Rolando A. Tenuto

Localização encefálica da blastomicose sul-americana. Considerações a propósito de 9 casos

The involvement of brain by the Paracoccidioides brasiliensis is rather frequent. Many authors call attention to the difficulty of diagnosis in almost every case, most of them being made through surgery or post mortem examination. The intracranial hypertension syndrome is the most common manifestation, almost always leading to the diagnosis of intracranial tumor. The laboratorial tests, including the spinal fluid examination, are very poor of specific data; the finding of the parasite or a positive test for blastomycosis in this fluid is rarely verified. This disease is only suspected when there are true paracoccidioidal lesions in other organs. Nine cases of South American blastomycosis of brain are reported, seven of which were submitted to surgery. From these seven, three had a fair evolution, beeing the only ones to survive. In the three cases, amphothericin B was used sometime after operation, intravenously or intratecally. The authors are favorable to the surgical management in blastomycosis of brain, whenever reliable localization signs of compression are found. After remotion of the granulomata, amphothericin B must be used in order to avoid recurrence of the disease.

Contribuição ao estudo das malformações occipito-cervical, particularmente da impressão basilar

The authors outline the development of the spine and skull, particularly of axis, atlas and occipital bone. As neuro-skeletal dysmorphisms, the occipito-cervical malformations belong to the neurodysplastic group. They are classified as skeletal anomalies, associated nervous malformations and meningeal reactions. Vertebralization of the occipital bone and occipitalization of atlas, subluxation of odontoid process, dysplasia of the occipital bone, dystrophia brevicollis and other anomalies are discussed. Special care is given to the study of basilar impression; its concept, history, incidence, clinical and neurological symptoms, radiological characterization (craniographic, perimyelographic and iodoventriculographic aspects) and surgical treatment are reviewed. The authors report five cases of occipito-cervical malformations, which are the first references in Brazilian literature. In case 1 the anomalies (manifestation of occipital vertebra and Arnold-Chiari deformity) were disclosed at an operation for cisticercosis of the posterior fossa. In the following four cases invagination of the basilar portion of the occipital bone (basilar impression) could be radiologically demonstrated; in case 2 a suboccipital craniectomy and a laminectomy of atlas and axis were performed but the patient died a week later and the necroscopic examination confirmed the neuro-skeletal anomalies. In all cases there were several associated malformations. In case 2 there were occipitalization of the atlas, fusion of the first and second cervical vertebrae, supernumerary rib of the seventh cervical vertebra, supernumerary lumbar vertebra, and Arnold-Chiari deformity; at necropsy it was found a syringomyelic cyst on the cervical cord and a fibrous dural ring over the foramen magnum. Case 3 showed the syndrome of Klippel-Feil, besides supernumerary ribs of the seventh cervical and first dorsal vertebrae, Arnold-Chiari malfotmation and probable aplasia of cell groups in the nuclei of oculomotor and facial nerves. In case 4 there were occipitalization of atlas and Chiari deformity. In case 5 vertebral reduction in the cervical region was evidenced but bony coalescence could not be proved. In the last four cases the constitutional morphologic type was determined. Electroencephalograms of cases 3, 4 and 5 showed no abnormalities; in case 1 slow bilateral occipital waves were recorded. The authors call attention to the importance of the diagnosis of these anomalies susceptible of successful surgical correction, which can simulate many nervous diseases of untoward course, like lateral amyotrophic sclerosis, syringomyelia (which can be actually associated to the bony anomaly, as in case 2), sclerosis of dorsal funiculi of the spinal cord, tabes, multiple sclerosis, heredocerebellar ataxia, etc. The authors also stress the clinico-radiological differences between basilar impression and platybasia, frequently taken as synonyms. Although both anomalies may coexist (as in cases 4 and 5) their association is not an absolute rule; on the contrary, in case 2 the basal angle was in the inferior limit of normality and in case 3 it was much lower. Emphasis is put on the value of the Chamberlains line and chiefly in the McGregor basal line for the correct diagnosis of basilar invagination of the occipital bone.

