PANDAS Disease

PANDAS Disease – PANDAS Disease is an autoimmune disease in which strep throat (Streptococcal Infection) triggers a misdirected immune response and results in inflammation on a child’s brain. PANDAS Disease can cause both neurological and psychiatric conditions.

PANDAS acronym means: Pediatric Autoimmune Neuropsychiatric Disorders associated with Streptococcus infections

What is PANDAS Disease?

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:

• 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 hrotat 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.

Other Juvenile Autoimmune Diseases:

• Celiac Disease affects 1-3% of the population
• Immune Thrombocytopenic Purpura (ITP) affects 5:100,000 children each year
• Juvenile Diabetes (Type 1 Diabetes) affects 2:10,000 children each year
• Juvenile Dermatomyositis (JDM) is rare and only affects about 3,000-6,000 in the US
• Juvenile Idiopathic Arthritis (JIA) affects 1: 1,000 children each year
• Juvenile Rheumatoid Arthritis (JRA) affects about 1:1,000 children each year
• Kawasaki Disease affects 1:10,000 under age 5 each year
• Rheumatic Fever affects 4:1000 each year

PANDAS Disease 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:

PANDAS Disease Diagnosis

SPECIALISTS

+ Pediatrician
+ Neurologist
+ PANDAS Specialist

BLOOD TESTS

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)

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

PANDAS Disease 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