Aggarwal, Hari Krishan Pt. B. D. Sharma Postgraduate Institute of Medical Sciences - Department of Medicine, India , Jain, Deepak Pt. B. D. Sharma Postgraduate Institute of Medical Sciences - Department of Medicine, India , Jain, Promil Pt. B. D. Sharma Postgraduate Institute of Medical Sciences - Department of Medicine, India , Kaverappa, Vipin Pt. B. D. Sharma Postgraduate Institute of Medical Sciences - Department of Medicine, India , Kumar, Ashwani Pt. B. D. Sharma Postgraduate Institute of Medical Sciences - Department of Medicine, India , Yadav, Sachin Pt. B. D. Sharma Postgraduate Institute of Medical Sciences - Department of Medicine, India

Tuberous sclerosis - A case report and review of literature

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Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome characterized by involvement of multiple system including central nervous system, kidney, skin, heart, lungs, and eye. It is frequently associated with skin lesions (96%); seizures (90%) and mental retardation (70%). Most common lesions associated with TSC are angiofibroma of face, ashleaf macules, shagreen patches, subependymal nodules, cortical tubers, mental retardation, and angiomyolipomas of kidney. The small benign tumours in the brain of affected individuals are the cause of neurological manifestations of the disorder. As prognosis depends on the involvement of brain, kidney and other vital organs; routine screening of a patient with tuberous sclerosis is required to determine the involvement of other organ systems. Combined skills of physician, radiologists and psychologist are required to manage a patient with TSC.

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Tuberous sclerosis , angiomyolipomas , shagreen patches , subependymal nodules , ashleaf macules , seizures

Cumhuriyet Medical Journal

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