[Specific aims of consultations for learning disorders]. / Objetivos específicos de la consulta de aprendizaje.

AIM: To describe the contribution made by consultations for learning disorders through their specific aims. DEVELOPMENT: The prognosis of children with specific learning difficulties, suffering from dysphasia, dyslexia, dyscalculia, dyspraxia, dysexecutive or dysattentional syndrome, partly depends on whether the methods and materials required for their re-educational and pedagogical adaptation are financed, made available and explained to the child's family in the cases that have been classed as the severest. This is the goal of the 37 reference centres that have been set up in France in the last five years. This work describes the objectives of this consultation, as well as specific ways in which useful information about the child can be shared. CONCLUSIONS: Consultation for learning difficulties involves an interdisciplinary assessment that allows the case of a child with learning disorders to be placed within a new set of dynamics.

[The specific role of the neuropaediatrician in visits for learning difficulties]. / Papel específico del neuropediatra en la consulta de dificultades del aprendizaje.

INTRODUCTION: Coordinating professionals for the initial assessment and treatment of children with learning difficulties is a complicated task. DEVELOPMENT: Initial contact with the sources of information, the history and physical examination, a fast neuropsychological evaluation and requesting suitable tests allow the professional to reach an evaluation that will lead to a proposal for re-education. Evaluating the results of the re-education, coordination among the re-educators and the school, and explanations for the family are essential conditions for favourable progress to be made. CONCLUSIONS: In a visit for learning disorders, the role of the neuropaediatrician is crucial, especially to optimise the performance of the team as regards time and efficiency, and to improve the quality of the service offered to the child.

[Non-verbal learning disorder. Clinical features to guide diagnosis]. / Trastornos del aprendizaje no verbal. Rasgos clinicos para la orientación diagnóstica.

AIMS: The development and multiplication of information about learning disorders leads to the need to systematise the knowledge available and to base it on the clinical data. DEVELOPMENT: Apart from trouble-free diagnoses, non-verbal learning disorders are characterised by their high comorbidity rate. They are associated to attention deficit with or without hyperactivity, motor coordination disorder, dyscalculia, problems with social development and also, to a certain extent, oral and written language disorders. Depending on countries, a child with a good fundamental intellectual capacity and good development of language, but motor clumsiness and low visuospatial skills with or without relational difficulties can be diagnosed in different ways. In this study, after briefly reviewing the literature on comorbidity and the contexts of the work, we propose a set of guidelines for basic examination that can be used in visits due to learning disorders, including suggestive history, areas that require detailed questioning, and central tests. CONCLUSIONS: Partly because of the comorbidity, following the introductory visit, only a multidisciplinary team can study a child with non-verbal learning disorder properly, using specific tests to pinpoint the profile of their difficulties; their strong points should also be stressed.

[Postictal paralysis during video-EEG monitoring studies]. / Paresia poscrítica durante estudios de monitorización de vídeo-EEG.

OBJECTIVE: To know the frequency of Todd s paralysis during the video EEG monitoring studies, to investigate in its pathophysiology, and to confirm its value to localise the epileptic focus. PATIENTS AND METHODS: We reviewed 114 monitoring studies, in 102 patients. RESULTS: Sixty patients had epileptic seizures. An obvious paresis was noted in four seizures of two patients (3 and 1, respectively). Both patients had frontal epilepsy. During the paralysis, in the first patient the EEG showed ictal discharges on the contralateral centrotemporal area. In the second patient, the EEG demonstrated slow waves in the contralateral frontal region. The ictal onset was contralateral to the paresis in all cases. No patient with pseudoseizures had paralysis. CONCLUSIONS: Postconvulsive paralysis are not frequent in video EEG monitoring studies. However, if present it points out to a contralateral seizure onset. In our series it happened in patients with frontal seizures. The EEG may help to clarify if it correspond to a true postictal phenomenon or to a ictal paralysis.

[Vagal stimulation in the treatment of epilepsy]. / Estimulación vagal en el tratamiento de la epilepsia.

