II. Types: Chronic Autonomic Failure

  1. Orthostatic Hypotension
    1. Diabetes Mellitus
    2. Amyloidosis
    3. Tabes Dorsalis (Syphilis)
    4. Multiple Sclerosis
    5. Acute Intermittent Porphyria
    6. Guillain-Barre Syndrome
    7. Connective Tissue Disorders
    8. Spinal Cord Injury
    9. Toxins
      1. Vacor rat poison
      2. Arsenic
      3. Mercury
    10. Renal Failure with Uremia
    11. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
    12. Wernicke Encephalopathy (chronic Alcohol Abuse)
    13. Medication Causes of Orthostatic Hypotension
  2. Primary Chronic Autonomic Failure (Idiopathic)
    1. Pure autonomic failure
    2. Multiple system atrophy (e.g. Shy-Drager Syndrome)
    3. Parkinson's Disease (or Lewy Body Dementia)
  3. Riley-Day Syndrome (Familial Dysautonomia)
    1. Peripheral and central degenerative disorder
    2. Decreased Lacrimation
    3. Transient skin blotching
    4. Episodic Hypertension
    5. Episodic fever
    6. Vomiting
    7. Altered Taste discrimination
    8. Decreased sensitivity to pain
    9. Emotional lability
  4. Chronic Orthostatic Intolerance
    1. Postural Orthostatic Tachycardia Syndrome (POTS Syndrome)
    2. Neurocardiogenic Syncope
  5. Hirschprung's Megacolon
    1. Congenital absence of parasympathetic Ganglion cells in colon wall
    2. Results in decreased colon motility, Constipation and a significantly dilated colon
  6. Other causes
    1. Mast Cell Activation Syndrome (Hypotension, Palpitations, Urticaria, Wheezing, Rhinitis, Pharyngitis, Conjunctivitis)
    2. Panic Disorder (psychogenic Ischemic Heart Disease)
    3. Neurogenic Essential Hypertension
    4. Congestive Heart Failure
    5. Chronic Fatigue Syndrome
    6. Sjogren Syndrome
    7. Systemic Lupus Erythematosus

III. Findings: Symptoms and Signs

  1. Postural Hypotension
  2. Nocturnal Diarrhea
  3. Impotence
  4. Incomplete voiding

IV. Diagnostics: Telemetry monitoring

  1. No overshoot Tachycardia on standing
  2. No variation in pulse with Valsalva Maneuver
  3. No Blood Pressure (<10 mmHg) increase with hand grip

V. Management: Orthostatic Hypotension

  1. See Vasovagal Syncope for assessment and management

VI. References

  1. Goldberg (2014) Clinical Physiology, p. 104-5
  2. Pryse-Phillips in Noble (2001) Primary Care, p. 1599
  3. Saper in Goldman (2000) Cecil Medicine, p. 2057-8
  4. Goldstein (2002) Ann Intern Med 137:753-63 [PubMed]

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Related Studies

Ontology: Dysautonomia (C0013363)

Definition (MSH) Disorders of the AUTONOMIC NERVOUS SYSTEM occurring as a primary condition. Manifestations can involve any or all body systems but commonly affect the BLOOD PRESSURE and HEART RATE.
Definition (NCI) An acute or chronic disorder, affecting the sympathetic or parasympathetic nervous system. It can be primary, the result of central nervous system degeneration, or secondary due to diabetes or alcoholism. Patients with the chronic form of this disorder usually have a progressive clinical course and a poor prognosis.
Concepts Disease or Syndrome (T047)
MSH D054969 , D001342
SnomedCT 15241006
Dutch dysautonomie, Dysautonomie
Japanese ジリツシンケイイジョウショウ, 原発性自律神経機能異常, 原発性自律神経異常症, 自律神経異常症, 自律神経機能異常, 自律神経異常症-原発性, 自律神経異常, 自律神経機能異常-原発性
English Primary Dysautonomias, Dysautonomias, Dysautonomias, Primary, Primary Dysautonomia, Dysautonomia, Primary, Primary Dysautonomias [Disease/Finding], dysautonomia, dysautonomias, Dysautonomia, NOS, Dysautonomia
Czech Dysautonomie, dysautonomie, primární dysautonomie
Spanish Disautonomías Primarias, Disautonomía, disautonomía
French Dysautonomies primitives, Dysautonomie, Dysautonomies primaires
Italian Disautonomia, Disautonomia primaria
German Dysautonomie
Portuguese Disautonomias Primárias, Disautonomia
Swedish Primära dysautonomier
Polish Dysautonomie pierwotne, Zaburzenia czynności układu wegetatywnego pierwotne, Pierwotne dysautonomie
Hungarian Vegetatív idegrendszeri zavar
Norwegian Primære dysautonomier, Dysautonomi, Dysautonomia, Primær dysautonomi

Ontology: Autonomic neuropathy (C0259749)

