Symptoms of autonomic neuropathy could include any except which of the following?
BackgroundAutonomic neuropathies are a collection of syndromes and diseases affecting the autonomic neurons, either parasympathetic or sympathetic, or both. Autonomic neuropathies can be hereditary or acquired in nature. Most often, they occur in conjunction with a somatic neuropathy, but they can also occur in isolation. Show
The autonomic nervous system modulates numerous body functions; therefore, autonomic dysfunction may manifest with numerous clinical phenotypes and various laboratory and neurophysiologic abnormalities. Although a patient may present with symptoms related to a single portion of the autonomic system, the physician must be vigilant for other affected parts of the autonomic system. In some forms, the degree and type of autonomic system involvement varies extensively. In some patients, the degree of autonomic dysfunction may be subclinical or clinically irrelevant; in others, symptoms may be disabling. Several clinically important features of autonomic neuropathies are treatable; therefore, the physician must be alert to these features. PathophysiologyThe pathophysiology of autonomic neuropathies is variable and depends upon the underlying medical conditions. We have chosen to classify the autonomic neuropathies into hereditary and acquired. The acquired autonomic neuropathies may then be subsequently subdivided into primary or secondary. Inherited Autonomic NeuropathiesAll forms of inherited autonomic neuropathies are rare. Familial amyloid polyneuropathy, the hereditary sensory autonomic neuropathies, Fabry disease, and the porphyrias are genetic diseases in which autonomic neuropathy is a common feature. Familial amyloid polyneuropathyFamilial amyloid polyneuropathy (FAP) is often caused by a genetic mutation of the transthyretin gene. Mutant transthyretin produced in the liver accumulates as amyloid deposits in the peripheral nervous system and autonomic nervous system. Rarely, a mutation in the gelsolin gene, which produces a protein important in cytoskeletal actin function, may also lead to amyloid deposition in autonomic nerves. Liver transplantation, currently the most effective treatment for FAP, may slow the development of autonomic neuropathy, but not in all cases. [1] Hereditary sensory autonomic neuropathyCurrently, 5 types of hereditary sensory autonomic neuropathy (HSAN) have been defined (see Table 1). These types differ in their presentation, the portions of the autonomic nervous system affected, their associated genes, and inheritance pattern. [2] HSAN I has an autosomal dominant inheritance, and the disease is characterized by distal limb involvement with marked sensory loss, including loss of pain sensation, making affected individuals more susceptible to injury. HSAN I has been associated with point mutations in serine palmitoyltransferase (SPT) at chromosome arm 9q22.1-q22.3. [3] SPT is the rate-limiting enzyme in synthesis of sphingolipids, including ceramide and sphingomyelin. Ceramide is necessary for regulation of programmed cell death in a number of tissues, including the differentiation of neuronal cells. HSAN II is inherited as an autosomal recessive condition and is more severe with a congenital onset. HSAN II has a pansensory loss with early ulcers, and nerves demonstrate a marked loss of myelinated and unmyelinated fibers. HSAN III (Riley-Day syndrome) is autosomal recessive in Ashkenazi Jews, with early childhood onset of autonomic crises. The genetic defect in HSAN III is in the inhibitor of kappa light polypeptide gene enhancer in B cells, kinase complex-associated protein (IKBKAP) at chromosome arm 9q31. HSAN III nerve pathology shows absence of unmyelinated fibers with essentially normal myelinated fibers. [4] Patients with HSAN IV present with widespread anhidrosis and insensitivity to pain. The genetic defect in HSAN IV is in the tyrosine kinase receptor A or nerve growth factor receptor at chromosome arm 1q21-q22. This defect is autosomal recessive. Recently, 2 novel missense mutations in the tyrosine kinase domain were found in a 10-year-old patient with HSAN IV. [5] This finding may provide a better understanding of the neuropathophysiology of HSAN IV. Patients with HSAN V present with pain insensitivity and preservation of other sensory modalities. Some patients with HSAN V have similar genetic abnormalities to those with HSAN IV. The genetic mutation has been isolated to the nerve growth factor beta gene. [6] Table. Types of HSAN (Open Table in a new window)
Fabry diseaseFabry disease is an X-linked recessive disorder with mutations in the gene for alpha-galactosidase. Somatic and autonomic neuropathy is due to accumulation of glycolipids. Attacks may be triggered by changes in temperature or exercise. Nerve pathology demonstrates loss of both small myelinated and unmyelinated fibers. [7] Acute intermittent porphyria and variegate porphyriaAcute intermittent porphyria and variegate porphyria can both have forms of peripheral neuropathy. Attacks can be triggered by exposure to particular drugs. During episodes, affected individuals present with acute polyneuropathy that may mimic Guillain-Barré syndrome. Autonomic dysfunction, particularly cardiac and vascular in nature, can be prominent. Acquired Autonomic NeuropathiesThe acquired autonomic neuropathies are much more prevalent than the inherited ones. Here, we subclassify the acquired autonomic neuropathies into primary and secondary disorders. Primary autonomic neuropathies are disorders that are idiopathic or that have autonomic neuropathy as a characteristic feature of the disease process itself. In the secondary autonomic neuropathies, an identifiable cause, such as a nutritional deficiency, may lead to autonomic neuropathy, but does not have autonomic neuropathy as a defining feature of the disease process. Subclassification can be somewhat artificial as the true mechanism of action is not clear in all cases, although it can be helpful when trying to develop an understanding of autonomic neuropathy. Primary acquired autonomic neuropathiesSee the list below:
Secondary acquired autonomic neuropathiesMetabolic derangements that may have an associated autonomic neuropathy are as follows:
Vitamin deficiencies, toxins, and drugs that may have an associated autonomic neuropathy are as follows:
Infectious diseases that may have an associated autonomic neuropathy are as follows:
Autoimmune conditions that may have an associated autonomic neuropathy are as follows:
EpidemiologyMortality/MorbidityFalls and loss of consciousness are significant contributors to morbidity associated with autonomic neuropathies. They may lead to injury, particularly in the elderly. Often, an autonomic neuropathy manifests with orthostatic hypotension, which has been associated with increased mortality in the middle aged and elderly. [46] As the autonomic nervous system is involved in involuntary control of almost every organ system, patients may have many other complaints that are discussed below. Many cases of autonomic neuropathy have a gradually progressive course, leading to a poor outcome. Patients with severe dysautonomia are at risk for sudden death secondary to cardiac dysrhythmia, as has been documented in GBS and diabetic neuropathy. Single-photon emission CT (SPECT) and positron emission tomography (PET) have demonstrated that cardiac sympathetic dysfunction is commonly present in both type I and type II diabetes mellitus. When associated with vascular complications, dysautonomia related to diabetic neuropathy is also associated with increased mortality. In other disorders, other forms of systemic dysfunction, such as with kidney failure in Fabry disease, may lead to mortality. RaceAutonomic neuropathies may be seen in all races and ethnicities. Certain subtypes may demonstrate an increased incidence in specific ethnic groups. These subtypes are addressed individually above. SexIn general, no predilection for autonomic neuropathies exists with regard to sex. POTS and connective tissue diseases are more common among females. Fabry disease is inherited as an X-linked recessive disorder; therefore, it manifests predominantly in males. AgeIn general, no predilection for autonomic neuropathies exists with regard to age. Age of onset is highly dependent upon the underlying pathophysiology. Patients with most forms of HSAN (except HSAN I) present at birth or in childhood.
Author Coauthor(s) Bashar Katirji, MD, FACP Director, Neuromuscular Center and EMG Laboratory, The Neurological Institute, University Hospitals Cleveland Medical Center; Professor of Neurology, Case Western Reserve University School of Medicine Bashar Katirji, MD, FACP is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Physicians, American Neurological Association Disclosure: Nothing to disclose. Specialty Editor Board Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference Disclosure: Received salary from Medscape for employment. for: Medscape. Chief Editor Nicholas Lorenzo, MD, CPE, MHCM, FAAPL Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Former Chief Medical Officer, MeMD Inc Nicholas Lorenzo, MD, CPE, MHCM, FAAPL is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for Physician Leadership Disclosure: Nothing to disclose. Additional Contributors Paul E Barkhaus, MD, FAAN, FAANEM Professor of Neurology and Physical Medicine and Rehabilitation, Chief, Neuromuscular and Autonomic Disorders Program, Director, ALS Program, Department of Neurology, Medical College of Wisconsin Paul E Barkhaus, MD, FAAN, FAANEM is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Neurological Association Disclosure: Nothing to disclose. What is a symptom of autonomic neuropathy?They might include: Dizziness and fainting when standing, caused by a sudden drop in blood pressure. Urinary problems, such as difficulty starting urination, loss of bladder control, difficulty sensing a full bladder and inability to completely empty the bladder.
What are 4 things the autonomic nervous system?The autonomic nervous system is a component of the peripheral nervous system that regulates involuntary physiologic processes including heart rate, blood pressure, respiration, digestion, and sexual arousal.
What are the three main body systems affected by autonomic neuropathy?Autonomic neuropathy is a group of symptoms that occur when there is damage to the nerves that manage every day body functions. These functions include blood pressure, heart rate, sweating, bowel and bladder emptying, and digestion.
What are the symptoms of autonomic failure?Due to the drop in blood pressure, people can have dizziness or faintness including syncope (loss of consciousness) in severe cases. Other related symptoms include visual disturbances, neck pain, breathing difficulty, and decrease in sweating which can lead to heat intolerance.
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