Foi avaliada a evolução neurológica, sob tratamento, de 76 casos de degeneração hepatolenticular. Dos 76 casos, 59 (77,6%) apresentariam melhora do quadro neurológico ou mantiveram-se assintomáticos, 8 (10,6%) permaneceram inalterados e 9 (11,8%) pioraram. Os principais fatores relacionados a resultados insatisfatórios foram: duração de tratamento insuficiente para permitir reversão de distúrbios neurológicos, uso irregular da medicação e predomínio de manifestações distônicas no quadro neurológico.

A series of 76 patients with hepatolenticular degeneration (HLD) followed up at the Hospital of the University of São Paulo Medical School between 1963 and 1988 have been studied focusing the evolution under treatment of neurological features. The assessment of neurological symptoms included 12. specific items, and it was made by a scoring system ranging from 0 to 3 (absent, mild, moderate and severe). Global score varied between 0 to 36. A functional disability scale (0, normal; 1, mild; 2, moderate; 3, severe) was also adopted. All patients received D-penicillamine but three of them developed serious renal toxic side-effects, and this drug was replaced by zinc (sulphate or acetate). Fifty-nine patients (77.6%) improved, 8 (10.6%) remained unchanged, and 9 (11.8%) got worse. The main features related to bad results were poor compliance, predominance of dystonic symptoms, and short time treatment (death due hepatic complications). Eight patients (10.5%) developed transient worsening of neurological symptoms during the first weeks of treatment. Three patients who have D-penicillamine replaced by zinc salts presented satisfactory evolution. From them, only one had neurological symptoms. Death due to infectious complications related to severe motor disability occurred in three patients, representing only 10.7% of fatal evolution.

As concentrações de Cu, ceruloplamina, Mg e S no soro de 8 pacientes com meningioma foram significantemente mais elevadas que os níveis normais. Os teores de Ca, P, Cu, Mg, Na e K no próprio tumor dos mesmos pacientes e de um outro foram comparados com as concentrações na dura máter normal do cérebro humano. Os valores de todos os elementos foram significantemente mais altos no tecido tumoral, exceto o conteúdo médio de Ca em 6 meningiomas meningoteliomatosos ou fibroblásticos, quando este grupo foi estudado à parte. Os tumores psamomatosos mostraram maiores concentrações de Ca, Cu e Mg que os outros tipos histológicos. Não foi verificada correlação significante entre as concentrações dos elementos no tecido neoplásico e a duração dos sintomas ou o volume do tumor, exceto uma correlação negativa entre os níveis de Cu e o volume do tumor.

The concentrations of Cu, ceruloplasmin, Mg and S in the blood serum of eight patients with meningioma were significantly higher than the normal levels. Ca, P, Cu, Mg, Na and K contents in the tumour itself of the same patients plus one were compared with the concentrations in the normal dura mater of human brain. The contents of all elements were significantly increased, except the mean Ca in six meningotheliomatous or fibroblastic meningiomas when this group was studied apart. The psammomatous tumours had higher concentrations of Ca, Cu and Mg than the other histologic types. No significant correlation was found between the concentrations of the element in the neoplastic tissue and the duration of symptoms or the tumour size, except a negative correlation between Cu levels and tumour size.

São apresentados 31 casos de leucoencefalite esclerosante subaguda, cujo diagnóstico se baseou no quadro clínico, no acentuado aumento das globulinas y no líquido cefalorraqueano, nas características alterações do eletrencefalograma e no estudo anátomo-patológico. Foram encontrados corpúsculos de inclusão intracelular mediante o estudo necroscópico de 8 entre 13 casos e o exame histopatológico de 1 entre 7 biopsias cerebrais. A média etária foi de 11,7 anos, com os limites de 3 a 22 anos. O sexo masculino predominou nitidamente sobre o feminino (23:8). Os pacientes provenientes da zona rural constituíram a maioria dos casos (77,4%). A análise da distribuição geográfica não demonstrou predomínio regional definido. Não foi observada influência estacional sôbre o início da moléstia. Em 16 casos acompanhados até o óbito, a duração média foi de 7 meses, com uma faixa de 2 a 20 meses. No quadro clínico sobressaiu a presença eonstante de mioclonias ou perdas de tono, e de alterações psíquicas. O primeiro sintoma foi representado por distúrbios psíquicos em 55,6% dos pacientes, por mioclonias em 35,5%o e por convulsões do tipo grande mal em 33,3%. Rigidez e sinais piramidais foram muito freqüentes, sendo rara a síndrome cerebelar. Alterações oftalmoscópicas (edema ou palidez de papila, coroidite) foram verificadas em um terço dos casos. Em 2 casos o início da moléstia coincidiu com uma epizootia de galinhas. Os valores do cobre sangüíneo, determinados em 8 casos, situaram-se sempre acima da faixa normal, enquanto a ceruloplasmina permaneceu dentro dos limites normais. Os valores do enxofre sangüíneo revelaram-se elevados em 3 pacientes examinados. Os níveis da magnesemia mostraram-se variáveis em 6 pacientes estudados.

