Neurocutaneous disorders are multisystem diseases affecting skin, brain, and other organs. Epilepsy is very common in the neurocutaneous disorders, affecting up to 90% of patients with tuberous sclerosis complex (TSC) and Sturge-Weber syndrome (SWS), for example. The mechanisms underlying the increased predisposition to brain hyperexcitability differ between disorders, yet some molecular pathwa...

On 9 November 2004 the Round Table Conference on Medical Care Management of Patients with Neurocutaneous Syndromes was held in Warsaw. The Conference was organised by the Polish Neurocutaneous Syndromes Clinics Initiating Group and the Children’s Memorial Health Institute in Warsaw. The heads of the departments involved in the treatment of patients with phakomatoses and delegates of the Polish ...

Neurocutaneous melanosis is a rare neurocutaneous syndrome defi ned by the presence of multiple and /or giant congenital cutaneous nevi and melanocytic deposits in the central nervous system with the infi ltration of leptomeninges. The major medical concern with giant congenital cutaneous nevi is high risk of developing cutaneous melanoma, leptomeningeal melanoma, and neurocutaneous melanosis. ...

Radiol Bras. 2016 Nov/Dez;49(6):406–413 412 http://dx.doi.org/10.1590/0100-3984.2015.0139 the lesions by the paramagnetic contrast agent. Examination of the cerebrospinal fluid showed that there was no infection with Cryptococcus or any other agents, indicating an effective response to the treatment. The combination of clinical and radiological worsening, despite an effective treatment response...

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We describe imaging findings in a 2-year-old girl with neurocutaneous melanosis and malignant cerebral melanoma. Because the cerebral melanoma in this child was of the amelanotic type, high-signal intensity on unenhanced T1-weighted images was not present. The cutaneous lesions played a crucial role in establishing a correct (presumed) histopathologic diagnosis on the basis of the imaging findi...