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Tuberous Sclerosis
Richard P. Morse, MD
Arch Neurol. 1998;55:1257-1258.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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INTRODUCTION
Embryological studies have shed light on the common origin of the nervous system and skin, both derived from ectoderm, and clinicians have delineated these associations most notably in disorders termed neurocutaneous syndromes.1 This essay traces the historical threads that were ultimately woven together into the concept of tuberous sclerosis as a neurocutaneous disorder. These citations focus on clinical and patient descriptions from the 19th and early 20th centuries.
EARLY DESCRIPTIONS: SKIN AND SYSTEMIC SIGNS
Although there were earlier possible descriptions of the dermatologic findings,1-2 tuberous sclerosis complex was not recognized as a neurocutaneous syndrome until the 19th century.
The most obvious manifestation being dermatologic, it is not surprising that the earliest records depict the characteristic facial angiofibromata. Pierre-Olive François Rayer, a French dermatologist, published a text in 1835 (on diseases of the skin)3 in which he described and illustrated in color a man with facial erythematous papules (Figure 1):
Figure appears in full text version.
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CEREBRAL PATHOLOGY
From the Department of Pediatrics, Division of Child Neurology, Duke University Medical Center, Durham, NC.
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