ABSTRACT
OBJECTIVE: To examine the cognitive functioning of children with multiple sclerosis (MS). METHODS: Six children with a diagnosis of clinically definite MS were evaluated using a neuropsychological test battery. RESULTS: The majority of the children showed deficits in at least two of the administered subtests, with IQ scores within the deficient classification. CONCLUSIONS: Verbal and non-verbal skills were equally impaired, and patients who were older at the moment of the onset of the disease had a better cognitive performance. Cognitive deficits should be regarded as a common occurrence in the course of MS in children.
Subject(s)
Cognition Disorders/diagnosis , Cognition Disorders/etiology , Cognition , Multiple Sclerosis/complications , Age of Onset , Child , Child, Preschool , Female , Humans , Intelligence Tests , Male , Neuropsychological Tests , Verbal LearningABSTRACT
AIMS: To conduct an updated review of the mechanisms of action, pharmacokinetics, clinical effectiveness and safety of atomoxetine in the treatment of the symptoms of ADHD. DEVELOPMENT: Atomoxetine is the first of the group of non-stimulant drugs to be approved by the US Food and Drug Administration to treat this disorder in children, adolescents and adults. Atomoxetine has a direct effect on noradrenalin and dopamine concentrations by exerting a strong and highly selective inhibiting action on the pre-synaptic noradrenalin transporter, with a minimum affinity for other transporters and receptors. After adjustment of the dosage for body weight, the pharmacokinetic parameters are similar across all age and gender groups. Maximal plasma concentration is reached one to two hours after oral administration. Data concerning the effectiveness and safety from the clinical trials and studies reported in the literature are discussed. CONCLUSIONS: Atomoxetine is an effective and well-tolerated drug when used for the pharmacological treatment of ADHD symptoms. Despite being a drug that has only recently been developed, evidence from the large number of comparative studies that have been carried out endorse its widespread use in the treatment of this syndrome.
Subject(s)
Attention Deficit Disorder with Hyperactivity/drug therapy , Propylamines , Atomoxetine Hydrochloride , Central Nervous System Stimulants/therapeutic use , Clinical Trials as Topic , Dopamine/metabolism , Humans , Methylphenidate/therapeutic use , Norepinephrine/metabolism , Norepinephrine Plasma Membrane Transport Proteins/metabolism , Propylamines/adverse effects , Propylamines/pharmacokinetics , Propylamines/therapeutic useABSTRACT
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.
Subject(s)
Neuronal Ceroid-Lipofuscinoses , Child, Preschool , Female , Humans , Magnetic Resonance Imaging , Male , Neuronal Ceroid-Lipofuscinoses/diagnosis , Neuronal Ceroid-Lipofuscinoses/genetics , Neuronal Ceroid-Lipofuscinoses/physiopathology , VenezuelaABSTRACT
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.
Subject(s)
Attention Deficit Disorder with Hyperactivity/epidemiology , Adolescent , Adult , Attention Deficit Disorder with Hyperactivity/classification , Attention Deficit Disorder with Hyperactivity/psychology , Child , Child, Preschool , Comorbidity , Educational Measurement , Female , Humans , Male , Obstetric Labor Complications/epidemiology , Pregnancy , Pregnancy Complications/epidemiology , Prenatal Exposure Delayed Effects , Prevalence , Severity of Illness Index , Venezuela/epidemiologyABSTRACT
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.
Subject(s)
Attention Deficit Disorder with Hyperactivity , Attention Deficit Disorder with Hyperactivity/classification , Attention Deficit Disorder with Hyperactivity/epidemiology , Attention Deficit Disorder with Hyperactivity/physiopathology , Diagnosis, Differential , Humans , Reproducibility of Results , Socioeconomic Factors , Venezuela/epidemiologyABSTRACT
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.
