PANDAS and PANS
PANDAS, an Overview
Pediatric Autoimmune Neuropyschiatric Syndrome Associated with Strep (PANDAS), or Pediatric Autoimmune Neuropsychiatric Syndrome (PANS), is a condition in which the body responds to a seemingly innocuous threat such as a bacterial infection (mainly streptococcus) or--as new research shows--to a viral or parasitic infection by producing abnormal autoantibodies against the basal ganglia. These autoantibodies interfere with basal ganglia function and produce a spectrum of symptoms that manifest as sudden behavioral changes including obssessive compulsive traits, behavioral tics, anxiety, mood disturbance, and others. The hallmark of this condition is the sudden onset of behavior change following a bacterial or viral infection. PANDAS / PANS is difficult to diagnose because it may follow a subclinical infection, meaning that your child may or may not have exhibited symptoms of being sick.
An experienced clinican can make the diagnosis of PANDAS and PANS based on clinical picture and history alone, but laboratory testing might also be helpful in confirming a diagnosis or PANDAS or PANS.
The most important component when treating PANDAS or PANS is to determine the etiology of the behavioral distrurbance. If, as is often the case, a bacterial infection caused the behavioral changes then the best and current treatment recommendation is a course of antibiotics. If a viral infection is found to be the cause, antivirals may be an option for some patients. The appropriate treatment and duration will be determined by your clinician. Our office recommends that whenever a child is placed on antibiotics (and for general health purposes, too!) supplementation with a good probiotic be provided.
PANDAS and PANS, FAQs!
What is it?
An abnormal autoimmune response to an infection (such as strep), or a virus that causes a sudden onset of symptoms including tics, OCD behavior, mood disturbance, etc.
Who gets it?
Genetically predisposed children exposed to an environmental trigger.
How is it diagnosed?
A clinician can make this diagnosis through a complete and thorough history and physical exam. A strep culture, and/or blood testing for strep titers may confirm a PANDAS diagnosis. Occasionally, other blood titer testng may also be appropriate.
How is it treated?
If a bacterial infection is the cause, then antibiotic therapy may be indicated. In the case that a virus was the trigger, antivirals may be appropriate for some patients.
How does PANDAS affect the brain?
Abnormal autoantibodies are produced in response to an infection or virus and these interfere with basal ganglia function.
What is a strep titer?
This antibody blood titer test shows immunological evidence of a past strep infection. The ASO titer rises 3-6 weeks after a strep infection, and the DNAase B titer rises 6-8 weeks after an infection.
Evidence of an Autoimmune Encephalitis
Evidence now supports that PANDAS is a sequelae of Group A Streptococcal infections and that PANDAS is a form of Autoimmune Encephalitis. As knowledge has evolved, several other terms have been used to describe what is being seen when the immune system mediates inflammation of the central nervous system, including:
PANDAS-- Pediatric Autoimmune Neuropsychiatric Disorder Associated with Strep
PITANDS-- Pediatric Infection-Triggered Autoimmune Neuropsychiatric Disorders
PANS-- Pediatric Acute Onset Neuropsychiatric Syndrome
CANS -- Childhood Acute Neuropsychiatric Syndrome
Post-Streptococcal Autoimmune Encephalitis (of the basal ganglia)
What is the Clinical Presentation of PANDAS?
The clinical presentation consistes of an acute onset of behavioral changes including OCD or eating disroder and/or tic disorder. There are episodic (relapsing-remitting) courses and there can be various associated neurological abnormalities present. The following list illustrates how wide and varied the associated neurological symptoms can be and how this diagnosis can be missed if symptoms are attributed to another etiology or considered as purely psychological.
Sleep disorders-- Insomnia, night terrors, refusal to sleep alone, restless legs, abnormal sleep study
Behavioral regression---Separation anxiety, "baby talk," tantrums
Inability to concentrate
Handwriting deterioration, fine motor concerns
Sensory hypersensitivity or insensitivity
Short-term memory loss or confusional state
Tics—motor and vocal
Abdominal pain, leg pain, joint pain
Urinary frequency, urgency, enuresis (night and/or daytime wetting)
Feeling of difficulty taking a deep breath
Blood pressure or heart rate fluctuations (postural tachycardia)
GI paresis (constipation, poor appetite, early satiety)
Terror stricken look or Hyper-alert appearance
Eating disorder (anorexia)
Abnormal EEG not associated with seizure activity
Why does PANDAS occur?
PANDAS and PANS result from a misdirected immune response to a foreign substance including a bacteria, virus, or parasites. For example, Streptococcal bacteria have molecular mimics on their cell wall. Antibodies mounted against Strep misrecognize the host as foreign and the ensuing inflammatory response from the cross-reactive antibodies is an autoimmune response. Well-known examples include: post-strep arthritis, glomerulonephritis and rheumatic fever, and particularly, Sydenham chorea. Studies from the NIH, as early as 1958, reported high rates of obsessive-compulsive symptoms that started two to four weeks before the chorea began.
As knowledge has progressed over the past 12 years, it has become clear that there are tiggers other than strep that can incite this autoimmune response. Published reports have postulated that stress as well as other types of infections can result in the neuropsychiatric conditions which include Mycoplasma bacteria, parasites, Lyme Disease, and viruses such as herpes simplex, common cold, and varicella viruses. Identification of a specific infectious agent may be difficult in certain cases, but the immune response against the infection is the cause of the symptoms in these vulnerable children.
The additions of CANS includes those that present with an acute and dramatic onset of neuropsychiatric symptoms not limited to OCD and tics, and may occur at any age.
Research suggests that PANDAS affects genetically vulnerable children exposed to environmental triggers such as those reviewed above.
Is there a Treatment?
Evidence suggests that treatment of Group A Strep (or other triggers) reduces OCD/tic exacerbations. Evidence-based treatment includes antibiotics, antivirals, and/or anti-parasitic medications depending on your child's individual trigger(s). The latest research indicates that if an early effect is noted once treatment begins, maintenance is often indicated through late adolescence and early adulthood. Earlier and individualized treatment produces better outcomes as we attempt to calm this aberrant immune response.
How is PANDAS Diagnosed?
A proper and detailed clinical history is perhaps the most important factor. Laboratory tests including ASO and anti-DNase B titers, and other individualized testing may be appropriate and can add support to a PANDAS diagnosis. Your child's clinician will help guide you through this process.
Read more about PANDAS or PANS at these sites:
Addressing Anxiety and OCD in Autism (and PANDAS, too!), an Autism Research Institute lecture by Dr. S. Chandra
Misdiagnosed bipolar: One girl's struggle through psych wards before Stanford doctors make bold diagnosis and treatment
Does this sound like your child?
Early treatment is often key in providing the best result for your child. If you recognize any of these symptoms, or have any questions specific to your child, please call our office to schedule a consult appointment with your child's provider.