Biomedical subjects
Publications and source records attributed to J M Prats-Viñas.
The diagnostic possibilities applicable in the focal epilepsies of the childhood are reviewed, as well as the methods diagnoses to arrive to their determination. Stress is made in that it is necessary to keep in more mind the atypical of the seizures that the present anomalies or not in the electroencephalogram.
INTRODUCTION: Only the organic and cryptogenic forms of frontal lobe epilepsy have been admitted by the ILAE, but according to several reports in the literature, it probably exists also an idiopathic form, at present not well recognized. OBJECTIVE: To study the differences between the organic and the cryptogenic forms of frontal epilepsy in our patients, that might indicate the presence of idiopathic cases within the presumed cryptogenic group. MATERIAL AND METHODS: All patients diagnosed of frontal lobe epilepsy, EEG registered, at the neuropediatric clinic of our hospital during 1993-1996, were selected: 30 patients under 14 years of age that were classified as organic (10 cases) or cryptogenic (20 cases) according to CT or MRI findings. The Mann-Whitney test and the Fisher exact test were performed for statistical analysis. Five patients with peculiar neurocognitive symptoms, epilepsy-related, are described in detail. RESULTS: Significative differences between the organic and cryptogenic forms of frontal lobe epilepsy were not found except for the tendency of cryptogenic EEG foci to spread beyond the frontal lobe, to generalize and to be associated with foci of other localizations. Epileptic status, either convulsive or not convulsive, were a frequent complication in both groups. CONCLUSIONS: The identification of an idiopathic form of frontal lobe epilepsy is difficult by its low prevalence as compared to the cryptogenic and organic forms, by the tendency of frontal seizures to become epileptic status, which predicts an intractable epilepsy, and by the clinical characteristics essential to frontal discharges: heterogeneity, frequent impairment of consciousness and specially the neurocognitive semiology, including dementia, that occurs as a critic or paracritic phenomenon, and that sometimes may develop or be accentuated by antiepileptic medication.
INTRODUCTION: The obstetric and neonatal technological advances have changed the frequency and syndromic classification of perinatal brain lesions. OBJECTIVE: To study all prevalent patients during 1996, with perinatal or late intrauterine brain lesions, in the outpatient neuropediatric clinic at our hospital. METHODS: Selection of patients according to neuroimaging findings, and/or neurological sequela with documented perinatal antecedent. EXCLUSION CRITERIA: a normal MRI, brain lesions of doubtful origin, or uncertain sequela with normal CT or echography studies. Descriptive study, and also analytical, using logistic regression to study the relationship between antecedents and sequela. RESULTS: A total of 111 patients with: brain lesions due to hypoxic-ischemic encephalopathy (22 cases), lesions due to prematurity (29), late intrauterine infection or neonatal meningitis as the only cause of fixed neurological impairment (12), unexpected vascular brain lesions during the neonatal period (11), late intrauterine brain lesions (37). The neurological sequela observed were: cerebral palsy (68%), epilepsy (47%), mental retardation (45%), learning disorders in (34%) of those of school age and free from mental retardation, strabism (26%), microcephaly (19%), visual impairment (14%), hyperkinesis (10%). The neuroimaging findings were: focal lesions (45%), hydrocephaly (28%), intraventricular haemorrhage (22%), white matter lesions (24%), venous thrombosis (2%). The multivariable analysis showed, as the most noticeable finding, the relationship between the antecedent of mechanical ventilation and late development of hyperkinesis (OR: 10), in this sample of patients with severe sequela. Three patients should be noted: a case of late-onset dystonia secondary to a late intrauterine brain lesion, a child with exclusively cerebellar symptoms due to a destructive cerebellar lesion of prenatal onset, and a patient with an acquired perinatal biopercular lesion and pseudobulbar palsy as the only long-term sequelae.
INTRODUCTION: Intraparenchymatous haemorrhages are rare in infancy, but their effects may be very harmful. The aetiology, clinical characteristics and prognosis regarding life and function are different from those in adults. OBJECTIVE: We decided to analyze the characteristics of this pathology in children and compare them with those in adults. MATERIAL AND METHODS: We reviewed the clinical histories of the patients under 13 years of age presenting with a spontaneous intraparenchymatous haemorrhage during a 10 year period. RESULTS: The study group was made up of 10 patients, 5 boys and 5 girls, aged between 23 days and 11 years. In 7 patients the aetiology was a burst arteriovenous malformation; in one a serious liver disorder due to alpha-l-antitrypsin deficiency; bleeding from an undiagnosed tumour caused another case and no aetiology was found in a further case. Two patients died, 6 patients had residual neurological defects and in the other two there were no sequelae. CONCLUSIONS: Intraparenchymatous haematomas in children have a high mortality (20%) and many sequelae. The figures are comparable with those for elderly persons aged over 70 and much greater than in the case of young adults. The commonest aetiology is an arteriovenous malformation, followed at a considerable distance by coagulopathies and tumours.
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INTRODUCTION: Arteriovascular malformations are anomalies of the embryonic development of cerebral vessels. They usually appear at between 10 and 30 years of age, being infrequent in infants. OBJECTIVE: Describe our experience of angiomas in small children. MATERIAL AND METHOD: The histories of all children under 10 years of age with the diagnosis of arteriovenous malformation admitted to our department between November 1984 and May 1995 were reviewed. RESULTS: Nine patients, aged between 3 months and 10 years; seven cases presented as intracranial haemorrhage, one with epileptic crises and another with general clumsiness and a hemicerebellar syndrome. The diagnosis was confirmed by angiography and/or study by the pathologist in all cases. Six patients were treated surgically and the other three by embolization. Of the latter, two also received radiosurgical treatment. The complications seen were; two children died, one during the acute phase and the other four years later as direct consequence of the haemorrhage. Six patients had residual neurological defects. One child had no complications. CONCLUSIONS: Intra-parenchymatous haemorrhage is the commonest manifestation of angiomas in children under 10 years of age. The high risk and serious consequences of bleeding make it necessary for treatment to be as radical as possible.
