The Brazilian database on pregnancy in multiple sclerosis.

OBJECTIVES: To report the results from the Brazilian database on multiple sclerosis (MS) and pregnancy. METHODS: Retrospective data from MS patients who became pregnant at any time of their disease were sent to a Brazilian database, using a specific file for this purpose. RESULTS: Data on 128 women (142 pregnancies) from 30 neurologists working in 21 cities in Brazil were collected. Patients' average age at pregnancy was 29.8 years (range 16-42). EDSS at start of pregnancy was 1.5±1.4; and the relapse rate in the year preceding pregnancy was 1.2±1.5. Exposure to medication at any time during pregnancy was high (69.7%): 48.6% to interferon beta; 14.1% to glatiramer acetate; and 7% to other immunomodulatory and immunosuppressive drugs. There was a significant decrease in relapse rate during pregnancy. The prevalence of complications was relatively low, with 4.9% of obstetric and 1.4% neonatal unfavorable outcomes. CONCLUSIONS: Our patients had low degrees of disability, short histories of disease, high drug exposure, and relatively high relapse rate in the year previous to pregnancy. Obstetric and neonatal outcomes were successful in over 90% of our patients.

Profile of the Brazilian scientific production in multiple sclerosis

This paper analyzes the profile of the Brazilian output in the field of multiple sclerosis from 1981 to 2004. The search was conducted through the MEDLINE and LILACS databases, selecting papers in which the term "multiple sclerosis" was defined as the main topic and "Brazil" or "Brasil" as others. The data were analyzed regarding the themes, the state in Brazil and institution where the papers were produced, the journals where the papers were published, journal's impact factor, and language. The search disclosed 141 documents (91 from MEDLINE and LILACS, and 50 from LILACS only) published in 44 different journals (23 of them MEDLINE-indexed). A total of 111 documents were produced by 17 public universities, 29 by 3 private medical schools and 1 by a non-governmental organization. There were 65 original contributions, 37 case reports, 20 reviews, 6 PhD dissertations, 5 guidelines, 2 validation studies, 2 clinical trials, 2 chapters in textbooks, 1 Master of Science thesis, and 1 patient education handout. The journal impact factor ranged from 0.0217 to 6.039 (median 3.03). Of 91 papers from MEDLINE, 65 were published by Arquivos de Neuro-Psiquiatria. More than 90 percent of the papers were written in Portuguese. São Paulo was the most productive state in the country, followed by Rio de Janeiro, Minas Gerais and Paraná. Eighty-two percent of the Brazilian output came from the Southeastern region.

Chemokines in the cerebrospinal fluid of patients with active and stable relapsing-remitting multiple sclerosis.

Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the human central nervous system. Although its etiology is unknown, the accumulation and activation of mononuclear cells in the central nervous system are crucial to its pathogenesis. Chemokines have been proposed to play a major role in the recruitment and activation of leukocytes in inflammatory sites. They are divided into subfamilies on the basis of the location of conserved cysteine residues. We determined the levels of some CC and CXC chemokines in the cerebrospinal fluid (CSF) of 23 relapsing-remitting MS patients under interferon-ss-1a therapy and 16 control subjects using ELISA. MS patients were categorized as having active or stable disease. CXCL10 was significantly increased in the CSF of active MS patients (mean +/- SEM, 369.5 +/- 69.3 pg/mL) when compared with controls (178.5 +/- 29.1 pg/mL, P < 0.05). CSF levels of CCL2 were significantly lower in active MS (144.7 +/- 14.4 pg/mL) than in controls (237.1 +/- 16.4 pg/mL, P < 0.01). There was no difference in the concentration of CCL2 and CXCL10 between patients with stable MS and controls. CCL5 was not detectable in the CSF of most patients or controls. The qualitative and quantitative differences of chemokines in CSF during relapses of MS suggest that they may be useful as a marker of disease activity and of the mechanisms involved in the pathogenesis of the disease.

