Autonomic Dysfunction

Aka: Autonomic Dysfunction, Dysautonomia, Autonomic Neuropathy, Chronic Autonomic Neuropathy

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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. Erectile Dysfunction
  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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