The Human Hypothalamus

The Human Hypothalamus

Neuroendocrine Disorders

1st Edition - July 4, 2021

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  • Editors: Dick Swaab, Ruud Buijs, Paul Lucassen, Ahmad Salehi, Felix Kreier
  • Hardcover ISBN: 9780128206836
  • eBook ISBN: 9780128206843

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The Human Hypothalamus: Neuroendocrine Disorders, Volume 181 in the Handbook of Clinical Neurology series, provides comprehensive summaries of recent research on the brain and nervous system as they relate to clinical neurology. This volume summarizes the role of the hypothalamus in neuroendocrine disorders, identifying the mechanism of action, disorder etiology, and best practices for assessment and treatment. Disorders covered include pituitary hypothalamic disorders of development and growth, hypothalamic tumor related disorders, hypothalamic autoimmune disorders and infection, disorders of vasopressin, water and sodium homeostasis, eating disorders, and gonadotropic hormone regulation disorders.

Key Features

  • Discusses the importance of the hypothalamus in human growth and development
  • Reviews hypothalamic related tumors, as well as pituitary, autoimmune, vasopressin and hormone regulation disorders
  • Includes metabolic and eating disorders
  • Identifies mechanisms of disease action and etiology
  • Provides best practice information for assessment and treatment