Subject(s)
Attention Deficit Disorder with Hyperactivity/epidemiology , Attention Deficit Disorder with Hyperactivity/diagnosis , Child , Cross-Sectional Studies , Education, Special , Female , Humans , Male , Population , Social Class , Venezuela/epidemiologyABSTRACT
OBJECTIVE: This study gives information about a parent training program and a clinical trial with a stimulant drug (methylphenidate) to reduce the symptoms of attention deficit hiperactivity disorder (ADHD) in a group of children in Venezuela. PATIENTS AND METHODS: 24 children, aged between 6 and 10 years and diagnosed as having ADHD, identified in ADHD screening days, were randomly assigned to two groups of treatment: parent training and a stimulant drug (methylphenidate). RESULTS: Both groups showed an improvement in their symptoms, to different degrees, after treatment. The differences were statistically significant, as evaluated by their parents, regarding the symptoms of inattention, hyperactivity and impulsivity. The teachers observed a significant improvement for the medication group with respect to the symptoms of hyperactivity and impulsivity, but no change in the inattention, whilst in the parent training group there was only significant difference in the ADHD index. There was no difference in the effectiveness of the two types of treatment. CONCLUSIONS: When the two programs of treatment were compared it was observed that both parent training and psychostimulant medication were effective in reducing the symptoms of ADHD. Although there was no difference in the effectiveness of the two programs, there was a tendency for medication to be more effective
Subject(s)
Attention Deficit Disorder with Hyperactivity/drug therapy , Central Nervous System Stimulants/therapeutic use , Methylphenidate/therapeutic use , Parents/education , Child , Female , Humans , Male , Pilot ProjectsABSTRACT
INTRODUCTION: Disseminated acute encephalomyelitis (DAE) is an autoimmune inflammatory condition, usually monophasic with gradual resolution, polysymptomatic, usually in relation to a virus, bacteria or immunizations but may also occur in the absence of any obvious infection. OBJECTIVE: To describe DAE, its aetiology, clinical features, diagnosis and treatment. Also to analyze these aspects with regard to a series of 13 children with DAE. RESULTS: The clinical condition started with fever in 61% of the patients. The commonest neurological findings were sensory deterioration, convulsions and motor disorders (paresias). The cerebrospinal fluid was abnormal in four patients. MR showed hyperintense lesions of multifocal distribution, predominantly in the subcortical white matter. All patients were treated with intravenous methylprednisolone at a dose of 20 30 mg/kg. Clinical follow up showed complete recovery in 11/13 patients and mild sequelae in two cases. CONCLUSIONS: Diagnosis of DAE is based on suggestive clinical data and MR findings. MR is very sensitive in detecting demyelinating lesions and is the method of choice for confirmation of the diagnosis. DAE is characterized by a satisfactory clinical course with simultaneous resolution of the demyelinating lesions.
Subject(s)
Encephalomyelitis, Acute Disseminated/diagnosis , Anti-Inflammatory Agents/therapeutic use , Ataxia/diagnosis , Ataxia/etiology , Brain/pathology , Child , Child, Preschool , Coma/diagnosis , Coma/etiology , Diagnosis, Differential , Electroencephalography , Encephalomyelitis, Acute Disseminated/complications , Encephalomyelitis, Acute Disseminated/drug therapy , Female , Humans , Infant , Injections, Intravenous , Magnetic Resonance Imaging , Male , Methylprednisolone/therapeutic useABSTRACT
Widespread cerebral atrophy and basal ganglia involvement are highly suggestive imaging features of the variants of late infantile type neuronal ceroid-lipofuscinosis. In the presence of clinical findings indicative of neuronal ceroid-lipofuscinosis, neuroimaging procedures are highly recommended to differentiate the variants from classic late infantile neuronal ceroid-lipofuscinosis. The clinical features and follow-up magnetic resonance imaging studies in a patient with the Costa Rican variant of late infantile neuronal ceroid-lipofuscinosis is presented. These procedures were of the utmost importance to observe the progression of the neurologic ailment and the extent of the cerebral and cerebellar abnormalities.