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.

Foi determinada a atividade cobre-oxidase (ceruloplasmina), pelo método de Houchin, no sôro de 65 indivíduos normais, de raça branca, com idades entre 20 e 45 anos. Foi obtido o seguinte valor médio neste grupo: 33,4 ± ± 3,1 mg/100 ml. Todavia, nos homens (n = 40) a média foi de 32,6 ± 3,1 mg/100 ml, enquanto nas mulheres (n = 25) foi de 34,6 ± 2,8 mg/100 ml; a diferença entre êstes subgrupos é significante. O sôro dialisado em 10 indivíduos normais perdeu 62,8 ± 0,8% da atividade cobre-oxidase. Não encontramos atividade cobre-oxidase na urina de 20 indivíduos normais nem no esperma de 2 outros indivíduos normais.

Copper-oxidase activity (ceruloplasmin) was determined, according to Houchin's method, in the blood serum of 65 normal white subjects, aged 20 to 45. The average concentration in this group was 33.4 ± 3.1 mgm./100 ml. In men (n = 40), however, the mean was 32.6 ± 3.1 mgm./100 ml, while in women (n = 25) it was 34.6 ± 2.8 mgm./100 ml; the difference between these means is significant. The dialysed blood serum of 10 normal subjects lost 62.8 ± 0.8 per cent of the copper-oxidase activity. No copper-oxidase activity was found neither in the urine of 20 normal subjects nor in the sperm of other 2 normal subjects.

O autor revê o histórico do tratamento cirúrgico das afecções extrapiramidais, desde Horsley (1890) até Buey e Buchanan (1931) e outros adeptos das intervenções sôbre o sistema "piramidal", e depois, a partir de Meyers (1940), as agressões sôbre os núcleos da base. Os efeitos favoráveis da palidectomia e da talamectomia sôbre o tremor e a rigidez parkinsonianos forçaram a reformulação dos conceitos sôbre a fisiopatologia do sistema extrapiramidal. O autor discute a localização da lesão responsável pela sintomatologia parkinsoniana, aceitando a opinião dos que consideram a destruição da substância negra como a base dos distúrbios hipercinéticos e tônicos, da mesma forma que a lesão do núcleo subtalâmico origina o hemibalismo. É feita a crítica do conceito clássico da sede preponderantemente palidal da lesão e da errônea interpretação jacksoniana dos fenômenos. É destacada a contribuição experimental e cirúrgica para o conhecimento da patofisiologia do pálido medial e dos núcleos ventrolaterais do tálamo. O tremor parkinsoniano pode ser considerado como resultante da hiperatividade da formação reticular pontomesencefálica (Jenkner e Ward), condicionada pela perda da ação inibidora do locus niger, associada à persistência da facilitação palidotalâmica. Na periferia, os estímulos reticulos-pinais "tremorogênicos" seguem a rota α. A rigidez parkinsoniana decorreria dos mesmos processos patológicos encefálicos, porém sua fisiopatogenia periférica é controvertida. Para alguns, resulta de hiperatividade γ, associada à hipersensibilidade das vias inibidoras dos motoneurônios (sistema δ de Hufschmidt). Outros pesquisadores, entretanto, aduziram dados experimentais em favor de uma paralisia parcial do sistema γ, com hiperatividade α. Finalmente, são referidas algumas teorias que tentam explicar o mecanismo básico do processo fisiopatológico nas afecções extrapiramidais, particularmente no parkinsonismo: descargas anormais de neurônios em degeneração (Cooper), hipersensibilidade de núcleos desaferentizados (Jenkner e Ward), desequilíbrio entre as estruturas lesadas e as indenes (Alberts e colaboradores).