Chemokines in the cerebrospinal fluid of patients with active and stable relapsing-remitting multiple sclerosis

Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the human central nervous system. Although its etiology is unknown, the accumulation and activation of mononuclear cells in the central nervous system are crucial to its pathogenesis. Chemokines have been proposed to play a major role in the recruitment and activation of leukocytes in inflammatory sites. They are divided into subfamilies on the basis of the location of conserved cysteine residues. We determined the levels of some CC and CXC chemokines in the cerebrospinal fluid (CSF) of 23 relapsing-remitting MS patients under interferon-ß-1a therapy and 16 control subjects using ELISA. MS patients were categorized as having active or stable disease. CXCL10 was significantly increased in the CSF of active MS patients (mean ± SEM, 369.5 ± 69.3 pg/mL) when compared with controls (178.5 ± 29.1 pg/mL, P < 0.05). CSF levels of CCL2 were significantly lower in active MS (144.7 ± 14.4 pg/mL) than in controls (237.1 ± 16.4 pg/mL, P < 0.01). There was no difference in the concentration of CCL2 and CXCL10 between patients with stable MS and controls. CCL5 was not detectable in the CSF of most patients or controls. The qualitative and quantitative differences of chemokines in CSF during relapses of MS suggest that they may be useful as a marker of disease activity and of the mechanisms involved in the pathogenesis of the disease.

Schistosomal myeloradiculopathy due to Schistosoma mansoni: report on 23 cases

Schistosoma mansoni infection is likely to be responsible for a significant proportion of cases of myelopathy occurring in areas where schistosomiasis is endemic. The aim of this study is to describe the clinical, laboratory and therapeutic data of 23 patients with schistosomal myeloradiculopathy. The medical records of 23 patients with schistosomal myelopathy admitted to two general hospitals of Belo Horizonte (MG), in Brazil, from 1995 to 1999, were reviewed retrospectively. Seventeen patients were male (74 percent). The mean age for the whole group was 27 years. Lower limb weakness and associated lumbar and/or lower limb pain were reported by 20 patients (87 percent), and 16 (70 percent) were unable to walk. All individuals presented urinary retention and 19 (83 percent) complained of intestinal dysfunction. The treatment was based on the association of antischistosomal drugs and corticosteroids. Five patients (22 percent) presented a full response to treatment, 13 (57 percent) partial response without functional limitations and 4 (17 percent) partial improvement with limitations or no response. Three out of the 4 patients who stopped steroids before 45 days of treatment developed recurrence of the symptoms and signs of myelopathy. Our cases demonstrate the severe presentation of the disease and the data disclosed here suggest that treatment with steroids should be kept for months after clinical improvement

[Recommendations for the use of immunomodulatory drugs in multiple sclerosis: the BCTRIMS consensus] [Brazilian Committee for Treatment and Research in Multiple Sclerosis]. / Recomendações quanto ao uso de drogas imunomoduladoras na esclerose múltipla: o consenso do BCTRIMS.

After brief considerations about clinical course and diagnosis in multiple sclerosis, the members of the BCTRIMS present some recommendations for the use of the immunomodulatory drugs in the treatment of this disease.

Coma and death in unrecognized Wernicke's encephalopathy. An autopsy study.

Eleven out of 36 autopsied cases of Wernicke's encephalopathy had developed coma. None of these patients had the diagnosis during life. There were six men and five women with ages ranging from 26 to 50 years (mean 36.6). Seven of these patients were heavy drinkers, three exhibited signs of severe malnutrition, whereas one was being evaluated for a disseminated gastric cancer and one was in treatment of hyperemesis gravidarum. Two patients were brought to the hospital after found unconscious at home. Neuropathological examination disclosed gross changes in the mammillary bodies in eight cases and microscopic changes in all cases. In one case there was atrophy of the anterior superior part of the vermis. Petechial hemorrhages were observed particularly in the walls of the third ventricle. Microscopically there were in addition to hemorrhages, glial proliferation, endothelial hypertrophy and necrosis of nerve cells and myelin. Central pontine myelinolysis was observed in one case. Wernicke's encephalopathy is a clinically underdiagnosed condition. Coma may mask its classical clinical picture or even be the sole manifestation. Although coma points to a poor outlook it may be reversed by thiamine administration. Any patient with coma of unknown etiology should be given parenteral thiamine.

