I searched MEDLINE from 1990 to 2004 with the search terms “peripheral autonomic nervous system diseases”, “autonomic neuropathy”, and “autonomic diseases”. Other search terms used included “diabetes”, “amyloid”, “immunology”, “infectious”, “inherited”, “genetic”, “toxic”, “investigation”, and “therapy”. I largely selected publications from the past 10 years but did not exclude commonly referenced and highly regarded older publications. I also searched the reference lists of articles
SeminarAutonomic peripheral neuropathy
Section snippets
Diabetic autonomic neuropathy
Diabetes mellitus is the most common cause of autonomic neuropathy in more developed countries.3 The autonomic neuropathy typically presents late in the course of diabetes and is generally accompanied by other features of distal sensorimotor polyneuropathy. Diabetic cardiovascular autonomic neuropathy can manifest as an increased resting heart rate when the cardiac vagus is affected. When sympathetic and parasympathetic fibres are involved, the heart rate is fixed and there is inadequate
Amyloid neuropathy
Amyloidosis is due to the deposition of insoluble fibrillar proteins in a β-pleated sheet configuration within the extracellular space of tissues and organs. Various proteins have been associated with amyloidosis. The current classification of the systemic amyloidoses is based on the biochemistry of the precursor protein.26, 27 Although the fibril precursor proteins differ, there are strong similarities among the clinical presentations and pathology of the neuropathies associated with the
Acute and subacute autonomic neuropathies
Guillain-Barré syndrome (acute inflammatory demyelinating polyradiculoneuropathy) is a monophasic illness with an immune basis that presents as an acutely evolving sensorimotor polyneuropathy of varying severity. The syndrome is commonly accompanied by autonomic manifestations such as sinus tachycardia, sinus pauses, other tachyarrhythmias and bradyarrhythmias, blood-pressure lability, bowel and bladder dysfunction, pupillomotor disturbances, sudomotor dysfunction, and vasomotor abnormalities.46
Immune-mediated and paraneoplastic autonomic neuropathies
Autonomic peripheral neuropathies have been associated with the presence of specific autoantibodies (panel 3). These neuropathies present subacutely in most cases, although acute and chronic presentations have been reported. Paraneoplastic autonomic neuropathy occurs frequently in association with anti-Hu antibodies. These antibodies are commonly found in patients with small-cell lung cancer, but they can also occur in non-small-cell lung cancer and malignant disorders of the gastrointestinal
Hereditary autonomic neuropathies
The hereditary autonomic neuropathies are listed in panel 4. Autonomic features are most prominent in the hereditary sensory and autonomic neuropathies and Fabry's disease.89 Other hereditary autonomic neuropathies include the triple A (Allgrove's) syndrome,90 Tangier disease,91 multiple endocrine neoplasia type 2b,92 and a sensory and autonomic neuropathy with arthropathy that is present in Navajo children.93, 94
Botulism
Binding of a neurotoxin from the anaerobic bacterium Clostridium botulinum to the presynaptic nerve terminal prevents release of synaptic vesicles containing acetylcholine and leads to this acute neuromuscular disorder (panel 5). The illness begins with gastrointestinal manifestations, followed by autonomic symptoms and a descending paralysis that spreads from the extraocular and bulbar muscles to the limbs.110 Autonomic symptoms result from cholinergic dysfunction; they include constipation,
Toxic neuropathies
Several industrial toxins, environmental toxins, marine toxins, and therapeutic drugs can cause autonomic dysfunction (panel 6). Autonomic-test abnormalities have been reported in individuals exposed to organic solvents,126 industrial-use acrylamide,127 arsenic,128 thallium,129 and other heavy metals.130 Clinically important autonomic abnormalities are rarely observed with exposure to these toxins. Distal hyperhidrosis and hypohidrosis are the most common autonomic abnormalities.127, 128
Treatment of autonomic neuropathy
Although many of the autonomic neuropathies are not reversible, the symptoms of autonomic dysfunction respond to several specific interventions. This topic has been covered in detail in several recent reviews.139, 140
Search strategy and selection criteria
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Autonomic neuropathy associated with sicca complex
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Idiopathic dysautonomia treated with intravenous gammaglobulin
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Unusual recovery from acute panautonomic neuropathy after immunoglobulin therapy
Mayo Clin Proc
Enteric neuronal autoantibodies in psuedoobstruction with small cell lung carcinoma
Gastroenterology
Autonomic failure and proximal skeletal myopathy in a patient with primary Sjogren syndrome
Mayo Clin Proc
Myasthenia gravis with autoimmune autonomic neuropathy
Auton Neurosci
Identification of a novel gene (HSN2) causing hereditary sensory and autonomic neuropathy type II through the Study of Canadian Genetic Isolates
Am J Hum Genet
Tissue-specific expression of a splicing mutation in the IKBKAP gene causes familial dysautonomia
Am J Hum Genet
Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis
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Heart rate variability: the time domain
Testing the autonomic nervous system
Semin Neurol
The peripheral nervous system and diabetes
The sympathetic nervous system in diabetic neuropathy: a clinical and pathological study
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The association between cardiovascular autonomic neuropathy and mortality in individuals with diabetes: a meta-analysis
Diabetes Care
Increased intraoperative cardiovascular morbidity in diabetics with autonomic neuropathy
Anesthesiology
QT interval prolongation and mortality in type 1 diabetic patients: a 5 year cohort prospective study
Diabetes Care
Cardiac sympathetic dysinnervation in diabetes: implications for enhanced cardiovascular risk
Circulation
Treatment of diabetic cystopathy
Ann Intern Med
Association of type and duration of diabetes with erectile dysfunction in a large cohort of men
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Impaired neurogenic and endothelium-mediated relaxation of penile smooth muscle from diabetic men with impotence
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Diabetes Care
Natural history of diabetic gastroparesis
Diabetes Care
Relationships of upper gastrointestinal motor and sensory function with glycemic control
Diabetes Care
Gastrointestinal tract symptoms among persons with diabetes mellitus in the community
Arch Intern Med
Pathogenesis of fecal incontinence in diabetes mellitus: evidence for internal-anal-sphincter dysfunction
N Engl J Med
Gustatory sweating in diabetes mellitus
Diabet Med
The systemic amyloidoses
N Engl J Med
Hereditary and acquired amyloid neuropathies
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Amyloidosis and neuropathy
Vascular and neural mechanisms of ACh-mediated vasodilation in the forearm cutaneous microcirculation
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A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine
N Engl J Med
Transthyretin-associated neuropathic amyloidosis: pathogenesis and treatment
Neurology
Late-onset familial amyloid polyneuropathy type I (transthyretin Met30-associated familial amyloid polyneuropathy) unrelated to endemic focus in Japan: clinicopathological and genetic features
Brain
The genetic amyloidoses with particular reference to hereditary neuropathic amyloidosis, type II (Indiana or Rukavina type)
Medicine (Balt)
Inherited predisposition to generalized amyloidosis: clinical and pathological study of a family with neuropathy, nephropathy, and peptic ulcer
Neurology
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