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Sexualstörungen bei vorwiegend zerebraler Histiocytosis X

Beitrag zur nosologischen Stellung des Hypothalamusgranuloms vom Typ Gagel-Ayala

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Zentralnervöse Sexualsteuerung

Part of the book series: Journal of Neuro-Visceral Relations ((NEURAL SUPPL,volume 10))

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Summary

Six autopsy cases of cerebral histiocytosis-X are reported. All six involved the hypothalamus, and in four of them diabetes insipidus was the initial manifestation of the disease. In two cases it was associated with severe and permanent sexual disorders. Morphologically, two cases were consistent with eosinophilic granuloma, two others with non-lipid histiocytosis, and a further two with lipoid granulomatosis (Hand-Schüller-Christian disease). In three cases, granulomas also involved other parts of the brain, and in two of them they were accompanied by xanthomatous transformation. Two patients suffered from extracerebral manifestations of this condition, i.e. eosinophilic granuloma of the lymph nodes and lipoid granulomatosis of the skeletal system, the latter representing the terminal manifestation of the disease.

From the analysis of these observations, a review of the actual slides of Gagel’s (1941) case, and a critical analysis of the relevant literature, it is concluded that “circumscribed hypothalamic granuloma of the Gagel-Ayala type” in most cases represents a localised or predominantly cerebral form of one or other type of histiocytosis-X.

The primary affection of the hypothalamic-hypophysial system is characteristic of this disorder, and its significance with regard to the function and structure of the gonads is demonstrated by two clinico-pathological case reports. These young women developed amenorrhoea and diabetes insipidus at 3½ and 7 years before death. At autopsy, in addition to severe lesions of other endocrine organs, total atrophy of the ovaries and mammary fibrosis were observed in one case. There was a complete destruction and granulomatous scarring of the tuber cinereum, infundibulum and posterior pituitary, but the anterior pituitary was intact. Observations on human patients with severe sexual disorders and atrophy of the gonads caused by isolated destruction of the hypothalamic sex centre or of its pathways to the anterior pituitary confirm the experimental data on the functional anatomy of the control of sexual functions by the central nervous system.

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Authors and Affiliations

  1. Department of Pathology and Oncology, Kansas University Medical Center, Kansas City, USA

    K. Jellinger, J. J. Kepes & Prof. Dr. F. Seitelberger (Vorstand)

  2. Neurologisches Institut, Universität Wien, Austria

    K. Jellinger, J. J. Kepes & Prof. Dr. F. Seitelberger (Vorstand)

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  1. K. Jellinger
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  2. J. J. Kepes
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  3. Prof. Dr. F. Seitelberger
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H. Orthner

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Jellinger, K., Kepes, J.J., Seitelberger, F. (1971). Sexualstörungen bei vorwiegend zerebraler Histiocytosis X. In: Orthner, H. (eds) Zentralnervöse Sexualsteuerung. Journal of Neuro-Visceral Relations, vol 10. Springer, Vienna. https://doi.org/10.1007/978-3-7091-4157-1_75

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  • DOI: https://doi.org/10.1007/978-3-7091-4157-1_75

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  • Print ISBN: 978-3-7091-4158-8

  • Online ISBN: 978-3-7091-4157-1

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