No, I am not talking about fun stuff like the newest cookware and cute animals. There is nothing ‘fun’ about pediatric autoimmune neuropsychiatric disorders. NOTHING!
As a parent we do everything we can to protect and provide for our children. There are so many sacrifices we make – money, time, vacations/activities, cars (oh the dreaded minivan), possibly careers/aspirations, etc. – but we do this without a blink of an eye. There is no greater joy as a parent than to raise our kids and watch them develop and become amazing adults. To this end (but within reason) there isn’t anything we wouldn’t do for them.
We spend countless hours reading books, asking for advice, and listening to experts on the best ways to raise our children but then the unimaginable happens…your child gets sick. And I’m not talking snotty-nose, flu-like sick, but rather unrecognizable, lost their mind, ‘crazy’ sick. That’s an awful way to say it but there really is no other way to describe it when your incredible, sweet, wonderful, smart, loving child goes to bed one night and wakes up a stranger….as if someone came in during the night and stole your child and replaced them with an unrecognizable alien. It feels like he/she has gone into a DEEP, DARK sleep and it happens in the blink of an eye…within hours to days. One day your child is fine and fully functional, the next day you are convinced you are living in the Twilight Zone and someone abducted your little one. It could read like a popular science fiction novel.
There are few things more painful than to watch your precious child become a prisoner to obsessive thinking, to the point of being nonfunctional, which leads to compulsive behaviors that make no logical sense. Irrational thoughts and worry consume them. As parents we often pride ourselves as always being able to take away our children’s ridiculous fears (like the boogie man) or we ‘kiss it and make everything better’ but in this case they trust their “OCB” (Obsessive-Compulsive Brain) as a more protective guardian….they believe it has become their better parent. There are no words, no reasoning, nothing you can say or do that will convince them that “OCB” is harming them. NOTHING! Just to complicate things more, they trust “OCB” more than they trust you but they also have fear of being separated from you, that something will happen to you, and that it will be their fault. None of it makes sense!
They become aggressive, angry, impulsive, age-inappropriate, verbally and physically abusive, and can’t sit still or turn themselves ‘off’, often leading to sleep disturbances and insomnia. Your straight A student now can’t add basic numbers or comprehend (or sit still for) a simple story. They may develop a tic or unusual motor movement that they are unaware of and certainly can’t stop. As if that’s not enough, they lose bladder control and begin urinating frequently and often in their clothing. It is UNBEARABLE and as parents we can’t fix it! Their brain is under attack….and worse, it is their own body attacking it. This is an autoimmune inflammatory process. I call it ‘Inflame Brain.’
A better way to explain Inflame Brain is to imagine an army (your immune system) fighting a war (against all possible foreign invaders like bacteria, viruses, toxins, etc.) but the army soldiers get confused and they start attacking their own base (tissues, including the brain) because it ‘looks’ really similar to the foreign invader. It is utter chaos! This is known as molecular mimicry.
In PANS/PANDAS, your body’s traditional protective mechanisms stop protecting but engage in a full-on assault of your brain.
Despair, failure, and hopelessness don’t even begin to describe the way you feel as a parent. Worse yet, friends, family, teachers…even your doctor may not understand this ‘new’ behavior and assume some parenting flaw or abuse is the cause. It is a devastating and an incredibly lonely place to be. Parental guilt is dangerous and your own health may deteriorate as a result of the stress, worry, lack of self-care, and lies you begin to believe.
This is an epidemic.
It is estimated that as many as 1 in 200 children are affected by PANS/PANDAS. This is an epidemic and few physicians are informed and able to provide support and the treatment that parents and these children desperately need.
Dr. Kiki Chang, the professor of psychiatry and behavioral sciences at Stanford University Medical Center, says, “We can’t say how many kids with psychiatric symptoms have an underlying immune or inflammatory component to their disorder. But given the burgeoning research indicating that inflammation drives mood disorders and other psychiatric problems, it’s likely to be a large subset of children and even adults diagnosed with psychiatric illnesses.” (Source: Brain Attack, Stanford Medicine, Fall 2014).
So what is PANS/PANDAS?
They are pediatric illnesses characterized by abrupt onset neuropsychiatric symptoms. Before we go into details we should start with a little science and history.
Group A Strep (GAS) is an important human pathogen and is associated with numerous infections but most commonly pharyngitis. Acute Rheumatic Fever (ARF) is a well-known complication of GAS associated throat infections. It is an autoimmune process where the body attacks the heart, joints, and brain. This is thought to be an example of proposed molecular mimicry. GAS is the foreign invader. Your immune system recognizes it as foreign and ‘memorizes’ the amino acid sequence and scans the body to make sure no other invaders got past. During this scan, this amino acid sequence mimics that of the heart, joints, and brain. Your immune system now views these tissues as foreign and begins to attack them.
Sydenham chorea is recognized as the principal neurologic manifestation of Acute Rheumatic Fever. Involuntary movements, obsessive-compulsive symptoms, and other neuropsychiatric symptoms such as hyperactivity and emotional lability characterize Sydenham chorea.
