Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)

What Causes Pediatric Acute-onset Neuropsychiatric Syndrome (PANS)?

Scientists don’t yet know precisely what causes PANS. However, researchers believe that it is triggered by an inappropriate response of the body’s immune system in most cases. In this type of condition, called an autoimmune disorder, the immune system attempts to fight off a bacterial or viral infection but mistakes some of the body’s own healthy cells for bacteria. When infection-fighting cells mistakenly attack brain cells, psychiatric symptoms result.

Scientists believe PANS sometimes is caused by triggers other than infections. Possible causes include psychological trauma or an underlying disorder such as cerebral vasculitis or neuropsychiatric lupus. However, other causes are likely to be discovered, and a PANS diagnosis does not require doctors to identify the disorder’s trigger.

Is Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Hereditary?

PANS likely is not a disorder that a child directly inherits from their parents. However, scientists believe some children may inherit genetic traits that make them susceptible to the autoimmune reaction that causes PANS and PANDAS. The condition probably occurs when the genetic predisposition coincides with external factors, including infections or other underlying conditions.

How Is Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Detected?

Early treatment of PANS may help lessen the severity of symptoms and prevent significant long-term impacts. Parents should consult a doctor if their child experiences signs of the condition, including:

• Sudden onset of obsessive-compulsive behaviors
• Change in eating behaviors in association with other psychiatric symptoms such as irritability, depression, or anxiety
• Behavioral changes

How Is Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Diagnosed?

There are no tests or exams that can directly diagnose PANS. However, if a doctor suspects PANS, they may perform several diagnostic steps to look for the characteristic features of the disorder and rule out other possible causes for the symptoms. The diagnostic criteria for PANS include:

• OCD and/or eating disorder symptoms are present.
• Symptoms appear suddenly, within 24-48 hours.
• Symptoms may improve and then worsen.
• At least two other neuropsychiatric symptoms are present, such as:
  • Anxiety
  • Rapid mood swings or depression
  • Irritability, aggression, or oppositional behaviors
  • Behavioral regression or age-appropriate behavior
  • Deterioration in school performance
  • Sensory or movement abnormalities
  • Unexplained physical symptoms such as insomnia or bedwetting
• Symptoms aren’t explained by another medical or psychiatric disorder.

Doctors diagnose PANS only when all other possible causes of the symptoms are ruled out. To reach this point, doctors will likely perform a series of diagnostic steps that may include assessment of the child’s medical history, physical and neurological exams, lab tests, sleep studies, imaging exams, or a psychiatric evaluation.

PLEASE CONSULT A PHYSICIAN FOR MORE INFORMATION.

How Is Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Treated?

PANS is usually treated with antibiotics even if the child is not known to have had a recent bacterial infection. Other treatment options may be required to treat the neuropsychiatric symptoms of PANS. Common treatment approaches include:

• Cognitive-behavioral therapy (CBT)
• Selective serotonin reuptake inhibitor (SSRI) medications to treat psychiatric symptoms. Children with PANS may be especially susceptible to side effects of these medications, so treatment with them should be carefully monitored.
• Immune-stabilizing treatments such as corticosteroids, nonsteroidal medications, plasma exchange, or immunoglobulin (IVIG) therapies. These treatments may carry the risk of significant side effects and are usually used only in the case of severe symptoms that don’t respond to other treatments.

How Does Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Progress?

In some cases where the triggering cause of PANS is identified and treated early, symptoms are entirely eliminated by treatment and don’t recur.

In other cases, symptoms may persist and worsen at times, a pattern referred to as episodic. In these cases, initial symptoms appear suddenly over a few days and gradually improve over weeks or months. When symptoms reappear, the new period of severe symptoms may last as long as several months.

When PANS symptoms are left untreated, long-term or permanent complications can result.

How Is Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Prevented?

Because some cases of PANS have an unknown trigger, there is no known way to prevent these cases. However, early identification and treatment of the underlying disorders associated with PANS may help reduce the risk of developing PANS symptoms.  

The best way to prevent autoimmune-associated PANS is to prevent infections, and the best way to avoid infections is to practice good hygiene habits and encourage them in your child. Good preventive practices include:

• Wash hands before you eat or prepare food
• Wash hands after using the bathroom
• Don’t share food, drinks, or utensils when someone in the house is sick
• Cover your mouth and nose with a tissue of the inside of your elbow when you sneeze or cough
• Use antibiotics according to your doctor’s direction when they’re prescribed for an infection

Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Caregiver Tips

• Learn about PANS. The disorder is not well understood, and people in your child’s life likely won’t understand the impact PANS has on them. Educate yourself about the condition’s impacts so you can be an effective advocate for your child.
• Be diligent about protecting your child. Practice good hygiene habits to help prevent future strep infections, and carefully follow all your doctor’s instructions concerning antibiotics and other medications.
• Get support from other families who are living with PANS. Online support groups can put you in touch with other people who understand the challenges the disorder poses.

