Horacio M. Canelas

Neurocisticercose: incidência, diagnóstico e formas clínicas

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 Sao 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 Sao 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.

Cysticercosis of the nervous system: less frequent clinical forms III- spinal cord forms

The spinal forms of cysticercosis are rather rare (2.7% of 296 cases of neurocysticercosis recorded in the Department of Neurology of the University of Sao Paulo Medical School). In a survey of the literature only 42 cases were found, most of them associated with cerebral symptoms. The reasons for this low incidence, as well as the possible routes followed by the parasite in its approach to the spinal cord, are discussed. After a review of the first cases reported in the literature, the authors refer the main syndromes (meningomyelitides, tabetiform pictures and spinal cord compressions) and some of the clinico-pathologic features of spinal cysticercosis. Nine cases of spinal cysticercosis are reported. The diagnosis was based on laboratorial data (mainly the complement fixation test for cysticercosis in the cerebrospinal fluid) or in the results of surgical therapy. Other cerebrospinal fluid findings (presence of eosinophile cells, protein contents, and the results of the manometric tests) are discussed. Myelographic block was demonstrated in 5 cases. Three of these patients were submitted to laminectomy, with variable results. The prevailing neurological picture was that of spinal cord and/or root compression (4 cases). Two patients showed a dorsal funiculi syndrome closely simulating tabes dorsalis. Two other patients presented a picture of meningomyelitis with no systematization. One patient had a syndrome suggestive of subacute combined degeneration of the spinal cord, but the presence of cerebral symptoms and the laboratorial data pointed to cysti-cercosis as the main disease process.

Blastomycosis of the nervous system

Os autores chamam a atencao sobre os erros de diagnostico nos casos de neuroblastomicose, seja nas formas meningoencefalicas, seja nas tumorais. Sao focalizadas a paracoccidioidose e a criptococose. Depois de caracterizarem clinicamente a paracoccidioidose, acentuam a raridade das formas nervosas (1,2% dos casos dessa micose autopsiados no Departamento de Anatomia Patologica da Faculdade de Medicina da Universidade de Sao Paulo). Pela revisao da literatura foi verificado haver apenas 12 casos registrados. 0 caso de Casiello e Klass e o unico em que o diagnostico foi feito em vida; os demais, ou constituem achado de necropsia, ou foram inesperadamente revelados pelo exame histopatologico realizado apos intervencoes cirurgicas. Os autores registram 2 casos de paracoccidioidose do sistema nervoso. No primeiro, tratava-se de meningomielorra-diculite cronica, sendo o parasito identificado no escarro; o segundo era portador de sindrome de compressao medular, cuja patogenia e discutida pelos autores, tendo sido a etiologia paracoccidioidica comprovada pelo exame da polpa e biopsia ganglionares. Consideracoes clinicas sobre a criptococose precedem o relato de um caso de meningoencefalomielite subaguda, no qual fora feito em vida o diagnostico de paracoccidioidose, pelo exame micologico do liquido cefalorraqueano; este paciente, embora medicado com doses macicas de sulfa e tiossemicarbarsona, veio a falecer, tendo o exame anatomo-patologico revelado tratar-se, na realidade, de lesoes nervosas produzidas pelo Crypiococcus neo-formans. Nos tres casos era incisivo o carater neurocirurgico da sintomatologia. Entretanto, ante o diagnostico de paracoccidioidose, foi instituido tratamento pelas sulfas, vacina especifica e tiossemicarbarsona. Os resultados foram excelentes no caso 2, em que a terapeutica pode ser instituida mais precocemente; melhoras tambem foram obtidas no caso 1. No caso 3, foram verificadas melhoras transitorias com o tratamento, porem, apos dois anos de molestia, o paciente veio a falecer. Os autores concluem que a blastomicose do sistema nervoso devera participar das cogitacoes neurocirurgicas. Entretanto, na paracoccidioidose, a sulfamidoterapia em doses elevadas, associada as vacinas antiblastomicoticas, deve constituir a primeira tentativa terapeutica. Para o tratamento da torulose sao indicadas a estreptotricina e a actidiona, embora a experiencia com o seu uso clinico ainda seja muito pequena para que se chegue a conclusoes definitivas. A cirurgia ficara reservada aqueles casos em que as condicoes topograficas ou evolutivas da lesao exijam solucao urgente.

Heart involvement in hepatolenticular degeneration

The case of a 10‐year‐old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. The microscopical examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin‐eosine and the histochemical method with rubeanic acid, showed a medium degree of myocardial damage and the presence of copper deposition in the tissue. Copper concentration on a myocardial biopsy fragment was almost 10 times the normal heart content. Comments are made on the relationship of the clinical, laboratory and pathological findings with the degree of copper storage on the myocardium.

