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Tuberous Sclerosis-Reply

Roger C. Duvoisin, M.C.
JAMA. 1961;176(12):1052-1053. doi:10.1001/jama.1961.03040250078026.
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To the Editor:—  The report of 3 cases of tuberous sclerosis without mental defect (JAMA175:869, 1961) was presented to emphasize the occurrence of relatively benign cases lacking the most familiar manifestations of the disease. It was not the occasion for a discussion of the several lesions which may appear in many different organs in this protean disorder. However, we appreciate Dr. Johnson's comments and indeed feel that the retinal lesion to which he refers deserves emphasis as an aid in detecting atypical or oligosymptomatic cases of tuberous sclerosis. Similarly, various cutaneous lesions such as the "shagreen skin," paraungual fibromata, cafe-au-lait spots, and anemic nevi may help betray the presence of a neurocutaneous syndrome.Unfortunately, the incidence among patients having tuberous sclerosis of the retinal lesion described by Van der Hoeve (Trans Ophthal Soc U K43:534, 1923) is as uncertain as that of its other manifestations. The


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