Sound familiar? It can be found in up to 30% of ARF cases, is often seen up to six months after the throat infection, and may be the only findings the patient presents with.
In the late 80’s, Dr. Susan Swedo was doing research on and writing about Obsessive-Compulsive Disorder and Sydenham’s chorea. In 1998, she along with her associates first described PANDAS. It has been described as the ‘half-cousin’ of Sydenham chorea; a neuropsychiatric illness as a result of GAS. PANDAS is a subset of PANS.
PANS is the acronym for “Pediatric Acute-Onset Neuropsychiatric Syndrome” and is characterized by obsessive-compulsive symptoms (OCD) or eating restrictions along with acute behavioral deterioration.
PANDAS is the acronym for “Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections.” It is a subset of PANS and was first reported by a team at the National Institute of Mental Health (part of NIH) in 1998 by Dr. Swedo. PANDAS has 5 distinct criteria for diagnosis, including abrupt “overnight” OCD or dramatic, disabling tics; a relapsing-remitting, episodic symptom course; young age at onset (average of 6-7 years); presence of neurologic abnormalities; and temporal association between symptom onset and Group A strep (GAS) infection.
PANS/PANDAS should be suspected when a prepubescent child has acute onset OCD or demonstrates food restriction behavior or a new tic accompanied by behavioral, emotional, or physical symptoms:
• These diseases affect children, typically prepubescent
• Acute onset
• Symptoms of an attack on the basal ganglia (a collection of nuclei deep within the forebrain responsible for movement and behavior)
○Neuropsychiatric symptoms that typically are seen:
♣ Obsessive Compulsive Disorder (OCD)
♣ Severe anxiety
♣ Behavior regression
♣ Food avoidance
♣ Deterioration in school performance
♣ Night-time sleep disturbances
♣ Hand writing difficulties
• Autoimmune/Autoinflammatory process as a result of a particular infection/possibly toxin
GAS is the known initiator for PANDAS while there is no specific trigger identified for the development of PANS. It is thought to be an abnormal immune response to any number of infectious triggers, including Mycoplasma pneumoniae, Influenza, Lyme disease, and others. Toxins are also hypothesized as other triggers.
What to do when PANS/PANDAS is expected:
• Get Help – not only for your child but for your family:
○You are your child’s advocate – Don’t accept the ‘brush off’ from a clinician – Start with finding a physician that not only believes in but understands PANS/PANDAS and knows how to treat it
• Find resources to help find a provider here
• Resources to find support
•Resource to help you understand OCD – Obsessive Compulsive Disorder – OCD is OCD no matter what the root cause (PANS/PANDAS or otherwise)
○You are your own advocate – Get yourself and your family in counseling to manage and deal with the significant stress and ripple effect this illness causes in the family
• Don’t forget self-care
• Deep breathing exercises
• Keeping a gratitude journal
• Time with friends and family – this is a very isolating illness otherwise – be intentional about this
• Get enough sleep
• Hydrate and make sure to eat
• Get A Workup – Start with an Infectious Disease Workup:
○ *** PANS and PANDAS are clinical diagnoses
• Based on whether presenting symptoms meet the diagnostic criteria, as set forth by National Institute of Mental Health (NIMH).
○ The diagnosis of PANDAS is based on evidence of recent or current streptococcal infection with onset of acute exacerbations. Streptococcal pharyngitis is confirmed with a properly performed throat culture or rapid antigen test. However, rapid throat cultures are known to be insensitive and frequently provide a false negative response. There are also other body sites (perianal, nasal, skin, and vaginal areas) where documented Group A Strep (GAS) infection needs to be considered
• Children may be asymptomatic with active GAS infection. This can initiate a rise in ASO and AntiDNAse-B titers. Antistrepolysin O (ASO) titer (a titer refers to the amount of something, in this case biological molecules in blood that indicate a previous infection) which rises 3-6 weeks after a strep infection, and Antistreptococcal DNAse B (AntiDNAse-B) titer, which rises 6-8 weeks after a strep infection.
○ Most PANS cases are suspected to be post-infectious in origin, although no single organism (other than GAS in PANDAS cases) has yet to be consistently associated with the onset of PANS.
• Most commonly observed are antecedent respiratory infections (rhinosinusitis, pharyngitis, and bronchitis)
• Mycoplasma pneumoniae has been associated with a number of postinfectious neurologic disorders
• Influenza is associated with a number of PANS cases, including influenza H1N1
• Epstein Barr virus infection has been reported to precede various neuropsychiatric disorders
• Borrelia burgdorferi (Lyme disease) has been associated with a wide range of postinfectious neuropsychiatric disorders, including OCD.
• Many other infectious agents including herpes simplex virus and varicella, and others particularly those with prolonged colonization, have the potential to lead to PANS.
