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Aim  Neurocutaneous melanocytosis is a rare neurocutaneous syndrome defined by the presence of large and/or multiple congenital cutaneous nevi and melanocytic deposits in the central nervous system. We sought to define the spectrum of central nervous system abnormalities in children with neurocutaneous melanocytosis. Method  We retrospectively reviewed cases of neurocutaneous melanocytosis referred to the pediatric neuro‐oncology service at our center from 2003 to 2010. Results  Of 14 patients (11 males, 3 females) identified, eight were living. Median age of survivors was 31 months (range 12mo–6y 10mo) while median age of death was 81 months (19mo–28y). Of the six patients who died, all had diffuse leptomeningeal melanocytic deposits and four had leptomeningeal melanoma. All patients had neuroimaging: six had findings suggestive of diffuse leptomeningeal melanocytosis; seven had multifocal melanocytic deposits; and one patient had normal neuroimaging but focal seizures. Spinal abnormalities were common: three patients had extensive dorsal spinal arachnoid cysts and one had a benign cervical spindle cell tumor. Seven patients had epilepsy. Three patients had profound developmental delay; the other 11 patients had no or mild delay. Interpretation  Children with neurocutaneous melanocytosis exhibit a wide range of intracranial and intraspinal abnormalities and variable clinical outcomes.

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Rationale and Objectives Delayed cerebral ischemia (DCI) is a devastating condition that occurs secondary to aneurysmal subarachnoid hemorrhage (A-SAH). The purpose is to compare CT perfusion (CTP) and digital subtraction angiography (DSA) for determining DCI in A-SAH. Materials and Methods A retrospective study of A-SAH patients admitted at our institution between December 2004 and December 2008 was performed. CTP and DSA were obtained at days 6–8 following aneurysm rupture. Both qualitative and quantitative analyses of CT perfusion deficits were performed. DSA was categorized as presence or absence of vasospasm. The reference standard for determining DCI was based on clinical deterioration or infarction on CT or MRI. The test characteristics of CTP and DSA were calculated and their graphs of conditional probabilities were constructed using Bayesian analysis. Results Fifty-seven patients were included; 79%(45/57) had DCI. Seventy percent(40/57) had CTP perfusion deficits; 80%(36/45) of the DCI and 33%(4/12) of no DCI patients. Sixty-three percent(36/57) had DSA demonstrating vasospasm; 73%(33/45) of the DCI and 25%(3/12) of no DCI patients. Quantitative analysis of the CTP data revealed a significant difference in cerebral blood flow values for the DCI (29.4 mL/100gm/min) and no DCI groups (40.5 mL/100gm/min, p=0.0213). The sensitivity, specificity, positive and negative predictive values for CTP were 0.80(95% CI 0.68–0.92), 0.67(95% CI 0.40–0.93), 0.90(95% CI 0.82–0.96), 0.47(95% CI 0.27–0.62) and for DSA were 0.73(95% CI 0.60–0.86), 0.75(95% CI 0.50–0.99), 0.92(95% CI 0.82–0.98) and 0.43(95% CI 0.26–0.53), respectively. Conclusion CTP and DSA have similar test characteristics and Bayesian analysis for determining DCI in A-SAH patients.