Resumo em Português:

E feita uma tentativa de relacionar os modernos conhecimentos da expressão genômica do sistema nervoso (SN) no homem, não sendo intensão deste artigo abordar os aspectos da evolução embrionário-fetal do SN do homem, e sim, enfocar o "equipamento neurológico" com o qual nascemos e como evolvemos para atingir a maturação. São, então, abordados os temas maturação e aprendizagem. Na maturação são ressaltados os aspectos de função e estrutura e, portanto, de mielinização do SN; no segundo aspecto - aprendizagem - são abordadas as várias teorias, dando-se ênfase à teoria do condicionamento, baseada em Pavlov. Após a revisão destas bases, o neurofenótipo da criança é dividido em 3 tipos: manifestações permanentes, manifestações reflexas transitórias e manifestações reflexas evolutivas. O neurofenótipo do RN normal se caracteriza por: 1 - atitude assimétrica tendendo ao padrão do reflexo tônico-cervical assimétrico; 2 - hipertonia generalizada em flexão dos 4 membros, contrastando com a hipotonia da musculatura paravertebral; 3 - presença, em 100% dos RN, dos reflexos miotáticos: patelares, adutores, tricipitais e naso-palpebrais, sendo obrigatória a simetria das respostas; 4 - reflexo cutâneo-plantar em extensão do hálux, com ou sem leque em 100% dos RN; 5 - atividades automáticas ou reflexas presentes em 100% dos RN; reflexo tônico-cervical assimétrico (fragmentado em 70%: completo em 30%), marcha reflexa, apoio plantar automático, sucção; 6 - reflexo dos "olhos de boneca" em 100%; 7 - reflexo fotomotor em 100%. A seguir são apresentadas as fases evolutivas do lactente normal separadas em 5 idades-chaves. Finalmente são apresentadas as conclusões do Exame Neurológico Evolutivo (ENE) do grupo de Lefèvre que mostra importantes correlações entre a maturação e certas funções, principalmente no que diz respeito, à sensibilidade pro-prioceptiva e às sensibilidades gnósicas.

Resumo em Inglês:

An attempt to the modern knowledge of the genomic expression of the nervous system (NS) is done but the authors do not want do demonstrate the evolutive espeçts of the embrionary-fetal development of the NS of man, but stress the "neurological equipament" which we were born with and how we get to maturity. Maturation and learning are focalized. In the subject of maturation the aspects of function and structure are shown. As to the second aspect - learning - many theories, are reviewed, being emphasized Pavlov's condicio-nement. After the revision of these basis the neurophenotype of children is divided in 3 types: 1) permanent manifestations with which the children are born and that are constant and practically do not modificate during their evolution (uncondicionated reflexes and the primitive sensibilities); 2) transitory reflex manifestations are archaic or reflex manifestations which disappear with the evolution returning just in pathological situations (tonic-neck assymetric reflex of Magnus-De Kleijn, Moro's reflex, Laudau's reflex and cutaneous-plantar in extension); 3) evolutive reflex manifestations - with which children are born, reflexes or automatics in the beginning, and which disappear with the evolution being replaced by the same activities, although in voluntary character, passing or not through a nitid inhibiting phase; lately some of these activities are automatized constituting in secondary automatisms (suction, prehension, automatic march, reflex of swimming). Myra y Lopez interpretate these functions in his terminology as "deflexes". The neurophenotype of the normal term NB is characterized by the following items, according to Lefèvre and Diament's works: 1 - Asymmetric attitude determined by the asymmetric tonic-neck reflex pattern; 2 - generalized hypertonia of the flexor muscles of the 4 limbs and of the thigh adductors; 3 - more frequent myotatic reflexes: patellar, adductor, tricipital and naso-palpebral, being necessary the symmetry of the responses; 4 - the plantar cutaneous reflex in extension of the hallux with or without spreading of the other toes present in 100% of the RN; 5 - automatic or reflex activities present in 100% of the RN; assymetric tonict-neck reflex (fragmentated in 70%, complete in 30%), reflex walking, automatic plantar support, suction; 6 - reflex of the "doll's eye" in 100%; 7 - photomotor reflex in 100%. The key-ages of the neurological evolution of the normal lactent determinated by us in 1967, are also expression of the normal neurophenotype and may be summarized as follows; A-1) from the RN until the end of the lst month: Vocal expression: inarticulated cry; initiation of the lallaction after the 1st month. Posture: asymmetric attitude, with or without asymmetric tonic-neck reflex: general hypertonia in flexion; hypotonia of the paravertebral muscles. Myotatic and superficial reflexes: more liverliness myotatic reflexes; the plantar-cutaneous reflex in extension of the hallux with or without spreading of the other toes: Primitive motor manifestations: the more frequent in the NB of normal term are suction, grasping of fingers and toes, Magnus-De Kleijn's TNR pattern, Moro's, walking reflex and plantar support. Ocular reflexes: photomotor reflex and doll's-eye proof. A-2) from the beginning of the 2nd month until the end of the third: Vocal expression: evident lallation. Posture: loss of asymmetric attitude; beginning of the symmetric attitude, head supine, recumbent position; beginning of complete and uncomplete upholding of the head; persistence of the hypertonia; general flexor hypertonia, although less pronounced. Superficial and myotatic reflexes: reduction of the liveliness of myotatic reflexes; persistence of the plantar-cutaneous reflex in extension of the hallux with or without spreading of the hallux. Archaic reflexes: persistence of the following primitive motor manifestations; suction, prehension of fingers and toes, Moro's plantar support and walking reflex; loss of Magnus-De Kleijn's reflex; beginning of higher incidence of Landau's reflex (I and II). Ocular reflexes: loss of answer to the doll's-eye proof from the end of the 3rd month on. A-3) from the beginning of the 4th month until the end of the 6th month: Vocal expression: estabilization of the lallation. Posture: beginning of the attitude 3 (or change of decubitus) and of the attitude 4 (change of position from laying to sitting); complete upholding of the head was observed in all examined children at the end of the 4th month; start sitting with support; beginning of the orthostatic position with support; reduction of the hypertonia in flexion. Locomotion: starting the voluntary walking with support in the 5th month. Coordenation; estabilization of the voluntary palmar prehension; beginning of the hand-handkerchief and hand-object coordination; beginning of the pincer's prehension in the 6th month. Superficial reflexes: beginning of cutaneous-plantar reflex in inconstant extension of the hallux. Archaic reflexes: loss of Moro's reflex not complete until the end of the 6th month; loss of the palmar prehension reflex until the beginning of the 6th month; loss of the plantar support until the beginning of the 5th month; loss of the walking reflex until the end of the 4th month; estabilization of the Landau's reflex I and II. Ocular reflexes: following the light stimulus from several direction in 100% of the children up to the 5th month. A-4) beginning at the 7th until the end of the 9th month: Vocal expression: beginning of the first words and phrase-words at the 8th month; gradual loss of lallation. Posture: spontaneous attitude related with the decubite (attitude 4); sitting without support in 100% from the 9th month on; evolution of the orthostatic position with support; stabilization of the normal tonus for the age or "phisiological hypotonia"; loss of hypertonia on generalized flexion of limbs starting in the beginning of the 7th month. Locomotion: beginning of crawling starting in the 8th month; beginning of walking with support. Coordenation: stabilization of the coordination hand-handkerchief on the face and hand-object; stabilization of prehension in pincers until the end of the 9th month. Superficial reflexes: stabilization of the plantar-cutaneous reflex in inconstant extension of the hallux and beginning of the answer in flexion in the 9th month. A-5) from the beginning of the 10th month until the 12th: Vocal expression: diseppearance of the lallaction at the end of the 11th month; predominance of the first words. Posture: spontaneous attitude (nº 4) respecting decubits; stabilization of the orthostatic position with support; beginning of the orthostatic position without support at the 11th month; predominance of "physiological hypotonia". Locomotion: stabilization of crawling; stabilization of walking with support; beginning of walking without support in the 11th month. Superficial reflexes: predominance of the plantar-cutaneous reflex in flexion at the 12th month. Archaic reflexes: disappearance of prehension reflex in the toes at 12 months; disappearance of palmar prehension at the end of the 11th month; stabilization of the prehension in pincers. Finally the conclusions of the Evolutive Neurological Examination of the group of Lefèvre showing important correlations between the maturation and certain functions, mostly regarding the proprioceptive and gnostic sensibilities are reported.

Resumo em Português:

Os autores baseiam o estudo do presente trabalho em 106 casos de tumores intracranianos em crianças, diagnosticadas e tratadas nos hospitais Santo Antonio e Beneficência Portuguesa de Porto Alegre no período de janeiro de 1962 a 1977. Comentam os achados clínicos e as relações entre a sede preferencial, idade, sexo e os vários vários tipos de neoplasias. Destacam a presença de tumores antes de um ano de idade.

