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Disease Profile
Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Unknown
Age of onset
Childhood
ICD-10
-
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
PANDAS; Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus; Pediatric autoimmune disorders associated with Streptococcus infections
Categories
Nervous System Diseases
Summary
Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections (PANDAS) is a neurological and psychiatric condition in which symptoms are brought on or worsened by a Streptococcal (strep) infection. PANDAS is a subtype of pediatric acute-onset neuropsychiatric syndrome (PANS). Signs and symptoms of PANDAS align with current guidelines for diagnosing PANDAS, and include:[1]
- The presence of obsessive-compulsive disorder (OCD) and/or tics (uncontrolled, sudden, repetitive movements or sounds), which are severe enough to interfere the ability to function.
- Symptoms appear between 3 years of age and puberty (although later onset of symptoms is possible).
- Symptoms appear abruptly and dramatically (typically within one to two days) and may repeatedly improve and then suddenly worsen (a relapsing and remitting course).
- There is an association between symptom onset or worsening and a group A Streptococcal (GAS) infection. Symptom flares must be associated with a positive throat culture and/or blood tests that show elevated
antibodies against a substance produced by GAS (called anti-GAS titers). Children with PANDAS often do not complain of a sore throat despite having an infection. After the initial infection, subsequent flares can be associated with other infectious or environmental triggers. - There are other neurologic abnormalities. When symptoms of PANDAS are present, a neurological exam will be abnormal. Abnormalities may include any of the additional neuropsychiatric symptoms that occur in people with PANS.
The underlying cause of PANDAS is unclear, but studies suggest that a strep infection causes an abnormal immune response resulting in neuropsychiatric symptoms. Management is centered on providing
Symptoms
Children may also experience the following signs and symptoms during a PANDAS episode:[4][6]
- 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")
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.
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 |
Loss of developmental milestones
Mental deterioration in childhood
[ more ] |
0002376 | |
Emotional lability |
Emotional instability
|
0000712 |
Impulsivity |
Impulsive
|
0100710 |
Irritability |
Irritable
|
0000737 |
Obsessive-compulsive |
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 |
Diagnosis
- 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.
Treatment
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
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
-
PANDAS Network
655 Oak Grove Avenue #1373
Menlo Park, CA 94026
E-mail: pandasnetwork@gmail.com
Website: https://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/ -
Pediatric Research and Advocacy Initiative (PRAI)
P.O. BOX 2198
Chesterfield, VA 23832
Website: https://praikids.org/
Organizations Providing General Support
-
American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.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/
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.
References
- PANDAS: PPN Diagnostic Guidelines. PANDAS Physicians Network. https://www.pandasppn.org/ppn-pandas-diagnostic-guidelines/. Accessed 10/2/2018.
- Michael E Pichichero. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UpToDate. Waltham, MA: UpToDate; June, 2016;
- PANDAS: Fact Sheet about Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. NIMH. August, 2015; https://www.nimh.nih.gov/health/publications/pandas/index.shtml.
- 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.
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