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Neurosarcoid is seldom recognized in children. In the absence of any large pediatric series, it has been assumed that the presenting signs and symptoms are identical in adults and children.To test the hypothesis that childhood neurosarcoid differs in presenting signs and symptoms from neurosarcoid in adults.We tabulated the initial neurologic signs and symptoms in all reported cases of childhood sarcoid with evidence of central nervous system involvement. These data then were compared with published studies of adult neurosarcoid.Twenty-nine cases (from the English, French, and German literature) had descriptions of presenting signs and symptoms. Ages were 3 months to 18 years; 48% (14 of 29) presented before 13 years. Seizures were the most common presenting symptom (38%, 11 of 29), and 73% of these children (8 of 11) were <13 years old at presentation. Twenty-one percent (6 of 29) had cranial nerve involvement at presentation, and all were >or=12 years old. Twenty-one percent (6 of 29) had hypothalamic dysfunction. Five children presented with headache, 4 with motor signs, and 3 with papilledema. Twenty-four percent (7 of 29) had mass lesions on imaging.Children with neurosarcoid present differently than do adults. Children are more likely to have seizures, less likely to have cranial nerve palsies, and perhaps more likely to have a space-occupying lesion. Our analysis of the cases available for review in the published literature suggests that children evolve to an adult pattern as they progress through adolescence.