The Human Hypothalamus in Health and Disease, Proceedings of the 17th International Summer School of Brain Research, held at the Auditorium of the University of Amsterdam

499 Pages·1992·42.82 MB·English

by  D.F. Swaab

PROGRESS IN BRAIN RESEARCH
VOLUME 93
THE HUMAN HYPOTHALAMUS IN HEALTH AND DISEASE
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PROGRESS IN BRAIN RESEARCH
VOLUME 93
THE HUMAN HYPOTHALAMUS IN
HEALTH AND DISEASE
Proceedings of the 17th International Summer School of Brain Research, held at the
Auditorium of the University of Amsterdam (The Netherlands), 26- 30 August, 1991
EDITED BY
D.F. SWAAB, M.A. HOFMAN, M. MIRMIRAN, R. RAVID
and F.W. VAN LEEUWEN
Netherlands Institute for Brain Research, Meibergdreef 33, I105 AZ Amsterdam (The Netherlands)
ELSEVIER
AMSTERDAM - LONDON - NEW YORK - TOKYO
1992
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V
List of Contributors
Ansseau, M., Department of Psychiatry, Academic Hospital of Liege, Domaine Universitaire du Sart
Tilman, B.35, 4000 Liege 1, Belgium.
Bergen, H., Rockefeller University, York Avenue and 66th Street, New York, NY 10021, U.S.A.
Braak, E., Department of Anatomy, Johann Wolfgang Goethe University, Theodor Stern Kai 7, 6000
Frankfurt am Main 70, Germany.
Braak, H., Department of Anatomy, Johann Wolfgang Goethe University, Theodor Stern Kai 7, 6000
Frankfurt am Main 70, Germany.
Bray, G.A., Pennington Biomedical Research Center, Louisiana State University, 6400 Perkins Road, Baton
Rouge, LA 70808-4124, U.S.A.
Breder, C.D., Department of Neurology, University of Chicago, 947 East 58th Street, Box 271, Chicago,
IL 60637, USA.
Champier, J., Laboratory of Anatomy and Pathology, Faculty of Medicine Alexis Carrel, Rue Guillaume
Paradin, 69372 Lyon CCdex 08, France.
Chan-Palay, V.L., Neurological Clinic, University Hospital, Frauenklinikstrasse 36, Zurich 8091,
Switzerland.
Charlton, H.M., Department of Human Anatomy, University of Oxford, South Parks Road, Oxford OX1
3QX, U.K.
Charnay, Y., Laboratory of Neuropathology, Be1 Air Clinic, Geneva, Switzerland.
Chigr, F., Laboratory of Anatomy and Pathology, Faculty of Medicine Alexis Carrel, Rue Guillaume
Paradin, 69372 Lyon CCdex 08, France.
De Wied, D., Rudolf Magnus Institute, Vondellaan 6, 3521 GD Utrecht, The Netherlands.
Epelbaum, J., INSERM U 159, Paul Broca Center, 75014 Paris, France.
Gooren, L.J.G., Department of Endocrinology, Free University, P.O. Box 7057, 1007 MB Amsterdam, The
Netherlands.
Goudsmit, E., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Heuser, I., Max-Planck Institute of Psychiatry, Clinical Institute, Kraepelinstrasse 10,8000 Munich 40, Ger-
many.
Hofman, M.A., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Holsboer, F., Max-Planck Institute of Psychiatry, Clinical Institute, Kraepelinstrasse 10, 8000 Munich 40,
Germany.
Jentsch, B., Neurological Clinic, University Hospital, Frauenklinikstrasse 36, Zurich 8091, Switzerland.
Jordan, D.; Laboratory of Anatomy and Pathology, Faculty of Medicine Alexis Carrel, Rue Guillaume
Paradin, 69372 Lyon CCdex 08, France.
Kok, J.H., Department of Neonatology, Academic Medical Center, Meibergdreef 15, 1105 AZ Amsterdam,
The Netherlands.
Kopp, N., Laboratory of Anatomy and Pathology, Faculty of Medicine Alexis Carrel, Rue Guillaume
Paradin, 69372 Lyon CCdex 08, France.
Kremer, H.P.H., Department of Neurology, Academic Hospital Leiden, P.O. Box 9600, 2300 RC Leiden,
The Netherlands.
..
Legros, J.J Department of Endocrinology, Academic Hospital of Likge, Domaine Universitaire du Sart
Tilman, B.35, 4000 Likge 1, Belgium.
McEwen, B.S., Laboratory of Neuroendocrinology, The Rockefeller University, 1230 York Avenue, New
York, NY 10021-6399, U.S.A.
VI
Martin, J.B., Department of Neurology, UCSF School of Medicine, Room S-224, 513 Parnassus Avenue,
San Francisco, CA 94143-0410, U.S.A.
Mengod, G., Department of Neurochemistry, CID-CSIC, Jordi Girona 18 - 26, Barcelona 08034, Spain.
Mirmiran, M., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Moore, R.Y ., Center for Neuroscience, University of Pittsburgh, CNUP Office, Biomedical Science Tower
W 1657, Pittsburg, PA 15261, U.S.A.
Najimi, M., Laboratory of Anatomy and Pathology, Faculty of Medicine Alexis Carrel, Rue Guillaume
Paradin, 69372 Lyon CCdex 08, France.
Neijmeijer-Leloux, A., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam,
The Netherlands.
Palacios, J.M., Department of Neurochemistry, CID-CSIC, Jordi Girona 18 - 26, Barcelona 08034, Spain.
Pfaff, D.W., Rockefeller University, York Avenue and 66th Street, New York, NY 10021, U.S.A.
Phillips 111, J.A., Division of Genetics, Department of Pediatrics, Vanderbilt University, Nashville, TN
37232, U.S.A.
Probst, A., Institute of Pathology, Department of Neuropathology, University of Basel, CH-4003, Basel,
Switzerland.
Rance, N.E., Department of Pathology, University of Arizona College of Medicine, 1501 N. Campbell
Avenue, Tucson, AZ 85724, U.S.A.
Rasmussen, D.D., Department of Reproductive Medicine 0802, University of California at San Diego, 225
Dickinson Street, San Diego, CA 92103-1990, U.S.A.
Ravid, R., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Repaske, D.R., Department of Pediatrics, Division of Endocrinology, CB 7220, 509 Burnett Womack,
