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Disease Profile

Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections

Prevalence
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

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ICD-10

-

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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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 antibiotics for GAS infection, and medication or behavioral therapy for neuropsychiatric symptoms.[2][3]

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.[4][5][6]

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
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

Diagnosis

A diagnosis of PANDAS is based on the presence of the following:[5][7]

  • 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

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.[5][6]

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.[5][6]

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

    Organizations Providing General Support

      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

        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

            References

            1. PANDAS: PPN Diagnostic Guidelines. PANDAS Physicians Network. https://www.pandasppn.org/ppn-pandas-diagnostic-guidelines/. Accessed 10/2/2018.
            2. Michael E Pichichero. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UpToDate. Waltham, MA: UpToDate; June, 2016;
            3. 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.
            4. PANDAS: Frequently Asked Questions about Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. National Institute of Mental Health. Accessed 3/30/2015.
            5. Michael E Pichichero, MD. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UptoDate. July 2013; Accessed 3/30/2015.
            6. Information About PANDAS. National Institute of Mental Health. Accessed 3/30/2015.
            7. 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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