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Biomedical subjects

M Castro-Gago

Publications and source records attributed to M Castro-Gago.

At least 19 recordsLinked to original sources
PubMed2006

[Aspects concerning behavioural development and socialisation and how they are linked to attention deficit hyperactivity disorder].

INTRODUCTION: Despite their importance, little attention has been given to the recognition of early markers of hyperactivity in the scientific literature and in everyday practice it can be difficult to separate some of the physiological behaviours displayed by older babies or preschool infants from pathological processes such as attention deficit hyperactivity disorder (ADHD), specific language impairment, autistic spectrum disorders, conduct disorders or bad habits with regard to discipline within the family context. DEVELOPMENT: Knowledge of some of the key points in behavioural development and sociability in children can help reach a correct interpretation of the manifestations observed in this age group. The development of linguistic competencies, a feeling of what is right and what is wrong, the development of an awareness of oneself and the capacity to infer that is established as progress is made in language are some of the keys to proper behavioural development and socialisation. The gene DRD4 is expressed in the prefrontal cortex and has been implicated in some subtypes of ADHD, as well as being linked to a novelty-seeking personality. Recent genetic evidence supports the notion of an association between certain polymorphisms in the DRD4 gene with resistant or disorganised bonds at early ages. CONCLUSIONS: The detection of several dysfunctional signs related to inadequate self-control that appear during behavioural development and socialisation may provide early keys to the future development of ADHD.

Attention Deficit Disorder with Hyperactivity↗
PubMed2005

[Benign seizures associated with mild gastroenteritis: apropos of two cases].

INTRODUCTION: The occurrence of benign seizures in association with viral gastroenteritis without dehydration or fever is well recognized in Asia, but it is virtually unknown in other parts of the world. This is a benign process that does not lead to a greater risk of epilepsy or developmental deterioration. CASE REPORTS: We describe two infants who were admitted to our department over a 1-year period with acute convulsions and mild gastroenteritis. The seizures were brief and did not recur after the first day. In both cases the outcome was excellent. CONCLUSIONS: This entity does not appear exclusively in Asia and its frequency may have been underestimated in Spain. This diagnosis should be borne in mind in patients with gastroenteritis and seizures to avoid intensive and/or prolonged antiepileptic treatment.

Child, Preschool↗
PubMed2005

[Aicardi-Goutières syndrome: report of two new cases].

INTRODUCTION: Aicardi-Goutières syndrome is an early onset autosomal recessive progressive encephalopathy, clinically characterized by acquired microcephaly, severe psychomotor delay and involvement of pyramidal and extrapyramidal tracts. Intracranial calcifications, especially at the level of the basal ganglia, white matter abnormalities, lymphocytosis and raised interferon (IFN)-alpha in blood and cerebrospinal fluid (CSF) form part of this syndrome. CASES REPORTS: We describe two unrelated infants (a 3-month-old boy and an 11-month-old girl) who both presented with hypotonia, microcephaly, and psychomotor delay. Mild choreic and dystonic movements, as well as progressive spasticity, were also observed in the girl. Extensive investigations revealed intracranial calcifications, mild CSF lymphocytosis in the boy, and raised IFN-a in blood and CSF in both patients. COMMENTS: Aicardi-Goutières syndrome should be kept in mind when investigating microcephalic and retarded patients with cerebral calcifications initially suggestive of TORCH infection. Appropriate genetic counseling should be provided.

Brain Diseases↗
PubMed2005

[Syncope in adolescents. Diagnostic and therapeutic approach].

Syncopal episodes are frequent in adolescence. Syncope is usually a benign, self-limiting condition but it may be a warning sign of serious disease that must be diagnosed and appropriately treated. The present article provides a review of the basic principles of the differential diagnosis of syncope in the adolescent patient and treatment recommendations.

Adolescent↗
PubMed2003

Nutritional status of mentally retarded children in north-west Spain. I. Anthropometric indicators.

AIM: To evaluate the nutritional status of mentally retarded children in the region of Galicia in north-west Spain, on the basis of anthropometric variables. METHODS: The following variables were determined in a sample of 128 mentally retarded children (81 M, 47 F): birthweight, bodyweight, height/length, head circumference, mid-arm circumference, mid-arm muscle circumference, triceps skinfold thickness, subscapular skinfold thickness, arm area, arm muscle area, arm fat area, arm lean-to-fat ratio, Shukla's nutrition index and Quetelet's body mass index (BMI). A preliminary statistical analysis indicated that most information content could be maintained considering only eight of these variables. A factor analysis of the resulting 8 x 128 (variables by subjects) data matrix was performed, identifying two factors (FA1 and FA2) that together explained 82% of total variance. Taking these factors as indicators of nutritional status, the data were analysed for possible effects of age, gender, socioeconomic and family environment, intelligence quotient (IQ), presence/absence of cerebral palsy, quality of diet, appetite and antiepileptic use. RESULTS: The analysis suggested that most subjects were in the normal nutrition range, but about 33% showed either borderline or definite malnutrition. Mean score on FA2 showed a significant negative correlation with age. Children with cerebral palsy had lower mean scores on both factors, and scores on both factors varied with IQ. Children with definite malnutrition had a significantly lower IQ than those in the normal nutrition range. Mean score on both factors varied with appetite and quality of diet. The mean FAI score of children from inland-rural areas was significantly lower than that of children from coastal or urban areas. Mean FA1 score increased with increasing age of the parents. The prevalence of obesity was 13% when obesity was defined on the basis of Shukla's nutrition index, and 19% when defined on the basis of FA1 score. CONCLUSION: Malnutrition as revealed by anthropometric variables occurs with a high prevalence among mentally retarded children. The prevalence increases with age, increasing IQ deficit and cerebral palsy.

Adolescent↗
PubMed2003

Nutritional status of mentally retarded children in northwest spain: II. Biochemical indicators.

AIM: To evaluate the nutritional status of mentally retarded children in the region of Galicia, northwest Spain, on the basis of serum biochemistry variables. METHODS: The following serum biochemistry variables were determined in a sample of 128 mentally retarded children (81 boys and 47 girls): albumin, prealbumin, retinol-binding protein, transferrin, ferritin, ceruloplasmin, iron, calcium, phosphorus, copper, total cholesterol, HDL cholesterol, LDL cholesterol, triglycerides, alkaline phosphatase, transaminases and carnitine. A preliminary statistical analysis indicated that most information content could be maintained taking into consideration only five of these variables. A factor analysis of the resulting 5 x 128 variables-by-subjects data matrix was then performed, identifying three factors (FB1, FB2 and FB3) that together explained 74% of total variance. Taking these factors as indicators of nutritional status, the data were then analysed for possible effects of age, gender, socioeconomic and family environment, IQ, presence/absence of cerebral palsy, quality of diet, appetite and use of antiepileptics. RESULTS: The analysis suggests that most subjects were in the normal nutritional range, but that about 37% showed either borderline or definite malnutrition. The FB1 score showed a significant, positive correlation with age, while FB2 score was negatively correlated with age. The FB1 score showed a significant positive correlation with socioeconomic class, and was significantly higher among children from inland-rural areas than among children from coastal or urban areas. The FB1 score was also positively correlated with parents' mean age. Finally, the results suggest that antiepileptic treatment with phenobarbital or diphenylhydantoin led to reductions in serum levels of calcium and phosphorus. CONCLUSION: Malnutrition as revealed by biochemical variables is highly prevalent among mentally retarded children, and the prevalence is affected by socioeconomic and family environment variables.

Child↗
PubMed2001

Usefulness of the head-upright tilt test for distinguishing syncope and epilepsy in children.

PURPOSE: Episodic loss of consciousness in children, whether or not associated with hypertonia or short-duration clonic movements, presents a diagnostic challenge to the pediatrician and child neurologist. We provide some evidence of the usefulness of the head-upright tilt test for investigating the causes of transient loss of consciousness in children, and for distinguishing between syncope, convulsive syncope, and epilepsy. METHODS: We studied nine children previously diagnosed as epileptic on the basis of compatible clinical events and epileptiform findings in routine EEGs who were treated over the long term with antiepileptic drugs, but whose clinical records suggested syncope or convulsive syncope rather than epilepsy on reevaluation. All subjects underwent head-upright tilt testing. RESULTS: The tilt-test result was positive in all nine cases, with the patients reporting the same symptoms as in the previously considered epileptic attacks. CONCLUSIONS: Inadequate histories and misuse/overinterpretation of EEG results often lead to misdiagnosis of epilepsy in children. The head-upright tilt test is a useful and reliable diagnostic technique, allowing syncopal events to be induced and evaluated under controlled conditions. In a subset of patients, it may help to distinguish epilepsy from simple or convulsive syncope.

Adolescent↗
PubMed2001

Three new patients with congenital unilateral facial nerve palsy due to chromosome 22q11 deletion.

We report three unrelated patients with congenital facial nerve palsy and chromosome 22q11 deletion, a condition hitherto poorly recognized. In the first case, facial palsy was associated with aortic coarctation, ductus arteriosus, and ostium secundum atrial septal defect. In the second case, facial palsy was associated with ostium secundum atrial septal defect, obstruction of the ureteropelvic junction, double ureteropelvic-calicial system, and distal metaphyseal widening of the forearm and leg bones. In both cases, facial palsy was the presenting feature. In the third case, an ostium secundum atrial septal defect was also present, but involvement of cranial nerves III, VI, and VIII, in addition to hypoplastic structures of cerebellar and cerebral peduncles, were the predominant features. There were no inherited deletions within chromosome band 22q11 and the de novo deletions detected in each case belonged to the paternally derived chromosome 22. Association of facial nerve palsy and congenital heart disease versus cardiofacial syndrome are different only on clinical grounds, so both conditions can be genetically identical and form part of the spectrum of defects associated with chromosome 22q11 deletions. We recommend investigation for chromosome 22q11 deletions in patients with complete nerve facial palsy.

Chromosome Deletion↗
PubMed2001

Concentrations of nucleotides, nucleosides, purine bases, oxypurines, uric acid, and neuron-specific enolase in the cerebrospinal fluid of children with sepsis.

To determine the effects of sepsis on cerebral energy metabolism, the cerebrospinal fluid adenosine monophosphate, inosine monophosphate, inosine, adenosine, guanosine, hypoxanthine, xanthine, and urate concentrations were determined by high-performance liquid chromatography and the neuron-specific enolase levels by means of an enzyme immunoassay method in 32 children with sepsis, without meningitis, aged between 2 months and 13 years, and in 160 age-matched controls. The septic group had significantly higher cerebrospinal fluid concentrations of inosine, adenosine, xanthine, and urate than controls. These results suggest that sepsis could provoke some degree of neuronal hypoxia and significant alterations of cerebral energy metabolism homeostasis.

Adolescent↗
PubMed2001

Congenital hydranencephalic-hydrocephalic syndrome with proliferative vasculopathy: a possible relation with mitochondrial dysfunction.

We report the case of a fetus aborted at gestation week 20 because of hydranencephalic-hydrocephalic syndrome. The fetus was the third pregnancy of a nonconsanguineous couple whose first child exhibited congenital hydranencephalic-hydrocephalic syndrome associated with muscle histology findings consistent with mitochondrial cytopathy and deficiency of complexes III and IV of the respiratory chain and whose second pregnancy had terminated in an elective abortion on detection of progressive hydrocephalus at gestation week 19. The third pregnancy had a normal course according to obstetric and ultrasonography examinations performed at gestation weeks 5, 10, and 15, and negative results were obtained in standard serologic and polymerase chain reaction (PCR) tests for prenatal infections of the mother. However, the ultrasonography examination at gestation week 18 revealed hydrocephalus, in response to which the parents requested an abortion, which was performed at gestation week 20; the fetus was male and with no evident external malformations. Histopathologic studies of the brain and medulla oblongata revealed proliferative vasculopathy (glomeruloid vessels, intracytoplasmic inclusions, and microcalcifications) and intracytoplasmic inclusions in the voluntary muscle. Microbiologic and PCR tests of hepatic and spleen tissue were negative for prenatal infections. In view of the precedent of a sister with mitochondrial dysfunction, these findings raise the pos sibility that at least some cases of familial syndrome of congenital hydranencephalic-hydrocephalic syndrome with proliferative vasculopathy can be attributed to alterations in the mitochondrial respiratory chain.

Abortion, Induced↗
PubMed2000

Cerebrospinal fluid purine metabolite and neuron-specific enolase concentrations after febrile seizures.

If febrile seizures cause significant compromise of neuronal metabolism (whether permanent or reversible), this should be reflected in an increase in the cerebrospinal fluid concentrations of neuron-specific enolase (NSE) and/or adenosine triphosphate (ATP) breakdown products. In the present study, AMP, IMP, inosine, adenosine, guanosine, adenine, guanine, hypoxanthine, xanthine, uric acid and NSE concentrations were determined in the cerebrospinal fluid of 90 children 1 h after febrile seizure (73 simple febrile seizures (SFS); 17 complex febrile seizures (CFS)), and in a control group of 160 children. There was no statistically significant difference between the SFS group and the control group for any of the substances determined, suggesting that SFS neither significantly depletes neuronal ATP concentration, nor significantly increases NSE concentration; thus, SFS do not appear to constitute a threat to neuronal integrity. However, patients with CFS showed significantly lower IMP concentrations and significantly higher adenine concentrations than controls, and significantly higher AMP concentrations than SFS patients; these results suggest that CFS may affect energy metabolism in the brain. However, NSE concentrations were normal in the cerebrospinal fluid of both SFS and CFS patients, suggesting that neither type of seizure causes significant neuronal damage, at least early after the seizure.

Adenosine Monophosphate↗
PubMed2000

The effects on lipid and apolipoprotein serum levels of long-term carbamazepine, valproic acid and phenobarbital therapy in children with epilepsy.

The aim of the present study was to assess the effect of long-term carbamazepine (CBZ), valproic acid (VPA) and phenobarbital (PB) treatment on serum lipids and apolipoproteins in epileptic children. Serum levels of total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C), very low-density lipoprotein cholesterol (VLDL-C) and triglycerides (TGs) were measured and the LDL-C/HDL-C and TC/HDL-C ratios were calculated in 320 children and adolescents (129 receiving CBZ, 127 receiving VPA and 64 receiving PB) suffering from various types of epilepsy. Additionally, in a subgroup of 181 children (68 CBZ; 78 VPA; 35 PB) apolipoprotein A-I (apoA-I), apolipoprotein B (apoB), HDL2-C and HDL3-C were measured and apoA-I/apoB and HDL2-C/HDL3-C ratios were calculated. Results of the measurements were compared with those of 169 age-and sex-matched healthy controls. None of the variables considered was significantly correlated with time elapsed since start of treatment or with drug concentration in serum. TC and LDL-C serum levels were high in children receiving CBZ or PB and low in those treated with VPA. Serum LDL-C level exceeded 130 mg/dl in 27.9% of CBZ-group, 31.8% of the subjects receiving PB, but only in 7% of those receiving VPA and in 11.8% of control group subjects. CBZ-treated children also showed high HDL-C and HDL3-C values. In the group receiving VPA, HDL2-C, HDL2-C/HDL3-C ratio and apo B were significantly lower than in the control group. Mean apoA-I levels were low in all treated groups: by contrast, in neither group did TGs, VLDL-C levels and TC/HDL-C or LDL-C/HDL-C ratios differ significantly from the corresponding control group. Our results suggest that the effects of long-term AED therapy on lipid profile and, particularly, on apolipoprotein serum levels increase risk of atherosclerosis-related disease. Moreover, these results confirm our previously reported increased risk in CBZ and PB-treated patients.