Subject(s)
Brain/pathology , Magnetic Resonance Imaging , Neuronal Ceroid-Lipofuscinoses/diagnosis , Atrophy , Child, Preschool , Chromosomes, Human, Pair 15 , Diagnosis, Differential , Disease Progression , Female , Genetic Linkage , Gliosis , Humans , Neuronal Ceroid-Lipofuscinoses/classification , Neuronal Ceroid-Lipofuscinoses/genetics , Neuronal Ceroid-Lipofuscinoses/pathologyABSTRACT
OBJECTIVE: We discuss the historical, epidemiological, clinical, complementary tests and neuropathological details of ceroid-lipofuscinoses in children. DEVELOPMENT: Initially, we review the basic concepts and historical details of the disorders, and the frequency and distribution of the different clinical forms. Subsequently, we review the subtypes and variants most commonly found in children, together with the elements necessary for diagnosis. Finally we analyze the neuropathological studies and their clinical correlation. CONCLUSIONS: The clinical diagnosis of ceroid-lipofuscinoses should be based on a clinical history showing disorders of vision, convulsions and regression of psychomotor functions. Neuroimaging findings, neurophysiological changes and ultrastructural studies confirm the diagnosis.
Subject(s)
Brain/pathology , Neuronal Ceroid-Lipofuscinoses/diagnosis , Brain/ultrastructure , Child , Child, Preschool , Diagnosis, Differential , Humans , Infant , Macular Degeneration/diagnosis , Macular Degeneration/etiology , Magnetic Resonance Imaging , Neuronal Ceroid-Lipofuscinoses/complications , Psychomotor Disorders/diagnosis , Psychomotor Disorders/etiology , Seizures/diagnosis , Seizures/etiologyABSTRACT
OBJECTIVE: To analyze the surgical neurology cases in which both the clinical and neuroimaging features suggested supratentorial meningioma, and resulted in different entities from the neuropathological point of view. PATIENTS AND METHODS: The clinical histories of patients harboring intracranial, supratentorial mass lesions diagnosed as meningioma operated in different hospitals of Maracaibo, Venezuela, during the period 1993-1997 were reviewed. RESULTS: Our analysis revealed 15 cases with different neuropathological diagnosis. They were distributed as follows: three cases of hemangiopericytoma, two cases each of anaplastic ependymoma, metastatic carcinoma, and solitary intracranial plasmacytoma, and one case each of plasma cell granuloma, pleomorphic xanthoastrocytoma, chondroma, actinomycetoma, meningeal fibroma and chronic inflammation not otherwise specified. CONCLUSION: A heterogeneous group of intracranial, supratentorial expansive mass lesions can masquerade as meningiomas both from the clinical and neuroimaging points of view.
Subject(s)
Brain/pathology , Meningioma/diagnosis , Supratentorial Neoplasms/diagnosis , Adolescent , Adult , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective StudiesABSTRACT
A total of 154 individuals belonging to three populations located at different altitude levels in northwest Argentina (San Salvador de Jujuy, 1,200 m; Tilcara, 2,500 m; Abra Pampa, 3,500 m) were studied for the GM, KM, HP, GC, PI and TF genetic systems. Individuals were selected on the basis of ethnocultural affiliation. Gene frequency values were found to be comparable to those reported for other South American populations. The populations studied showed a close genetic identity and an absence of interpopulation heterogeneity. Distribution of the GM phenotypes and haplotypes corresponds to historical data on human settlements in Jujuy Province. The presence of some alleles and the anthropological significance of the allele distribution are discussed, as are the effects of the admixture with Africans and Spaniards. The genetic pattern appears to be the result of a varying admixture due to the genetic isolation in populations located at various altitude levels.
