related to hypothalamic dysfunction; its main features are emaciation, despite a normal or slightly diminished caloric intake, and an alert appearance. DS has been Leonardo Salviati, M.D. ber 1991 and January 1996, are presented in this article. The children were boys, 2 Division of Diagnostic Imaging, Pediatric Neuroages 6, 7, and 18 months, respectively.
Oncology Program of the Department of Pediatrics
RESULTS.In all three patients, the initial contrast-enhancing MRIs of the head of the University of Padua, Padua, Italy.showed evidence of tumor dissemination. This finding prompted a study of the 3 Neurobiology Research Unit, Pediatric Neurospine, which in turn showed tumor deposits in all three subjects. Among the 43 Oncology Program of the Department of Pediatpatients younger than 16 years with low grade astroctyoma who consecutively rics of the University of Padua, Padua, Italy.entered the Neuro-Oncology Program during the study period, these 3 patients 4 Division of Pathology, Pediatric Neuro-Oncolwere the only ones who had disseminated tumors. ogy Program of the Department of Pediatrics of CONCLUSIONS. In this study, the hypothesis was formulated that DS and disseminthe University of Padua, Padua, Italy.ated hypothalamic-optic chiasm JPA tend to be more commonly associated than 5 Division of Child Neurology, Pediatric Neuropreviously stated. This study suggests that the initial contrast-enhanced MRI of Oncology Program of the Department of Pediatthe head of a child affected by DS and hypothalamic JPA must be looked at carefully rics of the University of Padua, Padua, Italy.for evidence of tumor dissemination, and that the spine must also be examined cludes old reports of nondiencephalic tumors associated with DS, 1,3 this rare neurologic disease is almost exclusively described in associa-