Neurocisticercose: formas clínicas pouco frequentes. II - formas do ângulo pontocerebelar

The localization of cysticerci in the lateral recess of the cisterna pontis may bring about a cerebello-pontine angle syndrome, eventually simulating a neurinoma of the nervus acusticus. The authors made a review of such cases reported in the literature. The differential diagnosis with the neurinomas of the auditory nerve is discussed under the clinical and radiological viewpoints. The authors report 7 cases of cysticercosis of the cerebello-pontine angle. The etiologic diagnosis was established in 5 cases by a positive complement fixation test for cysticercosis in the cerebrospinal fluid, and by surgery and/or postmortem examination in 2. All the cases showed symptoms of cochlear involvement, bilateral in 2; in 4 cases there was a vestibular impairment. Signs of unilateral involvement of the trigeminal nerve occurred in 6 cases. Facial nerve paralysis was present in 4 cases. Three patients had pharynx paralysis and 2, internal strabismus. In 3 cases the absence of cerebellar signs would suggest the diagnosis of neurinoma of the nervous acusticus if it were not for the clinical and subsidiary data, which pointed to neurocysticercosis. Signs of intracranial hypertension were evident in 5 patients. Convulsions occurred in 2 cases. Commenting on the results of radiological examination the authors emphasize that, in one case, there was an amputation of the tip of the petrous pyramid ipsilateral to the cerebello-pontine angle syndrome; other data, however, led the authors to reject the hypothesis of a neurinoma of the nervous acusticus, and the surgeon actually recovered a racemose cysticercus from the lateral recess of the cisterna pontis.

Congenital tumors of the nervous system I - Epidermoid and dermoid cysts

The authors study the tumors derived from inclusions of foetal ectodermal tissues, either of Malpighian epithelium (epidermoid cysts) or those also including dermal elements (dermoid cysts). The authors criticize the words cholesteatoma and pearly tumor, often applied to the former growths. The rareness of these cysts among nervous system tumors (particularly spinal epidermoids and diploic dermoids) is stressed. Sluggish development and incidence in youth are clinical features of both. The neurological symptomatology is extremely varied, since these tumors can be found in ?very site of the neuraxis. Some forms show peculiar signs which can point to their etiologic diagnosis. The authors report 12 cases of epidermoid (including 3 of the spinal canal) and 2 cases of dermoid cysts (including one of the diploe). Attention is called to eventual changes of the cerebrospinal fluid that may lead to erroneous diagnosis such as neurosyphilis and cysticercosis of the nervous system. The general rules and results of the surgical management are briefly analysed. In their own cases the authors had three post-operative deaths and three deaths not related to craniotomy (two preoperative and one due to an intervening cause: perforation of gastric ulcer); in the other cases improvement was achieved by surgery.

Cranioplastias e correção de rinoliquorréias com metilmetacrilato: considerações a propósito de 35 casos

The principal methods in the treatment of cranial bony defects are briefly reviewed and some advantages and disadvantages of them are discussed. According to several authors the results obtained with the use of methymethacrylate in cranioplasties are very satisfactory, the complications being unusual. The experience of the authors in 35 cases of skull defects treated with methylmethacrylate is presented: 32 cranioplasties, two corrections of cerebrospinal fluid rhinorrhea and one case in which cranioplasty and blockage of a cranionasal fistula was simultaneously made. The longest follow-up was of four years in only one case; in ten cases it was from one to three years and in the others the pursuing was less than a year. In four cases there was local suppuration, the remotion of the acrylic plate becoming necessary in three of them; in one case the plate was not removed, and the infection still remains. One patient died two months after the cranioplasty in consequence to a brain abscess. In 26 cranioplasties the cosmetic results were satisfactory; only in one case there was a slight displacement of the prothesis. In the three patients with rhinorrhea the drainage of cerebrospinal fluid ceased completely after surgery. In 29 cranioplasties the methylmethacrylate was applied directly into the skull defect without protection of the encephalic tissue; there were no signs of damage of the nervous system in any case. Only in three cranioplasties the Spences technique was used; in this technique the acrylic resin is molded within a polyethylene bag, thus being put in place after polymerization. In the cases of cerebrospinal fluid rhinorrhea the dural injuries were carefully repaired before the blockage of the bony defects with methylmethacrylate.

Gliomas múltiplos do cérebro

Os AA. apresentam um caso de gliomas multiplos do cerebro, com regressao dos sintomas clinicos apos intervencao cirurgica. A localizacao do tumor foi feita exclusivamente pelos dados clinicos. Chamava a atencao o fato de toda area cerebral descoberta pela cranictomia estar ocupada por tres grandes gliomas, que foram facilmente enucleados, e, mais profundamente, tres outros tumores menores. Houve regressao notavel da sintomatologia apos o ato cirurgico. O exame anatomo-patoloico mostrou tratar-se de ependimoma. Os AA. terminam a exposicao do caso tecendo consideracoes de ordem clinica e anatomo-patologica, e admitindo, como ponto de origem dos gliomas no caso em apreco, os germes ependimais deslocados durante o desenvolvimento embrionario, pois os tumores estavam em pleno cortex cerebral, sem conexao com os ventriculos.

Tumores da região pineal: considerações sobre 12 casos

Twelve cases of tumours of the pineal region were analysed under the clinical, laboratorial and radiological viewpoints. In 6 cases the diagnosis was based only on the clinical and radiological (particularly ventriculogramortem examination. The survival from 3 to 9 years, in 4 cases submitted to the conservative treatment including intra or extracranial shunts follow by cobalto-therapy, supports the indication of the procedure adopted.

Metástases extracranianas de tumores meningencefálicos

Os autores apresentam 3 casos de tumores intracranianos com metastases extracranianas; no primeiro caso o quadro clinico e a documentacao histopatologica permitem sua inclusao dentre os casos provavelmente validos. Os outros dois casos sao admitidos como questionavelmente validos.

Tumores congénitos do sistema nervoso II - Craniofaringiomas

Os craniofaringiomas, embora raros entre os tumores intracranianos, representam o tipo mais frequente de neoplasia congenita do sistema nervoso. Os autores referem a constituicao e a ulterior destruicao parcial do ducto hipofisario, de cujos restos esses tumores derivam. Expoem as classificacoes dos craniofaringiomas sob os pontos de vista macroscopico (cisticos, semicisticos e solidos), histopatologico (cistos epiteliais mucosos, adamantinomas e epiteliomas planocelulares) e topografico (selares, supra-diafragmaticos e intracrânio-selares). A proposito da incidencia, ressalta a maior frequencia nas duas primeiras decadas, embora os craniofaringiomas nao sejam exclusivos dos jovens. Predominam ligeiramente no sexo masculino. A sintomatologia e estudada de acordo com a situacao supradiafragma-tica (manifestacoes predominantemente hipotalamo-quiasmaticas) ou selar (disturbios das funcoes gonado e somatotrofica da hipofise). As alteracoes radiologicas sao de grande valor para o diagnostico, particularmente as que se revelam no craniograma (calcificacoes e erosoes selares), pneumencefalografia, pneumo e iodoventriculografia, e arteriografia cerebral. No tocante ao tratamento dos craniofaringiomas, os autores referem as controversias existentes sobre os resultados da radioterapia. O tratamento cirurgico comporta tecnicas variadas, desde a puncao transesfenoidal (hoje quase abandonada), ate a craniotomia frontal ou frontotemporal. O problema da excisao radical e discutido, salientando-se as dificuldades na sua consecucao, dadas as aderencias do tumor com as vias opticas na regiao quiasmatica, as arterias regionais e o hipotalamo. A elevada mortalidade operatoria e atribuida principalmente a manipulacao do hipotalamo e ao colapso hipofisario. E estudado com minucias o emprego do ACTH ou cor-tisona associado a cirurgia. Sao de prever bons resultados do tratamento com radio-isotopos. Os autores apresentam 26 casos de craniofaringiomas, 14 supradiafrag-maticos e 12 intracrânio-selares. Foi observada certa correlacao entre a idade dos pacientes e a duracao da sintomatologia. E estudada detidamente a sintomatologia neurologica (constituida principalmente por paralisias de nervos cranianos e sindromes piramidal e/ou cerebelar), oftalmologica (edema de papila, diminuicao da acuidade visual, hemianopsias) e endocrino-hipotalâmica (pan-hipopituitarismo, diabetes insipido, obesidade). Sao analisados os resultados dos exames radiologicos simples e contrastados, da eletrencefalografia e do liquido cefalorraqueano. O tratamento cirurgico (puncao do cisto e excisao parcial da capsula, em 5 casos associado a curetagem intracistica) foi instituido em 21 pacientes, tendo sido realizadas, ao todo, 32 intervencoes (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 pode ser estabelecido o tipo histopatologico da neoplasia: 2 eram adamantinomas, 11 carcinomas planocelulares e 6 do tipo misto. Em 2 outros casos havia caracteres de malignidade.

Lipomas intradurais raqueanos: Considerações sôbre 4 casos operados

Four cases of spinal intradural lipomas are reported. The rarity of these tumors is confirmed by the small number of cases reported in the medical literature, represented by 1 or 2% of the spinal tumors. In the Neurological Clinic of the University of Sao Paulo Medical School, in a total of 165 intraspinal tumors which were operated on between 1945 to 1964, the incidence was 2.4%. The clinical and histopathological data, as well as the paraclinical tests, are analysed. The surgical technique is discussed, the authors drawing the conclusion that the results are poorly rewarding.