Introduction to Clinical Aspects of the Autonomic Nervous System

Description

Introduction to Clinical Aspects of the Autonomic Nervous System: Sixth edition, Volume 2 is an all-encompassing reference to the autonomic nervous system's function, dysfunction, and pathology. This volume describes the role of the autonomic nervous system in circadian rhythms, sleep and wakefulness, aging, exercise, and its role in pain perception. Additional chapters focus on disorders causing autonomic dysfunction, including spinal cord injuries, autonomic neuropathies, trophic disorders, and progressive autonomic failure. Other chapters are dedicated to autonomic adaptations in space and hypoxia and autonomic testing in the laboratory. Readers will be well-equipped to care for patients with autonomic disorders and guide their research endeavors.

Provides an extensive reference on the autonomic nervous system and its crucial functions
Discusses all aspects of autonomic physiology and pathology, including autonomic failure, spinal cord injuries, autonomic neuropathies, trophic disorders, and other forms of autonomic dysfunction
Outlines the role of the autonomic nervous system in several physiological processes, including sleep, wakefulness, aging, and pain perception
Details autonomic function testing and the effects of space exploration and hypoxia on the autonomic nervous system. This volume includes a chapter on the autonomic nervous system during the COVID-19 pandemic

Front Cover 2
Introduction to Clinical Aspects of the Autonomic Nervous System 5
Copyright Page 6
Contents 7
Foreword 17
Acknowledgments 19
Introduction 21
1 Circadian rhythms 23
- 1.1 Introduction 23
- 1.2 Effect of light on the circadian system 24
- 1.3 Effects of melatonin on the circadian system 26
- 1.4 Other circadian rhythms in human 27
- 1.5 Aging and circadian rhythms 29
- 1.6 Circadian disturbances and the COVID-19 pandemic 33
- 1.7 Molecular and circuit-based aspects of the circadian system 33

