Antonio B. Lefèvre

Prevalence of sleep-talking in childhood

This report is on the prevalence of somniloquy, as revealed by a populational survey of 2,022 schoolchildren aged from 3 to 10 years. Data were collected by means of a multiple choice questionnaire aimed at the investigation of sleep characteristics (sleep characteristics questionnaire-SCQ) and submitted to statistical analysis. The objective was to detect the prevalence of sleep-talk and influence of age, sex and socioeconomic class on its frequency. About half the children of all ages presented somniloquy at least once a year, but less than 10% presented it everyday. No clearmarked correlation with age, sex or socioeconomic class was found. These results do not confirm the generally accepted notion that there is a higher prevalence in the beginning of the first decade of life and suggest also the necessity of further quantitative and objective assessment of this phenomenon.

Evaluation of flurazepam and placebo on sleep disorders in childhood.

The clinically observed results in 40 patients, from 1 to 15 years old, presenting sleep disturbances, in a comparative and statistically approached study of flurazepam 15mg daily against placebo, are reported. Placebo was administered, followed by the drug, during 14 days each. The chief complaints were sleepwalking, sleep-talking, sleep terror, sleep-related bruxism, sleep-related headbanging, insomnia and excessive movements during sleep. A significant effect of flurazepam on sleepwalking, sleep-talking, bruxism, sleep terror and excessive movement during sleep, was observed. The insomniac headbanging patients were not enough for statistical analysis. Flurazepam side effects were excessive drowsiness during daytime in 3 cases; irritability, 3 cases; nausea and vomiting, 2 cases, and were not correlated with age. Placebo side effects were similar, except for nausea and vomiting which were not observed. It was necessary to discontinue flurazepam in 2 cases, because of excessive drowsiness during daytime, which did not improve when reducing the dose.

Subacute sclerosing leucoencephalitis: an epidemiological, clinical and biochemical study of 31 cases

Thirty-one cases of SSLE are reported. The diagnosis was based on the clinical picture, the marked increase of gammaglobulins in the cerebrospinal fluid, the typical electroencephalographic pattern, and the pathological examination. Intracellular inclusion bodies were found at the postmortem examination of 8 among 13 cases and in 1 of 7 cerebral biopsies. The mean age was 11.7 years, with the limits of 3 and 22 years. Males prevailed markedly over females (23:8). Patients from rural regions were the bulk of the series (77.4%). No definite regional predominance was demonstrated by the analysis of the geographic distribution. A seasonal influence was not evidenced. In 16 patients followed up to death, the average duration of the disease was 7 months, with a range from 2 to 20 months. In the clinical picture the constancy of myoclonus or losses of tonus, and psychiatric disorders was evidenced. The first symptom was the psychiatric alteration in 55.6% of the cases, myoclonus in 35.5% and grand mal seizures in 33.3%. Rigidity and pyramidal signs were very frequent, while the cerebellar syndrome was rare. Ophtalmoscopic alterations (edematous or pale disk, choroidits) were found in a third of the cases. In two cases an epizootic of household chicken was coincident with the onset of the disease. The blood copper concentrations determined in 8 cases were always above the normal range, while ceruloplasmin remained within the normal limits. The blood sulfur levels were high in 3 patients studied. The blood magnesium levels were variable in 6 patients studied.

Subacute sclerosing panencephalitis in only one member of a monozygotic twin pair: Clinical, laboratory, electroencephalographic, and immunological study of both twins

Abstract Male monozygotic twins, one of whom developed subacute sclerosing panencephalitis (SSPE) are reported in detail. Both patients had measles at the age of 4 years and 6 months. The diagnosis of SSPE was based on clinical, electroencephalographic and laboratory findings. Titration of antibodies against measles was carried out in both cases. The healthy twin, who had been admitted with a clinical picture initially similar to that displayed by his brother, showed clinical manifestations mimicking those of the affected sib, but these vanished 4 days after his admission and were attributed to transient conversion hysteria. The genetic factors involved and the biological response of the antibodies against measles are discussed.

Distúrbios psíquicos na neurocisticercose em crianças

Six cases of neurocysticercosis in children are reported. These 6 patients were selected from a total number of 54 cases, in view of their particular symptomatology, represented by terrifying visual hallucinations (zoopsias) and psichic disturbances. Five of the patients also had a total loss of sight. The clinical characteristics of the patients are reported. The relatively satisfactory response to treatment in 5 cases is commented.