INTRODUCTION: The vagal nerve stimulation is a new technique for the treatment of drug resistant epilepsies. DEVELOPMENT: In 1997, it was approved in United States by the FDA to be used in adults with refractory focal epilepsies not candidates for epilepsy surgery. Its mechanism of action is unknown. The results in the controlled studies indicated a decrease of 30 50% in the seizure frequency in around 50% of the patients. Although more experience is needed to corroborate these results, it seems reasonable as a treatment for patients with difficult epilepsies, especially when the response to the antiepileptic drugs is poor or they are producing secondary effects, and the resection of the focus is not possible.

[Lamotrigine in the adult-onset epilepsy: efficacy and long-term safety]. / Lamotrigina en las epilepsias del adulto: eficacia y seguridad a largo plazo.

BACKGROUND: LTG is a new antiepileptic drug that is nowadays very often used in epileptic patients. OBJECTIVES: To determine efficacy and safety of Lamotrigine (LTG) in the first five years after its marketing in patients at a third level university hospital, as well as its impact on the management of classic antiepileptic drugs (AED). PATIENTS AND METHOD: We reviewed retrospectively our Epilepsy Unit Database. One hundred patients were treated with LTG in a 5-year period. Efficacy was evaluated comparing seizure frequency in a 6-month period before and after LTG. The type of epilepsy, side effects, blood levels and concomitant treatments were considered in the analysis. RESULTS: LTG was effective in all groups of epileptic patients studied. Eighteen percent of patients became seizure-free. Seventeen percent of patients improved more than 50%. Fifty-seven percent of patients remained treated with LTG after four years of follow-up. Side effects were mild, but frequent; only four patients discontinued LTG because of adverse effects. Serum levels were usually high, but showed no relation with clinical efficacy. The mean number of AED taken per patient increased. CONCLUSIONS: LTG is a safe an effective drug in epilepsy. It has a clear impact in the management of the epileptic patients.

Gait epilepsy. A case report of gait-induced seizures.

Reflex epilepsy includes a group of epileptic syndromes in which seizures are induced by a stimulus, either simple (visual, somatosensory, olfactory, auditory) or more complex (e.g., eating, thinking, reading). We document a case of reflex epilepsy in which focal seizures are triggered exclusively by gait. The patient is a young boy whose walking was impaired by abnormal motor phenomena on the left side. These phenomena were elicited by gait and were accompanied by a distinctive ictal pattern with centro-temporal discharges. After comparing this patient with others reported in the literature, we determined that he has an unusual type of reflex epilepsy for which we coined the term "gait epilepsy." This disorder must be considered when physicians are making a differential diagnosis in patients who have symptoms that suggest paroxysmal kinesigenic dystonia (PKD) or selective epileptic gait disorder.

[Surgical treatment of epilepsies: criteria for the selection of patients and results]. / Tratamiento quirúrgico de las epilepsias: criterios de selección de pacientes y resultados.

INTRODUCTION: Approximately 20% of all epileptic patients are not satisfactorily controlled by the available antiepileptic drugs. Some of these patients have epileptic syndromes which could potentially be treated by surgery. DEVELOPMENT: The technological advances applied to diagnostic and therapeutic methods have improved the identification of epileptic patients who may benefit from surgery. Up to 80% of the patients with focal epilepsies symptomatic of well defined lesions may become free of seizures after excision of the lesion or epileptogenic focus. Other forms of epilepsy, such as the so-called catastrophic infantile forms, may improve temporarily when techniques such as hemispherectomy or callosotomy are used. The morbidity and mortality of these surgical procedures are minimal. The results depend on correct selection of the patients. A strict protocol for rigorous evaluation of the patients should be used, with the collaboration of neurologists, epileptologist neuropaediatricians, neuropsychologists, neurophysiologists, neuroimaging specialists, psychiatrists and neurosurgeons. There should first be clear answers to three key questions: 1. Who is a good candidate? 2. How should the selection be made? and 3. When is the best time for evaluation? CONCLUSIONS: At present it seems clear that the surgery of epilepsy is used less than it could be. It is therefore necessary to encourage the development of specialist units to select patients and treat them, and to develop the means whereby patients can obtain this highly specialized attention.

