Maria Irmina Valente

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.

Distúrbios neuropsiquiátricos por lesões bilaterais do lobo temporal: síndrome de Klüver e Bucy

Clinical and paraclinical study of a 4 years old child, female, who, after an acute encephalopathy process of unknown etiology, presented neuro-psyciatric syndrome similiar to these described by Kluver and Bucy in monkeys, after bilateral temporal lobectomy. The clinical picture was characterized by hipermetamorphosis, oral tendency, psychic blindness, with preservation of visual acuity and, apparently, of the visual fields, complete aphasia, absence of reaction to auditive and olfactory stimuli, absence of tactiles participation in object recognition and considerable emotional changes (there were no fear or anger reactions and no smiling or laughing were noticed). The patient was observed over a period of 7 months, during which the symptoms improved slightly. Pneumo-ventriculography, pneumocysternography and isotopoencephalometry showed that the fronto-temporal region was bilaterally affected. This finding, however, could not make clear the nature of the lesions and explain whether they resulted from lesions of the present disease or whether they were due to a brain disgenesy, since the patient had convulsions since her second year of age. In view of the importance of this case, some of the main psychopatholo-gical symptoms were commented in relation to brain physiopatological aspects.

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.

Desenvolvimento neuropsicomotor da criança desnutrida: II subnutrição

The cases of 5 children with severe malnutrition, marasmic type, aged four to fourteen months are reported. The patients were sturied at admission in the hospital and 45-60 days and 4-5 months later. Eeach study included: hemoglobinemia, cholesterolemia, glycemia, weight, height, Gesell test, neurological examination, pneumoencephalogram, electroencephalogram and cerebrospinal fluid examination. Biochemical and physical recuperation was very good. Gesell test show an important and homogeneous deficiency, even at the third study. All patients presented cortical atrophy at the pneumoencephalogram in the first study: 4 patients were re-studied 4-5 months later and only one become normal. No changes in electroencephalogram and cerebrospinal fluid were found. It was not possible to evaluate the significancy of the poor psychosocial environment from where the patients came in the aetiology of the mental deficiency.

Skeletal age in children with cerebral palsy

The skeletal age of thirty children with cerebral palsy was studied and related to the chronologic age, height and weight. In 10 cases the thyroid function was evaluated. The results allow the following suggestions: 1) the skeletal age and the somatic development were proportionally delayed and both probably related to the poor nutritional state of the children; 2) the proportionality between skeletal age and weight among the male children, and between the skeletal age and weight as well as height among the female children, were altered; 3) the disorganizing action of the cerebral palsy should not depend on hypothyroidism; 4) the noted alterations are more conspicuous among the male children.

Tromboflebite intracraniana em criança: confirmação diagnóstica pela sinugrafia

E. F., branca, brasileira, do sexo feminino, com 30 meses de idade, residente em Santos. Antecedentes familiares normais; nascimento e evolução neuro-psico-motora normais; como antecedentes mórbidos, apenas amigdalites freqüentes. Nos primeiros dias de dezembro de 1954 a criança começou a ficar sonolenta, reduzindo sua atividade habitual, preferindo manter-se deitada, sem apresentar qualquer sintoma mórbido; em 8-12 teve hipertermia. Exame médico feito nessa ocasião teria revelado amigdalite e otite agudas, medicadas com antibióticos. A o fim de 3 dias, não tinha mais hipertermia, porém apresentava nítido déficit motor em ambos os membros inferiores e afasia de expressão sem sinais de comprometimento do componente perceptive A paciente ficou intensamente prostrada, porém não chegou a perder a consciência; tinha cefaléia, porém não teve vômitos nem convulsões. Com esse quadro foi internada na Santa Casa de Santos, onde permaneceu de 15-12-54 até 13-155, ocasião em que foi transferida para a Clínica Neurológica do Hospital das Clínicas (reg. 394.635). O exame por ocasião da internação mostrou: criança em bom estado nutritivo, apresentando furunculose na região cervical e couro cabeludo, hiperemia do orofaringe, sem hipertermia e sem outras anormalidades ao exame físico geral.

Estado atual do gargulismo

The authors relate the recent advances on the diagnosis of gargoylism emphasizing the value of biochemic and hematologic tests. Four cases are presented. The first one was considered as gargoylism plus hypothyroidism before employment of the recent methods of diagnosis; afterwards however it showed to be hypothyreoidism only. In the second case it was impossible to distinguish whether it was gargoylism or Nieman-Pick disease, as the Dorfman-Steiness test was not then available. The other two were actually gargoylism confirmed by laboratory tests. The authors enhance the importance of the citochemical blood analysis and the Dorfman-Steiness test.

Síndrome de Hunter-Hurler (gargoilismo): apresentação de um caso com estudo eletroforético

Case report of a Hunter-Hurler syndrome (gargoylism). The diagnosis was based on the clinical picture, although the grotesque facies, one of the main characteristics, was missing. The presence of typical vertebral alterations and the milk diffused opacity of the cornea, are sufficient to assure the diagnosis. The electrophoretic analysis of the serum proteins do not allow the authors to make definite statements because only one case was studied; however they point out the fact, considered very important by them, that there was a lipoprotein without electrophoretic mobility which indicating the presence of an instable lipoprotein complex in the blood, may suggest the possibility of lipoid deposit formation in the cells.