Neurocutaneous melanosis (NCM) is a rare congenital disorder characterized by the presence of large or multiple congenital melanocytic nevi in association with benign or malignant proliferation of melonocytes in the leptomeninges. NCM is believed to occur as a consequence of an error in morphogenesis in neural ectoderm in the developing embryo. Animal models suggest that aberrant expression of the hepatocyte growth factor/scatter factor (HGF/SF) may be involved in the pathogenesis in the NCM. While the majority of patients with NCM have large congenital melanocytic nevi in a posterior axial distribution, a significant proportion of patients present with multiple smaller nevi in the absence of a single larger lesion. Neurologic manifestations generally occur within the first two years of life, and are often related to increased intracranial pressure. Associated structural anomalies of the CNS have been reported in NCM, particularly the Dandy-Walker complex. The long-term clinical significance of characteristic magnetic resonance findings in neurologically asymptomatic patients is unclear. Approximately half of NCM patients develop CNS melanoma. The prognosis of symptomatic patients remains poor. (C) 2004 Elsevier Inc. All rights reserved.
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Univ Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, ItalyUniv Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, Italy
Ruggieri, Martino
Polizzi, Agata
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Univ Catania, Dept Educ Sci, Chair Pediat, Catania, ItalyUniv Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, Italy
Polizzi, Agata
Catanzaro, Stefano
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Univ Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, Italy
Univ Catania, Unit Neonatol & Neonatal Intens Care Unit NICU, AOU Policlin, PO San Marco, Catania, ItalyUniv Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, Italy
Catanzaro, Stefano
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Lo Bianco, Manuela
Pratico, Andrea D.
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Univ Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, ItalyUniv Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, Italy
Pratico, Andrea D.
Di Rocco, Concezio
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Int Neurosci Inst INI, Pediat Neurosurg, Hannover, GermanyUniv Catania, Dept Clin & Expt Med, Unit Rare Dis Nervous Syst Childhood, Sect Pediat & Child Neuropsychiat, Catania, Italy
机构:University of Catania,Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry
Martino Ruggieri
Agata Polizzi
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机构:University of Catania,Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry
Agata Polizzi
Stefano Catanzaro
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机构:University of Catania,Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry
Stefano Catanzaro
Manuela Lo Bianco
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机构:University of Catania,Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry
Manuela Lo Bianco
Andrea D. Praticò
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机构:University of Catania,Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry
Andrea D. Praticò
Concezio Di Rocco
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机构:University of Catania,Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry