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J Campos-Castello

Publications and source records attributed to J Campos-Castello.

7 recordsLinked to original sources
PubMed2006

[The neuropsychology of epilepsy: what factors are involved?].

INTRODUCTION: The epileptic child is three times more likely to have cognitive problems from other neurological pathologies, depending on three facts: the effect exerted by the epilepsy itself, any associated previously-existing neuropsychosocial deficits, and the side effects of the antiepileptic drug (AED). A certain degree of deterioration is universally accepted, although the factors involved in its production are not specified, but are generally accepted as being due to multiple causes. We analyse the relation between neuropsychology and epilepsy in neuropaediatrics. DEVELOPMENT: The relation between epilepsy and behaviour must be seen as the exception rather than the rule, unless there are co-existing personality disorders and/or mental deficiency. The cognitive effects of AED depend on the drug, on the dosages employed and the polypharmacy, and these effects can be both adverse and favourable. The differences observed between one drug and another are open to criticism, although monitored administration of suitable doses generally produces moderate adverse side effects, as evaluated using reliable appraisal methods (MIDDRA). Scaling time in the introduction of the drug is important, especially with some new AED. The mechanisms governing production vary, but an understanding of the mechanism of action improves cognitive functioning thanks to the control over the seizures. In infancy, idiopathic cognitive reactions are produced. The main disorders are a diminished reaction and information processing time with alterations affecting memory, attention and language. CONCLUSIONS: Epilepsy is associated to a number of different, generally moderate, cognitive problems. The age of onset, the type of syndrome, its aetiology, the response to treatment and polypharmacy are multifactorial elements conditioning the side effects. There is a need for batteries of tests capable of forecasting the future and controlling the progression of cognition during treatment. The side effects of AED affecting cognition and behaviour are generally moderate, but they may be important in the case of a particular child.

Anticonvulsants↗
PubMed1999

Aicardi-Goutières syndrome: monogenic recessive disease, genetically heterogeneous disease, or multifactorial disease?

Aicardi-Goutières syndrome (AGS) is a severe progressive familial encephalopathy, which is usually diagnosed shortly after birth. Using the principle of homozygosity mapping, genome-wide screening of five consanguineous families was performed to search for a homozygous region shared by all affected individuals. A total of 364 markers with an average spacing of 9.9 cM were genotyped, but no homozygous region common to all affected individuals could be found. Regions of homozygosity in affected sibs could only be identified within each family individually. This may reflect genetic heterogeneity, possibly related to clinical heterogeneity, since several syndromes are clinically difficult to distinguish from AGS. Involvement of a small number of genes and/or of an external factor, such as infection, may also explain the absence of a homozygous region common to all affected individuals.

Abnormalities, Multiple↗
PubMed1990

Rett's syndrome: a neurophysiological study.

Thirteen girls with Rett's syndrome were evaluated with wakefulness and sleep polygraphic records and EMG studies. The main EEG findings were paroxysmal activity during sleep, multifocal in younger and unifocal in older girls, and "pseudorhythmic flattening". In the opinion of the authors, the changes with age are related to evolution of the disease or maturational changes of CNS. The affectation of the peripheral nervous system was demonstrated in almost half the patients.

Age Factors↗
PubMed1986

Congenital muscular dystrophy and cerebral CT scan anomalies. Results of a collaborative study of the Société de Neurologie Infantile.

We present the results of a collaborative study on the association of congenital muscular dystrophy with central nervous system anomalies revealed by CT scan investigation of 10 patients. In seven children, an abnormal hypodensity of the cerebral white matter is found; in four of these patients, this radiological anomaly is either isolated, or associated with a moderate intellectual impairment; in one case, severe mental retardation and ocular changes had occurred; in the other two cases, the muscular disease was progressing slowly, in association with microcephaly, epilepsy, and moderate mental retardation. Three children were afflicted with a severe early encephalopathy and congenital muscular dystrophy, and presented signs of cortical and subcortical atrophy on CT scan. Two of these patients corresponded to different types of cerebro-ocular dysplasia-muscular dystrophy syndromes, and the third patient of Fukuyama's congenital muscular dystrophy. These observations are discussed and compared with those reported in the literature. The authors emphasize the need to investigate possible cerebral CT scan anomalies in congenital muscular dystrophies, and to look for muscular changes in some prenatal encephalopathies.

Atrophy↗
PubMed1984

Sleep in the Prader-Willi syndrome. Clinical and polygraphic findings.

Nine patients with the Prader-Willi syndrome, ranging in age from 3 to 21 years, were examined clinically as well as studied in the sleep laboratory. They had striking disturbances of sleep-wakefulness patterns. All patients except one had the symptom of excessive daytime sleepiness. The most striking finding was the presence in five patients of rapid-eye-movement (REM) sleep occurring at sleep onset (SOREM). None of the patients had the condition of sleep apnea. One patient, however, demonstrated severe hypoventilation during REM sleep; the lowest value recorded for O2 saturation was 40%, with a consistent value below 50% for as long as ten to 15 minutes. Previous findings have indicated that the Prader-Willi syndrome is of hypothalamic origin. We hypothesize that both the SOREM and O2 desaturation findings in our patients with the Prader-Willi syndrome are also a result of hypothalamic changes.

Adolescent↗
PubMedDate not supplied

[Arteriovenous malformations in children: a report of four cases].

INTRODUCTION AND AIMS: Vascular malformations are the most frequent cause of intracranial haemorrhage (IH) after the neonatal period that are not due to traumatic injury. Arteriovenous malformations (AVM) are the vascular malformations that most often give rise to symptoms in infancy and are the most common cause of IH in children over one year of age. CASE REPORTS: We reviewed the medical records of all patients under the age of 16 years diagnosed with AVM from the year 2000 to the present. Four cases aged between 7 and 15 years were found, all of whom were examined with computerised tomography and/or magnetic resonance imaging and cerebral arteriography scans. One patient started with headaches and another had learning disabilities. The other two began with IH, the most common clinical manifestations of which were a diminished level of awareness and vomiting. The final diagnosis was established by means of arteriography in all cases. Surgical treatment was carried out in three cases (using surgery, catheter embolisation and stereotaxic radiosurgery) and the patients' progress following the intervention was excellent. CONCLUSIONS: Spontaneous haemorrhage constitutes the most common presenting symptom of AVM. However, there are sometimes earlier manifestations that are a challenge to diagnosis and which should be the target of future research, since preventing IH would lead to a drastic reduction in the morbidity and mortality rate of AVM.

Adolescent↗