The author gives the historical background of the surgical therapy of the extrapyramidal diseases, from Horsley (1890) to Bucy and Buchanan (1931) and other neurosurgeons who adopted the attack on the "pyramidal" tract, and, afterwards, beginning with Meyers (1940), the operations at the level of the basal ganglia. The good results of pallidectomy and thalamectomy on the parkinsonian tremor and rigidity imposed a reformulation of concepts on the physiopathology of the extrapyramidal system. The author discusses the site of the lesion responsible for the parkinsonian symptomatology, accepting the view that the dyskinesia and the rigidity are caused by a lesion of the substantia nigra, in the same way as lesions of the subthalamic nucleus are responsible for the hemiballism. The author criticizes the classificai concept of the prevailing pallidal site of the lesion and the erroneous jacksonian interpretation of the phenomena. The experimental and surgical contributions to the study of the pathological physiology of the medial pallidum and the ventrolateral nuclei of the thalamus are emphasized. The parkinsonian tremor can be regarded as the result of hyperactivity of the pontomesencephalic reticular formation (Jenkner and Ward), owing to the loss of the inhibitory drive of the locus niger, associated to the sparing of the pallidothalamic facilitation. In the periphery the tremorogenic reticulospinal impulses follow the α route. The parkinsonian rigidity would depend on the same encephalic pathologic processes, but its peripheral physiopathogenesis is controversial. Some authors believe that it results from γ hyperactivity, associated to oversensibility of the inhibitory pathways of the motoneurons (HufSchmidt's 5 system). Other investigators, however, have brought to light experimental data pointing to the partial paralisys of the γ sytem, associated to hyperactivity of the α drive. Finally, the author refers to some theories which try to explain the basic mechanism of the physiopathologic process in the extrapyramidal diseases, especially in parkinsonism: abnormal discharges of degenerating neurons (Cooper), oversensibility of the deafferented nuclei (Jenkner and Ward), imbalance between damaged and preserved neural structures (Alberts et al.).

A localização de cisticercos no recesso lateral da cisterna pontina poderá levar à constituição de uma síndrome do ângulo pontocerebelar, simulando eventualmente um neurinoma do acústico. São revistos os casos dessa forma clínica de neurocisticercose descritos na literatura. O problema do diagnóstico diferencial com os tumores (neurinomas) do acústico é discutido sob os pontos de vista clínico e radiológico. Os autores apresentam 7 casos de cisticercose do ângulo pontocerebelar. O diagnóstico etiológico foi estabelecido pela positividade da reação de fixação do complemento para cisticercose no liqüido cefalorraqueano em 5 casos e, em 2, pela cirurgia e/ou necropsia. Em todos os pacientes havia sintomas de lesão coclear, bilaterais em 2; lesão vestibular foi comprovada em 4 casos. Em 6 pacientes ocorreram sinais indicativos de lesão unilateral do nervo trigêmeo. Comprometimento do nervo facial foi observado em 4 casos. Existia faringoplegia em 3 casos e estrabismo interno em 2. Em 3 casos não foram observados sinais cerebelares, o que faria com que o diagnóstico propendesse para o de tumor do acústico, não fôssem outras características clínicas e subsidiárias. A síndrome de hipertensão intracraniana era nítida em 5 pacientes. Convulsões ocorreram em 2 casos. A propósito do resultado dos exames radiológicos, os autores salientam que, num dos casos, foi verificada amputação da ponta do rochedo ipsolateral à síndrome do ângulo pontocerebelar; outros comemorativos do caso levaram, porém, a rejeitar a hipótese de tumor (neurinoma) do acústico, tendo a cirurgia comprovado o acêrto do diagnóstico clínico.

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.