Front Cover 2
Introduction to Clinical Aspects of the Autonomic Nervous System 5
Copyright Page 6
Contents 7
Foreword 17
Acknowledgments 19
Introduction 21
1 Circadian rhythms 23
- 1.1 Introduction 23
- 1.2 Effect of light on the circadian system 24
- 1.3 Effects of melatonin on the circadian system 26
- 1.4 Other circadian rhythms in human 27
- 1.5 Aging and circadian rhythms 29
- 1.6 Circadian disturbances and the COVID-19 pandemic 33
- 1.7 Molecular and circuit-based aspects of the circadian system 33
- 1.8 Circadian system and neurodegeneration 33
- References 34
2 Sleep and wakefulness 39
- 2.1 Introduction 39
- 2.2 Normal sleep physiology 39
- 2.3 The neurobiology of wakefulness and sleep 44
  - 2.3.1 Wakefulness 44
  - 2.3.2 Induction and maintenance of sleep 46
  - 2.3.3 Sleep–wake transition 47
  - 2.3.4 Body temperature 48
- 2.4 Rapid eye movement sleep and rapid eye movement sleep behavior disorder 48
  - 2.4.1 Physiology of rapid eye movement sleep 48
  - 2.4.2 Rapid eye movement sleep behavior disorder 49
- 2.5 Sleep deprivation 51
- 2.6 Excessive sleep 52
  - 2.6.1 Can healthy young individuals sleep too much? 52
  - 2.6.2 Narcolepsy 53
  - 2.6.3 Other syndromes associated with hypersomnia 55
- 2.7 Abnormal apneic periods during sleep 56
- 2.8 Neurodegeneration and sleep 60
- 2.9 Treatment of sleep disorders 61
- 2.10 Some nocturnal disorders associated with various stages of sleep 61
- References 63
3 The pupil 67
- 3.1 Anatomy and physiologic function of the pupil 67
  - 3.1.1 Pupillary constriction 70
  - 3.1.2 Pupillary dilation 73
  - 3.1.3 Pharmacology 74
  - 3.1.4 Other pupillary reflexes 74
  - 3.1.5 Pupillometry 81
- 3.2 Disordered pupillary function 85
  - 3.2.1 Horner syndrome 85
  - 3.2.2 The Argyll Robertson pupil 91
  - 3.2.3 The tonic pupil 93
  - 3.2.4 The large pupil 95
- References 103
4 Trophic disorders 107
- 4.1 Congenital absence of muscles 107
- 4.2 Congenital neuromuscular disorders with localized weakness 108
- 4.3 Congenital neuromuscular disorders associated with contractures and deformity about joints 109
  - 4.3.1 Congenital elevation of the shoulder (Sprengel’s deformity) 109
  - 4.3.2 Deformities of several joints (arthrogryposis multiplex congenita and amyoplasia congenita) 110
- 4.4 Disorders affecting the skin and subcutaneous tissue 110
  - 4.4.1 Hypermelanosis (increased brown melanin pigmentation) 111
- 4.5 Trophic disorders appearing after birth 111
- 4.6 The influence of the nervous system on the triple response of Lewis 113
- 4.7 The influence of the nervous system on myoedema 116
- 4.8 Lesions of the peripheral nervous system 118
- 4.9 Neuroarthropathies 120
- 4.10 Hypertrophic osteoarthropathy (Bamberger–Marie syndrome) 125
- 4.11 Interactions between central and peripheral neurons and their target tissues 125
- References 127
5 Pain perception and the autonomic nervous system 131
- 5.1 Causalgia 131
- 5.2 Reflex sympathetic dystrophy—complex regional pain syndrome 134
- 5.3 Hyperalgesia 137
- 5.4 Raynaud phenomenon 140
- 5.5 Pathophysiology 142
- 5.6 Associated disorders 142
- 5.7 Autonomic faciocephalalgia (Hortons syndrome, histaminic cephalalgia, and cluster headaches) 145
- 5.8 Raeder’s syndrome (paratrigeminal syndrome) 146
- 5.9 Referred pain 147
- 5.10 Erythromelalgia 151
- 5.11 Baroreceptor function and pain perception 153
- References 153
6 Biofeedback and operant conditioning 159
- 6.1 Interoception 165
- References 167
7 Aging and exercise 169
- 7.1 Aging and the autonomic nervous system 169
  - 7.1.1 Introduction 169
  - 7.1.2 Nutritional effects on autonomic nervous system function 170
  - 7.1.3 Fluid intake and thirst 172
  - 7.1.4 Vasomotor control 173
  - 7.1.5 Autonomic control of cerebral blood flow 175
  - 7.1.6 Thermoregulation 176
  - 7.1.7 Ethnic thermoregulatory differences 179
  - 7.1.8 Sweat-gland activity in the aged 180
  - 7.1.9 Neurogenic control of the circulation 181
  - 7.1.10 Continuous sympathetic nerve discharges 182