Osteoarthropathy of hepatolenticular degeneration.

Ninety‐six joints of 25 patients with hepatolenticular degeneration have been studied by means of scintigraphs with technetium (99mTc), a method which can disclose early synovial inflammation and postinflammatory articular degeneration. Signs of synovitis were found in 22 joints of 10 patients (40%) and degenerative changes were present in 15 joints of eight patients (32%). Eleven patients complained of joint pains; in four of them they were the presenting symptom. the radiological study showed osteoporosis in 22 patients (88%); flexion deformity was found in two cases, anomalous osteophytes in two, and signs of growth arrest, features of rheumatoid arthritis and aseptic necrosis in one case each. the pathogenesis of osteoarticular changes in Wilsons disease is discussed.

[Schistosomiasis with spinal meningo-radicular lesions].

Case report of a patient recently arrived from Pernambuco (a region where schistosomiasis is endemic) with a very suggestive history of bilharzial contamination, presenting hepatosplenomegalia and a neurological picture including generalized weakness, specially in the lower limbs, absence of deep reflexes, hypotonus, tactile and thermalgesic hypesthesia in the hands and lower limbs, and marked impairment of proprioception in the distal segments of all limbs. Ova of Schistosoma monsoni were found in the feces. Positive intradermic reaction and complement fixation test in the blood. The assumption of schistosomiasis of the spinal cord was strengthened by a meningo-parenchymatous type of change in the cerebrospinal fluid, including definite pleocytosis with eosinophilia, and also by a strongly positive complement fixation test in the fluid. The patient died before anti-monial treatment could be started. The post mortem study revealed changes which usually occur in visceral schistosomiasis, but as ova were not found in the tissues, the diagnosis was maintained if not confirmed. The study of the nervous system showed normality of the brain; in the spinal cord it was found chronic leptomeningitis, radiculitis, bilateral degeneration of roots in the cauda equina, and secondary bilateral partial degeneration of the fasciculus gracilis.

DISORDERS OF COPPER METABOLISM IN EPILEPSY.

Except for hepatolenticular degeneration and multiple sclerosis, copper metabolism has not been duly evaluated in nervous diseases. Concerning epilepsy, almost nothing is seen in the literature, ncithcr when the most renowned textbooks (Pen f i e ld & Jasper, 1954; L e n n o x & L e n n o x , 1960; T o w e r , 1960) are reviewed, nor when a survey is made of papers on copper metabolism in various disease processes (McElroy & Glass, 1950; Scheinberg & S t e r n l k b , 1960; Ade l s t e in & Vallee, 1961). Only in some few articles on disorders of copper metabolism in multiple sclerosis ( P l u m & Hansen , 1960) and in mental discases (Hei l ineyer & Ulrich, apud K e u p , 1954; Escobar & Niet to , 1957), or clse in studies on ceruloplasmin (O’ReilIy, 1961), some data regarding epilepsy arc found. On the other hand, in cases of acute or chronic copper intoxication no reference to seizures is made ( A d e l s t e i n & Val lee , 1961). In the present paper we report the results of the determination of copper and ceruloplasmin in the blood and cerebrospinal fluid (CSF) of epileptic patients, either in the interictal period, or immediately after the fit, and also in patients with mental diseases, before and after they were submitted to electroshock.

Subacute sclerosing leucoencephalitis: an epidemiological, clinical and biochemical study of 31 cases

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.

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.

Abdominal ultrasonography in hepatolenticular degeneration: a study of 33 patients

The ultrasonographic study of 33 patients with hepatolenticular degeneration showed the following main changes: disorders of the hepatic echotexture (29 cases), changes of the splenic dimensions (21), liver shrinkage (10), cholelithiasis (8), hepatomegaly and ascites (1). The disorders of liver echotexture exhibited different patterns, from slight to severe changes of the hepatic echogenicity, associated with anatomic distortions of the liver, such as alterations of outline and decrease of dimensions. Liver shrinkage was always accompanied by splenomegaly. The scarcity of hepatomegaly may be explained by the fact that the ultrasonography was performed after the onset of treatment with penicillamine, or by other factors still unknown, copper perhaps possessing a stronger fibrogenetic action than other hepatotoxic agents. Cholelithiasis was very frequent in females (6 of 13 patients), its incidence tending to increase with age. Regarding males there was no increase when the incidence was compared to the general population. The two sole males were young, an unusual finding in normal men.