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A 2-year-old girl affected with the late infantile form of metachromatic leukodystrophy had a persistent and moderate metabolic acidosis. Renal functional studies demonstrated the presence of decreased tubular reabsorption of sodium, bicarbonate and some amino acids. Other tubular functions, including distal urinary acidification and concentrating mechanism were normal. Glomerular filtration rate was moderately decreased. Metachromatic inclusions were demonstrated along the nephron by histochemistry and electron microscopy. Tubular dysfunction in metachromatic leukodystrophy could have been overlooked until now given the severity of the neurological picture.
OBJECTIVE AND DEVELOPMENT: We review the different cognitive and behaviour disorders in whose genesis the cerebellum has been thought to play a part. These disorders include infantile autism, Williams' syndrome, attention-deficit hyperactivity disorder and congenital cerebellar hypoplasia. We also consider the consequences of an acquired cerebellar lesion in the light of the following neuropsychological sequelas. It would seem that the cerebellum is involved in the functioning of the executive system due to its relationship with the prefrontal operative system and in the function of the working memory, and may share some of the characteristics of the acquired frontal syndrome.
OBJECTIVE: To report on the heterogeneity with regard to the clinical course of the acute disseminated encephalomyelitis (ADEM). CASE REPORT: A 5 year old boy suffered of acute disseminated encephalomyelitis of unknown origin. This child suffered two episodes of different neurologic symptoms separated by several weeks. Based on the clinical manifestations and typical appearance of magnetic resonance imaging findings and the absence of oligoclonal bands in CSF immunoglobulins, multiple sclerosis (MS) was ruled out. CONCLUSION: We postulate that the recurrent symptoms in our patient could be explained as a multiphasic disseminated encephalomyelitis (MDEM). Favourable outcome after simultaneous treatment with methylprednisolone and intravenous immunoglobulin is emphasized in this report.
OBJECTIVE: To review the concept of benign myoclonic epilepsy of infancy in the literature as compared with our series of patients. PATIENTS AND METHODS: We review the literature and describe seven personal patients, three males and four females, diagnosed with benign myoclonic epilepsy of infancy according to Dravet's criteria. RESULTS: Six of the seven patients were followed along 6 26 years, three of whom showed an unfavourable evolution of their intellectual and behavioral development. Three of the seven patients, not necessarily the same just mentioned, presented with generalized seizures later during their follow up: tonic clonic in one, Petit Mal status in another, and absences with marked eyelid myoclonia in the third. Four of the seven patients showed well defined eyelid myoclonias simultaneously occurring with the arms and head myoclonic seizures at the beginning of the illness, without inferring a prognostic value of their intellectual development or their response to antiepileptic treatment.
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OBJECTIVE: We review the complicated forms of different types of idiopathic benign partial epilepsy pointing out their relation to continuous point and wave tracings in slow sleep (POCS), the epileptic opercular syndrome and the Landau Kleffner syndrome. We insist on the role of some anti epileptic drugs in triggering the problem and withdrawal of these drugs as the most important aspect of the treatment of these complications replacing them by CLB in all cases. DEVELOPMENT: We describe various clinical cases which illustrate the problem
CASE REPORT: A young patient with epilepsy and limbic encephalitis unrelated to neoplasm. RESULTS: The patient in a subacute fashion showed frequent partial seizures with neuropsychological deficits mainly in recent memory capability, with an no simultaneous affectation of both temporal lobe and adjacent structures. After 6 years there was no evidence of underlying malignancy.
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INTRODUCTION: Tourette syndrome shows a fluctuating evolution, often masked by its comorbidity. OBJECTIVE: To study the clinical factors predicting the initial remission of tics in children with Tourette syndrome. Patients and methods. All patients attended during the last 5 years at a Child Neurology hospital based out patient clinic, with the diagnosis of Tourette syndrome according to DSM IV criteria, were selected. OUTCOME MEASURE: total remission of tics during at least 3 months, evaluated during the patient s second visit to our clinic. Demographic, clinic and therapeutic variables were studied. Statistical analysis was based on the Student t test or non parametric tests, as necessary. RESULTS: 53 patients, 44 males and 9 females. Age at starting tics: 6.9 2.2 years, time of evolution: 2 years (range: 1 9.4). Comorbidity in 51%: 34% with attention deficit hyperactivity disorder (ADHD), 17% with obsessive compulsive disorder (OCD) and school underachievement: 26%. Familial antecedents of tics, OCD, or ADHD: 49%. Tics remission at second visit to our clinic: 41.5%. Patients without remission were those with an earlier onset of tics (p=0.085), longer time of evolution (p< 0.05), or school underachievement (p= 0.024). Remission was not statistically associated with treatment. OCD and ADHD were associated with school failure but were not related to the tics evolution. CONCLUSION: The short term (at second visit), temporal (minimum 3 months) total remission of Tourette syndrome was not related to treatment but to previous duration of the syndrome and to factors (other than OCD and ADHD) that lead to school failure.