(See also Overview of the Autonomic Nervous System Overview of the Autonomic Nervous System The autonomic nervous system regulates physiologic processes. Regulation occurs without conscious control, ie, autonomously. The 2 major divisions are the Sympathetic system Parasympathetic... read more .)
Pure autonomic failure, previously called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome, denotes generalized autonomic failure without central nervous system (CNS) involvement. This disorder differs from multiple system atrophy Multiple System Atrophy (MSA) Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. It includes 3 disorders previously thought to be distinct... read more because it lacks central or preganglionic involvement. Pure autonomic failure affects more women, tends to begin during a person’s 40s or 50s, and does not result in death.
Pure autonomic failure is a synucleinopathy Pathophysiology (due to synuclein deposition); synuclein can also accumulate in patients with Parkinson disease Parkinson Disease Parkinson disease is a slowly progressive, degenerative disorder characterized by resting tremor, stiffness (rigidity), slow and decreased movement (bradykinesia), and eventually gait and/or... read more , multiple system atrophy Multiple System Atrophy (MSA) Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. It includes 3 disorders previously thought to be distinct... read more , or dementia with Lewy bodies Dementia with Lewy Bodies and Parkinson Disease Dementia Lewy body dementia includes clinically diagnosed dementia with Lewy bodies and Parkinson disease dementia. Dementia with Lewy bodies is chronic cognitive deterioration characterized by cellular... read more . (Synuclein is a neuronal and glial cell protein that can aggregate into insoluble fibrils and form Lewy bodies.) Some patients with pure autonomic failure eventually develop multiple system atrophy or dementia with Lewy bodies.
Symptoms and Signs of Pure Autonomic Failure
The main symptom is
There may be other autonomic symptoms, such as decreased sweating, heat intolerance, urinary retention Dementia with Lewy Bodies and Parkinson Disease Dementia Lewy body dementia includes clinically diagnosed dementia with Lewy bodies and Parkinson disease dementia. Dementia with Lewy bodies is chronic cognitive deterioration characterized by cellular... read more , bladder spasms (possibly causing incontinence Urinary Incontinence in Adults Urinary incontinence is involuntary loss of urine; some experts consider it present only when a patient thinks it is a problem. The disorder is greatly underrecognized and underreported. Many... read more ), erectile dysfunction Erectile Dysfunction Erectile dysfunction is the inability to attain or sustain an erection satisfactory for sexual intercourse. Most erectile dysfunction is related to vascular, neurologic, psychologic, and hormonal... read more , fecal incontinence Fecal Incontinence Fecal incontinence is involuntary defecation. Diagnosis is clinical. Treatment is a bowel management program and perineal exercises, but sometimes colostomy is needed. (See also Evaluation of... read more or constipation Fecal Incontinence Fecal incontinence is involuntary defecation. Diagnosis is clinical. Treatment is a bowel management program and perineal exercises, but sometimes colostomy is needed. (See also Evaluation of... read more , dry eyes and/or mouth, and pupillary abnormalities.
Rapid eye movement (REM) sleep behavior disorder Rapid eye movement (REM) sleep behavior disorder Parasomnias are undesirable behaviors that occur during entry into sleep, during sleep, or during arousal from sleep. Diagnosis is clinical. Treatment may include drugs and psychotherapy. (See... read more may occur.
Diagnosis of Pure Autonomic Failure
Diagnosis of pure autonomic failure is by exclusion. The norepinephrine level is usually < 100 pg/mL supine and does not increase with standing. Postural orthostatic tachycardia syndrome Postural orthostatic tachycardia syndrome (POTS) Orthostatic (postural) hypotension is an excessive fall in blood pressure (BP) when an upright position is assumed. The consensus definition is a drop of > 20 mm Hg systolic, > 10 mm Hg... read more can be differentiated because with standing, it does not usually cause hypotension, the norepinephrine level increases, and heart rate increases by > 30 beats/minutes or to 120 beats/minutes within 10 minutes.
Treatment of Pure Autonomic Failure
Treatment of pure autonomic failure is symptomatic:
Orthostatic hypotension Treatment Orthostatic (postural) hypotension is an excessive fall in blood pressure (BP) when an upright position is assumed. The consensus definition is a drop of > 20 mm Hg systolic, > 10 mm Hg... read more : Volume expansion, vasopressors, and support hose are used. Sometimes fludrocortisone 0.1 to 0.4 mg orally once a day and/or alpha-adrenoreceptor stimulation with midodrine 10 mg orally 3 times a day is given, but midodrine may increase supine blood pressure more than is desirable. Raising the head of the bed about 10 cm reduces nocturnal polyuria and supine hypertension and may reduce morning orthostatic hypotension. Alternatively, droxidopa can be used. Droxidopa has a mechanism similar to that of midodrine but a longer duration of action.
Constipation Treatment Constipation is difficult or infrequent passage of stool, hardness of stool, or a feeling of incomplete evacuation. (See also Constipation in Children.) No bodily function is more variable and... read more : A high-fiber diet and stool softeners are recommended. Sometimes enemas are needed.
Bladder spasms Neurogenic Bladder Neurogenic bladder is bladder dysfunction (flaccid or spastic) caused by neurologic damage. Symptoms can include overflow incontinence, frequency, urgency, urge incontinence, and retention.... read more : Oxybutynin chloride 5 mg orally 3 times a day or tolterodine 2 mg orally twice a day can be used to treat bladder spasms. For urinary urgency, tamsulosin 0.4 to 0.8 mg once a day or the beta-3 adrenergic agonist mirabegron (which does not increase orthostatic hypotension) 25 to 50 mg once a day may be given.
Urinary retention Treatment Urinary retention is incomplete emptying of the bladder or cessation of urination. Urinary retention may be Acute Chronic Causes include impaired bladder contractility, bladder outlet obstruction... read more : Self-catheterization of the bladder may be needed. Sometimes drugs that induce bladder contraction (eg, bethanechol 10 to 50 mg orally 3 or 4 times a day) are given.
Sweating abnormalities: Hot conditions should be avoided. For dry mouth, good dental care and regular dental check-ups are important. Artificial tears may help patients with dry eyes.
Erectile dysfunction Treatment Erectile dysfunction is the inability to attain or sustain an erection satisfactory for sexual intercourse. Most erectile dysfunction is related to vascular, neurologic, psychologic, and hormonal... read more : Drugs such as sildenafil 50 mg orally as needed or tadalafil 2.5 to 5 mg once a day and various physical means can be used.
Pure autonomic failure, like Parkinson disease, multiple system atrophy, and dementia with Lewy bodies, is a synucleinopathy.
The main symptom is orthostatic hypotension.
Diagnose by excluding other disorders that cause similar symptoms.
Use treatments specific for the symptoms present.
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