Resumo em Português:

Os craniofaringiomas, embora raros entre os tumores intracranianos, representam o tipo mais freqüente de neoplasia congênita do sistema nervoso. Os autores referem a constituição e a ulterior destruição parcial do ducto hipofisário, de cujos restos êsses tumores derivam. Expõem as classificações dos craniofaringiomas sob os pontos de vista macroscópico (císticos, semicísticos e sólidos), histopatológico (cistos epiteliais mucosos, adamantinomas e epiteliomas planocelulares) e topográfico (selares, supra-diafragmáticos e intracrânio-selares). A propósito da incidência, ressalta a maior freqüência nas duas primeiras décadas, embora os craniofaringiomas não sejam exclusivos dos jovens. Predominam ligeiramente no sexo masculino. A sintomatologia é estudada de acôrdo com a situação supradiafragmá-tica (manifestações predominantemente hipotálamo-quiasmáticas) ou selar (distúrbios das funções gônado e somatotrófica da hipófise). As alterações radiológicas são de grande valor para o diagnóstico, particularmente as que se revelam no craniograma (calcificações e erosões selares), pneumencefalografia, pneumo e iodoventriculografia, e arteriografia cerebral. No tocante ao tratamento dos craniofaringiomas, os autores referem as controvérsias existentes sôbre os resultados da radioterapia. O tratamento cirúrgico comporta técnicas variadas, desde a punção transesfenoidal (hoje quase abandonada), até a craniotomia frontal ou frontotemporal. O problema da excisão radical é discutido, salientando-se as dificuldades na sua consecução, dadas as aderências do tumor com as vias ópticas na região quiasmática, as artérias regionais e o hipotálamo. A elevada mortalidade operatória é atribuida principalmente à manipulação do hipotálamo e ao colapso hipofisário. É estudado com minúcias o emprêgo do ACTH ou cor-tisona associado à cirurgia. São de prever bons resultados do tratamento com rádio-isótopos. Os autores apresentam 26 casos de craniofaringiomas, 14 supradiafrag-máticos e 12 intracrânio-selares. Foi observada certa correlação entre a idade dos pacientes e a duração da sintomatologia. É estudada detidamente a sintomatologia neurológica (constituída principalmente por paralisias de nervos cranianos e síndromes piramidal e/ou cerebelar), oftalmológica (edema de papila, diminuição da acuidade visual, hemianopsias) e endócrino-hipotalâmica (pan-hipopituitarismo, diabetes insípido, obesidade). São analisados os resultados dos exames radiológicos simples e contrastados, da eletrencefalografia e do líqüido cefalorraqueano. O tratamento cirúrgico (punção do cisto e excisão parcial da cápsula, em 5 casos associado à curetagem intracística) foi instituído em 21 pacientes, tendo sido realizadas, ao todo, 32 intervenções (6 pacientes apresentaram uma ou mais recidivas). Em 10 casos foi associado tratamento pelo ACTH ou cortisona. A mortalidade geral foi de 38,5%. Faleceram 5 dos 20 pacientes operados e com seguimento (mortalidade de 25%). Em 19 casos pôde ser estabelecido o tipo histopatológico da neoplasia: 2 eram adamantinomas, 11 carcinomas planocelulares e 6 do tipo misto. Em 2 outros casos havia caracteres de malignidade.

Resumo em Inglês:

Although craniopharyngiomas are rare intracranial tumors, they constitute the most frequent congenital neoplasms of the nervous system. The authors summarize the development and the further disintegration of the hypophyseal duct, the vestiges of which give rise to the tumor. The classifications of the craniopharyngiomas from the macroscopic (cystic, semi-cystic and solid), histopathological (mucoid epithelial cysts, adamantinomas and squamous cell epitheliomas) and topographic (sellar, supradiaphragmatic and intracraniosellar) standpoints are discussed. The incidence of the growth is higher in the first two decades and in males. The symptomatology is studied according to the site of the tumor, either supradiaphragmatic (hypothalamo-chiasmatic manifestations) or sellar (hypophyseal genital and somatotrophic failure). Radiological changes are very important for the diagnosis, mainly those disclosed by the craniogram (calcifications and sellar erosions), pneumoencephalography, pneumocisternography, air and iodized oil ventriculography, and cerebral angiography. Roentgentherapy of craniopharyngiomas is still a matter of controversy. The surgical management is discussed in detail, the difficulties of the radical excision being emphasized. The high operative mortality rate is ascribed to manipulation of the hypothalamus and to hypophyseal collapse. The association of ACTH and cortisone is advised. Local treatment with radioisotopes seems a promising procedure. The authors report 26 cases of craniopharyngiomas: 14 were supradiaphragmatic and 12 intracraniosellar growths; not one of them was exclusively sellar. Some correlation between age and duration of symptoms is shown. The neurological signs (mainly paralysis of cranial nerves, pyramidal and/or cerebellar syndromes), the ophthalmological manifestations (choked disk, impairment of vision and visual field defects) and the endocrine and hypothalamic disorders (hypopituitarism, diabetes insipidus, obesity) are studied in detail. The results of simple and contrasted radiologic examinations, electroencephalography and cerebrospinal fluid examination are discussed. Twenty-one patients were submitted to surgery (puncture of the cyst and partial excision of the capsule, supplemented in 5 cases by evacuation of the cyst); 32 operations were performed (6 patients had one or more recurrences). In 10 of the most recent cases treatment with ACTH or cortisone was associated. The case mortality rate was 38.5%; there were 5 deaths among 20 patients operated on who could be followed-up (a mortality rate of 25%). The histologic pattern of the tumors was established in 19 cases, including 2 adamantinomas, 11 squamous cell epitheliomas and 6 mixed type craniopharyngiomas. Two other cases displayed malignant features.

Resumo em Inglês:

In the past few years somewhat new yet often differing points of view have been affirmed refering to Petit Mal epilepsy, with regard to its clinical, eletrencephalographic and evolutive aspects. The classical concept of PM epilepsy as established by Lennox and Gibbs, with its well defined clinical and EEGraphic characteristics is continually being modified with new research work on clinical and EEGraphic aspects. Numerous clinical cases have been published whose evolution clashes with that classically described and likewise numerous are those cases where the EEG shows signs of focal electric activity besides spike and wave dis-rythmia. Cases have been known where surgical exerese on the focus causes temporary desappearance of the spike and wave disrythmia. The present concept now holding sway is that PM epilepsy is a kind of reaction of infantile encephalon to diverse harmful causes. From this it will be seen we are very far from the concept that PM epilepsy is a pure form of idiopathic epilepsy. For these reasons we decided to re-examine those cases observed between 1950 and 1960. With this in mind we chose 141 PM epilepsy cases, from among over 23000 epileptics, all examined under a standard criterion by a small group of senior assistants. All cases suspected of Psm epilepsy were excluded. The analysis of this group of patients was compared to that of another group with GM as well as PM seizures (104 patients); in 52 of these cases, studies were made concerning the evolutive, clinical and EEGra-phic aspects. METHOD: Clinical criterion was used for selection of the cases. The results of the analysis from the clinical and EEGraphic point of view were exhibited in demonstrative graphs. From the clinical point of view we strove to find out positive hereditary elements, organic cerebral injury factor (intellectual deficit, mood and behaviour disturbances, neurologic or somatic signs, as well as other development anomalies), the presence of febrile convulsions before the setting in of clinical PM syndrome and the eventuality of abnormal birth. We listed the patients according to their sex and investigated the statement that there is a prevalence of PM seizures among women. We attempted to analyse the incidence of cases according to the patient's age, as well as the incidence regarding the onset age of the illness. With regard to the EEG we likewise attempted to analyse those elements enabling us to weigh up the lesional factors in the brain (abnormalities in the background activity, disrythmias with focal characteristics or other paroxysmal abnormalities of a lesional type) and the bilateral and synchronous paroxysmal disrythmias classically known as forming part of the Petit Mal epileptic picture. Out the group of patients presenting pure PM seizures (141 cases) we obtained a clinical and EEGraphic sequence in 37, during a period of time ranging from 1 to 13 years. Of the group of patients presenting GM seizures as well as PM (104 patients) we investigated, in a comparative manner, 15 cases in a cliincal and EEGraphic sequence, during a period ranging from 1 to 9 years. RESULTS: The analysis of the clinical and electroencephalographic aspects in the first group of patients (141 cases with PM seizures) has enabled us to point out - a) A greater occurrence of this disease between the ages 6 and 12 - though there were 48 patients between the ages of 11 and 20 and 16 patients above 20 years old; b) in the majority of cases the disease set in between the age of 6 and 10 (47 cases); in some cases however the disease started up under two years of age; in 31 patients the onset was after 11 years of age; c) as far as sex distribution, there was no significant difference; d) barely 1 case proved to be abnormal in the somatic examination congenital cardiopathy) ; e) the occurrence of psychic disturbances among patients was extremely low; f) there were no cases with past history of abnormal birth; g) back-ground activity was with in the limits of normality in the great majority of cases; h) incidence of bilateral and synchronous spike and wave 3 c/s, was fairly high; slower disrythmia of this type was present in 4 cases only; i) larval disrythmias by spikes or multiple-spikes and slow ware occurred quite often and appeared both in the mioclonic form of PM as well as in the other forms of the disease; j) diffuse and irregular slow sharp waves, were found in 7 patients and sharp focal waves in 4; these alterations would always seem to appear associated with bilateral and synchronous disrythmias. The analysis of these same elements in the second group (104 patients with PM plus GM seizures) has enabled us to note the following details: a) later age of onset; b) in 5 cases there was child-birth asphyxia; c) higher incidence of cases presenting bilateral and synchronous disrythmias with a frequency below 3 c/s. The development aspects of our patients was not uninteresting. Out of 37 patients with pure PM seizures we noted: a) that the age of onset did not on the whole favour or unfavour the evolution; b) the great majority of cases developed favorably: 18 patients have no more seizures, 11 still present a very minimum number of seizures; 6 later had GM seizures associated; one patient showed a rather serious impairment of his seizures; c) in cases of favorable development,' synchronous disrythmias by spike and wave 3 c/s disappeared, which was not what happened in the group of cases showing unfavorable development; d) total disappearance of disrythmias was only noted in those cases of favorable development; e) there were cases, both among the favorable and unfavorable development groups, where the initial disrythmias perceptibly modified with time and became diffuse sharp waves; f) background activity was normal in the great majority of cases; g) some of our patients developed with clinical fits even over the age of 30. Out of 15 patients with GM plus PM seizures and on whom development studies were made, we noted that the results differed very little from those of the previous group. We should like to point out, however, that disappearance of the spike and wave 3 c/s disrythmias did not occur, even in those cases presenting favorable clinical development. COMMENTS: After discussing the most interesting facts stated in the analysis of their cases and co-relating them to litterature data found, with particular reference to the works presented at the Congress of Brussels by Jung, Bickford, Droogleever-Fortuyn, Gibbs and Walker, the AA have come to the following conclusions: 1) there are clinical and electroence-phalographic indications that the syndrome "Petit Mal Epilepsy" is a clinical manifestation of diverse brain affections; 2) the starting up of the Petit Mal syndrome occurred at an age below 11 years in the majority of cases; however, there are others where the onset occurred after this age and in some cases even above the age of 20; 3) the persistence of Petit Mal clinical syndrome with electroencephalographic manifestations of the complex spike and wave 3 c/s. type can occur in many patients after the age of 15 and up to the age of 40, which fact is in complete disaccord with the classical idea of Petit Mal epilepsy good prognosis after puberty.