Os autores salientam a raridade dos déficits motores com distribuição mono ou hemiplégica, conseqüentes a lesões encefálicas de natureza cisticer-cótica. Ressaltam, outrossim, a dificuldade dos problemas diagnósticos que eventualmente se impõem nesses casos. Discutindo a patogenia dêsses processos, apontam dois mecanismos principais: as paralisias determinadas por focos encefálicos, constituídos por granulomas, cisticercóticos ou à distância, e os acidentes cerebrovasculares ocasionadas pelas alterações vasculares, cuja importância é realçada. São apresentados 19 casos de neurocisticercose, incluindo 17 formas hemiplégicas e 2 monoplégicas. Em todos, o diagnóstico etiológico foi estabelecido pela positividade da reação de fixação do complemento para cisticercose no liqüido cefalorraqueano, corroborado, em 2 casos, pela cirurgia e pela necropsia. Convulsões foram registradas em 10 casos (focais em 7 e generalizadas em 3). Sintomas de hipertensão intracraniana foram assinalados em 4 pacientes. A hemiplegia instalou-se sob forma de icto em 14 pacientes. Em 3 casos houve elementos altamente sugestivos de patogenia predominantemente vascular; em 3, a sintomatologia relacionou-se diretamente ao granuloma parasitário, embora deva ter havido participação do processo angiopático; em 1 caso, tratava-se, provavelmente, de paralisia pós-convulsiva; em 3, embora não houvesse suficientes dados complementares, os elementos clínicos levaram a salientar a importância do fator vascular na determinação da hemiplegia. Em 5 pacientes o déficit motor instalou-se de modo lento e progressivo. Em 2 casos foram encontrados e extirpados cisticercos situados no córtex cerebral, tendo a necropsia revelado, em um deles, amolecimento isquêmico na região parietal contralateral à hemiplegia; em 1 havia eisticercose da fossa craniana posterior, associada a lesão cortical traduzida por intensas alterações eletrencefalográficas; 2 casos careciam de elementos subsidiários que pudessem orientar melhor quanto ao mecanismo patogênico da hemiplegia. Os autores salientam a possibilidade de, em alguns dêsses casos, os comemorativos clínicos poderem ter conduzido ao diagnóstico errôneo, ora de tumor cerebral, ora de acidente cerebrovascular de etiologia indeterminada. Em tais eventualidades, os pacientes ficariam privados dos possíveis benefícios do tratamento adequado, cirúrgico ou medicamentoso.

The authors emphasize the low frequency of motor impairment with mono or hemiplegic distribution, due to cysticercotic lesions of the brain. It is also stressed the difficulty of the diagnostic problems posed by some of these cases. Two pathogenic mechanisms are pointed out: the paralyses caused by granulomatous brain foci, near or far from the parasite, and the cerebrovascular infarctions induced by the marked changes in blood vessels. Nineteen cases of cysticercosis of the central nervous system including 17 hemiplegic and 2 monoplegic forms are reported. In every case the etiology was established by a positive complement fixation test for cysticercosis in the cerebrospinal fluid, and was supported, in 2 cases, by suvgery and post mortem examination. Convulsions were referred by 10 patients (7 times focal and 3 generalized). Symptoms of intracranial hypertension were present in 4 patients. The hemiplegia set up like an ictus in 14 patients. In 3 cases the data were highly suggestive of a vascular pathogeny; in 3 the symptomatology was directly related to the parasitic granuloma, although the vascular disease must have shared in the process; in 1 the paralysis was probably post-epileptic; in 3, although subsidiary data were not sufficient, the clinical facts gave emphasis to the role of the vascular factor in the onset of the hemiplegia. In 5 patients the motor impairment was slow and progressive. In 2 cases cysticerci were found on the cerebral cortex and removed; the post mortem examination showed, in one of them, an ischemic softening of the parietal cortex contralateral to the hemiplegia; in 1 case there was cysticercosis of the nervous structures of the posterior cranial fossa, associated to a cortical lesion which induced severe electroencephalographic changes; 2 cases lacked subsidiary data which could help to disclose the pathogenic mechanism of the hemiplegia. The authors give emphasis to the possibility that, in some of these cases, the clinical data might have led to a wrong diagnosis, either of a brain tumor or of a cerebrovascular disease with unknown etiology. In such instances, the patients would be deprived of the eventual benefits of a proper treatment, either with drugs or surgery.