Clinical neurologists, researchers in neurology

Table of Contents

  • Cover image
  • Title page
  • Table of Contents
  • Copyright
  • Available titles
  • Foreword
  • Preface
  • The HCN volumes on the Human Hypothalamus
  • Contributors
  • Contents of related volumes
  • Chapter 1: Introduction: The human hypothalamus and neuroendocrine disorders
  • Section 15: Structural Disorders of the Hypothalamo–Pituitary Region
  • Section 16: Tumors of the Hypothalamus
  • Section 17: Neuroimmunological Disorders
  • Section 18: Drinking Disorders
  • Section 19: Eating Disorders
  • Section 20: Reproduction and Sexual Behavior
  • Section 15: Structural disorders of the hypothalamo-pituitary region
  • Chapter 2: Pituitary stalk interruption syndrome
  • Abstract
  • Introduction
  • Epidemiology
  • Magnetic Resonance Imaging findings
  • Clinical Manifestations
  • Hormonal Profile and Evolution of Hormone Deficiencies
  • Treatment
  • Pathogenesis of Pituitary Stalk Interruption Syndrome
  • Genetics of Pituitary Stalk Interruption Syndrome
  • Environmental Factors and PSIS
  • Conclusions and Perspectives
  • Chapter 3: Empty sella syndrome: Multiple endocrine disorders
  • Abstract
  • Introduction
  • Classification
  • Epidemiology
  • Pathogenesis of Empty Sella
  • Presenting Clinical Manifestations
  • Diagnosis
  • Differential Diagnosis
  • Treatment Strategies
  • Follow-Up
  • Prognosis
  • Conclusion
  • Chapter 4: Pituitary dysfunction after aneurysmal subarachnoidal hemorrhage
  • Abstract
  • Introduction
  • Epidemiology
  • Treatment and Management of aSAH
  • Neuroendocrine Dysfunction in aSAH Patients
  • Neuroendocrine Dysfunction in the Acute Phase after aSAH
  • Neuroendocrine Dysfunction in the Chronic Phase after aSAH
  • Neuroendocrine Dysfunction in aSAH Patients: Changes from the Acute to the Chronic Phase
  • Conclusions
  • Chapter 5: Septo-optic dysplasia
  • Abstract
  • Introduction
  • Etiology and Pathogenesis
  • Development of the Forebrain and Pituitary Gland
  • Neuropathological Studies
  • Epidemiology
  • Clinical Presentation
  • Diagnostic Stage
  • Clinical and Surgical Management
  • Prognosis and Outcome
  • Conclusion
  • Section 16: Tumors of the hypothalamus
  • Chapter 6: Hypothalamic hormone-producing tumors
  • Abstract
  • Introduction
  • Tumor Classification, Clinical, and Morphological Features
  • Therapeutic Approaches
  • Chapter 7: Craniopharyngiomas primarily affecting the hypothalamus
  • Abstract
  • Introduction
  • Conclusions
  • Sources of funding
  • Disclosure of potential conflict of interest
  • Section 17: Neuroimmunological disorders
  • Chapter 8: The stress-axis in multiple sclerosis: Clinical, cellular, and molecular aspects
  • Abstract
  • Introduction
  • The Stress-Axis in MS
  • Determinants of Stress-Axis Responsiveness
  • Pathological, Cellular, and Molecular Effects of Stress-Axis Responsiveness
  • Clinical Correlates of HPA Axis Activity in MS
  • Outlook
  • Chapter 9: Neuroendocrine manifestations of Langerhans cell histiocytosis
  • Abstract
  • Introduction
  • Epidemiology
  • Diagnostic Criteria
  • Neuroendocrine Manifestations of LCH
  • Management of LCH
  • Conclusions
  • Chapter 10: Neuroendocrine manifestations of Erdheim–Chester disease
  • Abstract
  • Introduction
  • Manifestations of Hypothalamus, Pituitary Stalk, and/or Pituitary Gland Lesions
  • Manifestations of Posterior Pituitary Hormone Deficiencies (Arginine Vasopressin and/or Oxytocin Deficiencies)
  • Manifestations of Anterior Pituitary Hormone Deficiencies
  • ACTH Deficiency (Hypothalamic–Pituitary–Adrenal Axis)
  • TSH Deficiency (Hypothalamic–Pituitary–Thyroid Axis)
  • GH Deficiency
  • Gonadotropin Deficiency (Hypothalamic–Pituitary–Gonadal Axis)
  • Hyperprolactinemia
  • PRL Deficiency
  • Conclusions
  • Chapter 11: Hypothalamitis and pituitary atrophy
  • Abstract
  • Introduction
  • Pathogenesis
  • Clinical Presentation
  • Diagnosis
  • Differential Diagnosis
  • Treatment
  • Prognosis
  • Conclusion
  • Chapter 12: Narcolepsy Type I as an autoimmune disorder
  • Abstract
  • Narcolepsy Type 1
  • Genetic Associations in Narcolepsy Type 1
  • Environmental Factors Linked to Disease Development
  • Pathologic Findings Postmortem in Narcolepsy Type 1
  • Immune System Chances in Narcolepsy Type 1 Patients
  • Animal Models of Narcolepsy Type 1
  • Is Narcolepsy Type 1 an Autoimmune Disease?
  • Chapter 13: Neuromyelitis optica, aquaporin-4 antibodies, and neuroendocrine disorders
  • Abstract
  • Neuromyelitis Optica Spectrum Disorders
  • Circumventricular Organs and the Hypothalamus as Target of Aquaporin-4 Autoimmunity
  • The Spectrum of Neuroendocrine Disorders in Neuromyelitis Optica
  • Chapter 14: Antibodies against the pituitary and hypothalamus in boxers
  • Abstract
  • Traumatic Brain Injury: Epidemiology, Causes, and Outcomes
  • TBI and Neuroendocrine Abnormalities
  • Autoimmunity and Hypothalamo-Pituitary Dysfunction
  • Boxing and Autoimmunity
  • Acknowledgment
  • Chapter 15: Autoimmune diabetes insipidus
  • Abstract
  • Introduction
  • Autoantibody Studies
  • Summary and outlook
  • Section 18: Drinking disorders
  • Chapter 16: Neuroimaging of central diabetes insipidus
  • Abstract
  • Introduction
  • Neuroanatomy and Neurophysiology of the Hypothalamic–Neurohypophyseal Axis
  • Causes of Central Diabetes Insipidus
  • Discussion
  • Summary and future directions
  • Chapter 17: Differential diagnosis of familial diabetes insipidus
  • Abstract
  • History
  • Physiology
  • Pathophysiology
  • Familial DI
  • Differential Diagnosis of Familial DI
  • Conclusions
  • Chapter 18: The vasopressin–aquaporin-2 pathway syndromes
  • Abstract
  • Defective Vasopressin–AQP2 Pathway in Nephrogenic Diabetes Insipidus (NDI)
  • Altered Vasopressin–AQP2 Pathway in the Syndrome of Inappropriate Secretion of Antidiuretic Hormone (SIADH)
  • Altered Vasopressin–AQP2 Pathway in Nephrogenic Syndrome of Inappropriate Antidiuresis (NSIAD)
