Associate Professor, faculty of Neurology, School of Medicine, Isfahan University of Medical Sciences
Abstract
Tuberous Sclerosis(TSC) Neurocutaneous syndromes or phakomatosis are inherited or sporadic conditions that feature lesions of both skin and nervous system TSC is inherited as an autosomal dominant and affects the brain, skin, kidney, heart and other organs. Cutaneous lesions of TSC include hypomelanotic maculae, the shagreen patch, ungual fibromas and facial angiofibroma (adenoma sebaceous); Neurological features of TSC include mental retardation, seizures and behavioral abnormalities; Retinal features of TSC include retinal hamartomas; Systemic features include cardiac rhabodomyoma, renal angiomyolipoma, and dyspnea and spontaneous pneumothorax
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