Definition (NCI) An inherited or acquired peripheral neuropathy affecting the autonomic nervous system. It results in disruption of the involuntary body functions. Inherited causes include Fabry disease and porphyrias. Acquired causes include diabetes, uremia, hepatic disorders, vitamin deficiencies, toxins, and drug toxicities.
Concepts Disease or Syndrome (T047)
SnomedCT 277879009
English AUTONOMIC NEUROPATHY, Autonomic neuropathy NOS, autonomic neuropathy, neuropathy autonomic, autonomic neuropathies, Autonomic neuropathy, Autonomic neuropathy (disorder), Autonomic Neuropathy
Italian Neuropatia autonomica, Neuropatia autonomica NAS
Dutch autonome neuropathie NAO, autonome neuropathie
French Neuropathie du système nerveux autonome SAI, Neuropathie du système nerveux autonome
German autonome Neuropathie NNB, autonome Neuropathie
Portuguese Neuropatia vegetativa NE, Neuropatia vegetativa
Spanish Neuropatía autonómica NEOM, neuropatía autónoma (trastorno), neuropatía autónoma, Neuropatía autonómica
Japanese 自律神経ニューロパチーNOS, ジリツシンケイニューロパチーNOS, 自律神経ニューロパチー, ジリツシンケイニューロパチー
Czech Autonomní neuropatie NOS, Autonomní neuropatie
Hungarian Vegetatív neuropátia k.m.n., Vegetatív neuropátia

Ontology: Autonomic nervous system disorders (C1145628)

Definition (MEDLINEPLUS)

Your autonomic nervous system is the part of your nervous system that controls involuntary actions, such as the beating of your heart and the widening or narrowing of your blood vessels. When something goes wrong in this system, it can cause serious problems, including

  • Blood pressure problems
  • Heart problems
  • Trouble with breathing and swallowing
  • Erectile dysfunction in men

Autonomic nervous system disorders can occur alone or as the result of another disease, such as Parkinson's disease, alcoholism and diabetes. Problems can affect either part of the system, as in complex regional pain syndromes, or all of the system. Some types are temporary, but many worsen over time. When they affect your breathing or heart function, these disorders can be life-threatening.

Some autonomic nervous system disorders get better when an underlying disease is treated. Often, however, there is no cure. In that case, the goal of treatment is to improve symptoms.

