Resumo em Inglês:

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.

Resumo em Português:

Entre os aspectos biológicos da neurocisticercose, têm sido mais exploradas as alterações do líqüido cefalorraquidiano (LCR) em vista do seu valor diagnóstico. Para analisar os conhecimentos quanto ao quadro liquórico da afecção são apresentados 03 achados referentes a 62 caco3 acompanhados na Clínica Neurológica da Faculdade de Medicina da Universidade de São Paulo. Em todos êstes casos a infestação do sistema nervoso central (SNC) e/ou de seus envoltórios pelo cisticerco foi comprovada pela necropsia ou durante intervenção cirúrgica (biopsia). A análise do material e da literatura sôbre o assunto permite as seguintes conclusões: 1 - Entre os exames complementares, o exame do LCR é aquêle que permite com maior freqüência o diagnóstico em vida da neurocisticercose. A demonstração da presença de anticorpos específicos é o elemento fundamental para o diagnóstico liquórico; a ecsinofilorraquia complementa esse dado e tem valor sugestivo. 2 - Na neurocisticercose, a eosinofilorraquia costuma ser tanto mais intensa quanto mais nítida a pleiocitose liquórica; entretanto, a presença de células eosinófilas no LCR pode decorrer' de outras causas e sua ausência não infirma o diagnóstico. A eosinofilorraquia permite avaliar a intensidade da reação hiperérgica desencadeada pelo parasito e possibilita orientar o diagnóstico em casos duvidosos. 3 - Na cisticercose há formação de anticorpos específicos, demonstráveis por meio de reações de precipitação e de fixação do complemento; esta última é a mais largamente utilizada. Os anticorpos são semelhantes aos que aparecem no parasitismo por outros cestóideos. Quando localizados no SNC e/ou em seus envoltórios, são os cisticercos os cestóideos que, com maior freqüência e em maior intensidade, desencadeiam reações imunitárias, determinando o aparecimento de anticorpos específicos no LCR. 4 - A evolução do quadro liquórico é variável e nem sempre acompanha a evolução clínica. Quando esta é satisfatória, costumam desaparecer progressivamente as alterações do LCR; se estas se mantiverem por longo tempo, o prognóstico é mais reservado, especialmente quando aparece hipoglicorraquia. Em alguns casos, após intervenções cirúrgicas há rápida remissão das alterações do LCR; provavelmente isto ocorre nos casos em que a infestação do encéfalo era discreta, ou mesmo única. Quando ocorre rotura da vesícula parasitária durante a intervenção cirúrgica, o liqüido contido em seu interior acarreta exacerbação transitória das alterações do LCR. 5 - As alterações do proteinograma do LCR na neurocisticercose são do tipo verificado em processos inflamatórios subcrônicos e crônicos do SNC e de seus envoltórios, caracterizando-se especialmente por aumento de γ-globulina. Êste aumento está relacionado à intensidade da reação imunopatológica e provavelmente é devido à produção local dessa globulina, que parece ser a carreadora dos anticorpos específicos. O aumento de γ-globulina é precoce, podendo preceder a formação dos anticorpos específicos e costuma regredir lentamente nos casos de evolução satisfatória. Quando a evolução é má, o teor dessa globulina costuma aumentar progressivamente.

Resumo em Inglês:

The CSF changes in neurocysticercosis have been extensively studied in view of their diagnostic importance. The CSF changes in 62 human cases of neurocysticercosis are presented. In all cases the diagnosis of cysticercosis was ascertained either by surgical procedure or by autopsy. The following observations are made from the study of this material, and from the pertinent literature: 1 - More cases had their diagnosis made during life by the CSF examination alone than by any other procedure. The demonstration of specific antibodies in the CSF is the main diagnostic element. The presence of eosinophile cells is only suggestive of the diagnosis, and is complementary to the immunologic data. 2 - The occurrence eosinophile in the CSF is more pronounced in cases of higher total cell count. The presence of eosinophiles in the CSF may be secondary to other causes, and its absence does not invalidate the diagnosis of cysticercosis. The hyperergic reaction to the parasite can be evaluated through the eosinophile count, which contributes to the diagnosis in doubtful cases. 3 - Specific antibodies are present in the CSF in human cysticercosis, demonstrated by complement fixation and precipitation tests, the former being used more often. The antibodies appearing in cestoid parasitism are similar as a general rule. The immune reaction to the Cysticercus cellulosae in the CNS is greater in frequency and intensity, as compared to the other cestoids. 4 - The CSF changes are variable and do not always follow the clinical progress. The improvement in the clinical picture usually is followed by progressive disappearence of the CSF changes. The prognosis is worse with persistence of the CSF changes, particularly with a low sugar content. After surgical excision of cysticerci the CSF changes occasionally disappear, probably due to a small degree of parasitism, totally removed by surgery. Extravasation of the vesicular fluid of a cysticercus during an operation induces transient increase in the CSF changes. 5 - The CSF protein electrophoresis pattern in neurocysticercosis is similar to the pattern observed in sub-chronic or chronic inflammatory processes of the CNS and its coverings. An increase in the γ-globulin fraction is found and it is related to the intensity of the immune reaction. This globulin fraction probably contains the specific antibodies and the possibility of local production of this globulin is to be considered. The rise in γ-globulin appears early in the disease, and may precede the formation of specific antibodies. It regresses slowly when the clinical progress is good. There is a progressive increase in cases of poor evolution.

Resumo em Português:

É apresentado um caso de gargulismo, em paciente masculino, de 11 anos de idade, com alterações clínicas, radiológicas e anátomo-patológicas típicas. A substância anormal, provàvelmente mucopolissacáride, se acumulava em células de SRE, células hepáticas, células cartilaginosas e células nervosas. Histológicamente esta substância varia de aspecto, o que foi considerado como indício de ativo estado metabólico. As alterações encontradas foram correlacionadas com os achados clínicos e radiológicos.

Resumo em Inglês:

A typical case of gargoylism with clinical, X-rays and pathological findings, occurring in an eleven year old boy is reported. An abnormal substance of mucopolysacharide character was present in RES, hepatic, cartilage and nervous cells. Histologically, this substance showed morphological variations which were interpreted as an indication of metabolic activity. The several morphological features of the case were correlated with clinical and X-rays data.

Resumo em Português:

É relatado o caso de paciente com dor lombar que se irradiava para o membro inferior esquerdo e com anestesia da heminádega do mesmo lado. Pela intervenção cirúrgica verificou-se uma profusão do IV disco lombar e três cistos, localizados um em cada raiz posterior de S2, S3 e S4. Além disso, a raiz de S1 estava espessada. Os conceitos sobre a patogenia dos cistos são discutidos.

Resumo em Inglês:

Case report of patient with low back pain irradiating to the left lower limb, with anesthesia in the ipsilateral gluteal region. Operation confirmed protrusion of the disk in the 4th intervertebral space and showed a cyst in each of left dorsal roots S2, S3 and S4; dorsal root St of the same side was strongly thickened. The various etiopathogenic concepts of perineurial cysts are discussed.