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 ...

Tuberous sclerosis is 2nd most common neurocutaneous syndrome. Bilateral renal angiomyolipoma in tuberous sclerosis is a rare entity. Reporting two cases of tuberous sclerosis with bilateral renal angiomyolipomas.

Sturge Weber syndrome is a rare sporadic neurocutaneous syndrome. It has extra -oral manifestations mostly in form of unilateral port wine stain on face that are ipsilateral to intra-cranial lesions (mostly lepto-meningeal angioma), seizures, glaucoma and intra-orally by hemangioma gingiva. We case report child with facial nevus opposite lesion, intra-oral gum hypertrophy. Other than contralate...

epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. multicystic kidney disease has been very rarely reported in this syndrome. here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with wilms' tumor. according to this ...

Introduction: Sturge-Weber Syndrome (SWS) is a rare neurocutaneous syndrome that is manifested by overt neurological and covert psychiatric features. Although the syndrome is known to be neurocutaneous, multiple organs and systems are involved. Case Presentation: A 45-year-old male, with type I SWS was admitted to the psychiatric ward with manic-like symptoms. The case had a history of repeated...

Hypomelanosis of Ito (HI) is a rare neurocutaneous disorder most likely caused by chromosomal mosaicism. HI patients may suffer from numerous clinical manifestations, but the expression of the disease is highly variable. Ophthalmologic, musculoskeletal, neurologic, and dental anomalies may be associated with the syndrome. The dental abnormalities found in HI include talon cusps, a single maxill...

objective incontinentia pigmenti (ip) (bloch_sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. the diagnosis of ip is performed based on clinical features and the family history with the support of histological findings. we repor...

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. ...