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PURPOSE Neurofibromatosis type 1 (NF1), a common genetic disorder, predisposes patients to the development of both benign and malignant tumors. Although the most common central nervous system (CNS) tumor is a low-grade pilocytic astrocytoma of the optic pathway, there have been sporadic reports of NF1 patients with more aggressive CNS lesions. We(More)
Optic pathway glioma (OPG), seen in 15% to 20% of individuals with neurofibromatosis type 1 (NF1), account for significant morbidity in young children with NF1. Overwhelmingly a tumor of children younger than 7 years, OPG may present in individuals with NF1 at any age. Although many OPG may remain indolent and never cause signs or symptoms, others lead to(More)
Optic pathway gliomas (OPGs) occur in 15%-20% of children with neurofibromatosis type 1 (NF1); up to half become symptomatic. There is little information regarding ophthalmologic outcomes after chemotherapy. A retrospective multicenter study was undertaken to evaluate visual outcomes following chemotherapy for NF1-associated OPG, to identify risks for(More)
BACKGROUND Although corticosteroids are commonly prescribed in the treatment of bronchiolitis, there is no evidence on the efficacy of these drugs in this disorder. We designed a randomised, double-blind, prospective study to assess the efficacy of dexamethasone in infants with bronchiolitis who require hospital management. METHODS Infants younger than 12(More)
Identification of new optic pathway tumors (OPTs) and progression of pre-existing OPTs in children with neurofibromatosis 1 (NF1) have been reported infrequently after age 6. The authors present eight children with NF1 (mean age 12.2 years) seen in three NF1 centers who had either late-onset (four of eight) or late-progressive (seven of eight) OPT.(More)
OBJECTIVE The goal of the Response Evaluation in Neurofibromatosis and Schwannomatosis Visual Outcomes Committee is to define the best functional outcome measures for future neurofibromatosis type 1 (NF1)-associated optic pathway glioma (OPG) clinical trials. METHODS The committee considered the components of vision, other ophthalmologic parameters(More)
Low grade gliomas affecting the visual pathway, commonly referred to as optic pathway gliomas (OPGs), have a relatively high survival rate but can cause significant vision loss. While previous treatment outcomes for tumors of the central nervous system have focused primarily on changes in tumor size or patient survival, more recently preservation of vision(More)
In his very informative and thoughtful discussion of a patient with copper deficiency, Dr Samuels refers to the checklist concept in considering the differential diagnosis. He cites a Google search of myeloneuropathy and malnutrition as helping in suggesting the diagnosis. 1 He further notes that searching on common nonspecific neurologic signs and symptoms(More)