Childhood Post-Infectious Neuroimmune Disorders are characterized by the acute onset of debilitating symptoms including:
- Significant change in emotional stability and behavior
- New-onset anxiety symptoms (obsessive compulsive symptoms, trichotillomania and restricted eating)
- New-onset tics and movements disorders
- Profound changes in mood regulation (panic attacks, rage, aggression, and suicidal thoughts)
- Major decline in motor function impacting handwriting and learning
In this group of disorders, psychiatric illness arises from an organic etiology; symptoms are indicative of an infectious trigger resulting in a misdirected immune system. A child beset by emotional or behavioral symptoms is exhibiting an aberrant response to infection. PANDAS/PANS is believed to be the result of a child’s immune system becoming misdirected and attacking neural structures of the brain and nervous system known as the basal ganglia.
Often, the underlying cause is not observed by the treating clinician, and, without appropriate medical intervention, the child’s functioning deteriorates in school, home, and/or community settings.
Children are frequently misdiagnosed, because of a lack of understanding and awareness, with a litany of other psychiatric disorders including: Attention Deficit Disorder, Generalized Anxiety Disorder, Tourette Syndrome, Obsessive Compulsive Disorder (OCD), Oppositional Defiant Disorder, eating disorders, and Bipolar Disorder.
PANDAS and PANS
Families with children experiencing neuroimmune illness with psychological and emotional symptoms have their lives turned upside down. Their previously healthy, happy children change dramatically overnight, or over the course of months. The burden on families is enormous. Based on a survey of 300 families of PANDAS patients at Massachusetts General Hospital (MGH), 70% of respondents traveled over 50 miles to obtain medical care for their children. 30% had visited the emergency department at least once due to the severity of their child’s symptoms (with an average of four ER visits!), and fully 20% of respondents reported needing to hospitalize their children. In addition, families spent an average of $20,000 out-of-pocket for treatments not covered by insurance. These are the lucky families getting care at MGH, one of the very few clinics in the United States actively diagnosing and treating PANDAS. Many more families nationwide have children who are undiagnosed or are on waiting lists and are not yet receiving effective care for their children.
Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infections (PANDAS) was first described at length in 1998 to describe sudden the onset of OCD and other physical and behavioral changes in children following streptococcal infection. This disease appears to share many characteristics with Sydenham’s Chorea and Autoimmune Encephalitis. Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) shares many similar symptoms but is triggered by an infection or environmental factor other than streptococcus. Neither condition is well understood, and it is imperative for research to move forward more quickly to develop much needed more effective therapies for children.
Because of the insufficiency of medical diagnosis and medical recognition of the disorder, the incidence and prevalence of PANDAS and PANS are unknown. As recognition grows locally, e.g. in certain US locales where children in multiple families have been diagnosed, it is becoming increasingly evident that these are not rare disorders and are more likely quite common. It is also likely that in areas of the world where Strep infection is most prevalent, these disorders may be even more common.
Although there are simple diagnostic tests available for streptococcal (“strep”) infection itself, children with PANDAS or PANS don’t display classic strep symptoms, and therefore a strep test isn’t ordered, causing patients to go undiagnosed for prolonged periods following infection.
Successful treatments for PANDAS include antibiotics and/or gamma globulin, to treat the underlying streptococcal infection, in combination with non-steroidal anti-inflammatory (NSAID) drugs such as ibuprofen or naproxen. Response is usually rapid, although in children susceptible to recurrent streptococcal infection, symptoms often reappear and require additional treatment. In addition, not all children respond to antibiotic and NSAID treatment, necessitating more aggressive therapies. And gamma globulin is currently in short supply in the US, creating further challenges for treatment and insurance reimbursement. More research is needed to understand which treatments are appropriate for different subsets of children, and to devise better treatment strategies based on the underlying biological cause.