Autonomic neuropathy is a collection of diseases and syndromes in which autonomic nervous system, parasympathetic, sympathetic or both are affected. Autonomic neuropathies can either be hereditary or acquired in nature; acquired can further be divided into primary and secondary diseases. Since autonomic nervous system innervates the whole body, autonomic neuropathy can present with various clinical manifestations affecting the entire body. The degree of autonomic dysfunction can be varied, from being subclinical in some patients to full-blown symptoms that are associated with a severe disability.(1)
Life Expectancy Of Someone With Autonomic Neuropathy
Increased morbidity is associated with falls and loss of consciousness in autonomic neuropathies. These can lead to injuries, especially in elderly patients. In many cases, the course of autonomic neuropathy is usually progressive that can have a poor prognosis. Kidney failure in Fabry disease has increased the incidence of death. Severe dysautonomia is associated with an increased risk of sudden death due to cardiac dysrhythmia, which is mostly seen in diabetic neuropathy and Guillain Barre Syndrome.
Cardiac sympathetic dysfunction is seen in both type 1 and types 2 diabetes mellitus and when it is associated with vascular complications and dysautonomia associated with diabetic neuropathy; it leads to increased risk of death.(1)
Patients with inadequately treated or untreated diabetes have higher rates of morbidity and complications associated with neuropathy than patients with controlled diabetes. The treatment of diabetic neuropathy is also a difficult task as most of the medicines do not provide complete symptomatic relief. Various clinical trials are underway to find new therapies for symptomatic treatment and delay in disease progression. Mortality is higher in patients with cardiovascular autonomic neuropathy associated with diabetes mellitus. The overall mortality for a 10 year period is 27% for patients with cardiovascular autonomic neuropathy in diabetes mellitus patients; whereas, it is 5% in patients with diabetes mellitus without any evidence of cardiovascular autonomic neuropathy.(2)
Causes Of Autonomic Neuropathy
The various conditions and diseases that can lead to autonomic neuropathy include inherited causes and acquired causes. Inherited autonomic neuropathies include familial amyloid polyneuropathy, hereditary sensory autonomic neuropathy, Fabry disease, and acute intermittent porphyria and variegate porphyria. These hereditary conditions are usually very rare; whereas, acquired diseases are usually common.(1)
Primary acquired autonomic neuropathies include pandysautonomia, idiopathic distal small fiber neuropathy, chronic idiopathic anhidrosis, amyloid neuropathy, Holmes Adie syndrome, and Ross syndrome, and postural orthostatic tachycardia syndrome. Secondary acquired autonomic neuropathies include diabetic autonomic neuropathy, uremic neuropathy, hepatic disease-related neuropathy, alcohol-related, vitamin deficiency and nutrition-related neuropathy, toxic and drug-induced autonomic neuropathy, Lyme disease, botulism, Chagas disease, HIV infection, diphtheria, leprosy, celiac disease, Sjogren’s disease, systemic lupus erythematosus, rheumatoid arthritis, Lambert Eaton myasthenic syndrome, Guillain Barre syndrome, inflammatory bowel disease, and paraneoplastic autonomic neuropathy.(1)
Symptoms Of Autonomic Neuropathy
The symptoms related to autonomic neuropathy are generalized and include most of the systems, including ocular, cardiovascular, gastrointestinal, renal, respiratory, urogenital and sudomotor. These include blurring of vision, tunnel vision, sensitivity to light, difficulty focusing, reduced lacrimation, loss of pupillary size over time, palpitations, nausea, tremulousness, presyncope with lightheadedness, tinnitus, headache, chest pain, shortness of breath, constipation, diarrhea, early satiety, increased gastric motility, dysphagia, bowel atony, bowel incontinence, gastroparesis in diabetes mellitus, hyposalivation, altered taste sensation, presyncope with urination and excretion, nocturia, bladder urgency and frequency, enuresis, incomplete bladder voiding, urinary retention, urinary incontinence, impotence, loss of ejaculation, retrograde ejaculation, inability to reach orgasm, non specific sexual dysfunction in both the sexes, pallor, anhidrosis or hypohidrosis, hyperhidrosis, gustatory sweating, hypothermia, hyperpyrexia, burning feet, pruritus, dysesthesia, allodynia, hyperalgesia, nocturnal exacerbation of symptoms, dry skin, loss of distal leg hair, brittle nails, and cold feet, and respiratory dysfunction.(1)
Orthostatic hypotension is often the first symptom that is noted and it is the most disabling symptom related to autonomic neuropathy. It is considered to be present if the systolic blood pressure increases more than 20 mm Hg or drops 10 mm Hg in the presence of presyncope symptoms.(1)
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