Subject(s)
Altitude , Gene Frequency , Genetic Markers/physiology , Immunoglobulin Gm Allotypes/genetics , Indians, South American/genetics , Adolescent , Adult , Argentina , Female , Genetics, Population , Haplotypes , Humans , Immunoglobulin Allotypes/genetics , Male , Population Surveillance , Rural Population , Sampling StudiesABSTRACT
Cortical dysplasias are a spectrum of malformations of the cerebral cortex that arise during development and are associated with epilepsy and mental retardation. New neuroimaging techniques have helped an in vivo" diagnosis of these conditions and the definition of specific clinical syndromes. This article reviews the etiology of these disorders, and briefly discusses the clinical and neuroimaging findings, and the prognosis of the epilepsy secondary to these malformations.
Subject(s)
Cerebral Cortex/abnormalities , Chromosome Aberrations/diagnosis , Epilepsy/etiology , Intellectual Disability/etiology , Adolescent , Adult , Child , Chromosome Disorders , Electroencephalography , Epilepsy/diagnosis , Female , Humans , Infant , Infant, Newborn , Intellectual Disability/diagnosis , Magnetic Resonance Imaging , Male , Prognosis , SyndromeABSTRACT
Hallervorden-Spatz disease is a rare, autosomal-recessive hereditary condition characterized by early onset of progressive movement alterations such as dystonia, rigidity, and choreoathetosis, which is usually associated with pyramidal signs and mental deterioration. The authors report two cases for which diagnosis of Hallervorden-Spatz disease was based on clinical manifestations that appeared during the first year of life, illness progression, and late-stage magnetic resonance imaging findings. The possibility that these two cases, along with other previously described rare instances with similar clinical features, be considered as a variant of subtype of the early-onset type of Hallervorden-Spatz disease is suggested. The need to differentiate these cases from cases of static encephalopathy with mental retardation and motor impairment is also stressed.
Subject(s)
Magnetic Resonance Imaging , Pantothenate Kinase-Associated Neurodegeneration/diagnosis , Child , Chromosome Aberrations/genetics , Chromosome Disorders , Diagnosis, Differential , Dominance, Cerebral/physiology , Female , Genes, Recessive/genetics , Globus Pallidus/pathology , Humans , Neurologic Examination , Pantothenate Kinase-Associated Neurodegeneration/geneticsABSTRACT
El articulo reseña los aspectos mas relevantes a tener en cuenta,relacionados con la seguridad personal,la salud publica y el medio ambiente en cualquier recinto en el que se manipule cloro.Debido al elevado riesgo que supone su manejo en condiciones no seguras,es de suma importancia conocer las precauciones que deben ser adoptadas,para evitar situaciones peligrosas que pueden afectar tanto a las personas directamente involucradas en su manejo,como a las poblaciones cercanas a las industrias en las cuales se utiliza,ya sea como materia prima o como producto final.Por ultimo y desde esta perspectiva se analiza el accidente ocurrido por una fuga de cloro en Terragona,España
Subject(s)
Chlorine , Environmental Hazards , Industrial Safety , Public Health , Occupational HealthABSTRACT
El articulo reseña los aspectos mas relevantes a tener en cuenta,relacionados con la seguridad personal,la salud publica y el medio ambiente en cualquier recinto en el que se manipule cloro.Debido al elevado riesgo que supone su manejo en condiciones no seguras,es de suma importancia conocer las precauciones que deben ser adoptadas,para evitar situaciones peligrosas que pueden afectar tanto a las personas directamente involucradas en su manejo,como a las poblaciones cercanas a las industrias en las cuales se utiliza,ya sea como materia prima o como producto final.Por ultimo y desde esta perspectiva se analiza el accidente ocurrido por una fuga de cloro en Terragona,España
Subject(s)
Chlorine , Environmental Hazards , Public Health , Occupational Health , Industrial SafetyABSTRACT
The genetic structure of two African-Ecuadorian communities, Rio Cayapas and Viche (Esmeraldas province, northwest Ecuador), was studied on the basis of ACP1, ADA, AK1, CA2, ESD, GLO1, G6PD, PGD, and PGM1 subtypes and thermostability, PGM2, HBbeta, F13A, F13B, ORM1, AHSG, C6, C7, and APOC2 gene frequency, and migration data on 255 individuals. The fixation index of Wright (F(ST)), correspondence, and genetic distance analysis were applied to compare the genetic relationships between these communities and other American populations of African ancestry. F(ST) values from the migration data and surname origins suggest that Rio Cayapas is genetically more isolated and shows less mobility and admixture than does Viche. The genetic admixture estimates indicate a large contribution of African genes to the gene pool of both communities (74.3% to 58.4%), whereas the proportion of the Amerindian component differs significantly (14.5% in Rio Cayapas to 27.6% in Viche).
