Table of Contents

Part II. Neuropathology of the Human Hypothalamus and Adjacent Brain Structures.

17. Vascular supply and vascular disorders. 17.1 Blood supply to the hypothalamus and pituitary. 17.2 Vascular lesions of the hypothalamus. 17.3. Choroid plexus of the third ventricle. 18. Disorders of development and growth. 18.1 Anencephaly. 18.2 Transsphenoidal encephalocele and empty sella syndrome. 18.3 Congenital midline defects: optic nerve hypoplasia and septo-optic dysplasia (De Morsier's syndrome). 18.4 Dystopia of the neurohypophysis. 18.5 The optic chiasm. 18.6 The growth hormone axis in development and aging. 18.7 Hydrocephalus. 18.8 Septum pellucidum abnormalities. 19. Tumors. 19.1 Symptoms due to hypothalamic tumors. 19.2 Germinoma and teratoma. 19.3 Hamartoma. 19.4 Glioma. 19.5 Craniopharyngioma, Rathke's cleft cysts and xanthogranuloma. 19.6 Dermoid and epidermoid tumors. 19.7 Pineal region tumors. 19.8 Tuberous sclerosis (Bourneville-Pringle syndrome) and tumors of the hypothalamus. 19.9 Metastases. 19.10 Other tumors. 20. Hypothalamic infections. 20.1 Inflammatory conditions affecting the hypothalamus. 20.2 Encephalitis lethargica (Von Economo's encephalitis). 20.3 Acquired immunodeficiency syndrome (AIDS). 21. Neuroimmunological disorders. 21.1 Neurosarcoidosis of the hypothalamus. 21.2 Multiple sclerosis (MS) and the hypothalamus. 21.3 Langerhans' cell histiocytosis (Hand-Schüller-Christian disease; histiocytosis-X).
21.4 Other neuroimmunological hypothalamic disorders and lesions. 22. Drinking disorders. 22.1 Pathology of the neurohypophysis. 22.2 Diabetes insipidus. 22.3 Primary polydipsia and adipsia. 22.4 Nocturnal diuresis. 22.5 Vasopressin hypersecretion in diabetes mellitus. 22.6 Inappropriate secretion of vasopressin. 22.7 Wolfram's syndrome. 23. Eating disorders. 23.1 Prader-Willi syndrome. 23.2 Anorexia nervosa and bulimia nervosa. 23.3 Other eating disorders. 24. Reproduction, olfaction and sexual behavior. 24.1 Disorders of gonadotropic hormone regulation. 24.2 Olfaction, anosmia, the vomeronasal organ (Jacobson's organ) and the embryology of LHRH neurons. 24.3 Kallmann's syndrome. 24.4 Klinefelter's syndrome or testicular dysgenesis. 24.5 Sexual differentiation of the brain and sexual behavior. 25. Hypothalamic lesions following trauma and iatrogenic disorders. 25.1 Head/brain injury. 25.2 Neuroleptic malignant syndrome. 25.3 Hypothalamic injury by radiation. 25.4 Lesion of the pituitary stalk. 26. Hypothalamic involvement in psychiatric disorders. 26.1 Psychiatric symptoms due to tumors of the third ventricle. 26.2 Attacks of laughter (gelastic epilepsy). 26.3 Ventromedial hypothalamus syndrome and the effect of lesions on aggression. 26.4 Depression and mania. 26.5 The hypothalamus in mental deficiency. 26.6 Obsessive-compulsive disorder. 26.7 Anxiety disorders. 26.8 Fatigue syndromes. 26.9 Aggressive behavior. 27. Schizophrenia and autism. 27.1 Schizophrenia. 27.2 Autism. 28. Periodic disorders. 28.1 Kleine-Levin syndrome (periodic somnolence and morbid hunger). 28.2 Spontaneous periodic fever, hypothermia, Shapiro syndrome and periodic Cushing's syndrome. 28.3 Acute intermittent porphyria. 28.4 Narcolepsy. 28.5 Epileptic seizures. 29. Neurodegenerative disorders. 29.1 Alzheimer's disease and the hypothalamus. 29.2 Dementia with argyrophilic grains. 29.3 Parkinson's disease. 29.4 Huntington's disease. 29.5 Wernicke's encephalopathy, Korsakoff's psychosis and Marchiafava-Bignami disease. 29.6 Adrenomyeloneuropathy, adrenoleukodystrophy and hypothalamic-pituitary dysfunction. 29.7 Other neurodegenerative disorders. 30. Autonomic disorders. 30.1 Temperature regulation. 30.2 Disturbed thermoregulation. 30.3 Cardiovascular regulation. 30.4 Cardiovascular disturbances. 30.5 Circumventricular organs: lamina terminalis, subfornical organ and autonomic regulation. 30.6 Micturition. 30.7 Sleep. 31. Pain and addiction. 31.1 Opioid peptides and other addictive compounds. 31.2 Pain and the hypothalamus. 31.3 Headache. 32. Miscellaneous hypothalamic syndromes. 32.1 Idiopathic hypothalamic syndrome of childhood, a paraneoplastic syndrome. 32.2 Hypothalamic atrophy, Leigh's disease and Cornelia de Lange's syndrome. 32.3 Diencephalic idiopathic gliosis. 32.4 Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome. 32.5 Agenesis of the diencephalon. 32.6 Tourette's syndrome. 33. Brain death and 'dead' neurons. References. Subject Index for Part I and Part II.