NIH: National Institute of Neurological Disorders and Stroke

Definition (MSH) Diseases of the parasympathetic or sympathetic divisions of the AUTONOMIC NERVOUS SYSTEM; which has components located in the CENTRAL NERVOUS SYSTEM and PERIPHERAL NERVOUS SYSTEM. Autonomic dysfunction may be associated with HYPOTHALAMIC DISEASES; BRAIN STEM disorders; SPINAL CORD DISEASES; and PERIPHERAL NERVOUS SYSTEM DISEASES. Manifestations include impairments of vegetative functions including the maintenance of BLOOD PRESSURE; HEART RATE; pupil function; SWEATING; REPRODUCTIVE AND URINARY PHYSIOLOGY; and DIGESTION.
Definition (CSP) condition in which there is a deviation from or interruption of the normal structure or function of the parasympathetic or sympathetic divisions of the autonomic nervous system; autonomic dysfunction may be associated with hypothalamic diseases, brain stem disorders, spinal cord diseases, and peripheral nervous system diseases; manifestations include impairments of vegetative functions including the maintenance of blood pressure, heart rate, pupil function, sweating, reproductive and urinary physiology, and digestion.
Concepts Disease or Syndrome (T047)
MSH D001342
ICD9 337.9, 337
ICD10 G90.9 , G90
SnomedCT 267588005, 192922002, 15241006
English ANS Disease, ANS Diseases, Autonomic Nervous System Diseases, Unspecified disorder of autonomic nervous system, Disorders of the autonomic nervous system, Autonomic Diseases, Autonomic Disease, AUTONOMIC NERVOUS SYSTEM DISORDERS, Autonomic Nervous System Disorders, Autonomic nervous sys.dis NOS, Autonomic nervous system disorder NOS, Disord of autonomic nrvous sys, Disorder of autonomic nervous system, unspecified, autonomic disorder, DIS AUTONOMIC NERVOUS SYSTEM, AUTONOMIC NERVOUS SYSTEM DIS, ANS DIS, NERVOUS SYSTEM DIS AUTONOMIC, CENTRAL AUTONOMIC NERVOUS SYSTEM DIS, AUTONOMIC DIS, autonomic nervous system disorder (diagnosis), autonomic nervous system disorder, ANS disorder, ANS (Autonomic Nervous System) Diseases, Central Autonomic Nervous System Diseases, Nervous System Diseases, Autonomic, Disorders of the Autonomic Nervous System, Autonomic nerve dis NEC, Disorder of the autonomic nervous system, unspecified, Autonomic Nervous System Diseases [Disease/Finding], ans disease, autonomic disorders nervous system, autonomic diseases nervous system, diseases ans, autonomic disorders, dysautonomia, disease autonomic, autonomic disease, autonomic disease nervous system, autonomic diseases, autonomic nervous disorders, disease ans, AUTONOMIC CNS DIS, Autonomic Central Nervous System Diseases, Autonomic nervous system disorder NOS (disorder), Autonomic nervous system--Diseases, Disorders of autonomic nervous system, Disorder of autonomic nervous system (disorder), Disorder of autonomic nervous system, Disorder of vegetative system, Autonomic nervous system disorders, autonomic; nervous system, disorder, disease (or disorder); autonomic nervous system, disease (or disorder); nervous system, autonomic, disease (or disorder); nervous system, vegetative, nervous system; disorder, autonomic, Disorder of autonomic nervous system, NOS, Disorder of vegetative system, NOS
French SYSTEME NERVEUX AUTONOME, Affections du système nerveux autonomique, Affection non précisée du système nerveux autonomique, Affections du système nerveux autonome, Maladies du système nerveux autonome, Maladies du système nerveux végétatif, Maladies du SNA, Maladies du système nerveux autonome (SNA)
German VEGETATIVES NERVENSYSTEM, unspezifische Stoerung des autonomen Nervensystems, Stoerungen des autonomen Nervensystems, Krankheit des autonomen Nervensystems, nicht naeher bezeichnet, Krankheiten des autonomen Nervensystems, Affektionen des autonomen Nervensystems, Vegetatives Nervensystem, Krankheiten, Krankheiten, autonomes Nervensystem, Nervensystem-Krankheiten, vegetative, Vegetative Nervensystem-Krankheiten, Störungen des vegetativen Nervensystems
Italian Patologie del sistema nervoso autonomo, Disturbo non specificato del sistema nervoso autonomo, Malattie del SNA, Malattie del sistema nervoso autonomo centrale, Disturbi del sistema nervoso autonomo, SNA (sistema nervoso autonomo) malattie, Malattie del sistema nervoso autonomo
Spanish Alteraciones del sistema nervioso autónomo, Trastorno no especificado del sistema nervioso autónomo, Trastornos del sistema nervioso autónomo, trastorno del sistema nervioso autonómico, TRASTORNOS DEL SISTEMA NERVIOSO VEGETATIVO, trastorno del sistema nervioso autónomo, SAI, trastorno del sistema nervioso autónomo, SAI (trastorno), trastorno del sistema vegetativo, trastorno del sistema nervioso autonómico (trastorno), trastorno del sistema nervioso autónomo, trastorno del sistema nervioso autónomo (trastorno), Enfermedades del Sistema Nervioso Autónomo Central, Enfermedades del Sistema Nervioso Autónomo
Dutch niet-gespecificeerde aandoening van autonome zenuwstelsel, aandoeningen van het autonome zenuwstelsel, aandoening; autonoom zenuwstelsel, aandoening; zenuwstelsel, autonoom, aandoening; zenuwstelsel, vegetatief, autonoom; zenuwstelsel, aandoening, zenuwstelsel; aandoening, autonoom, Aandoening van autonoom zenuwstelsel, niet gespecificeerd, autonome zenuwstelselaandoeningen, Aandoeningen van autonoom zenuwstelsel, Autonoom-zenuwstelselziekte, Autonoom-zenuwstelselziekten, Centraal-autonoomzenuwstelselziekte, Stoornis van het autonome zenuwstelsel, Zenuwstelselziekte, autonoom-, Ziekte, autonoom-zenuwstelsel-, Ziekten, autonoom-zenuwstelsel-
Portuguese Afecções do sistema neurovegetativo, Afecção NE do sistema nervoso vegetativo, DISTURBIOS DO SISTEMA NERVOSO AUTONOMO, Afecções do sistema nervoso vegetativo, Doenças do Sistema Nervoso Autônomo Central, Doenças do Sistema Nervoso Autônomo, Transtornos do Sistema Nervoso Autônomo
Japanese 自律神経障害, 詳細不明の自律神経系の障害, ジリツシンケイショウガイ, ショウサイフメイノジリツシンケイケイノショウガイ, 植物神経系疾患, 自律神経系疾患
Swedish Autonoma nervsystemets sjukdomar
Finnish Autonomisen hermoston sairaudet
Czech Poruchy autonomního nervového systému, Blíže neurčená porucha autonomního nevového systému, Poruchy vegetativního nervového systému, autonomní nervová soustava - nemoci, nemoci vegetativní soustavy, nemoci autonomního nervového systému, vegetativní nervový systém - onemocnění
Korean 자율신경계통의 장애, 상세불명의 자율신경계통 장애
Croatian Not Translated[Autonomic Nervous System Diseases]
Polish Choroby układu nerwowego autonomicznego
Hungarian Autonom idegrendszer betegségek, Az autonom idegrendszer nem meghatározott betegsége, Az autonom idegrendszer betegségei
Norwegian Forstyrrelser i det autonome nervesystemet, Sykdommer i det autonome nervesystemet