Resumo em Inglês:

The authors analyze 106 intracranial tumors occurring in patients from 0 to 15 years of age. This study was carried on during the period from 1962 up to 1977. In 85 cases the diagnostic work up was made on clinical, radiological, and histological grounds; the remaining 21 cases had their diagnosis based solely on clinical and radiological evidence. Observations involving incidence according to age, sex, localization and histological types are made. The signs and symptoms and supplementary examination are discussed. The frequency of primary intracranial tumors in children under 1 Year of age is stressed.

Resumo em Português:

Foi estudado no soro de pacientes com síndrome de Down e suas respectivas mães, a presença de anticorpos antitireóide (antitireoglobulina e antimicrossomal), tendo em vista dados de literatura mostrando uma eventual correlação entre a presença destes anticorpos, no soro de mães com filhos, mongolóides. Os anticorpos antitireoideanos foram dosados em 40 pacientes com síndrome de Down, cuja idades variavam 5 meses a 21 anos, sendo 24 masculinos e 16 femininos e, nas respectivas mães, cujas idades variaram de 22 a 66 anos de idade. Em nenhum dos soros analisados, quer dos mongolóides bem como de suas respectivas mães, conseguiu-se detectar os anticorpos antitireoideanos estudados.

Resumo em Inglês:

The presence of antithyroid antibodies (antithyroglobulin and antimicrosomal) in the serum of patients with Down's syndrome and their respective mothers were studied based on data from the literature, showing a possible correlation between the presence of these antibodies in the serum of mothers and the birth of mongoloid children. Antithyroid antibodies were studied in 40 patients with Down's syndrome, with ages ranging from 5 months to 21 years old, 24 males and 16 females and in the respective mothers whose ages varied from 22 to 66 years. In none of the analyzed sera of the mongoloids as well of their mothers, the authors were able to detect the antithyroid antibodies studied.

Resumo em Espanhol:

Fueron estudiados 80 pacientes portadores de la forma crónica de la enfermedad de Chagas mediante el empleo de técnicas convencionales de electromiografia de detección. Los hallazgos evidenciaron en el 79% de los pacientes signos de denervación antigua tanto en miembros superiores como inferiores, aúnque con predominio en estos últimos. En ningún caso hubo manifestacionrs clínicas concomitantes.

Resumo em Inglês:

An electromyographical investigation of 80 patients with chronic Chagas' disease was made. It was found that 79% of the studied patients had EMG manifestations of old and chronic denervation of the upper and lower limbs without clinical features of nervous system involvement.

Resumo em Espanhol:

90 pacientes afectados por enfermedad de Chagas crónica fueron estudiados electrofisiológicamente procediéndose a cuantificar Ias unidades motoras actuantes en los músculos de la eminencia tenar, eminencia hipotenar, soleo y extensor corto de los dedos. Se halló que muchos de ellos poseían un número reducido de unidades, en tanto que buena parte de las sobrevivientes mostraban su tamaño aumentado. No se encontró alteración en los valores de velocidad. de conducción nerviosa motora o sensitiva ni tampouco en las cifras de latencia residual motora. La respuesta muscular al estímulo repetitivo del nervio fué igualmente normal. Los hallazgos hechos sugirieron que las alteraciones musculares halladas en estos pacientes pueden, obedecer a la existencia de un defecto primario en el soma de la motoneurona alfa medular.

Resumo em Inglês:

An electrophysiological study has been made of the thenar, hypothenar, soleus and extensor digitorum brevis muscles and their inervation in 90 patients with chronic Chagas' disease. Some of them showed a reduced number of functional motor units with increased size of many of the surviving units. No decremental muscle response was found to repetitive nerve stimulation. Motor and sensory conduction velocities as well as motor terminal latencies were on the normal range. These findings suggested that the muscle changes resulted from a primary defect of the alpha spinal motoneurone soma.

Resumo em Espanhol:

En 7 pacientes afectados por enfermedad de Chagas crónica se procedió al estúdio biópsico del músculo gastrocnemio. Los pacientes seleccionados mostraran como única anormalidad la presencia de tests positivos en suero para la enfermedad de Chagas. Agrupamiento de fibras musculares de tipo I y II fué observado en 5 de aquellos; en 2 de estos últimos 5 se encontro, además, fibras anguladas que se teñian intensamente con NADH y esterase no especifica. Las observaciones hechas sugirieron la presencia de denervaçión seguida por reinervación, cuadro que puede ser observado con asiduidad en el músculo esquelético de pacientes con afecciones denervatorias bien compensadas y en los que no existen evidencias clinicas de ese compromiso.

