[Progressive myoclonic epilepsy: clinical characteristics of 18 patients]. / Epilepsia mioclónica progresiva: caracterización clínica de 18 pacientes.

INTRODUCTION: The term progressive myoclonic epilepsy (PME) includes a groups of heterogeneous conditions, with genetic causes, characterized by having different types of seizures, basically myoclonic, and other neurological findings due to a progressive lesion of the central nervous system. OBJECTIVE: To demonstrate the aetiology and clinico-encephalographic changes seen in patients with PME. PATIENTS AND METHODS: A retrospective, descriptive study was done of patients attended for PME in the Instituto de Neurología y Neurocirugía de Cuba between 1990 and 1995. Eighteen patients were included. All were interviewed and had a physical examination, EEG and the specific complementary tests for each aetiology. RESULTS: There was a predominance of neural ceroid lipofuschinosis in 10 patients (55.5%), and in 9 of these the illness started before the age of 9 years. The second most frequent condition was myoclonic epilepsy with red-torn fibres (16.6%) and Unverricht-Lundborg disease (16.6%). The latter began in late childhood or adolescence. The most marked clinical characteristics were epilepsy, which was difficult to control and intellectual deterioration in 100%, followed by cerebellar signs in 88.8%. Myoclonias were the commonest type of seizures (94.4%) and many children presented with prior tonic-clonic seizures (88.8%). CONCLUSION: Response to treatment was poor but the best results were obtained using valproate either alone or associated with benzodiazepines.

[Tumors of the posterior fossa in children]. / Tumores de fosa posterior en el niño.

INTRODUCTION: Primary tumours of the CNS form 20% of the neoplasias seen in children. They are the second commonest type of cancer seen in childhood, after leukemia. A significant proportion of paediatric cerebral tumours (> 50%) are intratentorial. OBJECTIVE: To find the frequency and clinicopathological behavior of posterior fossa tumours in children seen at our hospital. PATIENTS AND METHODS: From the hospital records, a descriptive, retrospective study was made of 112 children with tumours of the posterior fossa who were attended at the Institute of Neurology and Neurosurgery between January 1980 and December 1997. The variables: age, sex, clinical findings, site and extent of the tumour and its histological description were recorded. The mean, standard deviation, frequency distribution and chi-squared test were used, depending on the type of variable to be analyzed. RESULTS: The age of the patients when the diagnosis was made was between 8 months and 15 years (mean = 8.32 years). The male/female ratio was 59/53 (1.1/1). The commonest site of the tumours was the cerebellum (58.9%). The predominant histological types were medulloblastoma (33.92%) and astrocytoma (19.64%) in the cerebellum, glioma in the brain stem (10.71%) and ependymoma in the i.v. ventricle (8.09%). CONCLUSIONS: Endocranial hypertension and the cerebellar syndrome were the predominant clinical findings in medulloblastoma, cerebellar astrocytoma and ependymoma. In brainstem gliomas there was often involvement of several cranial nerves and long tracts.

[Factors to consider in the evaluation of intellectual achievement of an epileptic child]. / Factores que deben considerarse en la valoración del rendimiento intelectual en el niño epiléptico.

In this paper, factors related to the intellectual achievement of epileptic children are reviewed. These should be taken into account by medical and paramedical staff when making the intellectual prognosis of each patient. The factors related to epilepsy include: The type of crisis and epileptic syndrome, the aetiology of the epilepsy, genetic factors, age of onset, duration of the disorder and frequency of crises, electroencephalographic alterations, a history of status epilepticus, the antiepileptic drugs and their metabolic effects. The most important psychosocial factors are: The school, the family and the patient's personality. The significance of the multifactorial effects is emphasized.

[Influence of epilepsy-related factors on scholastic achievement in an epileptic child]. / Influencia de algunos factores relacionados con la epilepsia sobre los resultados escolares del niño epiléptico.

We studied 30 children aged between 5 and 12 years (mean = 8.7 years: standard deviation = (+)-1 9678) who suffered from partial epileptic crises, with or without secondary generalization, cryptogenic, who had had this disorder for more than one year, and who attended ordinary schools. The objective was to ascertain the educational results obtained, and to evaluate the effect of some of the factors related to epilepsy, in these results. The scholar's family was interviewed in all cases to record: the age of onset and duration of the illness, the frequency of crises, attendance at school and results obtained in the previous school year. The school results were: 5 children had results E (16.67%). 10 (33.33%) were classed as MB, 6 (20%) as B, 5 had R (16.67%) and 4 (13.33%) were evaluated as M. It is concluded that the factors most closely related to poor school results in the group studied were: a frequency of more than three crises in the previous year, partial crises with secondary generalization and failure to attend school because of crises.

[Intellectual achievements of the epileptic child in primary school]. / Rendimiento escolar del niño epiléptico que asiste a escuelas primarias.

Thirty children aged between 5 and 12 (M = 8.7 years, DS = +/- 1.9678) who suffered from cryptogenic partial crises, with or without secondary generalization, for more than one year and attended primary schools for normal children were studied to determine their intellectual achievements. Evaluation of intellectual output (WISC) was done for all these children. These findings were then compared with a control group of 30 children matched for age, sex and school grade using the mean comparison tests. In the group studied there was an average IQ of 90.03 and in the control group of 97.00 with a significant difference between both groups (p < 0.05). The implementing and subtest keys (p < 0.05) also showed significant differences. It is concluded that there is a significant difference in the quantitative expression of intellectual output between the two groups in spite of there being a normal mean and that the frequency of borderline cases is significantly greater in the group of epileptic children.