Resumo em Português:

Registrando 5 casos de dissinergia cerebelar mioclônica e 3 casos de epilepsia mioclônica os autores fazem revisão de alguns problemas referentes a essas afecções, especialmente no que se refere às mioclonias que são analisadas à luz dos conhecimentos anátomo-patológicos e eletrencefalográficos. Sob o ponto de vista anátomo-patológico é chamada a atenção para o caráter dinâmico das lesões, de forma que, num dado momento, surpreende-se um quadro histológico que pode estar em franca evolução; isso explica a variedade dos achados anátomo-patológicos registrados na literatura, mas sempre com uma base anatômica comum, localizando-se as lesões produtoras de mioclonias no circuito olivodentorrúbrico (triângulo de Guil-lain e Mollaret). Os autores tentam explicar as mioclonias em geral, por lesão de um ou de vários pontos compreendidos nesse circuito, ressaltando a importância, provàvelmente fundamental, da substância reticular do tronco cerebral. Na opinião dos autores, os achados eletrencefalográficos concordam com os dados anátomo-patológicos, no que diz respeito à topografia das lesões. Pelo estudo eletrencefalográfico, durante injeção de substância curari-zante (cloreto de succinilcolina), com conseqüente supressão dos fenômenos musculares, os autores verificaram que essa substância altera a morfologia das descargas disrítmicas e diminui seu número. A diminuição da freqüência das descargas é interpretada pelos autores como podendo resultar de um número maior de estímulos dirigidos aos motoneurônios que ao córtex, partindo de estruturas subcorticais responsáveis pelas mioclonias; durante a ação do cloreto de succinilcolina seriam registrados apenas os estímulos corticais, mais raros. Outra possibilidade aventada pelos autores seria a ação anticonvulsionante do cloreto de succinilcolina, o que ainda não foi demonstrado. Embora ambas as afecções, em certos casos, apresentem um quadro clínico muito semelhante, a histopatologia permite separá-las em entidades clínicas distintas.

Resumo em Inglês:

Five cases of myoclonic cerebellar dyssynergia and 3 cases of myoclonic epilepsy are reported. The authors make a revision of the problem, specially about myoclonic manifestations. Myoclonus is analyzed pathologically and electroencephalographically. From the pathological point of view the dynamic feature of the lesions is emphasized, so that, at a given time, it is possible to have a histological picture that is in progression. This explain the variety of pathological descriptions in the literature, even though all of them have a common anatomical basis, the olivodentorubral triangle being the site of the lesions producing myoclonus. The authors try to explain myoclonus as caused by lesions of one or several points in this triangle, emphasizing the importance of the reticular formation of the brain stem. It is the opinion of the authors that the electroencephalographic patterns agree with the pathological findings in what concerns the site of the lesions. The authors verified that, by making an electroencephalographic record with the aid of a curare-like drug (succynilcholine chloride), which suppresses the muscular component, the morphology of the waves is changed and the frequency of the paroxysms is diminished. The decrease in the frequency of discharges is interpreted as being the result of a greater number of stimuli directed to the motoneurons than to the cerebral cortex, starting in subcortical structures which are responsible for the myoclonus; during the action of succynilcholine chloride only the cortical discharges, which are rarer, would be registered. Or else, this curare-like drug would have an anticonvulsivant action which has not yet been demonstrated. Although both diseases, in some cases, have a very similar clinical picture, it is possible, on histopathological grounds, to regard them as two distinct clinical conditions.