Cysticercosis of the nervous system is a tribute paid to underdevelopment. The tables of incidence show that although it is practically unknown in some countries, it represents a curse to the less fortunate habitants of Asia, Africa and Latin America. The Latin American countries pay a heavy toll; known statistics show a ponderous incidence in Mexico, Chile, Peru and Brasil. The statistics referring to the States of São Paulo and Rio de Janeiro, Brasil, where a rather thorough study of the problem has been made through the past years, portray the seriousness of the problem. Autopsy studies show an incidence of 0.12 to 1.10 percent in general hospitals, and of 0.40 to 3.60 percent in neuropsychiatric hospitals. In the Department of Neurology of the University of São Paulo School of Medicine, the incidence ranged from 2.90 to 3.39 percent in ward patients, and from 0.31 to 0.55 percent in out-patients. This Department had 41,328 patients from 2-14-1945 to 8-31-1961, of which 4,900 were ward patients. A total of 276 cases of neurocysticercosis was observed. A correlation between the incidence of neurocysticercosis and hygienic-social conditions is made evident by the fact that only one fourth of our patients came from large cities, while the remaining came from the hinterland, where living conditions are notedly worse. The differences in incidence regarding to sex and race were only apparent. Regarding to age incidence, 54 cases were younger than 10 years, and about half of the cases were in the 21 to 40 age group. The youngest patient was 14 months, the oldest 69 years old. The diagnosis of neurocysticercosis was based on at least one of the following data: a) recovery of the parasite during surgery and/or autopsy; b) positive complement fixation test (CFT) for cysticercosis in the cerebrospinal fluid, with or without increase in eosinophiles; c) positive CFT for cysticercosis in the blood associated to X-ray demonstration of intracranial parvinodular calcifications. The CFT for cysticercosis in the cerebrospinal fluid, performed in 271 patients, was positive in 240 (88.6 percent). The same test in the blood was positive in 70.2 percent of the cases. The correlation between these data was not significant. Intracranial mottling calcifications were found in only 19.2 percent of the cases. Therefore, in 240 cases the diagnosis was based on cerebrospinal fluid findings, confirmed by surgery and/or autopsy in 58 patients. In 13 cases, despite a normal cerebrospinal fluid, the diagnosis was made by a positive CFT for cysticercosis in the blood, associated to the finding of intracranial parvinodular calcifications. In the remaining 23 cases the parasite was recovered during surgery and/or autopsy. Regarding to its clinical features, the disease was manifested under three separate forms: acute (meningoencephalitis, cerebral edema, ventricular obstruction, ischemic softening secondary to endarteritis), subacute and chronic (specially the purely convulsive clinical pictures). The symptoms had been present for less than 1 month in 40 cases, from 1 to 12 months in 116 cases, and for more than 1 year in 115 cases; in 5 cases this information was not ascertained. Our cases were classified, regarding to the clinical symptomatology, in hypertensive, convulsive, with focal or diffuse neurologic manifestations, and psychic. These forms were found as single manifestations in the following rates: 46.6 percent of the convulsive, 35.9 percent of the hypertensive, 24.5 percent of the cases with focal or diffuse neurologic symptoms, and none of the psychic. There was association of intracranial hypertension and convulsive seizures in 69 cases (25.0 percent). In 26 cases (9.4 percent) there were, in addition, psychic symptoms. In 24 cases (8.7%) focal or diffuse neurologic manifestations were associated to intracranial hypertension and convulsions. In general, the hypertensive forms were more common than the convulsive forms. Hypertensive cases prevailed in the Emergency Service (85.1 percent of cases), while in the Out-patient Department the convulsive forms were more frequent (87.8 percent). In 85 hypertensive cases the site of cerebrospinal fluid block was investigated either by X-ray, surgery or autopsy, and was determined to be ventricular in 23 cases, and cisternal in 46 cases. Racemous cysticerci more often cause obstructive forms. Seizures were present in 148 patients (53.6 percent), 79 of which having not intracranial hypertension. The incidence of generalized and focal seizures was approximately the same. Psychomotor epilepsy was clinically diagnosed in 5 cases, 2 of which presented temporal foci in the EEG examination. The electroencephalograms of 148 cases were analyzed regarding to topography of findings and clinical manifestations. Diffuse alterations and bilateral occipital foci were more common with intracranial hypertension, while isolated foci were more frequent with purely convulsive cases. Normal readings were also common with the latter (44.0 percent). Focal or diffuse neurologic manifestations were associated to intracranial hypertension and/or convulsions in 74 patients (26.8 percent), but were found as single symptomatology in only 24 patients (8.7 percent). In this group ataxia was the main finding, being cerebellar (33 cases) or mixed (3 cases). Central paralytic forms (monoplegia, hemiplegia and tetraplegia) were observed in 24 cases, sometimes starting as a vascular ictus. Cranial nerve involvement was noted in 12 patients, usually as a cerebello-pontine syndrome. Spinal cord manifestations characterized 8 cases. Paralysis of vertical gaze was observed in 5 instances. Extrapyramidal forms were rare (4 cases), as well as neuralgias of the trigeminal or glossopharyngeal nerves (3 cases) and infundibulo-hypophyseal syndromes (2 cases). Difuse cerebrospinal symptomatology was observed in 2 cases. Psychic disturbances were evident in 63 patients (22.8 percent), usually associated to intracranial hypertension, isolated (25 cases) or joined to seizures (26 cases). The mortality rate was 25.9 percent. Of 63 operated patients, 28 died. The results of various schemes of drug therapy cannot be well evaluated, due to the possibility of spontaneous remission, and to difficulties of prolonged follow-up. In view of the poor surgical results, and debatable effects of medical treatment, emphasis should be given to prophylactic measures, through campaigns of Public Health.