  • Constant Tonic Action of Vasopressin–AQP2 Pathway in Polycystic Kidney Disease
  • Chapter 19: Adipsic diabetes insipidus
  • Abstract
  • Introduction
  • Physiology of Arginine Vasopressin and Thirst
  • Etiology of Adipsic Diabetes Insipidus
  • Associated Diseases
  • Management of Adipsic Diabetes Insipidus
  • Prognosis and Future Directions
  • Chapter 20: Animal models for diabetes insipidus
  • Abstract
  • Determinants of the Intake and Excretion of Water and Mineral Salts
  • Diabetes Insipidus: Characteristics and Types
  • Animal Models of Central and Nephrogenic DI
  • From Animal Models to Emerging Human Therapies for DI
  • Chapter 21: Nocturnal enuresis in children: The role of arginine–vasopressin
  • Abstract
  • Introduction
  • Nocturnal Enuresis as a Complex Condition: Elements of Pathophysiology
  • The Concept of Nocturnal Polyuria in Nocturnal Enuresis
  • Circadian Rhythms, Arginine–Vasopressin, and Nocturnal Polyuria
  • Nocturnal Polyuria Unrelated to AVP
  • Antidiuretic Treatment of Nocturnal Enuresis With Vasopressin Analogues
  • The Refractory Patient
  • AVP and the Genetics of Nocturnal Enuresis
  • Conclusions and Future Perspectives
  • Section 19: Eating disorders
  • Chapter 22: Monogenic human obesity syndromes
  • Abstract
  • Introduction
  • Leptin—A Peripheral Hormone that Acts on Circuits in the Hypothalamus to Regulate Weight
  • Homozygous Mutations in the Genes Encoding Leptin and the Leptin Receptor
  • Genetic Disorders that Disrupt Melanocortin Signaling
  • Semaphorin 3 Signaling Affects the Development of POMC Neurons
  • SRC-1 and PHIP Modulate the Transcription of POMC
  • SIM1 and OTP Shape the Development of the PVN
  • BDNF, TrkB Affect Weight, Memory, and Behavior
  • SH2B1 Links Weight Regulation and Aggression
  • Conclusions
  • Chapter 23: Hypothalamic microinflammation
  • Abstract
  • Introduction
  • Hypothalamic Microinflammation Discovered in Animals
  • Hypothalamic Microinflammation: Human Relevance
  • A Few Inducers of Hypothalamic Microinflammation
  • Hypothalamic Microinflammation in Chronic Overnutrition
  • Hypothalamic Microinflammation in Early Aging
  • Hypothalamic Microinflammation: A Basis for Obesity
  • Hypothalamic Microinflammation: A Basis for Diabetes
  • Hypothalamic Microinflammation: A Basis for Hypertension
  • Hypothalamic Microinflammation: A Basis for Aging
  • Concluding Remarks
  • Chapter 24: Glucose and fat sensing in the human hypothalamus
  • Abstract
  • Introduction
  • Hypothalamic Sensing of Glucose and Fats
  • Summary, Implications, and Future Perspectives
  • Concluding Remarks
  • Chapter 25: Hypothalamus and neuroendocrine diseases: The use of human-induced pluripotent stem cells for disease modeling
  • Abstract
  • Introduction
  • Development of Human Neuroendocrine Hypothalamus
  • Differentiation of Human-Induced Pluripotent Stem Cell Into Hypothalamic Neurons
  • Modeling Neuroendocrine Diseases (Obesity) in a Dish Using hiPSC-Derived Hypothalamic Neurons
  • Chapter 26: Prader–Willi syndrome: Hormone therapies
  • Abstract
  • Introduction
  • Genetics
  • Endocrine Treatments
  • Conclusions
  • Chapter 27: Transcriptomics of the Prader–Willi syndrome hypothalamus
  • Abstract
  • Introduction
  • Transcriptomics Analysis in Prader–Willi Syndrome
  • Conclusions and future research directions
  • Chapter 28: Disorders of hypothalamic function: Insights from Prader–Willi syndrome and the effects of craniopharyngioma
  • Abstract
  • Introduction
  • Craniopharyngioma
  • Prader–Willi Syndrome
  • Comparisons Between Hypothalamic CP and PWS
  • Conclusions
  • Chapter 29: Animal models for Prader–Willi syndrome
  • Abstract
  • Introduction
  • Phenoptypes in Mouse Models
  • Conclusion
  • Chapter 30: Is there a hypothalamic basis for anorexia nervosa?
  • Abstract
  • Introduction
  • Genetics and Epigenetics of Anorexia Nervosa
  • Structural and Functional Imaging and Brain Connectivity in Anorexia Nervosa
  • Hypothalamic Control of Energy Homeostasis/Peripheral and Hypothalamic Adaptations in Anorexia Nervosa
  • Peripheral Sensors of Nutritional and Energy Status Acting in the Hypothalamus
  • Hypothalamic and Neuroendocrine-Related Agents in Anorexia Nervosa
  • Neuroendocrine/Hypothalamic Adaptations in Animal Models of Undernutrition
  • Conclusion
  • Section 20: Reproduction, olfaction and sexual behavior
  • Chapter 31: Sexual differentiation of the human hypothalamus: Relationship to gender identity and sexual orientation
  • Abstract
  • Introduction: Programming of the Brain
  • Gender-Based Differences in Behavior
  • Mechanisms Involved in Sexual Differentiation of the Brain
  • Differences in the Hypothalamus in Relation to Sex and Gender Dysphoria
  • Conclusions
  • Acknowledgements
  • Chapter 32: Klinefelter syndrome or testicular dysgenesis: Genetics, endocrinology, and neuropsychology
  • Abstract
  • Definition
  • Prevalence
  • Testicular Dysgenesis and Hypothalamic–Pituitary–Gonadal Axis Function
  • Cancer
  • Metabolic Disorders
  • Neurologic Disorders in Klinefelter Syndrome
  • Neurocognitive Phenotype
  • Personality, Social Dysfunction, Psychiatric Disorders, and Sexual Orientation
  • Brain Structure and Function
  • Genetics and Epigenetics
  • Conclusion
  • Chapter 33: Neurobiology of puberty and its disorders
  • Abstract
  • Introduction
  • Overview of Operation of Mature Hypothalamic–Pituitary–Gonadal Axis
  • Chronology of Pubertal Development and Stages
  • Ontogeny of Hypothalamic GnRH Pulse Generation (Fetal to Pubertal)
  • Neurobiology of Central Restraint
  • Control Systems Governing Timing of Brake
  • Energy Balance and Puberty
  • Disorders of Puberty
  • Disorders Associated With Early Puberty
  • Approach to the Child with Precocious Pubertal Development
  • Disorders of Delayed Puberty
  • Pituitary-Dependent Hypogonadotropism (Pituitary Hypogonadism)
  • Approach to the Child With Delayed Pubertal Development
  • Conclusion
  • Index

