Autonomic Neuropathy Clinical Presentation

Updated: May 22, 2017
  • Author: Steven D Arbogast, DO; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Presentation

History

Most of the primary autonomic disorders are chronic in nature, with symptoms often initiating in an insidious fashion. However, in acute autonomic neuropathies, the onset can be dramatic with presentation as a generalized dysautonomia. In general, patients present with symptoms of both sympathetic and parasympathetic dysfunction, with or without symptoms of somatic nervous system dysfunction. [47] Some symptoms, such as those of orthostatic intolerance, are common in autonomic neuropathies, whereas other symptoms, such as complete anhidrosis, are rare as a primary manifestation.

Orthostatic hypotension is often the first recognized symptom and is typically the most disabling. [7] However, other autonomic symptoms can occur before syncope, and these include impotence or ejaculatory dysfunction, decreased sweating, and urinary incontinence. For example, in Sj ö gren syndrome, dry mouth and eyes along with anhidrosis are typically the initial symptoms in affected patients. Detailed family history may yield information about possible inherited forms of autonomic neuropathy. In some cases, involvement may be subtle in certain family members, thus escaping detection. Careful attention to use and dosage of prescription medication as well as over-the-counter nutritional and other health or dietary supplements is important.

A thorough history and review of systems may reveal many of the following complaints.

  • Facial - Facial pallor, anhidrosis
  • Ocular - Blurring then graying of vision, blacking out, tunnel vision, sensitivity to light, difficulty with focusing, reduced lacrimation, and loss of pupillary size over time (which is often correlated with loss of visual symptoms)
  • Cardiovascular - Orthostatic onset of palpitations, nausea, tremulousness, presyncope with light-headedness, visual blurring, tinnitus, headache, chest pain, and shortness of breath (Elderly patients may complain of coat hanger or lower extremity discomfort. These symptoms may be worse after a large meal or in severe illness.)
  • Gastrointestinal - Constipation, episodic diarrhea, early satiety, increased gastric motility, dysphagia, bowel atony, bowel incontinence, gastroparesis in diabetes mellitus (which may cause food stasis and subsequent vomiting) [48] , hyposalivation, altered sense of taste [49] , presyncope with micturition, and defecation [50]
  • Renal - Nocturia, bladder urgency, bladder frequency, enuresis, incomplete bladder voiding, urinary retention, and urinary incontinence
  • Sexual – Impotence, loss of ejaculation, and retrograde ejaculation in men; inability to achieve orgasm or nonspecific sexual dysfunction in both sexes
  • Sweating - Anhidrosis or hypohidrosis, compensatory hyperhidrosis, and gustatory sweating [48]
  • Temperature regulation - Hypothermia (from loss of shivering and inability to vasoconstrict to prevent heat loss) and hyperpyrexia
  • Extremities - Burning feet most commonly observed in small-fiber sensory neuropathy, pruritus, dysesthesia, allodynia, hyperalgesia, nocturnal exacerbation of symptoms, dry skin, and loss of distal leg hair, brittle nails, pallor, and cold feet
  • Respiratory – Impaired control of bronchomotor tone, leading to a depressed bronchoconstrictory response to cholinergic stimuli (caused by diabetes mellitus); impaired ventilatory and heart rate response to hypoxia, but not to hypercapnia, in patients with diabetes
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Physical

General techniques of the physical examination

Detailed neurologic examination should be performed to detect a somatic peripheral neuropathy. Motor examination should concentrate on the strength and muscle bulk of distal muscles, and on deep tendon reflexes. Sensory examination should include assessment of painful and temperature stimuli, as well as light touch, vibration, and proprioception to distal extremities. An important finding on sensory examination is a stocking and glove pattern of sensory loss, which suggests concurrent somatic neuropathy. Coordination and gait are important to assess for an ataxic component to any suspected peripheral neuropathy.

Specific abnormalities in autonomic functioning can be detected by using physical examination techniques, including the following:

  • Orthostatic vital signs are essential. Blood pressure and heart rate is measured after several minutes of baseline rest, 1 minute after sitting and 3 minutes after standing.
    • Orthostatic hypotension is present if systolic pressure increases more than 20 mm Hg or drops 10 mm Hg in the presence of presyncopal symptoms.
    • Postural tachycardia syndrome is present if tachycardia response is excessive (>30 bpm increase from baseline).
  • Examine the palms, soles, and axillae for sweat.
  • Examine pupillary responses to light and accommodation.
  • Examine for presence of Horner syndrome with light palpation of both sides of the face to determine unilateral anhidrosis, assessment of pupillary size to determine miosis, and assessment for ptosis. Of note, ptosis in Horner syndrome is due to a sympathetic defect to Mueller muscle, which is found in both superior and inferior eyelids; therefore, Horner syndrome can produce a ptosis of both upper and lower eyelids.
  • Examine the oral cavity for excessive dental caries in xerostomia.
  • Examine the conjunctiva and cornea for excessive scratches or signs of trauma due to xerophthalmia.
  • Palpate the lower abdomen for detection of a distended bladder.

Disease-specific findings on physical examination

See the list below:

  • Skin and mucosal membrane changes and ulcerations can be observed in leprosy, Lyme disease, HSAN, and diphtheria.
  • Angiokeratomas of the trunk or groin in a patient with a history of renal failure and previous strokes is suggestive of Fabry disease.
  • Somatic neuropathy, systemic infections, and other HIV/AIDS systemic manifestations can suggest HIV-associated neuropathy. [29]
  • Concomitant liver, renal, and cardiac disease may suggest amyloidosis.
  • Hepatomegaly, spider nevi, caput medusae, parotid hypertrophy, Dupuytren contracture, and other features of alcoholism may suggest a concurrent ethanol/nutritional neuropathy.
  • Occurrence of arthritis, rash, renal disease, pulmonary disease, xerophthalmia, and xerostomia can suggest a connective tissue disorder, such as rheumatoid arthritis, systemic lupus erythematosus, or Sj ö gren syndrome.
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Causes

The causes of autonomic neuropathy are varied. The discussion noted above in the Pathophysiology covers many of the common and uncommon causes of autonomic neuropathy.

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