  - 7.1.11 Pupillary changes with age 183
  - 7.1.12 Neurogenic bladder dysfunction in the aged 184
  - 7.1.13 Aging and disorders of sexual function 184
  - 7.1.14 Variations in sinus rhythm 185
  - 7.1.15 Orthostatic stress, age, and autonomic function 186
  - 7.1.16 Aging and adrenoceptor-mediated responses 188
  - 7.1.18 The impact of sex on sympathetic neurocirculatory regulation 189
  - 7.1.17 Postprandial hypotension 189
  - 7.1.19 Cellular senescence and the autonomic nervous system 190
- 7.2 Exercise and the autonomic nervous system 190
  - 7.2.1 Acute exercise 190
  - 7.2.2 Exercise training 193
  - 7.2.3 Thermoregulatory response to exercise 194
  - 7.2.4 Exercise, autonomic nervous system, opioids, and fatigue 195
  - 7.2.5 Sympathetic activity 198
  - 7.2.6 Parasympathetic function 199
  - 7.2.7 Vasopressin and atrial natriuretic factor 199
- 7.3 Exercise and aging 199
  - 7.3.1 Introduction 199
  - 7.3.3 Field studies on mountain neurology to mimic aging 201
  - 7.3.2 Exercise and neurodegeneration 201
  - 7.3.4 A call for “freestyle neurology” 206
- References 206
8 Autonomic neuropathies 213
- 8.1 Blood supply to peripheral autonomic nerves 213
  - 8.1.1 Historical aspects 213
  - 8.1.2 Vascular anatomy 214
  - 8.1.3 Vasa nervorum: structural aspects 216
  - 8.1.4 The blood–nerve barrier 217
  - 8.1.5 Innervation of vasa nervorum and the nervi nervorum 218
  - 8.1.6 Noradrenergic innervation of intraneural blood vessels 220
  - 8.1.7 Vasoactive intestinal polypeptide 223
  - 8.1.8 Substance-P 227
  - 8.1.9 Calcitonin gene-related peptide 228
  - 8.1.10 Neuropeptide-Y 229
  - 8.1.11 Serotonin 231
  - 8.1.12 Vasa nervorum and peripheral nerve ischemia 233
  - 8.1.13 Vasa nervorum in human autonomic neuropathies 235
- 8.3 Innervation of vasa nervorum and the nervi nervorum in human sural nerves 238
- 8.2 Pathogenesis of autonomic failure in peripheral nerve disease 238
- 8.4 Human autonomic neuropathies 242
  - 8.4.1 Presenting clinical features 242
  - 8.4.2 Clinical evaluation 243
  - 8.4.3 Paraclinical evaluation 244
  - 8.4.4 Classification 245
    - 8.4.4.1 Immune-mediated and inflammatory autonomic neuropathy 245
    - 8.4.4.2 Autonomic neuropathies associated with systemic autoimmunity 260
    - 8.4.4.3 Metabolic/nutritional 261
  - 8.4.6 Autonomic neuropathy in systemic disorders 271
    - 8.4.6.1 Infectious 273
    - 8.4.6.2 Toxic autonomic neuropathies 274
    - 8.4.6.3 Hereditary autonomic neuropathies 275
      - 8.4.6.3.1 HSAN I 275
      - 8.4.6.3.3 HSAN 3 276
      - 8.4.6.3.2 HSAN 2 276
      - 8.4.6.3.4 HSAN 4 285
      - 8.4.6.3.5 Fabry disease 286
      - 8.4.6.3.6 Cerebellar ataxia, neuropathy, vestibular areflexia (CANVAS) 287
      - 8.4.6.3.7 Charcot-Marie-Tooth disease 287
    - 8.4.6.4 Amyloid neuropathy 287
    - 8.4.6.5 Other causes of autonomic neuropathy 291
      - 8.4.6.5.1 Ross syndrome 291
    - 8.4.6.6 Others 292
    - 8.4.6.7 Peripheral nerve damage in central autonomic failure 294
    - 8.4.6.8 Autonomic dysfunction in Machado–Joseph disease 295
    - 8.4.6.9 Causalgia 296
    - 8.4.6.10 The postpolio syndrome 298
    - 8.4.6.11 Navajo neuropathy 299
    - 8.4.6.12 Navajo neuropathic arthropathy 302
    - 8.4.6.13 Miscellaneous disorders associated with autonomic dysfunction 303
      - 8.4.6.13.1 Hyperbradykininemia 303
      - 8.4.6.13.3 Cystic fibrosis 304
      - 8.4.6.13.2 Dysautonomia in mitral valve prolapse 304
      - 8.4.6.13.4 Weight loss and sympathetic nervous system activity 305
      - 8.4.6.13.5 Lesch–Nyhan syndrome 306
      - 8.4.6.13.6 Canine dysautonomia 307
    - References 308
  - 8.4.5 Treatment-induced neuropathy of diabetes 271
  - 9 Progressive autonomic failure 319
    - 9.1 Progressive autonomic failure—autonomic dysfunction in synucleinopathies 319
      - 9.1.1 Introduction 319
      - 9.1.2 Historical perspective 320
      - 9.1.3 Pathophysiology 321
      - 9.1.4 Pathology 325
      - 9.1.5 Epidemiology 328
      - 9.1.6 Clinical presentation 329
        
