[Botulinum toxin as a treatment for infantile cerebral palsy].

Objective: To review the results and adverse effects to botulinum toxin type A (BTA), Botox, in cerebral palsy (CP) spastic and/or dystonic in an open prospective study.

Material And Methods: The first 39 cases treated were analyzed. They received 1-2 doses and were followed up to 12 months.

[Monotherapy with vigabatrin in the treatment of West's syndrome].

Introduction: Vigabatrin (VGB) was specifically synthesized to enhance inhibitory GABAergic transmission by elevating GABA levels via irreversible inhibition of GABA transaminase.

Material And Methods: This study was conducted to determine the efficacy of VGB introduced as monotherapy in 26 children fulfilled the criteria for diagnosis of West syndrome. Duration of follow-up was 24 months.

Transient movement disorders in children.

Transient movement disorders are quite common in pediatrc practice, occurring mainly in infants. They are underdiagnosed but represent about 20% of cases of movement disorders (tics excluded) seen in a neuropaediatric department. Dystonia, tremor and myoclonus are the most common.

[Topiramate, a new antiepileptic drug].

Objective: The characteristics of the new anti-epileptic drug topiramato (TPM) are described: multiple mechanism of action, favourable pharmaco-kinetics, wide therapeutic range and is relatively well tolerated, Development. Its initial usefulness in patients with partial crises resistant to other anti-epileptic drugs has been shown. TPM is equally effective in primary generalized crises, and in patients with the Lennox-Gastaut syndrome, in whom no problem of tolerance has been seen.

L-2-Hydroxyglutaric aciduria: clinical, biochemical and magnetic resonance imaging in six Portuguese pediatric patients.

We present clinical, biochemical and cranial magnetic resonance imaging data of six pediatric patients with L-2-hydroxyglutaric aciduria. All the children have the same ethic origin and lived in the northern area of Portugal. Our findings reinforce the described phenotype of this rare metabolic disease with mental deficiency, severe cerebellar dysfunction, mild extrapyramidal and pyramidal symptoms, progressive macrocephaly and seizures.

[Experience with Health Quality of Life Questionnaire for the epileptic child (CAVE)].

Introduction: The practical application of a questionnaire designed to evaluate the quality of life of an epileptic child, known as the CAVE test, is described. This is the first scale of its kind. The test consists of 8 parameters: conduct, attendance at school, learning, independence, social relationships, frequency of crises, intensity of crises and parental opinion.

[When and how should the long-term anti-epileptic treatment be stopped].

Although for some decades it has been customary to stop long-term treatment of epilepsy in patients who have been free of crises for several years, there is still no general agreement as to when, how and in which cases such treatment should be stopped. Several factors have to be taken into account when making such a decision: the known toxicity of anti-epileptic drugs; the fact that 10-20% of the patients on such treatment have recurrences of their epileptic crises and that around 25% of the children and 40% of the adults relapse when long-term treatment is stopped. On the other hand, factors which reduce the risk of relapse have recently been identified.

[Sturge-Weber syndrome: experience with 14 cases].

Objective: The objective of this study was to review the cases of Sturge-Weber syndrome (SWS) diagnosed and followed-up in our center over the last 25 year period in order to evaluate their clinical characteristics, evolution and therapeutical response.

Patients And Methods: A retrospective review of the records of patients diagnosed of SSW (facial nevus flammeus at least over the first branch of the trigeminal nerve and ipsilateral leptomeningeal angioma) was performed.

Results: Fifteen patients were found in our records, with one of them being excluded from the study due to the lack of follow-up.

[Neonatal convulsions in health care. II. Prognostic factors].

Objective: To determine whether neonatal convulsions make up a homogeneous pathological group when it comes to establishing indices of prognosis.

Design: Descriptive study of retrospective cohorts.

Scope: Twenty five cases of neonatal convulsions out of the 12,427 new born babies in the province of Albacete in the period 1991-1993 and the follow-up of their development up to December 1994.

[Actual nosology of polyneuropathies in childhood].

The current nosological classification and terminology of the hereditary peripheral neuropathies in childhood are in a instable state. Linkage of the genes of these disorders to chromosomes 1, 8, 17 and X and the finding of altered peripheral myelin proteins are open the way to understand their physiopathology. The genetic heterogeneity and variability in the hereditary pattern obligates to reconsider the nosology of these disorders specially concerning the so called Déjerine-Sottas disease.

[Neonatal convulsions in health care. I. Incidence, etiology and clinical aspects].

Objective: To determine the incidence, etiology and course of neonatal convulsions in the Albacete Health District between 1991 and 1993.

Design: A descriptive study of retrospective cohorts.

Scope: 12,427 new born babies in the province of Albacete.

[Treatment of refractory infantile epilepsy with vigabatrin in a series of 55 patients].