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.

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.

Os autores apresentam os resultados imediatos e tardios do emprêgo do ACTH em 4 casos de neuropatias desmielinizantes aquílicas (assim chamadas degenerações combinadas da medula). Os efeitos imediatos foram satisfatórios em todos os casos, particularmente em relação à síndrome piramidal, mostrando-se mais resistente a síndrome neurítica. Os efeitos tardios foram, no conjunto, absolutamente idênticos aos imediatos, demonstrando que os benefícios se mantiveram por períodos de 3 a 6 meses; a análise separada das síndromes neurológicas evidenciou melhora ulterior dos sinais piramidais, estabilidade da sintomatologia neurítica e discreta recidiva da síndrome funicular dorsal. A instituição de tratamentos pelas vitaminas B1, e B12, em altas doses, após um período pós-ACTH de 6 meses (em 3 casos) não modificou o quadro clínico.

The authors report the early and late results of the use of ACTH in 4 cases of achylic demyelinating neuropathy (so-called subacute combined degeneration of the spinal cord). Daily doses of 12.5 or 15.0 mgm. of corticotropin were given by continuous drip method, without any other associated drug which could interfere in the results. By employing a semi-quantitative method for evaluation of the clinical effects, an average improvement of 26 per cent was found, the same results being observed soon after the treatment and by a follow-up from 3 to 6 months. The effects on the pyramidal syndrome were particularly satisfactory both early and lately; the improvement of the dorsal funicular syndrome was not so long lasting. Treatment with vitamins B1 and B12 six months after the use of ACTH, in 3 cases, caused no further change in the clinical picture.

