[Movement disorders: dystonias which are apparently psychosomatic. Torsion dystonias].

In neuropediatric clinical practice, disorders of movement include a wide diversity of conditions, amongst which the dystonias are uncommon in our practice, although they have to be considered amongst the possible diagnoses in some cases. The great variety of clinical symptoms and age of onset together with the nonspecific, erratic clinical course make diagnosis difficult. Some clinical pictures of genuine torsion disorders may be confused with hysterical conversion disorders, somatizations or Munchausen's syndrome.

[The evaluation of the effects of pharmacological treatment of children with attention deficit hyperactivity disorder].

In this study we approach problems related to the dosage and specific action of methylphenidate, its effects at cognitive and social levels, possible side-effects and limitations to its usage, and methods for evaluation of the response to treatment in the school environment. We make special mention of the importance of both parents and teachers being familiar with these problems.

[Children at risk for learning disorders].

Introduction: To find what is meant by normal learning, it is essential to understand learning difficulties not only from the anatomical but also from the functional point of view.

Development: We review the literature concerning risk factors for learning difficulties in order to treat them early on and attain satisfactory long-term development.

Conclusions: Learning difficulties are the largest groups of disorders in everyday neuropaediatric practice.

[Epileptic signs of metabolic origin].

Introduction: It is very difficult to identify metabolic causes of epileptic crises. This is partly because metabolic disorders are not fully understood and partly because the complementary tests required for their identification can be undertaken only in a few laboratories.

Development: We describe the characteristics of the metabolic disorders which may most frequently lead to epileptic crises occurring during the neonatal period, the first year of life, between the ages of one and six years and between six and fifteen years of age.

[Risk factors in cerebrovascular disease in childhood].

Introduction: The etiopathogenesis of cerebrovascular diseases in paediatrics is little known and very varied. Review of the literature gives little practical information about how to investigate a paediatric patient who presents with an acute cerebrovascular illness.

Objectives: To identify the risk factors for cerebrovascular accidents in the paediatric age group in our setting and establish guidelines as to how best to act.

[Economic costs of childhood epilepsy in Spain].

Introduction: Health expenditure increases continuously in all developed countries. Common disorders, including epilepsy are responsible for most of the health budget.

Objective: To quantify the economic effect of childhood epilepsy in Spain by analyzing its major components.

[Visual evoked potentials in children with tension headaches and migraine].

Objective: To determine whether visual evoked potentials (VEP) change, and to what degree, in different types of headache (migraine with or without aura, or tension headache).

Patients And Methods: We made a transversal study of 78 children (aged 3-14 years) studied between March 1997 and August 1998, classified into three groups according to HIS diagnostic criteria of 1988 and HIS-R 1997. A VEP of geometric pattern was done using the recording technique recommended by the International Society in their standards for VEP and the reference values were used for an amplitude of less than 5 microV and a latency of P100 +/- 15 ms.

[Ischemic anoxic cerebral crises in children. Electrophysiological aspects].

Introduction: Ischemic anoxic cerebral crises are linked to cardio-inhibition in response to vagal hypersensitivity which causes cerebral ischemic.

Objective: In this paper we proposed to describe the clinical and electrophysiological features seen during the ocular compression test.

Patients And Methods: We studied 200 children with clinical findings suggesting ischemic anoxic cerebral crises with positive results on ocular compression testing.

[Perinatal intracranial hemorrhage due to immune thrombocytopenia].

Introduction: Immune neonatal thrombocytopenia is caused by maternal antibodies (IgG) passing across the placenta, with subsequent destruction of foetal platelets. There are two forms, the iso-aloimmune forms, with an incidence of intracranial hemorrhage (ICH) in the neonatal period of 10-20%, and the autoimmune form with an incidence of only 1%.

Objective: To review the patients with this condition in a neonatal unit.

[Early mitochondrial encephalomyopathy due to complex IV deficiency consistent with Alpers-Huttenlocher syndrome: report of two cases].

Introduction: Complex IV or cytochrome c oxidase (COX) deficiency is the most common disorder involving complexes of the respiratory chain in the pediatric age. Exceptionally, it has been reported in association with Alpers syndrome or Alpers disease, and with its variant named progressive neuronal degeneration with liver disease or Alpers-Huttenlocher syndrome.

Objective: To report the cases of two infants with mitochondrial encephalomyopathy due to COX deficiency in whom the clinical, biochemical, neurophysiologic and neuroimaging characterization suggested an associated Alpers-Huttenlocher syndrome.

[Brain stem ischemia in a boy with resistance to C activated protein and elevated lipoprotein A].

Introduction: Activated protein C resistance is the most common hereditary coagulation abnormality. In the majority of cases it results from a point mutation Arg506-->Gln of the factor V gene, and characterized by a poor anticoagulant response to activated protein C.

Clinical Case: We report the clinical case of a 6-year-old obese boy, who presented with acute hemiparesis.

[Long-term efficacy and tolerance of topiramate in 44 children with resistant epilepsy].

Introduction: Topiramate (TPM) is a new antiepileptic drug with multiple modes of action which should theoretically represent a wide therapeutic spectrum. However, there is still little clinical experience of its use in children with epilepsy.

Patients And Methods: TPM was given during a period of 14.

[Vigabatrin and alterations of the visual field].

Introduction: Since 1997 when Ecke et al described peripheral constriction of the visual field in three patients taking vigabatrin (VGB), and persistence of the changes after the drug was stopped, study of the visual fields of persons taking this antiepileptic drug has increased.

Objective: To evaluate the ocular repercussions of monotherapy with carbamazepine (CBZ), valproate (VPA) and VGB in children with epilepsy.

Patients And Methods: We made a blind retrospective study to evaluate visual side effects using Goldmann's campimetry and direct ophthalmoscopy in 9 children treated for 2.

[Reflex anoxic cerebral crises in children].

Objective: Reflex anoxic cerebral crises are due to depression of nerve function caused by a vagotonic state or vagal hypersensitivity. In this paper we propose to review the physiopathology, clinical features and diagnostic procedures of these crises.

Development: There are three types of reflex anoxic cerebral crises: asphyxiating anoxic crises, ischemic anoxic crises and asphyxiating-ischemic anoxic crises.

[In children with refractory epilepsy: vigabatrin or lamotrigine?].

Introduction: Both vigabatrin (VGB) and lamotrigine (LTG) have been shown to be effective in the control of epileptic crises in double-blind randomized studies, as compared with placebos and as treatment in addition to the classical drugs.

Patients And Methods: In this study we made a retrospective analysis of the evolution of 97 children aged from 6 months to 16 years with resistant epilepsy, to whose treatment either VGB or LTG were added.

Results: Frequency of the crises was reduced by > 50% in 54.

[Rett's syndrome in the Spanish population].

Introduction And Objective: Rett syndrome was described in 1966 and became known through the English medical literature in 1983. There are typical and atypical forms. The objective of this study was to record the cases diagnosed in Spain and discover their clinical characteristics in order to describe its phenotype and geographical distribution.

[Various practical considerations in the management of movement disorders in children].

Disorders of movement in children often cause semiological and diagnostic difficulties to the paediatricians concerned. Based on personal experience, the author analyzes aspects which are particularly useful in overcoming these problems. Epidemiology, rules for history taking, indications as to the interpretation of clinical signs and use of complementary studies are considered.

[Clinical features of epilepsy in adolescents].

Objective: The epilepsy is a common neurologic disorder in adolescence. Its prevalence is 1.5-2% in these ages.

[Holmes-Adie syndrome. Clinical case].

Introduction: The Adie tonic pupil is an intrinsic, isolated and benign ophthalmoplegia producing defective accommodation and anisocoria. The affectation is unilateral in the 90% of cases and can appear at any age. If associated to patellar tendinous reflexes abolition, constitutes Holmes-Adie syndrome.

[Suggestions regarding new classification of epileptic seizures].

Introduction: A modification of the current classification of epilepsy is proposed taking the clinical and neurophysiological knowledge of recent years into account.

Development: In the first group (partial seizures) those of unknown type are added. In the second group (generalized seizures) typical and atypical absence attacks, tonic, atonic, clonic and tonic-clonic attacks are included.

[Risk factors in learning difficulties].

Objective: To review the literature of the risk factors in learning difficulties (LD) and to assess these factors in children diagnosed as having LD in the Neuropaediatric Department of the Hospital Universitario Infantil La Fe in Valencia, in 1996 and 1997.

Material And Methods: A retrospective study was done of children diagnosed in the Hospital Infantil Universitario La Fe in Valencia as having learning difficulties. The different factors related to LD were assessed.

[3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].

Introduction: 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMG-CoA lyase) is an inborn error of ketogenesis and Leucine catabolism. HMG-CoA lyase catalyses the final step in leucine degradation, converting HMG-CoA to acetyl-CoA and acetoacetic acid. Clinical manifestations include hepatomegaly, lethargy or coma and apnoea.

[Diagnostic focus on the child with myoclonic seizures in isolation or associated with other types of seizures].

Myoclonus may be observed in children with mild or severe epileptic syndromes. Both types are seen at characteristic ages, together with other factors: aetiology, family history, hereditary pattern, effect on psychomotor development and EEG-EMG findings. In children with progressive or degenerative encephalopathies and myoclonus, better known as progressive myoclonic epilepsy, there are also specific clinical data, together with biological and genetic markers which permit identification.

[Tiagabine: a GABA-ergic anti-epileptic drug].

Introduction: Tiagabina (TGB) is a new anti-epileptic drug which inhibits the uptake of GABA by the neurons and glia and prolongs the time GABA remains at the synapse.

Development: Experimental studies suggested the efficacy of TGB in generalized tonic-clonic crises, in focal crises and in myoclonic crises. This has been confirmed in the clinical trials carried out in patients and after free use of the drug after being put on sale.