        9.1.6.1 Neurogenic orthostatic hypotension 329
        
        9.1.6.2 Gastrointestinal dysfunction 330
        
        9.1.6.4 Thermoregulatory dysfunction 331
        
        9.1.6.3 Genitourinary dysfunction 331
        
        9.1.6.5 Survival and autonomic dysfunction in synucleinopathies 332
      - 9.1.7 Differential diagnosis 332
      - 9.1.8 Autonomic testing 336
        
        9.1.8.1 Autonomic reflex screen 336
        
        9.1.8.3 Ambulatory blood pressure monitoring 338
        
        9.1.8.2 Fractionated plasma catecholamines 338
        
        9.1.8.4 Genitourinary testing and gastrointestinal motility studies 339
      - 9.1.9 Treatment 339
        
        9.1.9.1 Multidisciplinary approach 339
        
        9.1.9.2 Symptom management 339
        
        9.1.9.3 Disease-modifying approach 343
      - 9.1.10 Conclusion 343
    - 9.2 Progressive autonomic failure—others 344
      - 9.2.1 Human T-cell leukemia virus (HTLV) and autonomic failure 344
      - 9.2.2 Genetic forms of autonomic failure 345
        
        9.2.2.1 Dopamine-ß-hydroxylase (DBH) deficiency 345
        
        9.2.2.2 Congenital absence of norepinephrine due to CYB561 mutations 348
        
        9.2.2.3 Familial autonomic ganglionopathy caused by rare CHRNA3 genetic variants 348
        
        9.2.2.4 Other genetic disorders associated with autonomic failure 349
        
        9.2.2.5 Familial dysautonomia (see chapter on peripheral autonomic neuropathies) 349
        
        9.2.2.5.1 Waardenburg’s syndrome 349
        
        References 350
      - 10 Spinal cord injuries 361
        
        10.1 Introduction 361
        
        10.1.1 Anatomy 361
        
        10.1.2 Cardiovascular autonomic testing 362
        
        10.1.3 Thermoregulatory function following spinal cord injury 365
        
        10.1.4 Orthostatic hypotension following spinal cord injury: understanding clinical pathophysiology 368
        
        10.1.5 Hypertension following spinal cord injury 370
        
        10.1.6 Respiratory function 371
        
        10.1.7 Bladder function 371
        
        10.1.8 Gastrointestinal function 372
        
        10.1.9 Sexual function 374
        
        10.1.10 Pupillomotor abnormalities 374
        
        10.1.11 Trophic disorders 375
        
        10.1.12 Phantom experiences following spinal cord injury 376
        
        10.1.13 Triple response of Lewis in patients with spinal cord injury 376
        
        10.2 Autonomic dysreflexia 377
        
        10.2.1 Introduction 377
        
        10.2.2 Clinical manifestation and autonomic testing 379
        
        10.2.3 Management of autonomic dysreflexia 381
        
        References 383
      - 11 Autonomic adaptation to hypoxia: mountain medicine 387
        
        11.1 Cardiovascular function at altitude 388
        
        11.2 Cerebrovascular function at altitude 390
        
        11.3 Oxygenation during exercise in Tibetan and Han-Chinese 393
        
        11.4 Sleep 394
        
        11.5 Neuropeptides, altitude, and exercise 396
        
        11.5.1 Opioids 396
        
        11.5.2 Endothelin (ET) 397
        
        11.5.3 Neuropeptide-Y (NPY-LI) 400
        
        11.6 Water and electrolytes 400
        
        11.7 Autonomic reflexes and altitude 401
        
        11.7.1 Pupillary function, age, and altitude 401
        
        11.7.2 Heart rate variability 401
        
        11.7.3 Swallow-induced tachycardia 402
        
        11.7.4 Skin flare 403
        
        11.8 Circulating catecholamines 404
        
        11.9 Thermoregulatory and vasomotor effects of high altitude 406
        
        11.10 Altitude hypoxia: exercise and cardiovascular responses 408
        
        11.11 Pentoxifylline 413
        
        11.11.1 Pentoxifylline—physiology and pharmacology 413
        
