Clinical involvement of the nervous system in visceral larva migrans due to

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Summary:Purpose: Familial periventricular heterotopia (PH) represents a disorder of neuronal migration resulting in multiple gray-matter nodules along the lateral ventricular walls. Prior studies have shown that mutations in the filamin A (FLNA) gene can cause PH through an X-linked dominant pattern. Heterozygotic female patients usually remain asymptomatic until the second or third decade of life, when they may have predominantly focal seizures, whereas hemizygotic male fetuses typically die in utero. Recent studies have also reported mutations in FLNA in male patients with PH who are cognitively normal. We describe PH in three male siblings with PH due to FLNA, severe developmental regression, and West syndrome.Methods: The study includes the three affected brothers and their parents. Video-EEG recordings and magnetic resonance image (MRI) scanning were performed on all individuals. Mutations for FLNA were detected by using polymerase chain reaction (PCR) on genomic DNA followed by single-stranded conformational polymorphism (SSCP) analysis or sequencing.Results: Two of the siblings are monozygotic twins, and all had West syndrome with hypsarrthymia on EEG. MRI of the brain revealed periventricular nodules of cerebral gray-matter intensity, typical for PH. Mutational analyses demonstrated a cytosine-to-thymidine missense mutation (c. C1286T), resulting in a threonine-to-methionine amino acid substitution in exon 9 of the FLNA gene.Conclusions: The association between PH and West syndrome, to our knowledge, has not been previously reported. Males with PH have been known to harbor FLNA mutations, although uniformly, they either show early lethality or survive and have a normal intellect. The current studies show that FLNA mutations can cause periventricular heterotopia, developmental regression, and West syndrome in male patients, suggesting that this type of FLNA mutation may contribute to severe neurologic deficits.

-In spite of the steady increase in the incidence of Staphylococcus aureus infections, it remains a relatively uncommon cause of meningitis. To our knowledge, no series of community-acquired S. aureus meningitis (CASAM) restricted to children has been published. So far in this retrospective study we report our experience with CASAM in children, hospitalized from 1983 to 1998 at Nossa Senhora da Glória Children's Hospital (HINSG). During the sixteen-year study period, 2,319 new cases of acute pyogenic meningitis were diagnosed at HINSG. Community-acquired S. aureus was identified as the causative agent in 30 patients (1.3 percent). The predominantly spinal localization of the agent is stressed. In contrast with publications which analyze adults, it has a better prognosis.

Objective: To identify prognostic factors predicting a fatal outcome in HIV-negative children with neurotuberculosis based on clinical, epidemiological, and laboratory findings. Method: The clinical records of all in-patients diagnosed with neurotuberculosis from 1982 to 2005 were evaluated retrospectively. The following prognostic parameters were examined: gender, age, close contact with a tuberculosis-infected individual, vaccination for bacillus Calmette-Guérin, purified protein derivative (PPD) of tuberculin results, concomitant miliary tuberculosis, seizures, CSF results, and hydrocephalus. results: One hundred fortyone patients diagnosed with neurotuberculosis were included. Seventeen percent of the cases resulted in death. The factors that were correlated with a negative outcome included lack of contact with a tuberculosis-infected individual, negative PPD reaction, coma, and longer hospitalisation time. A multiple logistic regression analysis was performed to identify which of these factors most often resulted in death. conclusion: Coma at diagnosis, lack of tuberculosis contact, and a non-reactive PPD were the most important predictors of fatality in patients with neurotuberculosis. Key words: tuberculosis, meningitis, central nervous system, prognosis, child.Fatores prognósticos de letalidade da neurotuberculose em crianças HIV-negativas resuMO Objetivo: Identificar elementos prognósticos para a letalidade da neurotuberculose na criança, a partir das manifestações clínicas, dados epidemiológicos e laboratoriais. Método: Registros de pacientes internados durante o período de 1982 a 2005 foram retrospectivamente avaliados. Os elementos prognósticos considerados foram: sexo, idade, história de contato íntimo com indivíduo com tuberculose, vacinação com o bacilo de Calmette-Guérin (BCG), teste tuberculínico (PPD), concomitância de tuberculose miliar, convulsões, resultados da análise do LCR e presença de hidrocefalia. resultados: 141 pacientes com diagnóstico de neurotuberculose foram incluídos. Dezessete por cento dos pacientes foram a óbito. Os fatores associados ao óbito foram história negativa de contágio, ausência de reatividade ao teste de PPD, coma e tempo de internação prolongado. Análise por regressão logística múltipla foi usada para investigar as relações entre os elementos prognósticos e o desfecho óbito. conclusão: Os fatores prognósticos na previsão de óbito nos pacientes com neurotuberculose foram a presença de coma no momento do diagnóstico, a ausência de história de contágio e a ausência de reação ao PPD. Palavras-chave: tuberculose, meningite, sistema nervoso central, prognóstico, crianças. Tuberculosis remains one of the most severe infectious diseases and more than eight million new cases are diagnosed worldwide each year. The disease is especially prevalent in developing countries affected by poverty, malnutrition, and over-

Os autores apresentam 69 casos de afecções respiratórias em crianças que atribuem a agentes não bacterianos, provavelmente virais. Usam para isto um critério clínico, outro morfológico, em uma revisão de 372 pneumopatias infecciosas em casos de autópsias. Caracterizaram morfologicamente a resposta à agressão viral pela presença de: infiltrado mononuclear intersticial, predominantemente peribronquilar; alterações degenerativas ou mesmo necrose e hiperplasia do epitélio respiratório; membrana hialina; descamação epitelial; células gigantes sinciciais alveolares e bronquiolares; inclusões nucleares e citoplasmáticas; edema proteináceo alveolar e septal, proliferação intersticial conjuntiva incipiente. Criticam o erro por excesso de diagnósticos de "pneumonia mononuclear intesticial" e o erro por falta quando o acometimento bacteriano dificulta o diagnóstico de lesão atribuível a vírus. Além disso realçam a importância de achado de bonquiolite aguda como fundamental para o diagnóstico. Estas lesões - ao lado de achados clínicos-radiológicos e epidemiológicos - cosntituem o que a experiência adquirida julga como reação do pulmão a vários vírus conhecidos (Adenovírus, Influenza, Parainfluenza, Vírus Sincicial Respiratório e Sarampo).
69 cases of respiratory diseases in children are presented and atributed to non-bacterial agents, probably viral. Using clinical and morphological criteria, a review of 372 cases of infections lung diseases was performed in autopsy material. The reaction due to the viral injury is characterized morphologically by interstitial mononuclear infiltration, mainly peribronchiolar; degenerative changes, necrosis and hyperplasia of the respiratory epithelium; hyaline membrane; epithelial desquamation; syncytial giant-cells, alveolar or bronquiolar; nuclear and cytoplasmic inclusions; proteinaceous edema, alveolar and septal; mild intersticial conective tissue proliferation. The overdiagnosis of "mononuclear intesticial pneumonia' is criticized; on the other hand, attention is called to the underdiagnosis of viral pneumonia when bacterial complication difficults its bronchiolitis for morphological diagnosis of viral disease of the lung is stressed. Those lesions - correlated the clinico-radiological and epidemiological findings - constitute which the acquired experience judge as the reaction of the lung to several known viruses (adenovirus, influenza, parainfluenza, sysncytial respiratory virus and measles)

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