Product details

  • No. of pages: 566
  • Language: English
  • Copyright: © Elsevier 2021
  • Published: July 4, 2021
  • Imprint: Elsevier
  • Hardcover ISBN: 9780128206836
  • eBook ISBN: 9780128206843

About the Editors

Dick Swaab

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.

Affiliations and Expertise

Netherlands Institute for Brain Research, University of Amsterdam, Amsterdam, The Netherlands

Ruud Buijs

Dr. Ruud M. Buijs is head of the Physiology department of the I.I.Biomedicas at the UNAM university and leader of the group Hypothalamic Integration Mechanisms. In that group, the scientists study how the brain and body interact with each other, and hereby the attention is focussed on autonomic and hormonal regulation of body functions under the influence of the biological clock of the brain.

Affiliations and Expertise

Instituto de Investigaciones Biomedicas, Universidad Nacional Autonoma de Mexico, Ciudad Universitaria, Mexico D.F.

Paul Lucassen

Paul J. Lucassen did his PhD in 1995 on Alzheimer’s Disease at the Netherlands Institute for Brain Research in Amsterdam. After a.o. a postdoc in Leiden, he became Full Professor of Brain Plasticity in 2011 at the Swammerdam Institute for Life Sciences (SILS) of the University of Amsterdam, The Netherlands. His group studies molecular, nutritional, pharmacological and environmental regulation of brain plasticity. They combine molecular tools, in vitro/vivo model systems, human brain tissue, cohort studies and brain imaging. A major focus is on adult neurogenesis and cognition in relation to; (early life) stress, exercise, enrichment, depression, brain insults and dementia.

Affiliations and Expertise

Swammerdam Institute for Life Sciences (SILS) - Center for Neuroscience, University of Amsterdam, Amsterdam, The Netherlands

Ahmad Salehi

Ahmad Salehi is affiliated with Stanford Medical School, Department of Psychiatry & Behavioral Sciences, in Palo Alto, CA, United States.

Affiliations and Expertise

Stanford Medical School, Department of Psychiatry and Behavioral Sciences, Palo Alto, CA, United States

Felix Kreier

Felix Kreier is a pediatrician and affiliated with OLVG Hospitals in Amsterdam, The Netherlands.

Affiliations and Expertise

OLVG Hospitals, Amsterdam, The Netherlands

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