Cognicion e impacto familiar en niños con epilepsias refractarias: Resultados preliminares en 100 casos / Cognition and familial impact of children with refractory epilepsy. Preliminary results in 100 cases

Objetivos: conocer el nivel cognitivo global y el impacto familiar en un grupo de niños con epilepsias de dificil control, posibles candidatos a tratamiento quirúrgico. Material y método: la muestra estuvo constituida por 100 niños (edad media 11.2 años). El nivel cognitivo global se evaluó con un test de inteligencia general (Stanford-Binet, Wechsler). El impacto sobre la familia se estimó a través de una encuesta donde se consideró la percepción subjetiva de los padres acerca del nivel de calidad de vida de sus hijos. Resultados. Nivel cognitivo: el 58 por ciento registra retraso mental de grado leve a grave (Organización Mundial de la Salud OMS) el 11 por ciento tiene un cociente intelectual promedio y el 31 por ciento restante se sitúa en la franja de normal bajo y limítrofe. Percepción subjetiva de calidad de vida: 12 por ciento mala o muy mala, 40 por ciento regular, 37 por ciento buena, y 11 por ciento muy buena. Conclusiones. coincidente con otras investigaciones los niños con epilepsias refractantes registran un nivel intelectual inferior al término medio, lo cual refleja un grado variable pero claramente significativo de afectación cognitiva. De acuerdo a la percepción de sus padres, la calidad de vida es mala o regular en el 52 por ciento de los casos. Estos datos confirman la importancia de buscar medidas terapéuticas más efectivas, incluyendo un eventual tratamiento quirúrgico con el objeto de evitar o detener el deterioro cognitivo y mejorar la calidad de vida (presente y futura)de estos niños

Cognicion e impacto familiar en niños con epilepsias refractarias: Resultados preliminares en 100 casos / Cognition and familial impact of children with refractory epilepsy. Preliminary results in 100 cases

Objetivos: conocer el nivel cognitivo global y el impacto familiar en un grupo de niños con epilepsias de dificil control, posibles candidatos a tratamiento quirúrgico. Material y método: la muestra estuvo constituida por 100 niños (edad media 11.2 años). El nivel cognitivo global se evaluó con un test de inteligencia general (Stanford-Binet, Wechsler). El impacto sobre la familia se estimó a través de una encuesta donde se consideró la percepción subjetiva de los padres acerca del nivel de calidad de vida de sus hijos. Resultados. Nivel cognitivo: el 58 por ciento registra retraso mental de grado leve a grave (Organización Mundial de la Salud OMS) el 11 por ciento tiene un cociente intelectual promedio y el 31 por ciento restante se sitúa en la franja de normal bajo y limítrofe. Percepción subjetiva de calidad de vida: 12 por ciento mala o muy mala, 40 por ciento regular, 37 por ciento buena, y 11 por ciento muy buena. Conclusiones. coincidente con otras investigaciones los niños con epilepsias refractantes registran un nivel intelectual inferior al término medio, lo cual refleja un grado variable pero claramente significativo de afectación cognitiva. De acuerdo a la percepción de sus padres, la calidad de vida es mala o regular en el 52 por ciento de los casos. Estos datos confirman la importancia de buscar medidas terapéuticas más efectivas, incluyendo un eventual tratamiento quirúrgico con el objeto de evitar o detener el deterioro cognitivo y mejorar la calidad de vida (presente y futura)de estos niños(AU)

[Instruments for evaluation of memory abilities in children]. / Técnicas para evaluar la memoria del niño.

OBJECTIVES: This is a review of the more relevant instruments to measure memory abilities of children and adolescents. The strengths, limitations and future developments of these instruments are discussed. CONTENTS: Memory disturbances are present in developing individuals as a consequence of perinatal damage, brain trauma, neuroinfections, epilepsy etc. Also mnesic deficits may be observed as an element of language and learning disorders. Probably all types of amnesic syndromes well known in adults do exist in children, but there are only a few observations documented in the literature. Here are described the available batteries (TOMAL, WRAML, CMS, BEM 144) and specific tests (RBMT C,VADS, CAVLT 2, etc.) that are useful for the diagnostic measurement of verbal and visual memory abilities in children and adolescents; a Spanish version and validation is available for many of these instruments. The use of tests must be preceded by structured clinical interviews and questionnaires for parents and teachers. CONCLUSIONS: In spite of all these instruments, the diagnosis must be based on clinical criteria; new developments of instruments adapted to children aged under five years have to be done. All aspects of memory functions shall be considered procedural, explicit (semantic an autobiographical), prospective, etc. , parallel forms and typifications on more large samples are claimed.

[Evaluation of executive functions in children]. / Evaluación de las funciones ejecutivas en el niño.

OBJECTIVES: This is a review of the available instruments to evaluate executive functions in children and adolescents. DEVELOPMENT: Executive functions (EF) give support for organization, anticipation, inhibition, working memory, flexibility, control and autoregulation of behavior. EF appear abnormal in several neurological an psychopathological conditions e.g. learning and behavior disorders, obsessive compulsive disorder, attention deficit/hyperactivity disorder, Tourette syndrome, Asperger syndrome, and temporal epilepsy. The large spectrum of the so called EF makes difficult to propose any simple and comprehensive instrument for evaluation. In spite of that, here are described the more useful tests adapted to developmental ages. CONCLUSIONS: The measure of FE in children and adolescents needs further developments of more solids theoretical models. Nevertheless, in the last recent years relevant progresses have been done, and several useful instruments of evaluation have been built for clinical practice and research.

Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type).

We report, on two, school-age girls with clinical and electroencephalographic features of early onset childhood epilepsy with occipital paroxysms (CEOP) of the "Panayiotopoulos type" that showed atypical evolution. Neurological examination and brain imaging were normal in both. One child presented at age 2.5 years episodes of oculocephalic deviation, and ictal vomiting during nocturnal sleep. The EEG showed left occipital spikes during wakefulness and sleep. One year later, frequent inhibitory seizures appeared in the lower limbs causing, "pseudoataxic gait". At the same time she presented with behavioral disturbances and aphasia. EEG showed bilateral spike-waves while awake and continuous spike-waves during slow sleep (CSWSS). After switching AEDs to benzodiazepines, control of seizures along with improvement of behavior, and partial restoration of cognitive functions were achieved. The CSWSS disappeared and the last EEG at age 8 years only showed only isolated right occipital spikes. The other girl had a personal and familial history of febrile seizures. At 4 years of age she presented the first non-febrile seizures during sleep, with oculocephalic deviation and ictal vomiting, followed by a generalized tonic-clonic seizure. Partial control of seizures was obtained with antiepileptic drugs. At age 7, the child began to have weekly episodes of oculocephalic version, occasionally with secondary generalization. Repeated inhibitory seizures and absences also appeared. EEG showed frequent bilateral spikes occupying predominantly the posterior regions while awake, and CSWSS. At 7.5 years the same electro-clinical picture persisted. Ethosuximide was added to sodium valproate and clobazam. Fifteen days later, the seizures disappeared and the EEG showed less frequent bilateral occipital spikes. She is now 9 years old and she has been seizure-free for 18 months. Her present neuropsychological profile shows mild mental retardation. The two children with typical electroclinical features of "Panayiotopoulos Type" CEOP developed an atypical evolution which, to our knowledge, has not been described previously.

Extratemporal epilepsy in children: candidate selection and surgical treatment.

From June 1988 to June 1998, 60 children with extratemporal epilepsies (EE), most of whom were symptomatic, underwent surgery. All patients were studied by means of CT scanning, MRI and scalp EEG. Video-telemetry was used in 40 cases. Intracranial electrodes were placed in 10. Intraoperative ECoG was used in the 35 children who underwent resective procedures and in the 25 in whom disconnection was performed. Surgical procedures were as follows: 24 lesionectomies, 25 disconnecting procedures, 7 polectomies and/or lobectomies, 3 corticectomies and 1 anatomical hemispherectomy. After at least 1 year's follow-up in 48 children, to date 38 are in Engel class I, 7 in class II, 1 in class III and 2 in class IV. That is to say, in 46 of the 48, surgical outcomes ranges from very good to at least worthwhile, as reflected in their classification in Engel class III.

[Rasmussen syndrome]. / Síndrome de Rasmussen.

INTRODUCTION: Rasmussen's encephalitis is a neurological disorder probably of immunological origin, characterized by intractable epilepsy, neurological deterioration and the presence of antibodies against R3 glutamate receptors. OBJECTIVE: To present the experience of an Argentinian Paediatric Neurology Service during the past 7 years. MATERIAL AND METHODS: We analyzed the clinical histories of 12 patients with RS as defined by the diagnostic criteria proposed by Hart et al. We evaluated the following parameters: neurological examination, neuropsychological assessment, types of crises, age of onset, frequency, distribution, electroencephalogram, video EEG, evoked potentials, neuro-radiological studies; CT and cerebral MR using gadolinium, SPECT, lumbar puncture and cerebral biopsy. RESULTS: The crises were of simple partial motor type in 12 cases, secondarily generalized partial crises in 7 cases, complex partial crises in 4 cases and partial and generalized motor states in 8 cases. RESULTS. The ictal EEGs during the continuous partial crises showed contralateral focal slow activity of the affected side of the body in 30% of cases. The neuro-radiological studies (CT and MR) showed signs of cerebral hemiatrophy in all 12 cases, hyper-intense images in the T2 sequences of the fronto-temporo-parietal regions in 5 cases, an asymmetric megacisterna magna in 1 case and hypoplasia of the cerebellar vermis in another case. CONCLUSIONS: The continuous partial crises showed little correlation with the electro-encephalography. Following administration of i.v. gammaglobulin there was a transient halt in progression of the neurological deterioration and control of the crises in 55% of the cases. Surgery continued to be the treatment recommended for control of the epileptic crises.

Acquired epileptic aphasia: neuropsychologic follow-up of 12 patients.

A study of specific neuropsychologic, neurolinguistic, and behavioral features of acquired epileptic aphasia or Landau-Kleffner syndrome was conducted in a group of 12 patients followed-up for 2-15 years (mean: 8 yr). Seventy-five percent had exhibited some language disturbance prior to acquired epileptic aphasia. Even when 9 patients had normal electroencephalographic findings in the long-term course of the disease, only 3 achieved normal language. No patient with persisting electroencephalographic abnormalities recovered normal or near normal language. The need to perform detailed neurolinguistic and neuropsychologic evaluations in the work-up and follow-up of children with acquired epileptic aphasia is stressed. An adapted neuropsychologic profile battery proved to be practical and objective for the follow-up of these patients.