Os autores chamam a atenção sôbre os erros de diagnóstico nos casos de neuroblastomicose, seja nas formas meningoencefálicas, seja nas tumorais. São focalizadas a paracoccidioidose e a criptococose. Depois de caracterizarem clìnicamente a paracoccidioidose, acentuam a raridade das formas nervosas (1,2% dos casos dessa micose autopsiados no Departamento de Anatomia Patológica da Faculdade de Medicina da Universidade de São Paulo). Pela revisão da literatura foi verificado haver apenas 12 casos registrados. 0 caso de Casiello e Klass é o único em que o diagnóstico foi feito em vida; os demais, ou constituem achado de necropsia, ou foram inesperadamente revelados pelo exame histopatológico realizado após intervenções cirúrgicas. Os autores registram 2 casos de paracoccidioidose do sistema nervoso. No primeiro, tratava-se de meningomielorra-diculite crônica, sendo o parasito identificado no escarro; o segundo era portador de síndrome de compressão medular, cuja patogenia é discutida pelos autores, tendo sido a etiologia paracoccidióidica comprovada pelo exame da polpa e biópsia ganglionares. Considerações clínicas sôbre a criptococose precedem o relato de um caso de meningoencefalomielite subaguda, no qual fôra feito em vida o diagnóstico de paracoccidioidose, pelo exame micológico do liqüido cefalorraqueano; êste paciente, embora medicado com doses maciças de sulfa e tiossemicarbarsona, veio a falecer, tendo o exame anátomo-patológico revelado tratar-se, na realidade, de lesões nervosas produzidas pelo Crypíococcus neo-formans. Nos três casos era incisivo o caráter neurocirúrgico da sintomatologia. Entretanto, ante o diagnóstico de paracoccidioidose, foi instituído tratamento pelas sulfas, vacina específica e tiossemicarbarsona. Os resultados foram excelentes no caso 2, em que a terapêutica pôde ser instituída mais precocemente; melhoras também foram obtidas no caso 1. No caso 3, foram verificadas melhoras transitórias com o tratamento, porém, após dois anos de moléstia, o paciente veio a falecer. Os autores concluem que a blastomicose do sistema nervoso deverá participar das cogitações neurocirúrgicas. Entretanto, na paracoccidioidose, a sulfamidoterapia em doses elevadas, associada às vacinas antiblastomicóticas, deve constituir a primeira tentativa terapêutica. Para o tratamento da torulose são indicadas a estreptotricina e a actidiona, embora a experiência com o seu uso clínico ainda seja muito pequena para que se chegue a conclusões definitivas. A cirurgia ficará reservada àqueles casos em que as condições topográficas ou evolutivas da lesão exijam solução urgente.

The authors emphasize the dangers of diagnostic errors in the cases of neuroblastomycosis, whether in the meningoencephalic or the tumoral forms. Paracoccidioidosis and cryptococcosis are studied. After a clinical characterization of paracoccidioidosis, the authors stress the scarceness of its nervous forms (1,2 per cent of 84 necropsies of this mycosis performed at the Department of Pathology of the Faculty of Medicine of São Paulo). In the literature there are but 12 cases reported. Casiello and Class' is the only case where the diagnosis was made during life; the other cases were necroscopic findings or unexpected histopathologic data after the patients had been operated on por presumptive tumors. The authors report 2 cases of paracoccidioidosis of the nervous system. In the first one, showing meningo-myelo-radiculitis, the parasite was identified in the sputum; the second patient exhibited a syndrome of spinal compression, whose pathogeny is discussed by the authors (lymphatic infiltration of the epidural cervical space? lymphatic infiltration of the spinal cord? allergic diffuse serous inflammation of the spinal cord caused by the ganglionar focus?) ; the paracoccidioidal etiology was attested by bactériologie examination and biopsy of cervical lymph nodes. After clinical comments on cryptococcosis, the authors report a case of subacute meningoencephalomyelitis, which was diagnosed as paracoccidioidal by the mycologie examination of the cerebrospinal fluid; this patient, although treated with high doses of sulfonamides and thiosemicarbarsone, died after two remissions of the symptomatology; the necroscopic examination disclosed the real nature of the neurological changes, which were diagnosed as torular granuloma. In all cases the neurosurgical aspect of the symptomatology was impressive. Neverthless, the diagnosis of paracoccidioidosis imposed the treatment with sulfadiazine, sulfamerazine, specific vaccine and thiosemicarbarsone. Results were remarkable in case 2, where treatment was earlier started; case 1 also showed improvement. In case 3, transient improvement followed the treatment, but, after two years of disease, the patient died. The authors conclude that nervous blastomycosis must be taken on account in neurosurgical considerations. In paracoccidioidosis massive doses of sulfa drugs associated to specific vaccine, however, will be the first therapeutic approach. In the treatment of cryptococcosis streptothricin and actidione are worth trying according to experimental studies. Surgery is indicated only in the cases where the site or developmental conditions of the disease demand urgent resolution.