imaging in the brain. These two cases may lead us to think that besides the Huntington Disease, the HSS is also an entity to be taken into consideration in a young patient with movement disorder, mental deterioration and progressive course.