• Don’t miss something – Consider other reasons for the symptoms
• Neuropsychiatric symptoms can be caused by several other etiologies including autoimmune encephalitis, systemic autoimmune conditions, and other inflammatory diseases
• Don’t accept a diagnosis that doesn’t make sense
• All too often children with PANS and PANDAS are misdiagnosed as having a psychiatric illness and started on psychotropic medications
• Because PANS/PANDAS symptoms can mimic other illnesses, it can be challenging to obtain the correct diagnosis but it is critical as studies have shown that when appropriate anti-infective and/or immunologic treatment initiated patients may experience complete resolution of or dramatically reduced symptoms.
• What should the specific evaluation/testing for PANDAS/PANS include?
•Meets criteria for PANS/PANDAS (as noted above)
• Physical exam to look for source of active infection
• Culture of tonsils, throat, (perianal, vaginal, nasal areas if suspected source)
• ASO titer
• Anti-DNAse antibodies
• Infectious disease workup to include (Mycoplasma pneumonia antibodies and PCR, Epstein Barr virus antibodies, Influenza, Lyme testing, and, if appropriate, herpes simplex, varicella, and possibly others)
• ANA (Antinuclear antibodies)
• Immunodeficiency workup (IgG with subclasses, IgA, IgM, IgE and Lymphocyte subsets (T, B, natural killer (NK) cells), CBC with manual differential, and post-vaccine titers if previously vaccinated
• Consider a Cunningham Panel – Autoimmune antibodies that bind to these sites potentially block the function of these antigens. This may lead to movement or neuropsychiatric disorders, OCD, and abnormal neurologic behaviors classically seen in PANS/PANDAS
– Molecular Labs – to measure circulating autoimmune antibodies against specific neuronal antigens. It consists of five tests:
1. Dopamine D1 receptor (DRD1)
2. Dopamine D2L receptor (DRD2L)
3. Lysoganglioside GM1
5. CaM Kinase II is a key enzyme involved in the upregulation of several neurotransmitters, including dopamine, and it is known to increase the responsiveness of the neurologic receptors to neurotransmitters.
• Possibly MRI to look for evidence of inflammatory changes in the basal ganglia, including volumetric changes
• Exclude non-infectious triggers such as toxins – heavy metals and environmental toxins
• Remember this is a clinical diagnosis and if the child meets the diagnostic criteria but laboratory values are normal, the diagnosis can still be made
• Keep Clean
○ Remember with PANS/PANDAS the immune system has gone awry and is in a state of chaos, so it is critical to avoid toxins that can trigger this abnormal immune response
○Make sure you read labels – Visit Environmental Working Group (www.ewg.org) to get information about what is considered safe and nontoxic in regards to products and foods
What to do once PANS/PANDAS is diagnosed:
• Start Treatment
○ Antibiotics to treat the specific infection – weeks to months of treatment may be necessary
○ Anti-inflammatory agents
• Start with an anti-inflammatory diet – consider removing pro-inflammatory foods like gluten, sugar, processed foods, and dairy
• Consider adding in anti-inflammatory supplements
• Omega-3 fats
• Other anti-inflammatory agents may be recommended
• NSAIDS – like Ibuprofen for a week or so
• Steroids – Prednisone
○ 5-15 days of Prednisone, if needed
○ Immune Modulating Treatment
• Consider Low Dose Naltrexone
• Intravenous Immunoglobulin therapy (IVIG) – In moderate to severe cases of PANS/PANDAS IVIG has been shown to be helpful with the inflammatory process seen in this autoimmune processes
– Mechanism is not completely clear
◊ Donor antibodies may retrain the abnormal antibody response
◊ Donor antibodies may overwhelm the abnormal antibodies
◊ May have a direct effect on the proliferation of the immune system (T-reg cells)
–Therapeutic apheresis (blood cleaning procedure)
– Plasma removal – plasma is the liquid portion of the blood which contains proteins including antibodies and other immune components
○ Treatment for OCD
• Treat the underlying, root cause as noted above
• Exposure and Response Prevention – a type of Cognitive Behavior Therapy (CBT)
• SSRIs (antianxiety/antidepressant medications such as Prozac) are not typically recommended initially and often don’t work in cases of PANS/PANDAS. Once again, behavioral therapy is the key
○ Consider prophylactic antimicrobial agents when exposed to infections
○ Tonsillectomy for recurrent throat infections should be considered
• Involve the School
○ Communication with the school is critical and will help reduce stress for the parent as well as provide education for the staff
○ Parents should request to be informed if there are cases of documented Strep within the classroom
○ Educate the school about the importance of proper hygiene
○ A great resource to provide the faculty
○ If necessary, a 504 or IEP (Individualized Education Plan) for the child should be requested
• Avoid exposure
○ As best as possible, keep affected children away from people who are currently ill as this might perpetuate symptoms
It is possible to become fully functional even after the diagnosis of PANS/PANDAS strikes your family?
• At Vine Healthcare we have a proprietary process through which we take our patients where we partner with them/their family in order to provide a measure of hope and healing and help them become Fully Functional™ for life….no matter what the diagnosis/health concerns.
Visit our website.
When the righteous cry for help, the LORD hears and delivers them out of all their troubles. The LORD is near to the brokenhearted and saves the crushed in spirit.