Schilder's myelinoclastic diffuse sclerosis.

We report here a case of Schilder's myelinoclastic diffuse sclerosis in a 14-year-old girl with sudden bilateral visual loss. Computed tomographic scan showed two large symmetrical lesions in the occipital lobes and a smaller hypodense area in the frontal lobe. Cerebrospinal fluid examination revealed increased immunoglobulin G fraction with the presence of oligoclonal bands. Ultrastructural study of a biopsy specimen disclosed a demyelinating disorder with no cytoplasmic inclusions. Steroid treatment was followed by a dramatic response, with almost complete visual recovery and shrinkage of the lesions.

[Pseudohypertrophic myopathy caused by cysticercosis. Report of a case]. / Miopatia pseudo-hipertrófica por cisticercose. Registro de caso.

A 25 years old woman was admitted with a history of apparent hypertrophy of the calves, specially on the left, slight pain in the legs and difficulty in walking. Electromyography showed giant motor unit potentials with complete interference pattern. Biopsy of both gastrocnemii was performed revealing a cysticercus among inflammatory infiltrate and changes of the muscle fibers. Review of the literature disclosed 12 other reported cases. The age ranged from 10 to 35 years with a median of 25 years. Pseudohypertrophic myopathy due to cysticercosis has been found twice more common in males than in females. History of epilepsy and muscle pain occurs in about one half of the cases and muscle weakness in about one third of them. Usually there is simultaneous involvement of the upper and lower limb girdles. Myotonia is rare but subcutaneous nodules are frequently found. Our case is unique in the literature in which the pseudohypertrophy was confined to the legs and electromyography showed giant motor unit potentials. The pathogenesis of this condition is discussed and attention is called to the differential diagnosis with other pseudohypertrophic muscular conditions such as pseudohypertrophic muscular dystrophy, myotonia congenita, trichinosis, hypothyroidism, amyloidosis and glycogenosis of type I (Pompe's disease) in its juvenile form.

The central nervous system leukemia: a clinical and pathological study.

Post-mortem clinical and pathological study of 18 cases of central nervous system leukemia showed that this complication occurred mostly in chronic myelogenous leukemia (38.8%). No diagnostic criteria was found. The great majority of signs and symptoms were related to either disturbances of the mental status or cranial nerves dysfunction. Cerobrospinal fluid may be found normal. CNS involvement may occur at any time during the course of systemic leukemia, when the disease is under apparently good therapeutic control as well as during relapse. Pathological findings in order of decreasing frequency were: parenchymal hemorrhage (61%); subarachnoid hemorrhage (55%); meningeal infiltrates (44%); leukostasis (28%); edema and herniation (28%); parenchymal infiltrates (22%); ischemic infarcts (17%); progressive multifocal leucoencephalopathy (10%); calcifications (5%); meningitis (5%). Total survival time ranged from 8 to 1980 days a median of 300 days. Survival time after CNS involvement ranged from 1 to 180 days with a median of 21 days.

Tuberculomas intracranianos: estudo clinico e patologico. / Intracranial tuberculoma: clinical and pathological aspects

Vinte e quatro tuberculomas intracranianos foram encontrados em 17 pacientes numa analise retrospectiva de 19.760 autopsias e 72.972 biopsias consecutivas, num periodo de 41 anos e 4 meses. Os 17 pacientes representaram 4,2% dos casos das lesoes expansivas intracranianas. A maioria dos tuberculomas se localizava nos hemisferios cerebrais, e um terco deles, no cerebelo. Cerca de 65% dos pacientes tinham idade inferior a 20 anos. O quadro clinico e semelhante ao das outras lesoes expansivas intracranianas estando relacionado a hipertensao intracraniana e a localizacao da lesao. Os dados encontrados sao comparados aos das varias series publicadas em diferentes paises

[Primary intracranial tumors: analysis of a series of consecutive autopsies and biopsies]. / Tumores primários intracranianos: análise de uma série de autopsias e biopsias consecutivas.

