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.

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

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

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

Leopard syndrome, a neural crest disorder: a case report.

A sindrome de Leopard e uma condicao autosoma dominante de variavel expressao fenotipica.Uma minoria apenas dos pacientes apresenta todos os componentes da sindrome, ou seja, lentigenes multiplas (L), alteracoes eletrocardiograficas (E), hipertelorismo ocular (O) estenose pulmonar (P), anormalidades genitais (A), retardo de crescimento (R), e surdez neurosensorial (D). Descrevemos o caso de uma crianca com todos os componentes da sindrome alem de outras anomalias nao descritas anteriormente, tais como macroglossia, alteracoes dentarias, megacolon, ectopia anal e hipertrofia do clitoris, impressao basilar e platibasia. A presenca de alteracoes dentarias e megacolon, representando envolvimento das papilas dentarias e do plexo mioenterico, derivados da crista neural,constitui nova evidencia que favorece a hipotese de ser a sindrome de Leopard resultante de anomalia dos elementos da crista neural