Anticonvulsants↗
PubMed1999

Neuron-specific enolase levels in the cerebrospinal fluid of neurologically healthy children.

Levels of neuron-specific enolase (NSE) levels in the cerebrospinal fluid (CSF) of children without neurological disease were assessed. CSF samples were obtained from 37 subjects aged between 1 month and 13 years. All subjects had undergone lumbar puncture for diagnostic purposes, and were subsequently shown not to be suffering any form of neurological disease. NSE levels in CSF were determined by an enzyme immunoassay method. NSE level ranged from below the detection limit to 4.8 ng/ml (1.52+/-1.01 ng/ml). The present results may be useful as a basis for defining reference levels of NSE in CSF in post-neonatal children.

Adolescent↗
PubMed1999

Long-term treatment of children with epilepsy with valproate or carbamazepine may cause subclinical hypothyroidism.

PURPOSE: The aim of the study was to evaluate serum thyroid hormone balance in children receiving long-term therapy with carbamazepine (CBZ), valproate (VPA), and phenobarbital (PB). METHODS: We determined serum levels of triiodothyronine (T3), thyroxine (T4), free thyroxine (FT4), thyroxine-binding globulin (TBG), and thyroid-stimulating hormone (TSH) in 148 healthy children and 141 children with epilepsy who had been receiving CBZ (61 patients), VPA (51 patients), or PB (29 patients) for 12-161 months. In view of TSH values, three categories of subclinical hypothyroidism were considered: I, TSH greater than the control-group mean + 2 SD (4.37 mIU/L in our study) and <6 mIU/L; II, TSH between 6 and 12 mIU/L; and III, TSH >12 mIU/L. RESULTS: In all treated groups, mean T4 and FT4 levels were lower than in the control group, whereas the CBZ- and VPA-treated children additionally showed reduced mean T3 and TBG levels and increased mean TSH levels. In the group receiving CBZ, 8.2% had TSH values higher than the normal-range maximum, by comparison with only 3.6% of healthy children. The increase in TSH levels was particularly marked in VPA-treated children, accounting for 26% of patients with subclinical hypothyroidism. CONCLUSIONS: Our results, in contrast to previous reports, suggest that CBZ and particularly VPA may induce subclinical hypothyroidism. This suggests a need for careful monitoring of TSH levels in children receiving CBZ or VPA.

Adolescent↗
PubMed1999

Congenital hydranencephalic-hydrocephalic syndrome associated with mitochondrial dysfunction.

We report the case of a 3-year-old girl, the only child of a nonconsanguineous couple without relevant antecedents, who was born with hydranencephalic-hydrocephalic syndrome diagnosed by ultrasonography at gestation week 28, and who was treated during the neonatal period by implantation of a ventriculoperitoneal shunt. She showed severe mental retardation, and died at age 4 years following an acute respiratory infection. Due to persistently high lactic acid levels in blood, muscle and skin biopsies were taken. Analysis of muscle biopsies revealed microscopic and ultrastructural alterations typical of mitochondrial disorders, and low levels of complexes III and IV of the mitochondrial respiratory chain. The enzymes of the pyruvate dehydrogenase complex showed normal activities in cultured skin fibroblasts. These findings raise the possibility that at least some cases of congenital hydranencephalic-hydrocephalic syndrome may be due to alterations in the mitochondrial respiratory chain.

Biopsy↗