Subject(s)
Black People/genetics , Gene Frequency , Gene Pool , Africa/ethnology , Demography , Ecuador , Emigration and Immigration , Enzymes/genetics , Ethnicity/genetics , Female , Humans , Male , Phenotype , Phylogeny , Proteins/geneticsABSTRACT
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.
Subject(s)
Encephalomyelitis, Venezuelan Equine/complications , Encephalomyelitis, Venezuelan Equine/epidemiology , Mental Disorders/etiology , Adolescent , Adult , Consciousness Disorders/etiology , Disease Outbreaks , Female , Fever/etiology , Headache/etiology , Humans , Male , Middle Aged , Retrospective Studies , Venezuela/epidemiologyABSTRACT
OBJECTIVES: To review the literature on the risk factors for bacterial meningitis (BM) and evaluate these factors in children diagnosed as having BM in the Paediatric Department of the University Hospital of Maracaibo between 1996 and 1998. PATIENTS AND METHODS: We made a retrospective study of children diagnosed as having BM in the University of Maracaibo. We evaluated different factors related to BM. RESULTS: From 1 January 1996 to 31 December 1998 a total of 152 children were diagnosed as having bacterial meningitis; 69.7% were boys and 30.3% were girls. The commonest causal germ was Haemophilus influenzae. Disorders of sensation (42.7%) and signs of meningism (32.8%) were the most frequent neurological alterations. The main laboratory findings were leucocytosis (51.3%), thrombocytosis (49.3%), pleocytosis (70.3%), cerebrospinal fluid protein (49.3%) and low cerebrospinal fluid glucose (72.8%). Twelve patients (7.5%) died. Of this group, 10 were under one year of age and had septic shock. CONCLUSIONS: BM represents a major group of hospital admissions in everyday paediatric practice. Although the mortality has decreased, an increased risk of sequelas must be borne in mind. Clinico-neurological examination on admission and simultaneous analysis of laboratory investigations allows identification of prognostic indicators of morbidity and mortality.
Subject(s)
Meningitis, Bacterial/diagnosis , Anti-Bacterial Agents , Child, Preschool , Drug Therapy, Combination , Female , Humans , Leukocytosis/cerebrospinal fluid , Male , Meningitis, Bacterial/drug therapy , Meningitis, Bacterial/mortality , Prognosis , Retrospective Studies , Risk Factors , Sensation Disorders/diagnosis , Thrombocytosis/cerebrospinal fluidABSTRACT
A 30 months-old boy developed bilateral nistagmus, tremor, gait disturbance, hypotonia and disartria. The diagnose of Leigh encephalopathy was suggested on the basis of clinical, neuroimaging and laboratory findings. Computed tomography and magnetic resonance imaging (MRI) at an early stage revealed bilateral and symmetric lesions in the putamen, appearing as hyperintense signal on T2-weighted images. Twelve months later a relatively large hypertense area in the posterior brainstem was observed. At this stage, the patient exhibited marked deterioration, dystonic manifestations, rigidity and respiratory disturbances. He died 6 months later for respiratory arrest during bronconeumonic infection. We believe MRI is a valuable means to allow assessment of the evolution of the disease.