PANS / PANDAS

Courtesy of New England Pans/ Pandas Association

 

PANS/PANDAS stands for Pediatric Acute-onset Neuropsychiatric Syndrome

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections

PANS/PANDAS is a clinical diagnosis and one of exclusion, based on history and examination, not simply one laboratory test.  PANS is thought to result from a variety of disease mechanisms and to have multiple etiologies, including but not limited to psychological trauma, underlying neurological, endocrine, and metabolic disorders or postinfectious autoimmune and neuroinflammatory disorders. Therefore, PANS diagnosis does not require a known trigger. However, common triggers are infections as well as non-infectious agents. 

Some children suffer debilitating flares while others function enough to continue to go to school but not remotely at the same functioning level. PANS/PANDAS symptoms may relapse and remit. During subsequent flares, symptoms can worsen and new symptoms may manifest. Initial triggers and secondary triggers may vary. Children are often misdiagnosed as having a psychiatric illness thus prescribed only psychotropic medications rather than treated correctly.

The most studied etiology to date is post infectious autoimmunity and neuroinflammation. Accordingly, PANS can have an encephalitic onset as result of an abnormal immune response to common infections like strep, mycoplasma, coxsackie, lyme, epstein barr and more.

 

PANDAS, a subset of PANS, describes cases with a documented association with group A Streptococcus (GAS) infections.  The antibodies to these infections that normally are created in response to infection mistakenly attack proteins in the brain resulting in neurologic or psychiatric symptoms.

 

PANDAS is based on 5 criteria including acute abrupt onset of OCD and/or severe tics which are often accompanied by comorbid symptoms seen in PANS. Not all patients present with strep throat. Onset can occur 4-6 months post strep infection if antibiotics did not eliminate the bacteria.

 

·         Incidence rate is likely to be between 1-2% of the pediatric population. At least 1 in 200 kids have PANS.  Average age of diagnosis is between 4-13 years of age.

·         Nationwide, 33% of children see more than five doctors before being correctly diagnosed.

·         PANS is frequently misdiagnosed as Tourette’s Syndrome, Autism, OCD, bi-polar disorder, ADHD, or Oppositional Defiance, anorexia, but PANDAS/PANS is a distinctly separate condition.

·         It is likely a lifelong condition unless properly treated. Recovery is possible if treated early and appropriately. PANS/PANDAS symptoms may relapse and remit.

·         Attention has been focused on the pediatric population, but adults have also been identified.

·         Not all kids will have all of the symptoms. Severity of symptoms differs from patient to patient. Some children suffer debilitating flares while others function enough to continue to go to school but not remotely at the same functioning level.

·         Diagnosing and treating PANDAS/PANS promptly may help prevent a temporary postinfectious pathological immune response from progressing into a chronic autoimmune condition.

 

PANS Criteria

 

  • Sudden & acute onset of OCD and/or severely restricted food intake
  • Concurrent severe & abrupt onset of symptoms from at least 2 of the neuropsychiatric categories below:
  • Anxiety, Separation Anxiety
  • Emotional Lability, Depression
  • Aggression, Irritability, Oppositional Behavior
  • Behavioral/Developmental Regresion
  • Deterioration of learning abilities related to ADHD
  • Sensory & Motor Abnormalities
  • Somatic Signs: sleep disturbances, enuresis, urinary frequency
  • Symptoms not better explained by a known medical or neurological disorder. It is a "diagnosis of exclusion".
  • There is no age requirement, typically symptoms start during grade school but post-pubertal cases are not excluded.

·         Can have Motor & Phonic tics (whooping, wringing hands)

·         Can have episodes of extreme anxiety or aggression.

·         Can have visual or auditory hallucinations identical to the psychotic symptoms seen in conditions such as schizophrenia, bipolar disorder, and lupus cerebritis.

·         Can have a decline in handwriting & math skills.

 

PANDAS Criteria

 

·         Significant OCD and/or debilitating/incapacitating Tic symptoms

·         Pediatric Onset – Symptoms have an evident onset between 3 years of age and puberty but post pubertal onset is possible. Pediatric onset specified as it is time of peak exposure and cross-species immunity of GAS infections.

·         Acute onset and episodic course: Defined as either a dramatic onset of OCD or tic symptoms or by relapsing-remitting symptoms that erupt with an acute change. Between episodes, symptoms may lesson but not return to pre-syndrome levels.

·         Associated with Streptococcal-A (GABHS) infection. Note: not all patients will have pharyngitis; strep may be in locations other than throat or patient may be a carrier without active infection. Secondary triggers can be due to exposure to strep or other pathogens.

·         Neurologic abnormalities (motoric hyperactivity, choreiform movement) during symptom exacerbation

·         In conjunction to OCD and/or tics, patients often concurrently experience the comorbid neuropsychiatric symptoms seen in PANS with the same acute and dramatic onset.

 

Treatment Basics

 

·         14-day course of B-Lactam Antibiotics

·         Consider 5-15 days of Prednisone

·         Consider IVIG or PEX

·         Consider continued full dose or prophylactic dose of antibiotics

·         CBT and/or counseling for residual OCD

·         Psychotropic medications can be considered if appropriate. Initial dose must but an extremely low amount with a very gradual taper up as needed to avoid activation, agitation, akathisia, and other adverse effects of the drugs.

 

Other Treatment Options:

 

·         Antifungals

·         Anti-Inflammatories

·         Antihistamines (H1 & H2 Blockers)

·         Extremely low dose SSRIs, increasing slowly

·         Tonsillectomy and Adenoidectomy

·         Dietary Changes

·         Vitamin D3, Omegas, etc

 

Additional Notes on Treatment

 

·         Even if the PANS patient shows no obvious signs of infection at diagnosis, a course of antibiotics for strep is suggested.

·         Patients should be monitored and treated for subsequent infections, including but not limited to the flu and sinus infections.

·         Family members and close contacts should be swabbed for strep even if asymptomatic at the time of patient's initial diagnosis and during exacerbations and treated if positive.

·         PANDAS patients with severe symptoms or recurring strep infections may require prophylactic doses of antibiotics to mitigate risk of neuronal injury.