Children with PANS suffer from different brain-related issues, a condition called co-morbidity. Here are a few of the disorders commonly associated with PANS:

• Obsessive-compulsive disorder (OCD) is common among children with PANS.
• Many children with PANS have a tic disorder such as Tourette syndrome.
• Eating disorders such as anorexia are also commonly co-morbid with PANS.
• Many children with PANS suffer from mood disorders such as depression.
• Anxiety disorders such as separation anxiety disorder are common.
• Trichotillomania (hair-pulling disorder) is often seen in children with PANS.

Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Brain Science

In an autoimmune disorder, such as most PANS cases, antibodies attack specific healthy cells because molecules on the surface of these cells are identical or similar to molecules on the surface of the virus or bacteria that the antibodies are trying to fight. The confusion causes the antibodies to destroy healthy cells as if they were viruses or bacteria.

Recent research has attempted to identify which brain cells are attacked by antibodies in the case of PANS and PANDAS. One study found that specific nerve cells called cholinergic interneurons in part of the brain called the basal ganglia appear to be affected more by autoimmune reactions than other brain cells are. Although it’s not yet clear how damage to cholinergic interneurons may cause PANS symptoms, problems with these cells have been associated with Tourette syndrome in previous studies.

Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) Research

Title: Neurobiologic, Immunologic, and Rheumatologic Markers in Youth With PANS

Stage: Recruiting

Principal investigator: Joanne Cheung   

Stanford University

Palo Alto, CA

This study investigates the neurologic, immunologic, and rheumatologic markers of Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS). PANS is a condition characterized by the abrupt, dramatic onset of obsessive-compulsive disorder (OCD) and/or eating restriction, accompanied by equally abrupt and severe co-morbid neuropsychiatric symptoms. These include anxiety, emotional lability, depression, irritability, aggression, oppositionality, deterioration in school performance, behavioral (developmental) regression, sensory amplification, movement abnormalities, sleep disturbance, and urinary frequency. PANS is thought to be caused by infection, inflammation, or alternate triggers associated with a brain response that leads to these symptoms. The purpose of this study is to examine specific neurologic, immunologic, rheumatologic, and genomic components in children with the acute-onset of psychiatric symptoms. This research may begin to uncover a much larger story of autoimmune processes involved in childhood psychiatric disorders. By better understanding the etiologic components of the psychiatric phenomenon, future treatments may be better targeted to underlying causes.

The investigators will recruit 500 children, 1-18 years old, at onset with PANS/PANDAS. They will be treatment-naive and within one month of onset/exacerbation. The 500 children with PANS will be gender- and age-matched to 100 healthy children to allow examination of immunologic, neurologic, genomic, and behavioral differences between these two groups of children.

Title: Study To Compare The Effect of Panzyga Versus Placebo in Patients With Pediatric Acute-onset Neuropsychiatric Syndrome

Stage: Recruiting

Contact: Patrick Murphy  

Octapharma Research Site

Little Rock, AR

This is a superiority study to compare the effect of Panzyga versus placebo in patients with Pediatric Acute-onset Neuropsychiatric Syndrome.

Title: Trial of Naproxen Sodium for the Treatment of OCD in Children With PANDAS

Stage: Recruiting

Principal investigator: Kyle Williams, MD PhD  

Massachusetts General Hospital

Boston, MA

This project aims to rigorously evaluate a potential treatment for inflammation-related Obsessive-Compulsive Disorder (OCD) symptoms in children. To accomplish this goal, the investigators will conduct a double-blind, randomized, placebo-controlled trial of Naproxen Sodium, a nonsteroidal anti-inflammatory drug (NSAID) in participants diagnosed with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections (PANDAS). This research fills a gap in the empirical evidence base for the treatment of PANDAS and will add to a growing literature of empirically-derived practices for PANDAS.

The investigators propose to systematically evaluate the effects of naproxen sodium on anti-obsessional and behavioral improvement and conduct the first randomized controlled trial of naproxen sodium in the treatment of children with PANDAS. The study will involve an 8-week, double-blinded randomized controlled trial to evaluate the efficacy of naproxen sodium (10mg/kg, by mouth, twice a day) versus placebo to treat OCD symptoms in children with PANDAS. The investigators will acquire completed data on 44 children with PANDAS for this pilot study (i.e., 22 randomized to active treatment; 22 randomized to placebo). The outcome will be assessed comparing pre- and post-treatment OCD symptom severity using a standardized, clinician-administered interview assessing OCD symptoms by an independent rater blind to treatment assignment.