Description This volume in the Handbook of Clinical Neurology (Series Editors: Michael J. Aminoff, Department of Neurology, University of
California, San Francisco, USA; Francois Boller, Bethesda, USA; Dick F. Swaab, Netherlands Institute for Neuroscience, Amsterdam, the
Netherlands)is the first major account of the human hypothalamus. Part II is system and disease oriented and deals with the clinic, and
with the neuropathology, neurobiology and genetics of vascular disorders, disorders of development and growth (including e.g. anencephaly,
septo-optic dysplasia and other midline defects), hypothalamic and pineal tumors, infections, neuroimmunological disorders such as neurosarcoidosis,
multiple sclerosis, and Langerhans-cell histiocytosis. After the neuropathology of the neurohypophysis has been dealt with, the background
of the different forms of diabetes insipidus and other drinking disorders, such as polydipsia and adipsia, inappropriate secretion of
vasopressin, and Wolfram syndrome are presented. Eating disorders such as, e.g. Prader-Willi syndrome, anorexia and bulimia nervosa,
are presented as typical hypothalamic diseases. Genetic disorders causing a defect in the migration of hypothalamic neurons are the
basis of Kallmann syndrome. Sexual differentiation of the brain is related to transsexuality and homo/heterosexuality.
Audience
Clinical neurologists and researchers in the neurosciences
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-Schuller-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.