Tillie Doyle
PANDAS and PANS are severe forms of obsessive-compulsive disorder (OCD) that appear suddenly in young children, accompanied by other distressing symptoms. PANDAS is short for pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections and is caused by a strep infection. PANS stands for pediatric acute-onset neuropsychiatric syndrome and is caused by other infections such as Lyme disease or influenza. While there is currently no cure for PANDAS or PANS, treatments include antibiotics, cognitive behavioural therapy, and habit reversal training for tics.
| Characteristics | Values |
|---|---|
| What is PANS? | Pediatric Acute-onset Neuropsychiatric Syndrome |
| What is PANDAS? | Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections |
| Symptoms | Tics, obsessive-compulsive behaviour, severe eating restrictions, cognitive, neurological or behavioural symptoms, anxiety, depression |
| Causes | Linked to strep infection, Lyme disease, influenza, chickenpox, mycoplasma, mononucleosis, walking pneumonia |
| Treatment | Antibiotics, cognitive behavioural therapy, psychiatric medication, exposure and response prevention, immunomodulatory treatment, Intravenous immunoglobulin (IVIG) therapy, Plasmapheresis (Apheresis) or Plasma Exchange (PEX) |
| Prevention | Do not share food, drinks or personal items, wash hands frequently and use hand sanitiser |
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What You'll Learn
Antibiotics to reduce symptoms
PANDAS (Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) and PANS (Paediatric Acute-onset Neuropsychiatric Syndrome) are rare conditions that cause a sudden onset of severe OCD symptoms and other sudden behavioural changes in children. PANDAS is thought to be related to a strep infection, whereas PANS results from other infections such as Lyme disease or influenza.
Antibiotics are a crucial component of the treatment for PANDAS and PANS, especially when the symptoms are triggered by an infection. Antibiotics are used to treat the underlying infection and reduce neuropsychiatric symptoms. The most effective antibiotics for GAS (Group A Streptococcus) infections are "beta-lactams", including penicillin, amoxicillin, and cephalosporins. Erythromycin, azithromycin, and clindamycin are also effective in treating GAS infections.
In a study, azithromycin and penicillin were used in the treatment of PANDAS, and improvements in neuropsychiatric symptoms were observed. The study found a 61% overall reduction in neuropsychiatric symptom exacerbations during the year of antibiotic treatment and a 94% reduction in GAS-triggered neuropsychiatric symptom exacerbations. Additionally, there was a decrease in the mean number of streptococcal infections.
Prophylactic antibiotics are recommended for PANDAS to prevent recurrent GAS exposure, which can trigger symptoms. Prophylactic antibiotics may also be considered for PANS, especially when there is a documented strep throat infection. The choice of antibiotics and dosage can be guided by AHA recommendations for prophylaxis in acute rheumatic fever.
The response to antibiotics can be rapid, with improvements in OCD, anxiety, and other comorbid symptoms of PANS and PANDAS within 24-48 hours. Typically, however, the response occurs after a week or two of therapy. If there is no improvement after 10-14 days, an alternative antibiotic treatment may be considered for an additional 10-14 days. If the antibiotics are effective in reducing symptoms, they may be continued for an additional 2-4 weeks.
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Cognitive behavioural therapy
PANS and PANDAS are clinical disorders characterised by a sudden onset of severe OCD symptoms, including obsessions and compulsions, as well as other behavioural changes. PANDAS is specifically linked to streptococcal infections, whereas PANS is thought to be caused by other infections such as Lyme disease or influenza.
CBT for PANS/PANDAS focuses on the function of the behaviour, rather than the form or symptom. The patient is taught to gradually and systematically face their fears, regardless of the symptom content. This approach can be beneficial even when obsessions and compulsions are rapidly changing, as it gives the patient a set of tools to fall back on when an OCD thought arises.
CBT is not always an appropriate treatment for PANS/PANDAS. Some patients may be unable to engage with behavioural interventions due to the severity of their symptoms. In such cases, immunomodulatory treatments may be recommended first, and CBT may be introduced at a later stage.
It is important to note that treatment should be tailored to the individual and adjusted over time. A licensed healthcare provider should be consulted for diagnosis and treatment.
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Exposure and response prevention
ERP is one of the three complementary interventions used to treat PANS and PANDAS completely and comprehensively. The other two interventions are medication and treating the underlying infection.
ERP is a form of therapy that aims to reduce ritual behaviours exhibited by children with PANS or PANDAS. These ritual behaviours are called compulsions and are characterised by repetitive actions and the inability to stop. For example, a child may walk in and out of a doorway multiple times, attempting to do it "right".
ERP is a behavioural therapy that teaches patients how to manage their symptoms. It is a form of CBT, which is a type of therapy that focuses on the relationship between thoughts, feelings, and behaviours. CBT helps patients understand how their thoughts and feelings influence their behaviours and teaches them how to manage their symptoms by changing their thoughts and feelings.