Tratamento do estado de mal epiléptico em crianças pelo Valium

Considering the results obtained in 40 children with status epilepticus, the favorable effects of Valium administered intravenously are emphasized. The drug administered in small doses, undiluted, quantum satis and without relation to weight and age of the patient, gave good results in all cases, the control of the convulsive crisis being very fast. There were not observed toxic manifestations.

Granuloma eosinófilo da coluna cervical com manifestação neurológica, liquorica e radiológica atípica

A case of eosinophilic granuloma of the cervical spine in a ten year-old white boy is reported. The patient complained of posterior cervical pain at the level of C5-C6 and right hemiparesis that progressed to mixed tetraparesis (lower motor neuron in upper limbs and upper motor neuron in lower limbs). Examination of the cerebrospinal fluid (CSF) disclosed an inflammatory reaction; radiologic studies of the cervical spine showed an irregular aspect with demineralization at the base of spinous process of C6. The patient was treatet intermitently with corticosteroids (prednisone); there was an improvement of neurologic symptoms and the examination of the CSF was normal within a few days, each time he took the drug. Neurologic symptons and abnormalities of the CSF recurred after discontinuation of corticotherapy. Examination of a fragment of the spinous process and neighboring tissues of C6, taken by biopsy, disclosed eosinophilic granuloma. Radiotherapy was then indicated, plus corticosteroids. There was remission of all neurologic symptoms and signs. The unusual features of the case are discussed in relation to others reported in the literature.

Fenilcetonúria: estudo clínico e mediante biópsia cerebral

Six cases of phenylketonuria (PKU) are reported. The patients belong to three different families — three siblings. Genealogic, clinic and laboratorial studies have been done, including serum phenylalanin. The important aim was to show the normality of the histopathology performed through cerebral biopsy in four cases. Histochemical study has not been done. The normal cerebral biopsy did not show correlation with mental level, electroencephalographic alterations or with cerebral atrophies which were found in two cases by air encephalography. All cases have been treated with special diet (Lofenalac) and some food with low phenylalanin level. After and before the diet phenylalanin determinations have been done, nevertheless, it is very soon indeed to conclude anything by now.

Estudo clínico do RO-5-4023 no tratamento de epilepsias

Seventy six epileptic patients (children and adults) submitted to treatment with RO-5-4023 were studied. RO-5-4023 was administered alone (34 cases) or in association with other antiepileptic drugs. The average dose was 5 mg/day. The cerebrospinal fluid examination was performed in all cases with normal results. In 32 cases the elertoencephalogram was made before and after the treatment. The study allows the authors to draw the following conclusions: 1) the results of therapy were good in 61 cases (81%); 2) the better results were obtained in cases of generalized epilepsy (GM and PM) and in cases of focal non convulsive epilepsy; 3) the results were better when RO-5-4023 was administered in association with other antiepileptics drugs; 4) the electroencephalogram normalized in 46,8% of the 32 cases controled before and after essay; 5) side effects occurred in 32,8% of the cases, consisting of drowsines, ataxia, excitation, fatigue and agressivity; these manifestations disappeared within 10 days after the begining of the treatment.

Hidrocefalia ou hidranencefalia: valor da transiluminação do crânio no diagnóstico diferencial. Estudo anátomo-clínico de dois casos

Report of two anatomical-clinical cases, one being an hydranencephalic and the other of congenital hydrocephaly. The neurological examination showed in both cases a similar picture which characteristics recalling those found in a normal newborn. In both cases a transillumination of the skull and a carotidangiography were performed in order to study the cerebral circulation. The conclusions are as follows: 1) Transillumination has not been safe method for differential diagnosis between hydrocephaly and hydranencephaly, since in case 2 (hydrocephaly) was obtained a clear transillumination whereas in case 1 (hydranencephaly) it was absolutely negative; it must be emphasized that the cranial fluid of the hydranencephalic case was cloudy thus explaining possibly the insuccess of transillumination. 2) No analogy was found between the arterial distribution demonstrated by angiography and the anatomical-pathological findings of the hydranencephalic case, i.e., the anterior and medial cerebral arteries were well visualized, but necropsy showed absence of the portions of the hemispheres that are supplied by those arteries (fronto-parieto-temporal portions); on the contrary the territory supplied by the posterior cerebral artery was quite well developed, but the this artery was reduced to a short, tortuous and slender tuft of vasa. 3) Owing to the impossibility to take EEG tracings in the hydranen-cephalic case it was not possible to evaluate the importance of EEG in the differential diagnosis between hydrocephaly and hydranencephaly.