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ESSENTIALS OF DIAGNOSIS
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Abnormalities of blood pressure, heart rate, sweating, intestinal motility, sphincter control, sexual function, respiration, or ocular function, occurring in isolation or any combination
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Dysautonomia may occur as a result of central or peripheral pathologic processes It is manifested by a variety of symptoms that may occur in isolation or in various combinations and relate to abnormalities of blood pressure regulation, thermoregulatory sweating, gastrointestinal function, sphincter control, sexual function, respiration, and ocular function Syncope, a symptom of dysautonomia, is characterized by a transient loss of consciousness, usually accompanied by hypotension and bradycardia It 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
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A Central Neurologic Causes
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Disease at certain sites in the central nervous system, regardless of its nature, may lead to dysautonomic symptoms Postural hypotension, which is usually the most troublesome and disabling symptom, may result from spinal cord transection and other myelopathies (eg, due to tumor or syringomyelia) above the T6 level or from brainstem lesions such as syringobulbia and posterior fossa tumors Sphincter or sexual disturbances may result from cord lesions below T6 Certain primary degenerative disorders are responsible for dysautonomia occurring in isolation (pure autonomic failure) or in association with more widespread abnormalities (multisystem atrophy or Shy-Drager syndrome) that may include parkinsonian, pyramidal symptoms, and cerebellar deficits
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B Peripheral Neurologic Causes
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A pure autonomic neuropathy may occur acutely or subacutely after a viral infection or as a paraneoplastic disorder related usually to small cell lung cancer, particularly in association with certain antibodies, such as anti-Hu or those directed at neuronal nicotinic acetylcholine receptors Typically, presenting symptoms include postural hypotension, impaired thermoregulatory sweating, xerostomia or xerophthalmia, abnormal gastrointestinal motility, dilated pupils, or acute urinary retention Dysautonomia is often conspicuous in patients with Guillain-Barr syndrome, manifesting with marked hypotension or hypertension or cardiac arrhythmias that may have a fatal outcome It may also occur with diabetic, uremic, amyloidotic, and various other metabolic or toxic neuropathies; in association with leprosy or Chagas disease; and as a feature of certain hereditary neuropathies with auto-
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somal dominant or recessive inheritance or an X-linked pattern Autonomic symptoms are prominent in the crises of hepatic porphyria Patients with botulism or the LambertEaton myasthenic syndrome may have constipation, urinary retention, and a sicca syndrome as a result of impaired cholinergic function
CMDT 2008
Clinical Findings
A Symptoms and Signs
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, 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 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 are common postictally
tion, sleeping in a semierect position (which minimizes the natriuresis and diuresis that occur during recumbency), and fludrocortisone (01 02 mg daily) Vasoconstrictor agents may be helpful and include midodrine (25 10 mg three times daily) and ephedrine (15 30 mg three times daily) Other agents that have been used occasionally or experimentally are dihydroergotamine, yohimbine, and clonidine; refractory cases may respond to erythropoietin (epoetin alfa) or desmopressin Patients must be monitored for recumbent hypertension Postprandial hypotension is helped by caffeine There is no satisfactory treatment for disturbances of sweating, but an air-conditioned environment is helpful in avoiding extreme swings in body temperature
Freeman R Autonomic peripheral neuropathy Lancet 2005 Apr 2-8;365(9466):1259 70 [PMID: 15811460] Mathias CJ Multiple system atrophy and autonomic failure J Neural Transm Suppl 2006;(70):343 7 [PMID: 17017551]
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