RESUMO
JC virus (JCV) is an opportunistic virus known to cause progressive multifocal leukoencephalopathy. Anti-JC virus (Anti-JCV) antibody prevalence in a large, geographically diverse, multi-national multiple sclerosis (MS) cohort was compared in a cross-sectional study. Overall, anti-JCV antibody prevalence was 57.6%. Anti-JCV antibody prevalence in MS patients ranged from approximately 47% to 68% across these countries: Norway, 47.4%; Denmark, 52.6%; Israel, 56.6%; France, 57.6%; Italy, 58.3%; Sweden, 59.0%; Germany, 59.1%; Austria, 66.7% and Turkey, 67.7%. Prevalence increased with age (from 49.5% in patients < 30 years of age to 66.5% in patients ≥ 60 years of age; p < 0.0001 comparing all age categories), was lower in females than in males (55.8% versus 61.9%; p < 0.0001) and was not affected by prior immunosuppressant or natalizumab use.
Assuntos
Anticorpos Antivirais/sangue , Vírus JC/imunologia , Esclerose Múltipla/virologia , Infecções por Polyomavirus/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Criança , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/sangue , Esclerose Múltipla/tratamento farmacológico , Prevalência , Distribuição por Sexo , Adulto JovemRESUMO
Choroid plexus papilloma (CPP) is a rare, benign epithelial brain tumor of the nervous system seen particularly in infants. Familial cases are extremely uncommon. Some other form of malignant tumors was noted in the relatives of patients with CPPs, and some genetic defects regarding this coincidence were reported in the literature. These neoplasms are occasionally bilateral and hydrocephalus is an associated sign in most of the cases. We report three lateral ventricle CPPs in two siblings, at the age of 7 month and 2 years respectively. All tumors were resected with parietotemporal craniotomy and a superior temporal sulcus approach to the lateral ventricle. To avoid a concomitant need of ventriculoperitoneal shunt insertion, external ventricular drainage was inserted for a week in the postoperative period relieving symptoms of hydrocephalus. Search for a hereditary defect in the p53 gene of the second infant (7 months old) revealed no mutation. Postoperative courses were uneventful and the patients were followed for three years without any recurrence. Bilateral CPPS are rare and unusual in two siblings. A genetic predisposition such as the p53 mutation should be investigated in bilateral CPPs in particular.
Assuntos
Papiloma do Plexo Corióideo , Proteína Supressora de Tumor p53/genética , Derivação Ventriculoperitoneal , Sequência de Bases , Pré-Escolar , Humanos , Hidrocefalia/genética , Hidrocefalia/patologia , Hidrocefalia/cirurgia , Lactente , Ventrículos Laterais/patologia , Ventrículos Laterais/cirurgia , Imageamento por Ressonância Magnética , Masculino , Dados de Sequência Molecular , Papiloma do Plexo Corióideo/genética , Papiloma do Plexo Corióideo/patologia , Papiloma do Plexo Corióideo/cirurgia , IrmãosRESUMO
BACKGROUND: Pineoblastomas are very rare brain tumors in fetus and neonates, comprising only 0.9% of congenital brain tumors. The occurrence of multiple tumors of different histopathologic types in the same individual is a rare event, most often encountered in hereditary cancer syndromes. CASE REPORT: We report a female fetus presented with a congenital pineoblastoma at the 32nd week of gestation, with hydrocephalus and concurrent parameningeal embryonal rhabdomyosarcoma in early infancy. RESULTS: Cytogenetic analysis showed normal karyotype in the peripheral blood of the patient, and p53 mutational analysis revealed no germ line mutations. DISCUSSION: This is the first case with concurrent congenital pineoblastoma and parameningeal embryonal rhabdomyosarcoma in early infancy. We suggest that concurrence of these tumors could be due to mutations in other tumor suppressor genes or secondary to exposure to unknown in utero factors.