We present the results of treatment with vigabatrin in the polytherapy of resistant infantile epilepsy. A retrospective review of 55 children with resistant epilepsy aged between 2 months and 15 years was carried out between January 1992 and January 1995. Semiologically, the crises treated were simple partial crises (CPS), complex partial crises (CPC), West's syndrome, the Lennox-Gastaut syndrome and polymorphic crises.

[Periventricular leukomalacia: its retrospective diagnosis in children with spastic diplegia].

Typical images of residual periventricular leukomalacia (PVL) have been searched for in a review of the CT scans of 135 children with spastic diplegia. Among the 135 children studied, 90 (66.6%) of them had had a CT scan with 51 (56.

[Acute intermittent familial ataxia: report of a new family].

INTRODUCTION. Acute intermittent familial ataxia is a rare disorder with autosomal dominant inheritance and unknown etiology which usually in childhood or adolescence. CASE 1.

[Ataxia telangiectasia: review of 13 new cases].

We report the review of 13 patients who were diagnosed of ataxia telangiectasia before 6 years of age. All of them manifested cerebelous ataxia, oculocutaneus telangiectasias (11), sinopulmonary infections (9), dystonia (9), oculomotor apraxia (9) and Burkitt linfoma (1). We analyse the most common presentation of the disease in early stages and the complementary studies performed.

[Quality of life in childhood epilepsy].

We describe a Quality Life scale in childhood epilepsy (CAVE) composed by 8 items, behaviour, school compliance, learning, autonomy, social relation, frequency and intensity of seizures and the parents opinions. Each item may be answered as very bad (5), bad (2), medium (3), good (4), or very good (5), and we can obtain full scores before and after the beginning of epileptic treatment and we can compare them. Although other Quality Life questionnaires exist for adult epilepsy, CAVE is the first scale applied for chronic childhood epilepsy.

[Clinical-radiological evaluation of infantile autism and epileptic syndromes associated with autism].

With the aim of ascertaining the existence of medical conditions associated with autism, the presence of pre- and perinatal factors, family antecedents as well as the prevalence rate of different epileptic syndromes in the juvenile autistic population, we carried out a retrospective observational study and looked at the medical history of 62 autistic children so diagnosed in the neuropaediatric service at Barakaldo Hospital Cruces in the past twenty years. All of them were diagnosed by a child neurologist following DSM-III-R criteria, having been by the same physician on at least one occasion. From the etiologic point of view, eight children (13%) presented specific syndromes, fifteen (23%) presented encephalopathies acquired pre-or perinatally, two (3.

Intrathecal chemotherapy-related myeloencephalopathy in a young child with acute lymphoblastic leukemia.

Since the mid-1960s intrathecal chemotherapy (methotrexate [MTX], cytarabine [Ara-C], or both, plus hydrocortisone) has constituted the standard approach to prophylaxis and treatment of central nervous system (CNS) leukemia and lymphoma. Intrathecal chemotherapy-related neurotoxicity has been described in a variable proportion of patients. At least 35 cases of subacute myeloencephalopathy with transient or permanent paraplegia/quadriplegia after intrathecal chemotherapy have been reported.

[A retrospective study of infant headache].

Headache either as an isolated syndrome or as part of a symptomatic grouping is a frequent reason for medical consultation or hospitalization during childhood and adolescence. We review 94 clinical histories of patients between three and thirteen years of age. Headache was the reason for being hospitalized in all cases.

[Neurological development of very low birth weight infants (<1,501 gr) at two ears of age].

The aim of this study is to analyse different aspects of the psychomotor development, during the first two years of the life, of 56 very low birth weight newborns (less of 1.501 g) that had completed a follow-up program. Of the results we have got, the most important are the following: 4 observations (7%) presented severe sequelae motor disability in a tetraparesis with sensorial defects in 3 of them and behavior disturbance (psychosis) with visual defect in the rest.

[Infantile spasms in children with Down's syndrome].

Two hundred eighty six infants with Down syndrome have been studied. Infantile spasms have been identified in nine of them, in which background, EEG pattern and its evolution, modalities of treatment and its effects, neuroimaging and development course have been revised. None of these patients had either familiar or personal pathological antecedents.

[Early infantile epileptic encephalopathy].

Early infantile epileptic encephalopathy (EIEE) with burst-suppression (Ohtaharas syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalised epilepsy. We present 4 cases of EIEE fulfilling the classic diagnostic criteria, excluding the cases with segmentary and erratic myoclonic, characteristic features of Early Myoclonic Encephalopathy. All the patients were females.

[Treatment of the partial epilepsy in the first two years of life. Experience in 26 cases].

We performed a retrospective study with 26 patients with partial epilepsy (16 male, 10 female), aged between 1 and 24 months. We excluded those patients with seizures secondary to cerebral and extracerebral acute diseases, febrile convulsions, infantile spasms and other epileptic syndromes frequently diagnosed at this age. Etiology was of prenatal origin in 8 cases, perinatal in 9, postnatal in 3, and 6 were cryptogenic.