Parsonage Turner syndrome

Información en español Title

Other Names:

Acute brachial neuritis; Acute brachial neuritis syndrome; Acute brachial radiculitis syndrome; Acute brachial neuritis; Acute brachial neuritis syndrome; Acute brachial radiculitis syndrome; Acute shoulder neuritis; Idiopathic neuralgic amyotrophy See More

Categories:

Nervous System Diseases

Summary Summary

Parsonage Turner syndrome (PTS) is characterized by the sudden onset of shoulder and upper arm pain followed by progressive (worsening over time) weakness and/or atrophy of the affected area. The pain is felt along the path of one or more nerves and often has no obvious physical cause. The network of nerves involved in this syndrome is called the brachial plexus and it controls movement and sensation in the shoulders and arms. The cause is still unknown (idiopathic). However, researchers believe that most cases are due to an autoimmune response following exposure to an illness or environmental factor. Suspected triggers include viral and bacterial infections, surgery, vaccinations, injury, childbirth, strenuous exercise, certain medical procedures, and various health conditions. Treatment is symptomatic and may include pain relievers, corticosteroids and physical therapy.^[1]^[2]

PTS is one of two forms of neuralgic amyotrophy. The other form is hereditary and is caused by mutations in the SEPT9 gene in about 85% of cases.

Last updated: 6/15/2017

Symptoms Symptoms

Parsonage Turner syndrome is usually characterized by the sudden onset of severe pain in the shoulder and upper arm, which is often described as sharp, aching, burning, stabbing, or throbbing. In some cases, the pain may extend to the neck, lower arm and/or hand on the affected side. Rarely, both sides of the body are involved. Affected people typically experience constant pain that may become worse with movement. Intense pain can last from a few hours to several weeks, at which point the pain usually begins to subside. However, mild pain may continue for a year or longer.^[1]^[2] As the pain subsides, it typically is replaced by progressive weakness of the affected area, ranging from mild weakness to nearly complete paralysis. There may also be muscle wasting (atrophy), absent or reduced reflexes, or loss of sensation.^[1]^[2]

In some cases, nerves and muscles outside of the shoulder and upper arm region are affected, such as the lumbosacral plexus (causing symptoms in the legs) or phrenic nerve (affecting the diaphragm).^[3]

Last updated: 8/6/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Arthralgia	Joint pain	0002829
EMG abnormality		0003457
Muscle weakness	Muscular weakness	0001324
Polyneuropathy	Peripheral nerve disease	0001271
30%-79% of people have these symptoms
Paresthesia	Pins and needles feeling Tingling [ more ]	0003401
Scapular winging	Winged shoulder blade	0003691
Sprengel anomaly	High shoulder blade	0000912
5%-29% of people have these symptoms
Acrocyanosis		0001063
Cleft palate		0000175
Narrow mouth	Small mouth	0000160
Neurological speech impairment	Speech disorder Speech impairment Speech impediment [ more ]	0002167
Respiratory insufficiency	Respiratory impairment	0002093
Round face	Circular face Round facial appearance Round facial shape [ more ]	0000311
Short stature	Decreased body height Small stature [ more ]	0004322
Sleep disturbance	Difficulty sleeping Trouble sleeping [ more ]	0002360

Showing of 15 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 10/1/2018

Do you have updated information on this disease? We want to hear from you.

Cause Cause

The exact cause of Parsonage Turner syndrome is unknown. Researchers suspect that most cases are due to an autoimmune response following exposure to an illness or environmental factor. In many cases, no triggering event or underlying cause can be identified. Factors known to trigger some cases include:^[1]^[2]

Infections (both viral and bacterial)
Surgery
Vaccinations
Childbirth
Certain medical procedures, such as a spinal tap or imaging studies that require administration of radiologic dye
Strenuous exercise
Certain medical conditions, including connective tissue disorders and autoimmune disorders
Injury

Some researchers believe that Parsonage Turner syndrome is a multifactorial condition, which means that it is caused by an interaction between environmental and genetic factors. In this case, a person may have a genetic susceptibility to PTS due to one or more genes, but won't develop the condition unless they are exposed to certain environmental triggers (such as one or more of those listed above).^[1]^[2]

Last updated: 6/15/2017

Inheritance Inheritance

Parsonage Turner syndrome, also known as idiopathic neuralgic amyotrophy, is not inherited. However, there is an inherited form of neuralgic amyotrophy called hereditary neuralgic amyotrophy. This form is inherited in an autosomal dominant manner.^[3]

Last updated: 6/15/2017

Diagnosis Diagnosis

A diagnosis of Parsonage Turner syndrome often is suspected based on the presence of characteristic signs and symptoms. Specialized tests may be recommended to further investigate the shoulder pain and/or muscle weakness, and to rule out other conditions that can cause similar features. These tests may include nerve conduction studies (tests that determine the ability of a specific nerve to relay a message to the brain), electromyography, magnetic resonance imaging (MRI scan), and/or an X-ray.^[1]^[2]

Last updated: 6/15/2017

Treatment Treatment

Treatment for Parsonage Turner syndrome varies based on the signs and symptoms in each person.

Pain medications may be prescribed depending on the severity of the nerve pain. After the acute phase, different medications known as co-analgesics may be administered. These include gabapentin, carbamazepine, and amitryptiline. These drugs specifically treat nerve pain. Other techniques for pain management include application of heat or cold, and transcutaneous electrical nerve stimulation (a method of pain relief in which a special device transmits low-voltage electrical impulses through electrodes on the skin to an area of the body that is in pain).^[1]^[2]

Many people with PTS have physical therapy and/or occupational therapy to maintain muscle strength and range of motion once the pain begins to subside. Surgeries such as nerve grafting or tendon transfer to restore movement and function to the shoulder muscles and joint may be considered if other treatment options are not effective.^[1]^[2]

Last updated: 6/15/2017

Prognosis Prognosis

The long-term outlook (prognosis) for people with Parsonage Turner syndrome varies. Some people may experience only a single episode of pain, and fully recover the strength and functionality of their shoulder. Others may have multiple episodes throughout their lives. It has been reported that approximately 80% of people recover within 2 years of the start of symptoms, and approximately 90% recover within 3 years. Traditionally, it was believed that most people would recover around 70-90% of their original strength and function level. However, more recent research suggests that long-term complications are more common than previously thought. People with bilateral (affecting both sides of the body) involvement or multiple episodes are more likely to have a poorer prognosis.^[2]^[1]

Last updated: 6/15/2017

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

The Foundation for Peripheral Neuropathy
485 Half Day Road
Suite 350
Buffalo Grove, IL 60089
Telephone: +1-877-883-9942
Fax: +1-847-883-9960
E-mail: info@tffpn.org
Website: https://www.foundationforpn.org

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Parsonage Turner syndrome. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NCATS Rare Diseases Are Not Rare! Challenge
October 9, 2018
The NIH Undiagnosed Diseases Network Expands
September 26, 2018

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

About 3 months following the onset of Parsonage-Turner Syndrome, I developed numbness in my lower body. Gradually over the course of 8 weeks, about 90% of the sensation in my lower body returned. The Parsonage-Turner also damaged my esophagus and one vocal cord. It's been about 8 years and I continue to have the numbness and tingling in my feet. Is it possible that the Parsonage-Turner is causing the persistent neuropathy in my lower body? See answer
I have been recovering from Parsonage Turner syndrome for years. I still have some pain, the fatigue is still with me and my quality of life is compromised. Can this develop into something else? Also, is it normal that I still have fatigue after 5 plus years? See answer

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