        11.11.2 Pentoxifylline—physiological effects at high altitude 415
        
        11.11.3 Pentoxifylline—neurologic and thermoregulatory effects at high altitude 419
        
        11.11.4 Reflex vasoconstriction in the skin at altitude 422
        
        11.11.5 Poststrain (Valsalva) bradycardia 425
        
        11.11.6 Reflow after circulatory arrest 426
        
        11.11.7 Temperature difference—fingertips to palms 427
        
        11.12 Clinical effects of altitude exposure 432
        
        11.12.1 The pulmonary circulation 432
        
        11.12.2 J receptors 434
        
        11.12.3 High-altitude pulmonary edema 435
        
        11.12.4 Systemic blood pressure 437
        
        11.12.6 Hypoxia at sea level 438
        
        11.12.5 Saliva 438
        
        11.12.7 Chronic mountain sickness (CMS) 439
        
        11.12.8 Altitude neuropathy 443
        
        11.13 Freestyle neurology 443
        
        References 443
      - 12 The autonomic nervous system in space exploration 451
        
        12.1 Introduction 451
        
        12.2 Fluid shifts during spaceflight 452
        
        12.3 Changes in blood volume with microgravity 453
        
        12.3.1 Centralization of blood volume in microgravity 453
        
        12.3.2 Decreased circulating volume 455
        
        12.3.3 Metabolic and inflammatory factors and vascular changes 457
        
        12.4 Autonomic function testing 457
        
        12.5 Deconditioning and miscellaneous autonomic manifestations 458
        
        12.6 Altered central processing and brain changes in space 459
        
        12.7 Simulated microgravity 460
        
        12.8 Postflight autonomic changes 461
        
        12.9 Space motion sickness and disruption of vestibular-autonomic reflexes 462
        
        12.10 Bed rest deconditioning and orthostatic intolerance 463
        
        12.11 Effects of sex and gender on adaptation to space 466
        
        12.12 Countermeasures to combat orthostatic intolerance 467
        
        12.13 Brain and blood changes in humans during prolonged isolation 468
        
        References 469
      - 13 Testing autonomic function 477
        
        13.1 Key concepts 481
        
        13.2 Tests of cardiovascular autonomic function 482
        
        13.2.1 Heart rate and blood pressure measurements 486
        
        13.2.2 Blood flow measurements 487
        
        13.2.3 Baroreflex pathway testing 490
        
        13.2.4 Active standing and passive head-up tilting 491
        
        13.2.5 Lower body negative pressure 498
        
        13.2.6 The Valsalva maneuver 499
        
        13.2.7 Baroreflex sensitivity 501
        
        13.3 Efferent sympathetic pathway testing 503
        
        13.3.1 Microneurography 503
        
        13.3.4 Mental stress test 504
        
        13.3.2 Cold pressor test 504
        
        13.3.3 Isometric exercise (sustained handgrip) 504
        
        13.3.5 Inspiratory gasping 505
        
        13.3.6 Plasma catecholamines and pharmacologic testing 505
        
        13.3.7 Tests of cardiovagal function 507
        
        13.3.8 Resting heart rate variability 508
        
        13.3.9 Standing 508
        
        13.3.10 Deep breathing 509
        
        13.3.11 Diving response (cold face test) 509
        
        13.3.12 Carotid sinus massage 510

Author's affiliation

Otto Appenzeller: University of New Mexico School of Medicine, and New Mexico Health Enhancement and Marathon Clinics Research Foundation, USA
Guillaume J. Lamotte: Assistant Professor of Neurology Movement Disorders and Autonomic Disorders, Department of Neurology, University of Utah, USA
Elizabeth A. Coon: Mayo Clinic, USA

Volume 2

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