PANDAS

Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections

Other Names

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

Pathophysiology

• 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

• Signs and symptoms of PANDAS align with current guidelines for diagnosing PANDAS, and include:
• 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).

Association with Streptococcus infection

• 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.

Cause

The underlying cause of PANDAS is unclear, but studies suggest that a strep infection causes an abnormal immune response resulting in neuropsychiatric symptoms.

Onset and progression

• 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")

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, sertraline, or paroxetine) and cognitive behavioral therapy. Tic disorders are often treated with a variety of medications, as well.

IVIG and plasmapheresis

• 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.
• These treatments are often reserved for severely affected children who do not respond to other therapies.

External links

• [1]
• NIMH Director's Blog

PANDAS Resources
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NIH genetic and rare disease info

• NIH info on PANDAS

PANDAS is a rare disease.