Journal of child neurology
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Case Reports
Toxic pediatric parkinsonism: report of a child with metabolic studies and response to treatment.
We successfully treated a child with parkinsonism secondary to cytosine arabinoside with levodopa/carbidopa and attempted to elucidate the pathogenesis of the disorder by sequential analysis of cerebrospinal fluid biogenic amines, sequential magnetic resonance imaging, and fluorodopa positron emission tomography, which revealed both permanent and reversible biochemical and structural alterations. The parkinsonian disorder resolved in 9 months. Metabolic changes and structural abnormalities on imaging studies were chronologically correlated with the clinical course.
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The use of alternative therapies in immigrant children with stroke and cerebrovascular diseases has not been reported. We identified immigrant and first-generation Chinese-Canadian children with stroke and cerebrovascular diseases in a pediatric stroke clinic and used standardized questionnaires to interview their parents about alternative therapy use. The chi-square test was used to assess the associations of parental educational level and foreign birth in the child with alternative therapy use. ⋯ Three families reported feeling uncomfortable discussing alternative therapy use with their physicians. We did not see an association between alternative therapy use and parental educational level or foreign birth in the child. Physicians should be aware that parents of children with stroke may be using alternative therapies to treat their children and may be unwilling or unable to report all therapies used.
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Pelizaeus-Merzbacher disease and X-linked spastic paraplegia type 2 are two sides of the same coin. Both arise from mutations in the gene encoding myelin proteolipid protein. ⋯ The diverse disease spectrum is mirrored by the underlying pathogenesis, in which a blockade at any stage of myelin proteolipid protein synthesis and assembly into myelin spawns a unique phenotype. The continuing definition of pathogenetic mechanisms operative in Pelizaeus-Merzbacher disease and spastic paraplegia type 2, together with advances in neural cell transplant therapy, augurs well for future treatment of the severe forms of Pelizaeus-Merzbacher disease.
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Sturge-Weber syndrome is a neurocutaneous disorder classically presenting with a facial port-wine stain, vascular eye abnormalities, and an ipsilateral occipital leptomeningeal angioma. Children with Sturge-Weber syndrome often develop progressive neurologic problems. Data on the pathophysiology of Sturge-Weber syndrome are briefly reviewed. ⋯ Studies suggest that complex molecular interactions contribute to the abnormal development and function of blood vessels in Sturge-Weber syndrome. Neurologic deterioration in Sturge-Weber syndrome is likely secondary to impaired blood flow to the brain and is worsened by the presence of seizures. Insights from related areas are discussed, and future research studies are suggested.