Dysautonomic symptoms include syncope, postural hypotension, paroxysmal hypertension, persistent tachycardia without other cause, facial flushing, hypohidrosis or hyperhidrosis, vomiting, constipation, diarrhea, dysphagia, abdominal distention, disturbances of micturition or defecation, erectile dysfunction, apneic episodes, and declining night vision. In syncope, prodromal malaise, nausea, headache, diaphoresis, pallor, visual disturbance, loss of postural tone, and a sense of weakness and impending loss of consciousness are followed by actual loss of consciousness. It is usually accompanied by hypotension and bradycardia and may occur in response to emotional stress, postural hypotension, vigorous exercise in a hot environment, obstructed venous return to the heart, acute pain or its anticipation, fluid loss, and a variety of other circumstances. Although the patient is usually flaccid, some motor activity is not uncommon, and urinary (and rarely fecal) incontinence may also occur, thereby simulating a seizure. Recovery is rapid once the patient becomes recumbent, but headache, nausea, and fatigue commonly persist.
B. Evaluation of the Patient
The extent and severity of autonomic dysfunction should be determined, and the presence of associated neurologic symptoms and signs ascertained. Bedside testing of autonomic function includes examination of pupillary reactivity, examination of the skin for areas of excessive or reduced sweating and of the hands and feet for color or temperature changes, as well as assessment of blood pressure and heart rate in the supine position and 2 minutes after standing. With dysautonomia, postural hypotension is not accompanied by a compensatory rise in heart rate. Specialized tests include the cardiovascular response to the Valsalva maneuver and deep respiration, tilt-table testing, the thermoregulatory sweat test, the quantitative sudomotor axon reflex test, and the quantitative direct and indirect axon reflex test. Tests of gastrointestinal motility and urodynamics may be helpful when symptoms of dysmotility, incontinence, or urinary retention are present.
The neurologic examination should focus on detecting signs of parkinsonism, cerebellar dysfunction, disorders of neuromuscular transmission, and peripheral neuropathy. All patients should be tested for vitamin B12 deficiency and diabetes. Patients with acute or subacute isolated dysautonomia should undergo testing for ganglionic acetylcholine receptor, anti-Hu, voltage-gated potassium channel complex, and voltage-gated calcium channel antibodies. For those with evidence of peripheral neuropathy, nerve conduction studies; electromyography; and testing for HIV, amyloidosis, Sjögren syndrome, and Fabry disease are indicated. If there is evidence of central pathology, imaging studies will exclude a treatable structural cause. If the neurologic examination is normal, reversible, nonneurologic causes of symptoms must be considered. Isolated postural hypotension and syncope may relate to a reduced cardiac output, paroxysmal cardiac dysrhythmias, volume depletion, various medications, and endocrine and metabolic disorders such as Addison disease, hypothyroidism or hyperthyroidism, pheochromocytoma, and carcinoid syndrome.