Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections

Title

Other Names:

PANDAS; Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus; Pediatric autoimmune disorders associated with Streptococcus infections

Categories:

Nervous System Diseases

This disease is grouped under:

Pediatric acute-onset neuropsychiatric syndrome

Summary Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.

Orpha Number: 66624

Disease definition

PANDAS is an acronym for Pediatric Autoimmune Neuropsychiatric Disorders Associated with a group A beta-hemolytic Streptococcal infection and applied to a subgroup of children with obsessive-compulsive disorder (OCD) and/or tic disorders.

Epidemiology

The prevalence is unknown but the boy-to-girl ratio is 2.6:1.

Clinical description

The current diagnostic criteria for the PANDAS are: presence of OCD and/or a tic disorder, very young age at onset (prepubertal), sudden and dramatic onset of symptoms, association between streptococcal infections and episodic relapsing-remitting exacerbations manifesting as neuropsychiatric symptoms (motor hyperactivity or adventitious movements including choreiform movements or tics). The increased severity of symptoms usually persists for at least several weeks, but may last for several months or longer, followed by a slow, gradual improvement. The major distinctive feature of PANDAS is the temporal association between neuropsychiatric symptom exacerbations and streptococcal infections. Additional neuropsychiatric symptoms occur frequently: emotional lability, separation anxiety, anorexia, impulsivity, distractibility and motor hyperactivity characteristic of attention deficit hyperactivity disorder (ADHD). Comorbid disorders include major depression (36%), major dysthymia (6%) and separation anxiety disorder (20%).

Etiology

The etiology is uncertain. One theory is that streptococcal infections trigger an antibody response in some children that causes changes in the basal ganglia. No specific genetic factors have been identified.

Diagnostic methods

Diagnosis of PANDAS is clinical. Neuroimaging studies may reveal increased basal ganglia volumes.

Management and treatment

Management includes standard interventions for obsessive-compulsive and tic disorders: cognitive-behavioural therapy, reversal therapy in the case of tic disorders and pharmacologic therapy (neuropsychiatric drugs, antibiotics to prevent infections and intravenous immunoglobulin therapy).

Visit the Orphanet disease page for more resources.

Last updated: 11/30/2006

Symptoms Symptoms

PANDAS is an acronym for Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus. It describes the abrupt onset or worsening of symptoms in children who have obsessive-compulsive disorder or a tic disorder (i.e. Tourette syndrome), following a group A streptococcal infection (strep infection). Following a strep infection, children affected by PANDAS typically experience a sudden onset of motor or vocal tics; obsessions; and/or compulsions. These severe symptoms typically last from several weeks to several months. After a gradual improvement, children may not experience any additional problems until they contract another strep infection.^[1]^[2]^[3]

Children may also experience the following signs and symptoms during a PANDAS episode:^[1]^[3]

Moodiness and irritability
Separation anxiety
ADHD symptoms
Sleep disturbances
Night-time bed wetting and/or day-time urinary frequency
Fine motor changes (handwriting)
Joint pain
Concentration difficulties and loss of academic abilities
Developmental regression (i.e. temper tantrums, "baby talk")

Last updated: 3/30/2015

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.

Showing of 22 |

Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Tics	Tic disorder	0100033
30%-79% of people have these symptoms
Attention deficit hyperactivity disorder	Attention deficit Attention deficit disorder Attention deficit-hyperactivity disorder Attention deficits Childhood attention deficit/hyperactivity disorder [ more ]	0007018
Chorea		0002072
Depressivity	Depression	0000716
Developmental regression	Loss of developmental milestones Mental deterioration in childhood [ more ]	0002376
Emotional lability	Emotional instability	0000712
Impulsivity	Impulsive	0100710
Irritability	Irritable	0000737
Obsessive-compulsive trait	Obsessive-compulsive traits	0008770
Oppositional defiant disorder		0010865
Personality changes	Personality change	0000751
Sleep disturbance	Difficulty sleeping Trouble sleeping [ more ]	0002360
5%-29% of people have these symptoms
Agoraphobia	Fear of open spaces	0000756
Anorexia		0002039
Arthralgia	Joint pain	0002829
Claustrophobia		0025253
Clumsiness		0002312
Encopresis	Stool holding Stool soiling [ more ]	0040183
Enuresis		0000805
Phonophobia	Fear of loud sounds	0002183
Recurrent streptococcus pneumoniae infections		0005366
Separation insecurity		0031468

Showing of 22 |

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

Last updated: 11/1/2018

Cause Cause

Although the exact underlying cause of PANDAS has not been proven, studies suggest that it may be an autoimmune disorder. Normally, when a person is exposed to bacteria, the immune system makes proteins called antibodies to fight off the infection. In autoimmune disorders, the body's immune system mistakenly attacks and destroys its own, healthy body tissue.^[4] PANDAS may occur when a group A streptococcal infection (strep infection) triggers an abnormal immune response, causing the body to mistakenly attack the tissues of the central nervous system (brain and spinal cord).^[4] However, the role of autoimmunity in PANDAS is controversial.^[5]

Last updated: 7/5/2016

Diagnosis Diagnosis

A diagnosis of PANDAS is based on the presence of the following:^[2]^[4]

Obsessive-compulsive disorder or a tic disorder (i.e. Tourette syndrome)
Symptoms develop before age 12
Abrupt onset of symptoms and "episodic" course (relapses are separated by periods of time in which children have less severe or no symptoms)
Association with group A streptococcal infection (in some cases, the affected person has no obvious symptoms of a strep infection; however, a throat culture and/or blood tests may show evidence of a current or recent infection)
Association with other neuropsychiatric symptoms (i.e. separation anxiety, ADHD, developmental regression, etc)

There are currently no tests available to confirm a diagnosis of PANDAS.

Last updated: 3/31/2015

Treatment Treatment

The treatment of PANDAS is symptomatic. Children who are diagnosed with a group A streptococcal infection are typically treated with antibiotics whether or not they develop symptoms of PANDAS. Obsessive-compulsive disorders are usually managed with medications (i.e. SSRI medications such as fluoxetine, fluvoxamine, sertaline, or paroxetine) and cognitive behavioral therapy. Tic disorders are often treated with a variety of medications, as well.^[2]^[3]

Some researchers suspect that therapies such as plasmapheresis and/or intravenous immunoglobulin (IVIG), which are often used to treat autoimmune disorders, may be a treatment option for children with PANDAS. Plasmapheresis is a procedure that removes antibodies from the blood. IVIG can sometimes be used to alter the function or production of abnormal antibodies. Although some studies have demonstrated that both of these therapies are effective for the treatment of severe, strep triggered OCD and tics, a number of the children in these studies experienced severe side-effects. Thus, these treatments are often reserved for severely affected children who do not respond to other therapies.^[2]^[3]

Last updated: 3/31/2015

Statistics Statistics

The incidence and prevalence of PANDAS are not known, although it is rare. In one prospective study, only 10 cases were identified among 30,000 throat cultures (1 in 3000) positive for group A streptococci (GAS). Since then, the annual incidence has ranged between 0 per 10,000 cultures to 10 per 30,000 cultures, depending upon the strain of GAS and other factors. Despite its rarity, some researchers suggest that it may account for ≥10% of childhood-onset obsessive-compulsive disorder (OCD) and tic disorders.^[5]

Last updated: 7/5/2016

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

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

The PANDAS Network maintains a list of healthcare providers in the United States that treat PANS/PANDAS. They also have a list of International Providers.
The PANDAS Physicians Network (PPN) has a Practitioner Directory as an informational resource. The directory includes the names of PPN members who have given their permission to be identified publicly. Inclusion in the directory is not an endorsement or a warranty of the doctors and medical professionals or the care they provide. Practitioners in the directory offer an expertise in a wide range of specialties.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

PANS Research Consortium
Website: https://med.stanford.edu/pans/research/PANS-consortium.html

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

Child Mind Institute
445 Park Avenue
New York, NY 10022
Telephone: (212) 308-3118
Website: http://childmind.org/
International OCD Foundation
18 Tremont Street, Suite 308
Boston, MA 02108
Telephone: (617) 973-5801
Fax: (617) 973-5803
E-mail: info@iocdf.org
Website: https://iocdf.org/
PANDAS Network
655 Oak Grove Avenue #1373
Menlo Park, CA 94026
E-mail: pandasnetwork@gmail.com
Website: http://pandasnetwork.org
PANDAS Physicians Network (PPN)
117 Eastbend Court
Mooresville, NC 28117
Toll-free: 855-347-4921
Fax: 1-800-641-7954
E-mail: support@pandasppn.org
Website: https://www.pandasppn.org/parent-information/
PANDAS/PANS Advocacy & Support
212 Tupelo Avenue
Naperville, IL 60540
Telephone: 630-479-7143
E-mail: service@pas.care
Website: http://www.pas.care/
Contact form: http://www.pas.care/contact.html
Pediatric Research and Advocacy Initiative (PRAI)
P.O. BOX 2198
Chesterfield, VA 23832
Website: http://praikids.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

The International OCD Foundation website provides advice for practitioners and parents as well as a fact sheet about Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections.
The National Institute of Mental Health (NIMH) has information on this topic. NIMH is part of the National Institutes of Health and is dedicated to understanding, treating, and preventing mental illnesses.
The PANDAS Network offers an information page on Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections. Please click on the link to access this resource.
The PANDAS Physicians Network provides information about PANS and PANDAS.

In-Depth Information

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 Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Chang K, Frankovich J, Cooperstock M, et al. Clinical evaluation of youth with pediatric acute-onset neuropsychiatric syndrome (PANS): recommendations from the 2013 PANS Consensus Conference. J Child Adolesc Psychopharmacol. 2015 Feb;25(1):3-13.
Thienemann M, Murphy T, Leckman J, et al. Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part I—Psychiatric and Behavioral Interventions. J Child Adolesc Psychopharmacol. 2017 Sep 1; 27(7): 566–573.
Francovich J, Swedo S, Murphy T, et al. Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part II—Use of Immunomodulatory Therapies. J Child Adolesc Psychopharmacol. 2017 Sept;27(7).
Cooperstock MS, Swedo S, Pasternack MS, Murphy TK. Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part III—Treatment and Prevention of Infections. J Child Adolesc Psychopharmacol. 2017 Sept;27(7).
Swedo SE, Frankovich J, Murphy TK. Overview of Treatment of Pediatric Acute-Onset Neuropsychiatric Syndrome. J Child Adolesc Psychopharmacol. 2017 Sep 1; 27(7): 562–565.

News & Events News & Events

News

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

I have three children who have been treated for PANDAS and PANS successfully, on several occasions. I am a nursing student and for my research paper, I chose to write about PANDAS because I am passionate about the subject, having faced tremendous difficulty in getting help for my children. I am wondering if you know about the prevalence and incidence of PANDAS, as I am not able to find much information about how many children are affected. I appreciate any help you can give me. See answer

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References References

PANDAS: Frequently Asked Questions about Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. National Institute of Mental Health. Accessed 3/30/2015.
Michael E Pichichero, MD. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UptoDate. July 2013; Accessed 3/30/2015.
Information About PANDAS. National Institute of Mental Health. Accessed 3/30/2015.
Williams KA, Swedo SE. Post-infectious autoimmune disorders: Sydenham's chorea, PANDAS and beyond. Brain Res. October 2014; [epub ahead of print]:Accessed 3/31/2015.
Michael E Pichichero. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UpToDate. Waltham, MA: UpToDate; June, 2016;