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This volume is part of the Handbook of Clinical Neurology series, the world's most comprehensive source of information in neurology. Now in its third generation, the series has an unparalleled reputation for providing the latest foundational research, diagnosis, and treatment protocols essential for both basic neuroscience research and clinical neurology.

About the Author

Dick Swaab (1944) earned his medical and doctoral degrees at the University of Amsterdam, where he became involved in brain research during his third year of medical school. He was Director of the Netherlands Institute for Brain Research from 1978 to 2005. Since 1979 he is Professor of Neurobiology at the Medical Faculty, University of Amsterdam.

In 1985, Dr. Swaab founded the Netherlands Brain Bank (NBB) to serve as a source of clinically and neuropathologically well-documented research tissue. Since its founding, the Brain Bank has provided samples from more than 4,000 autopsies to 500 research groups in 25 countries. He was director of the NBB until 2005.

He is Leader Research team Neuropsychiatric Disorders, Neth. Inst for Neuroscience, an institute of the Royal Netherlands Academy of Arts and Sciences (KNAW). Swaab is also appointed for 2011-2017 Chao Kuang Piu Chair of Zhejiang University, Hangzhou, P.R. China.

His major research interests focus on, sexual differentiation of the human brain in relation to gender identity and sexual orientation, aging of the brain, Alzheimer’s disease, the neurobiological basis of depression, suicide and eating disorders. He has published over 540 papers in SCI journals, authored more than 200 chapters in books, and edited more than 60 books. Swaab mentored 84 PhD students from which 16 are now full professor. He is “Companion in the Order of the Dutch Lion”, bestowed by her Royal Majesty Queen Beatrix of the Netherlands. In 2008 Swaab obtained the Academy medal for his role in national and international neuroscience.

Dick Swaab is author of the 2 volume monograph The Human Hypothalamus that appeared in the Handbook of Clinical Neurology series, Elsevier, Amsterdam (1000 pp) and the Dutch best seller We are our Brains (450.000 copies sold), that is translated in 14 languages. A children's version of the book (You are your brains) has also appeared in Dutch in 2013 and Russian (2014). Swaab's H-factor is 76.

Reviews

In these "collected works" hypothalamic involvement is demonstrated in lower, as well as many higher functions, and even in neurologic and psychiatric disorders, such as memory and attention deficits, mood and depression, narcolepsy, cluster headache, and aggression-all conditions one would, intuitively, not associate with the hypothalamus. If one book can change the traditional clinical perception of the hypothalamus as an evoluntionary ancient, endocrine structure of little importance, this monograph will.
"Journal of Neuropathology and Experimental Neurology, March 2005"