Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections

Alternate names

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

Definition

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.

Summary

• PANDAS is a subtype of pediatric acute-onset neuropsychiatric syndrome (PANS).
• A child may be diagnosed with PANDAS when:
• Obsessive-compulsive disorder (OCD), tic disorder, or both suddenly appear following a streptococcal (strep) infection, such as strep throat or scarlet fever.
• The symptoms of OCD or tic symptoms suddenly become worse following a strep infection.
• The symptoms are usually dramatic, happen “overnight and out of the blue,” and can include motor or vocal tics or both and obsessions, compulsions, or both.
• In addition to these symptoms, children may become moody or irritable, experience anxiety attacks, or show concerns about separating from parents or loved ones.

Epidemiology

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.

Cause

• Strep bacteria are very ancient organisms that survive in the human host by hiding from the immune system as long as possible.
• They hide themselves by putting molecules on their cell wall so that they look nearly identical to molecules found on the child’s heart, joints, skin, and brain tissues.
• This hiding is called “'''molecular mimicry'''” and allows the strep bacteria to evade detection for a long time.
• However, the molecules on the strep bacteria are eventually recognized as foreign to the body and the child’s immune system reacts to the molecules by producing antibodies.
• Because of the molecular mimicry by the bacteria, the immune system reacts not only to the strep molecules but also to the human host molecules that were mimicked; antibodies “attack” the mimicked molecules in the child’s own tissues.
• These antibodies that react to both the molecules on the strep bacteria and to similar molecules found on other parts of the body are an example of “cross-reactive” antibodies.
• Studies at the National Institute of Mental Health (NIMH) and elsewhere have shown that some cross-reactive antibodies target the brain—causing OCD, tics, and the other neuropsychiatric symptoms of PANDAS.

Signs and 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.

Children may also experience the following signs and symptoms during a PANDAS episode:

• 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")

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Tics(Tic disorder)

30%-79% of people have these symptoms

• Attention deficit hyperactivity disorder(Attention deficit)
• Chorea
• Depressivity(Depression)
• Developmental regression(Loss of developmental milestones)
• Emotional lability(Emotional instability)
• Impulsivity(Impulsive)
• Irritability(Irritable)
• Obsessive-compulsive trait(Obsessive-compulsive traits)
• Oppositional defiant disorder
• Personality changes(Personality change)
• Sleep disturbance(Difficulty sleeping)

5%-29% of people have these symptoms

• Agoraphobia(Fear of open spaces)
• Anorexia
• Arthralgia(Joint pain)
• Claustrophobia
• Clumsiness
• Encopresis(Stool holding)
• Enuresis
• Phonophobia(Fear of loud sounds)
• Recurrent streptococcus pneumoniae infections
• Separation insecurity

Diagnosis

A diagnosis of PANDAS is based on the presence of the following:

• 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)

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.
• 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.

NIH genetic and rare disease info

• NIH info on Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections

Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections is a rare disease.

Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections Resources
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