keepgoing
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A 10-year-old boy with history of epilepsy and mental retardation is brought to a specialty clinic for evaluation.Physical examination is remarkable for several ovoid hypopigmented areas on the trunk and large numbers of red and yellow papules on the face, particularly near the mouth. Biopsy of the papules demonstrates angiofibromata. This patient is most likely to have which of the following central nervous system pathologies?
A. Acoustic neuromas
B. Capillary hemangioblastomas
C. Herniation of cerebellar tonsils into foramen magnum
D. Large cortical hamartomas
E. Leptomeningeal angiomatosis
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| shalini.ravi
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e...where r u gettin these qs from KG?
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| oldkidontheblock
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A
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| new_n_lost
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D. Large cortical hamartomas Tuberous Sclerosis Classical Presentation
A. Acoustic neuromas NeuroFibromatosis 2
C. Herniation of cerebellar tonsils into foramen magnum Aronld- Chiari 1
E. Leptomeningeal angiomatosis Sturger - Weber Syndrome no skin manifestations.
B. Capillary hemangioblastomas - Classical VHL disease.
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| mytime
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several ovoid hypopigmented areas on the trunk ---------TS...ash-leaf like. Rest KG knows! 
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| keepgoing
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Hmm good try folks and yes MT u pointed to good signs...here is full answer...
The correct answer is D. The disease is tuberous sclerosis. The facial angiofibromata are also called adenoma sebaceum, and the hypopigmented patches on the trunk are called ash-leaf spots. This disease is one of the neurocutaneous disorders called phacomatoses. Tuberous sclerosis is inherited as an autosomal recessive trait, and epilepsy and mental retardation are commonly seen in this disorder. Large, firm, white hamartomatous nodules (tubers) are seen in the cortex and in subependymal sites. The tubers consist of aberrantly arranged neurons and/or glia. Patients may also have pancreatic cysts, renal angiomyolipomas, and cardiac rhabdomyomas. Rarely, an astrocytoma will arise in a tuber.
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| hanwin
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Classic tuberous sclerosis.
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