Resumo em Português:

Para o tratamento cirúrgico da trigeminalgia essencial, Frazier e Spiller propuseram, em 1921, a neurotomia retrogasseriana que, progressivamente aperfeiçoada, é ainda usada com resultados bons e permanentes. Esta intervenção, entretanto, comporta inconvenientes sérios, pois dela resultam seqüelas subjetivas (formigamentos) e objetivas (hipoestesia) na face; além disso, se a secção das vias trigeminais se estende a fibrilas correspondentes à região oftálmica, há risco de desenvolvimento de lesões ulcerativas na córnea (queratite neuro-paralítica). Para evitar êstes inconvenientes, outras operações foram propostas: tractotomia bulbar (Sjöqvist), tractotomia peduncular (Walker), descom- pressões do óstio posterior do cavo de Meckel (Taarnhoj), descompressões dos buracos grande oval e grande redondo (Shelden). Infelizmente, no cômputo final, nenhuma delas parece melhor que a operação de Frazier, algumas porque comportam recidiva em alta percentagem (operações des-compressivas), outras porque apresentam grande risco operatório. Recentemente King e col.10, 11, 12 e Crue e Sutin4, em uma série de observações, procuraram reproduzir experimentalmente a trigeminalgia em animais. De suas conclusões pode-se inferir que o quadro doloroso resulta de alterações orgânicas e funcionais dos núcleos sensitivos dos nervos cranianos e das primeiras raízes raquidianas e que as crises são provocadas pelo bombardeio dêstes núcleos por estímulos anormais, originados em processos patológicos periféricos de variada natureza. O nervo trigêmeo, sendo o de maior representação sensitiva na cabeça, é a sede mais freqüente dessa sintomatologia nevrálgica. A cura da moléstia só pode ser obtida mediante a supressão de parte ou de todos os influxos sensitivos que bombardeiam constantemente os núcleos sensitivos centrais. Na nossa experiência, a secção de parte das vias trigeminais (2/3 inferiores do plexo triangular) atrás do gânglio de Gasser, com preservação, portanto, do território oftálmico, é suficiente para a cura definitiva da moléstia, e quiçá de outras nevralgias cérvico-cefálicas. Em apoio desta afirmação apresentamos os resultados imediatos e tardios obtidos em 26 doentes operados no Departamento de Cirurgia da Faculdade de Medicina de Ribeirão Prêto, entre 1956 e 1960.

Resumo em Inglês:

Most surgical procedures proposed for treating the "tic douloureux" present some untoward side-effects. Very often retrogasserian neurotomy leaves behind troublesome losses of sensitivity with disabling sequels as corneal ulcers. Tractotomies are less dangerous, as far as sensitivity is concerned, but unfortunately they require more difficult technical procedures. Decompressive operations upon the "cavum Meckeli" have a very high recurrence rate. The recent experimental investigations performed by King et al. and by Crue and Sutin reinforce the concept that, in order to reduce the paroxystic cranio-facial pains, it is sufficient to relieve a hypothetic internuncial pool, by suppressing important peripheral sensitive impulses. Retrogasserian rhizotomy is the best operation, according to our judgement, but the neural section must always include nothing more than the caudal 2/3 neurofibrils : with this technique our 26 patients obtained improvement, even those painful complaints referred to areas with no correspondence to the projection of the nerves divided.

Resumo em Português:

Em 10 pacientes do sexo feminino, portadoras de hemorragia uterina disfuncional, foi investigada a existência de manifestações epilépticas (clínicas e eletrencefalográficas) : dos 10 casos, 7 apresentavam antecedentes e/ou sintomas de natureza provável ou certamente epilépticos: o eletrencefalograma foi anormal em 5 casos. Foi estudada a ação do Premarin por via intravenosa, na dose de 20 mg, durante registro eletrencefalográfico. Não houve piora do traçado durante ou após a injeção.

Resumo em Inglês:

In 10 patients with functional uterine bleeding, the existence of epileptic manifestations (clinical and electroencephalographic) was investigated: 7 among the 10 cases had a previous history and/or symptoms of a nature that was probably Or certainly epileptic; the electroencephalogram showed abnormalities in 5 cases. The action of intravenous Premarin (20 mg) was studied during electroencephalogram recording. There was no change in the normal electroencephalogram and in the patological ones there was not an increase of the abnormalities.