<prompt> A child presents to the clinic with history of seizures and mental retardation. Clinical examination reveals multiple hypopigmented macules. What is the likely diagnosis:Tuberous SclerosisNeurofibromatosisSturge Weber SyndromeLinear epidermal nevus syndrome  <response>: Answer is A Tuberous Sclerosis: The presence of hvopigmented macules in the background of seizures and mental retardation suggests the diagnosis of Tuberous Sclerosis. Often hypopigmented macules are the initial clue to tuberous sclerosis, a neurocutaneous disorder inherited in an autosomal dominant manner. Any child with unexplained seizures should be examined carefully for cutaneous clues, paicularly hypopigmented macules. The hypopigmented lesions are small, ovoid and scattered and their number varies. -- Oskis Pediatrics 4th861 The off white hypomelonotic macules are seen more easily in tanned or dark skinned individuals. The macules often are oval or ash leaf in shape and follow dermatomes. In infancy the presence of these macules accompanied by seizures is viually diagnostic of Tuberous Sclerosis Tuberous Sclerosis Autosomal Dominant Neurocutaneous Disorder Cutaneous Features Neurological Features Other Features Hypopigmented maculesAsh-leaf spotsdeg Seizuresdeg Renal Lesions - Seen in 90 of cases - Seizures are the most common Renal cysts or angiomyolipomas - Are small, ovoid, scattered and variable presenting symptom may occur Hematuria in number - Infantile spasms may be the obstruction - Larger lesions are known as Ash-leaf presenting feature during Cardiac Lesions spots as these may have jagged edges infancy Rhabdomyomas of the hea may resembling a leaf Mental Retardationdeg occur Shagreen Spotsdeg - Mental Retardation occurs in Pulmonary Lesions - Roughened, raised, leathery lesions with an orange peel consistency Adenoma Sebaciume - Facial skin hamaoma that develops in upto 50 of patients referred to teiary care - Patients with seizures are more prone to mental retardation Lymphagiomyomatosis is the classical pulmonary lesion Rarely cystic lung disease may occur Eye lesions The charachteristic brain lesion is a coical Tuber. The most common neurological early childhood -- 4-6 years of age - Appears as tiny red nodules over nose Retinal Hamaomas may occur Skeletal lesions and cheeks resembling acne Facial Angiofibromas Ungual Fibromas Facial angiofibromas may seen ungula fibromas are more common in toes Cafe- au- lait spots include manifestations seizures, seizures cognitive impairment and behavioral abnormalities . including autism. Cystic rarefaction of the bones or the fingers or toes. These may be seen occasionally T. S. Classical Triad described includes Seizures, Mental Retardation and Adenoma Sebacium occurs in 33 patients T.S should be suspected in any child presenting with unexplained seizures who shows hjpopigmented cutaneous macules. 