[Specific disorders in the language development: neurobiological basis]. / Trastornos específicos del desarrollo del lenguaje: bases neurobiológicas.

Studies of twins, familial aggregates and particular phenotypic conditions have shown an inherited basis for some dysphasias or specific developmental language impairments (SLI). This predisposition is usually multifactorial but the analysis of some families allows to postulate an autosomal dominant transmission of deficits in specific modular aspects of linguistic competences. Moreover, neuroimaging studies have shown modifications of normal volumetric interhemispheric asymmetries, and in group of SLI with receptive prominent disorder coexist epileptiform activity in wakefulness and non-REM sleep EEG; in some of these cases, antiepileptic drugs, specially steroids, can significantly ameliorate the language processing. As many patients with SLI have a difficulty for discrimination of subtle temporal indices, a hypothesis can also be made of a dysfunction in various subcortical structures (thalamus, basal ganglia, cerebellum) modulating the cerebral cortex in phonological processing.

A simple, effective and well-tolerated treatment regime for West syndrome.

The authors systematically treated 94 patients with West syndrome using the same protocol of sodium valproate and steroids, starting with hydrocortisone (HC) orally for two weeks. If seizures stopped, HC was withdrawn; if they persisted, tetracosactrin (TA; synthetic ACTH) was administered for another two weeks then HC was slowly withdrawn. 90 per cent of the symptomatic cases were controlled by HC, the remainder by TA. 65 per cent of symptomatic cases were controlled by HC; this rose to 78 per cent if patients treated by HC then TA were included. At 31 months follow-up, the percentage of favourable results was 72 per cent for cryptogenic and 60 per cent for symptomatic cases. For the latter, best results were obtained in patients with periventricular leukomalacia, postnatal distress and porencephaly. Patients suffering from sequelae of full-term distress or encephalopathy of unknown aetiology were the most resistant.

Lamotrigine in treatment of 120 children with epilepsy.

One hundred twenty children aged 10 months to 16 years 9 months were included in three studies with lamotrigine (LTG): a single-blind study (n = 60), a pharmacokinetic study (n = 23), and a compassionate group (n = 37). At 3 months, 11 patients had become seizure-free and 34 had > 50% decrease in seizure frequency. The best results involved absence epilepsy, Lennox-Gastaut syndrome (LGS), and other symptomatic generalized epilepsy. Forty-two patients were followed > 1 year, 22 for a mean of 2.2 years, and there was no significant increase in seizure frequency as compared with 3-month follow-up. Fourteen patients became seizure-free for > 6 months; all except 1 had generalized epilepsy. For 12 patients, treatment could be reduced to monotherapy, but for those with valproate (VPA) comedication LTG dosage had to be increased; 25% of patients with VPA monotherapy exhibited skin rash, appearing 3-18 days after starting LTG. For 4 patients, LTG could be reintroduced after VPA was withdrawn. Ten patients had ataxia and/or drowsiness and 2 had vomiting. For all other patients, tolerance was excellent.

Landau-Kleffner syndrome: a pharmacologic study of five cases.

Five children with Landau-Kleffner syndrome (epilepsy, acquired aphasia, and continuous spike-wave discharges during sleep), were treated with antiepileptic drugs (AEDs), sleep-modifying drugs, and corticosteroids. The pharmacologic profiles differed from those observed in focal epilepsies, resembling instead those of certain generalized epilepsies, such as West or Lennox-Gastaut syndromes. Phenobarbital (PB), carbamazepine (CBZ), and phenytoin (PHT) were ineffective or worsened the EEG and neuropsychological symptoms, whereas valproate (VPA), ethosuximide (ESM), and benzodiazepines were partially or transiently efficacious. Dextroamphetamine produced a dramatic but transient improvement in waking and sleep EEG in one of two children; aphasia did not change. Corticosteroid treatment resulted in improved speech, suppression of seizures, and normalization of the EEG in three of three children. Our own experience and data from the literature suggest that corticosteroids should be given in high doses as soon as the diagnosis is firmly established and should be continued in maintenance dose for several months or years to avoid escape. Early diagnosis, before mutism or global deterioration develops, appears to be essential for effective therapy with minimal neuropsychological sequelae.