Resumo em Inglês:

Gastrocnemius muscle biopsies were performed in 7 subjects with chronic Chagas' disease. On clinical and laboratory grounds the selected patients were judged to be healthy, being the only abnormality found the presence of positive serum tests for Chagas' disease. Fibre type grouping of either type I or type II was observed in 5 of the 7 patients. Furthermore, in 2 of the 5 patients showing muscle fibre groupings, angular fibres reacting with NADH and non-specific sterase were also found. These observations strongly suggest denervation associated with reinervation. This picture often can be observed in the skeletal muscle of patients with well compensated denervatory conditions who do not show clinical evidences of denervation.

Resumo em Português:

No estudo de 38 encéfalos de indivíduos que faleceram com leucemias. verificou-se que a infiltração neoplásica apareceu como lesão mais frequente (70,5%), seguida de hemorragia intracraneana (48,5%). O local mais frequente da infiltração leucêmica foi o espaço subaracnoideano (79,1%), seguido dos plexos coróides numa frequência de 57,8% que corresponde ao envolvimento de 11 dos 19 casos que foram submetidos a estudo histológico, e, por fim. a infiltração do tecido nervoso (50%). Esta infiltração neoplásica do SNC e principalmente o envolvimento dos plexos coróides, que aparece também independente de infiltração meníngea, frequentemente decorre sem manifestações neurológicas. Isto chama atenção para a necessidade do estudo citológico do líquido cefalo-raquidiano de rotina nos casos de leucemias, para melhor orientação terapêutica, visto que o SNC é local de difícil penetração de quimioterápicos administrados por via sistêmica e pode constituir foco de reativação da doença mesmo na fase de remissão terapêutica.

Resumo em Inglês:

In the study of 38 cases of leukemia, neoplastic infiltration of the brain was the most frequent lesion, occuring in 70,5%, compared to 48,5% for hemorrhage. The leptomeninges were the most frequent site of leukemic infiltration followed by the choroid plexus (57,8%) and the nervous tissue (50%). In few cases leukemic infiltration of choroid plexus was seen in the absence of meningeal involvement. Very often the leukemic infiltration of the CNS courses without clinical manifestations. The paper points out the importance of the cytologic study of the cerebro-spinal fluid as a routine procedure in cases of leukemia, since it is well known that the therapeutic agents have difficulty in penetrating the blood-brain barrier and that foci of CNS leukemic infiltration may represent points of reactivation of the disease even during the therapeutic remission.

Resumo em Português:

A síndrome de Lennox-Gastaut, descrita como variante do pequeno mal em 1950 por Lennox e Gibbs, vem merecendo, desde então, interesse de diversos autores sobre os aspectos clínicos e eletrencefalográficos. Tem sido considerado como difícil o problema terapêutico desta síndrome. Não querendo propor medicação ideal para esta condição, os autores apresentam, neste trabalho, resultados satisfatórios com o uso do barbexaclone. Mostrando a eficácia terapêutica, são considerados como parâmetros as manifestações convulsivas, as alterações psíquicas e os dados eletrencefalográficos.

Resumo em Inglês:

The Lennox-Gastaut syndrome has been a source of interest for many workers both from a clinical and electroencephalographic view point. Treatment has always posed problems. Without suggesting an ideal medication for this syndrome, the authors have obtained good results with barbexaclone. Taking into consideration the convulsive manifestations, psychic changes and electroencephalographic details as parameters the therapeutic efficacy of this drug was studied in seven cases.

Resumo em Português:

Relato de um caso de astrocitoma grau II com metástase medular ao nível da terceira vértebra dorsal. O nódulo intradural determinou sinais e sintomas de compressão medular. O diagnóstico clínico foi confirmado por mielografia descendente com lipiodol e mielocintilografia com I131 - albumina. A paciente foi submetida a laminectomia para exerese da metástase. A revisão da literatura mostra que o fator mais importante para a ocorrência das metástase dos tumores encefálicos é a invasão do sistema ventricular e/ou espaço subaracnóideo periencefálico pela neoplasia, sem relação com o ato operatório.

Resumo em Inglês:

A case of a right frontal astrocytoma with spinal metastatic lesion in the region of the third dorsal vertebra is reported. The metastatic nodule was removed six months after the craniotomy. In the literature concerning to the dissemination of tumors cells is suggested that there is not a causal relationship between CSF seeding and operative intervention. Acess to the ventricular system or basal cisterns is of primary importance in the production of metastases.

Resumo em Português:

É relatado o caso de um paciente de 6 meses de idade, de cor parda, internado por gastroenterocolite aguda, desnutrição e desidratação. Apesar do tratamento, a criança apresentou septicemia, com picos febris de até 38,6ºC, vindo a óbito no 12º dia de internação. Na necrópsia, além de broncopneumonia bilateral, observou-se hemorragia subaracnóidea fronto-parietal bilateral e extensa necrose recente da face inferior de ambos hemisférios cerebelares. Estes achados neuropatológicos foram clinicamente silenciosos. Ao exame histopatológico, nas áreas de necrose cerebelar, observaram-se numerosas hemácias falcizadas em hemorragias petequiais e, em menor quantidade, no interior de capilares. O interesse do presente caso prende-se à extrema raridade das necroses cerebelares na anemia falciforme. O mecanismo patogenético da necrose não ficou claro visto que não foram encontradas tromboses em artérias ou capilares. Provavelmente atuaram fatores coadjuvantes, tais como o estado infeccioso grave e a febre, além da anóxia provocada pela broncopneumonia.

Resumo em Inglês:

A 6 month-old mulatto boy was admitted on account of acute gastroenteritis, malnutrition and dehydration. In the hospital, the child developed septicemia, and temperature reached up to 38.6°C. Despite intensive antibiotic treatment, the patient died 12 days after admission. Necropsy disclosed bilateral bronchopneumonia, bilateral fronto-parietal subarachnoid hemorrhage, and extensive necrosis of the inferior half of both cerebellar hemispheres. On histopathological examination of the necrotic cerebellar cortex, numerous sickled erythrocytes were observed in petechial hemorrhages and, in lesser quantities, inside capillaries. Lesions of the central nervous system in sickle cell anemia most often involve the cerebral cortex, and a single extensive cerebellar infarction as present in this case seems extremely rare. The pathogenetic mechanism of the necrosis is unclear, since thrombosis was not observed either in large blood vessels or in capillaries. Possible contributory factors were the infectious condition (septicemia), fever, and anoxia caused by the extensive bronchopneumonia.

Resumo em Português:

É relatado o caso de paciente feminina de 65 anos de idade que apresentou sintomatologia neurológica com evolução de um mês, com progressão rápida para a morte. A autópsia demonstrou a presença de metástases cerebrais, múltiplas, cerebelares, de tronco cerebral e de hipófise, bem como metástases para a glândula tireóide e supra-renais. Os autores discutem a frequência de metástases na hipófise, especialmente nos casos de carcinoma de mama. Além disso tecem considerações a respeito da localização da metástase na hipófise e seu aspecto macro e microscópico.

Resumo em Inglês:

The case of a 65 old woman who had a rapidly progressive neurologic symptomatology is reported. The autopsy disclosed a carcinoma of the breast with metastases in brain, cerebellum, brainstem, pituitary, tyreoid gland and the adrenals. The frequency of the pituitary metastases is discussed, especially in cases of breast cancer. The macro and microscopic aspects of the metastatic pituitary gland are studied.

Resumo em Português:

É relatado o caso de uma criança de 12 anos de idade apresentando quadro de hipertensão endocraniana e síndrome cerebelar, cujos exames neurológico e neuroradiológicos foram sugestivos de neoplasia de fossa posterior. A cintilografia cerebral mostrou um quadro compatível com a existência de tumor da fossa posterior, da linha mediana, mais provavelmente meduloblastoma ou astrocitoma. Pela intervenção cirúrgica foi verificado tratar-se de cisticercose, sendo removido um cisto do 4ºventrícuio. Os autores sugerem que em áreas geográficas com alta prevalência de neurocisticercose na população infantil a hipótese da forma pseudotumoral seja lembrada, quando da tentativa de caracterização do tipo de lesão da fossa posterior, detectada pela cintilografia cerebral.

Resumo em Inglês:

The case of a twelve year old child with intracerebral hypertension and cerebellar syndrome in whom the neuroradiological and neurological examinations were sugestive of posterior cranial fossa tumor is reported. The brain scan depicted the scintigraphic pattern of midline neoplasms, most likely medullo-blastoma or astrocytoma. At surgery a Cysticercus cellulosae was removed from the fourth the fourth ventricle. The authors suggest that in geographical areas with high prevalence of neurocysticercosis in the infantile population the hypothesis of the pseudotumoral form of the disease be remembered when one attempts to characterize the type of neoplasms of the posterior fossa as detected by brain scan.