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The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions. The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum(More)
Two sisters with autosomal recessive cerebellar hypoplasia and severe nonprogressive retinal pigmentary disease are presented. This syndrome has been previously described in only 1 patient. The retinal changes may be difficult to discern and we suggest that all patients with congenital ataxia have a detailed ophthalmologic assessment, including(More)
Magnetic resonance imaging brain scans and neuropsychological assessments of 17 children who met the NIH consensus diagnostic criteria for neurofibromatosis Type 1 were carried out in order to determine if there is a relationship between presence of high intensity signal abnormalities on MRI scans and nonverbal cognitive deficits. Cranial MRI scans in 10(More)
The role of surgery in the management of sinus disease is in a state of evolution. With the advent of the endoscope, functional sinus surgery has become a reality. This type of surgery is recently being utilized as a therapeutic modality for sinus disease in children. The sinus surgeon should be aware of the differences between adult and pediatric sinus(More)
Seven adults with Listeria monocytogenes infection of the central nervous system had computed X-ray tomography of the head performed. One patient had a normal scan, three had hydrocephalus; cerebral edema, an arachnoid cyst and hyperemia were each noted in one patient. Atrophy of the vermis of the cerebellum was demonstrated on follow-up scans in two(More)