A retrospective study of primary intracranial tumors found in 19.760 consecutive autopsies and 72.972 consecutive biopsies during a period of over 41 years was carried out. Two hundred and five neoplasms comprising 69,7% of the primary intracranial tumors were located in the supratentorial compartment whereas 84 tumors (28,6%) were infratentorial in site. Gliomas constituted the largest group of primary tumors comprising 60,9% of the intracranial primary neoplasms. Most of gliomas were found in the cerebral hemispheres, particularly in the frontal lobes. Of the total number of gliomas 40,2% were astrocytomas, 29,6% glioblastomas, 10,6% ependymomas, 10,1% medulloblastomas, 5% oligodendrogliomas and 2,2% choroid plexus papillomas. Meningiomas constituted the second most common primary intracranial tumors being found in 56 cases (19%), most of them in parasagittal region, sphenoid ridge and anterior fossa. In the sella region there were 39 neoplasms including 21 pituitary adenomas, seven meningiomas, five craniopharyngiomas, four epidermoid cysts and two teratomas. Six capillary hemangioblastomas of the cerebellum and two chordomas of the clivus were also found. Sixty-five tumors occurred in patients under 15 years of age, 53,3% of them infratentorial in location--36% in the cerebellum, 9,3% in the brainstem and 8% in the fourth ventricle. The majority of these tumors were astrocytomas, medulloblastomas and ependymomas. The present data are discussed in relation to other pathological series found in the literature.

Tumores primarios intracranianos: analise de una serie de autopsias e biopsias consecutivas. / Primary intracranial tumors: analysis of a series of consecutive autopsies and biopsies

Entre 19.760 autopsias e 72.972 biopsias consecutivas, num periodo de 41 anos e 4 meses, foram encontrados 294 tumores intracranianos primarios representando 74,8% das lesoes expansivas intracranianas. Cerca de dois tercos destes tumores se localizavam no compartimento supratentorial. Os gliomas foram os tumores mais frequentemente observados, seguidos pelos meningiomas, adenomas hipofisarios, tumores de origem embrionarias e tumores vasculares. Na regiao selar foram diagnosticados 39 tumores, incluindo os tumores intracelares supraselares e paraselares. A maior parte destes tumores foi constituida pelos adenomas hipofisarios, seguidos pelos craniofaringiomas, meningiomas, cistos epidermoides e teratomas. Setenta e cinco tumores foram verificados em pacientes com idade inferior a 15 anos,sendo a maioria deles na fossa posterior, principalmente no cerebelo. Estes tumores foram representados pelos astrocitomas do cerebelo e tronco encefalico, meduloblastomas e ependimomas do quarto ventriculo. Os dados obtidos foram comparados com as varias series encontradas na literatura

Effects of tenotomy on the muscle of the turtle: a histochemical study.

Tenotomized turtle muscles exhibited some changes similar to those found in mammalian tenotomized muscles. These changes included presence of small rounded and angulated fibres with high oxidative reaction, acute necrosis of fibres with degenerative changes, perivascular cellular reaction, phagocytosis and the atrophy of all 3 types of fibres. In contrast to mammalian tenotomized muscles, no decrease in PAS positive material and phosphorylase activity was noticeable. Also, the central core fibres and nemaline rods were absent in tenotomized turtle muscles.

Primary pigmented carcinoma of the choroid plexus. A light and electron microscopic study.

Pigmented carcinoma of the choroid plexus was found in a 33-year-old man. Autopsy revealed no primary tumor in the skin or eyes. A slightly cystic pigmented tumor was present in the right lateral ventricle infiltrating the thalamus and striatum. Metastatic implants were found in both temporal lobes and the cerebellum. The spinal cord was covered by black meningeal nodules, and the cauda equina was completely encased by the tumor. Metastases were found in the liver, pancreas, and kidney. Microscopically the mass contained normal choroid plexus with psammoma bodies adjacent to carcinomatous epithelium forming prominent papillae and tubules. Melanin granules were found within the neoplastic cells and in the stroma. Electron microscopy demonstrated melanin granules in various stages of development in the cells, which were joined one to another by desmosome-like structures. This is the second reported case of pigmented carcinoma of the choroid plexus and the first with metastases outside the central nervous system.