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Precocious Puberty in Tay-Sachs Disease

  • Chapter
Sphingolipids, Sphingolipidoses and Allied Disorders

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 19))

Abstract

Various neurologic causes of sexual precocity have been reported. Among these have been destructive pineal tumors (6,l9,25), hamartomas or hyperplasia of the tuber cinereum (8,14,31), hypothalamic tumors (34,36,40,48,53), tumors of the optic chiasm (12), encephalitis (16), miliary tuberculosis and tuberous sclerosis (55), the Sturge-Weber syndrome, porencephalic cysts, brain damage, craniostenosis, microcephaly, arrested hydrocephalus, and so-called idiopathic cases in which non-specific, but grossly abnormal electroencephalograms have been found (27). In addition, at this institution it has been noted that children with Tay-Sachs disease not uncommonly manifest premature thelarche and/or pubarche (unpublished observations). In an effort to elucidate the pathogenesis of these complete and incomplete forms of precocious puberty, as well as to detect any other hitherto nsuspected hormonal abnormalities, detailed endocrine evaluations of 2 female children with Tay-Sachs disease were carried out.

This work was supported in part by a grant from the National Tay-Sachs and Allied Disease Association, Inc.

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Authors and Affiliations

  1. Dept. of Medicine & Isaac Albert Research Inst., Kings-brook Jewish Medical Center, Brooklyn, N.Y., 11203, USA

    Richard Relkin M.D.

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  1. Richard Relkin M.D.
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Editors and Affiliations

  1. Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center and Department of Pathology, Downstate Medical Center, State University of New York, Brooklyn, New York, USA

    Bruno W. Volk

  2. Department of Pathology, Miriam Hospital and Brown University, Providence, Rhode Island, USA

    Stanley M. Aronson

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Relkin, R. (1972). Precocious Puberty in Tay-Sachs Disease. In: Volk, B.W., Aronson, S.M. (eds) Sphingolipids, Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 19. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6570-0_47

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  • DOI: https://doi.org/10.1007/978-1-4757-6570-0_47

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4757-6572-4

  • Online ISBN: 978-1-4757-6570-0

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