ERP is often used in conjunction with other treatments, such as medication and treating the underlying infection. Medication is typically introduced with a "start low and go slow" approach, with beginning dosages for PANS and PANDAS being typically 1/4 or less than the typical doses. Antibiotics are also used to treat the underlying infection, especially in patients with severe symptoms and/or frequent flares, to reduce the risk of further infection and neuronal injury.
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Intravenous immunoglobulin therapy
Intravenous immunoglobulin (IVIG) therapy is an effective treatment for PANDAS and PANS, with the ability to reset" the immune system. IVIG is made from pooled human donor blood products, and as such, carries a risk of hidden infections from the donors. However, in moderate to severe cases, IVIG has been shown to significantly decrease symptom severity and shorten the course of illness.
The treatment involves the administration of immunoglobulins directly into a vein, typically immunoglobulin G (IgG). The dosage depends on the patient's weight, with the most common dosage being 750 milligrams per kilogram of body weight per day for two days (total dose 1.5g/kg). A child's symptoms may start to improve as soon as a day after the IVIG treatment, with the average recovery period being three weeks. In rare cases, children may have a delayed response, with symptoms abating up to two months after IVIG therapy.
Before the procedure, lab tests are conducted to evaluate immunoglobulin levels. A topical anesthetic is applied to minimize discomfort where the intravenous catheter is inserted. The patient is also premedicated with acetaminophen to reduce headache and/or swelling, and diphenhydramine to prevent allergic reactions. Intravenous steroids are also administered.
IVIG treatment comes with some risks and potential side effects. Severe allergic reactions may occur, and in rare cases, it may trigger hemolysis, leading to anemia, or Stevens-Johnson syndrome.
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Plasmapheresis or plasma exchange
Plasmapheresis, or plasma exchange, is a "blood cleaning" procedure that may be used in the treatment of PANS/PANDAS. It involves removing the patient's blood through an intravenous catheter and processing it in a plasmapheresis machine, which separates the blood's formed elements (red blood cells, white blood cells, and platelets) from the plasma (liquid portion of the blood containing proteins, antibodies, and other immune components). The plasma is then removed and replaced with equal volumes of albumin, which is mixed with the patient's blood components and returned to the body through a second intravenous catheter. This procedure is typically carried out in a pediatric apheresis center due to its invasive nature and associated risks.
The benefits of plasmapheresis for PANS/PANDAS have been demonstrated in clinical trials. Perlmutter et al. conducted a small randomized-controlled trial in 1999, which showed a significant improvement in patients undergoing plasmapheresis compared to a placebo group. On average, patients in the trial improved by 65%, with some children experiencing nearly complete symptom resolution within one to two weeks of completing the treatment series. The trial utilized five single-volume treatments over ten days, reducing circulating antibodies by 85-95%. More recently, Dr. Beth Latimer has reported therapeutic benefits with just three 1.5-volume exchanges performed over four to six days, reducing the number of procedures and related risks while increasing the probability of using peripheral catheters instead of a central line.
Plasma exchange is listed as an accepted first-line therapy by the American Society for Apheresis, either alone or in combination with other treatments for PANDAS exacerbation. It is particularly preferred in cases of severe, life-threatening symptoms due to its quick response rate. However, it is important to note that plasmapheresis is an invasive procedure with potential adverse events, such as mild vasovagal episodes related to needle insertion and cutaneous paresthesias caused by citrate-induced hypocalcemia. While rare, machine malfunction can result in blood loss, leading to anemia or temporary neutropenia.
Overall, plasmapheresis or plasma exchange is a promising treatment option for PANS/PANDAS, especially in severe cases. Its effectiveness has been demonstrated in clinical trials, and it is recognized as a first-line therapy by expert societies. However, it is important to carefully consider the potential risks and ensure that the procedure is performed in a specialized pediatric apheresis center.
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Frequently asked questions
PANS stands for Pediatric Acute-onset Neuropsychiatric Syndrome. PANDAS is a subgroup of PANS and stands for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. Both PANS and PANDAS are severe forms of obsessive-compulsive disorder (OCD) that appear suddenly in young children, accompanied by other confusing and distressing symptoms.
The symptoms of PANS and PANDAS include tics, obsessive-compulsive behaviour, and other mental and neurological symptoms. Children with PANS and PANDAS will have sudden and severe obsessions, compulsions, or both.
PANS and PANDAS can be treated with medicines and therapy. Antibiotics are a temporary measure to reduce or put into remission certain symptoms of PANS and PANDAS. They will not cure PANS or PANDAS but can help with symptoms that occur because of brain inflammation, including OCD, anxiety, tics and more.
PANS and PANDAS are rare. PANDAS is thought to affect select children who have had strep infections, such as strep throat or scarlet fever. PANS, on the other hand, can result from other infections such as Lyme disease or influenza.