University of North Carolina, Chapel Hill, NC 27599-7220, U.S.A.
Reppert, S.M., Children’s Service, Massachusetts General Hospital, 32 Fruit Street Boston, MA 021 14,
U.S.A.
Riskind, P.N., Neurology Service, Massachusetts General Hospital, Boston, MA 021 14, U.S.A.
Ronken, E., Rudolf Magnus Institute, Vondellaan 6, 3521 GD Utrecht, The Netherlands.
Saper, C.B., Department of Pharmacological and Physiological Sciences, University of Chicago, 947 East
58th Street, Box 271, Chicago, IL 60637, U.S.A.
Scanlon, M.F., Department of Medicine, University of Wales College of Medicine, Heath Park, Cardiff CF4
4XW, U.K.
Schwanzel-Fukuda, M., Rockefeller University, York Avenue and 66th Street, New York, NY 10021, U.S.A.
Scherbaum, W.A., Medical and Outpatient Clinic, University of Ulm, Robert Kochstrasse 8, D-7900 Ulm,
Germany.
Spengler, D., Max-Planck Institute of Psychiatry, Clinical Institute, Kraepelinstrasse 10, 8000 Munich 40,
Germany.
Summar, M.L., Division of Genetics, Department of Pediatrics, Vanderbilt University Medical Center,
T2404 Medical Center North, Nashville, TN 37232, U.S.A.
Swaab, D.F., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Van de Poll, N.E., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Van Cool, W.A., Department of Neurology, Academic Medical Center, Meibergdreef 15, 1105 AZ Amster-
dam, The Netherlands.
Van Goozen, S.H.M., Psychonomics, Faculty of Psychology, University of Amsterdam, Roeterstraat 15,
1018 WB Amsterdam, The Netherlands.
Van Leeuwen, F.W., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Van Zwieten, E.J., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Weesner, G., Rockefeller University, York Avenue and 66th Street, New York, NY 10021, U.S.A.
Wiegant, V.M., Rudolf Magnus Institute, Vondellaan 6, 3521 GD Utrecht, The Netherlands.
VII
Witting, W., Netherlands Institute for Brain Research, Meibergdreef 33, 1105 AZ Amsterdam, The
Netherlands.
Wood, M.J., Department of Human Anatomy, University of Oxford, South Parks Road, Oxford OX1
3QX, U.K.
Zheng, L.M., Rockefeller University, York Avenue and 66th Street, New York, NY 10021, U.S.A.
This Page Intentionally Left Blank
IX
Preface
The 17th International Summer School of Brain Research held in Amsterdam from
August 26 to August 30, 1991, was the first international multidisciplinary conference
on the human hypothalamus in health and disease. The summer school was organized
by the Netherlands Institute for Brain Research (NIBR), an institute of the Royal
Netherlands Academy of Sciences. The history of the NIBR dates back to the beginning
of this century. It was in 1901, at a meeting of the International Association of
Academies held in Paris, that the anatomist Wilhelm His proposed to place research
into the nervous system on an international footing. This proposal resulted in the for-
mation of the International Academies Committee, which set itself the task of
“organizing a network of institutions throughout the civilized world, dedicated to the
. . .”.
study of the structure and functions of the central organ This resulted in the
founding of several institutes for brain research, among them the NIBR, which was
founded in 1909. The committee pointed out that “the time is not far distant when the
study of the millions of brain cells will have to be divided amongst researchers in the
way the astronomers have been obliged to divide the millions of stars into various
groups”. The purpose of the summer school was, however, not to divide the millions
of hypothalamic neurons among the participants, but rather to share and assemble the
current knowledge on the human hypothalamus in health and disease.
The human hypothalamus is involved in growth, development, birth, rhythmicity,
water balance, thermoregulation, feeding, sexual behavior and many other functions.
Alterations in hypothalamic structures and functions are thought to be involved in
diseases such as anorexia nervosa, bulimia, depression, Cushing’s disease, diabetes in-
sipidus, Prader-Willi syndrome, polycystic ovaries syndrome and the malignant
neuroleptic syndrome as well as disturbances in sleep, circadian rhythms and
temperature regulation. In addition, the hypothalamus is affected in neurodegenerative
diseases and might be responsible for particular symptoms in, e.g., Alzheimer’s,
Parkinson’s and Huntington’s diseases, Down’s syndrome and possibly also in multi-
ple sclerosis. Moreover, the development of this brain region is presumed to be affected
by hormones given during pregnancy (e.g., diethylstibestrol (DES)), in adrenogenital
syndrome and in Turner’s, Klinefelter’s and Kallman’s syndromes. In spite of this im-
pressive list, only recently a start was made with collecting data on the normal develop-
ment, sexual differentiation and aging of the human hypothalamus, and the first infor-
mation on how the pathophysiology of each of these conditions is reflected in the mor-
phology of the human hypothalamus is now beginning to appear in the literature. Such
data should provide unique information on possible functions of the different
hypothalamic nuclei.
In the past few years, with the advent of powerful molecular, physiological,
neuroanatomical and morphometric techniques, such questions have been attacked