Healthcare Provider Information

 

  • Physicians of all specialties should be hyper-vigilent of abrupt personality changes in pediatric patients encompassing signs and  symptoms of anger or inappropriate rage, anxiety, phobias, sleep disorder, deterioration in learning abilities, anorexia, behavioral regression, hyperactivity with loss of concentration, or hallucinations. Physical symptoms may include tics (motor or vocal), urinary frequency (nocturnal enuresis), increased sensory response(light, sound, touch, or smell), dilated pupils (fear response), adventitious movements, and unexplained GI complaints (constipation, diarrhea). In short, any abrupt behavioral changes should alert health care professionals to first consider an organic basis for this change. Therefore, PANDAS must FIRST be ruled out before committing children to the chronic use of psychotropic medications. Furthermore, Haloperidol and Ativan should be avoided since worsening symptoms have been reported with the use of these medications.

 

  • Research currently indicates that multi-functional auto-antibodies, immune complexes and cytokines which breech the blood brain barrier attacking neurons in the basal ganglia are involved in the pathogenesis of this disorder.

 

  • At this time, there are no commercially available tests to accurately diagnose this disorder, although streptococcal exotoxin screening  can be performed with antistreptolysyn O (ASO) and anti-Dnase B titers at the time of clinical presentation.  A throat culture is also recommended to diagnose a current pharyngeal streptococcal infection [Swedo2004] although, streptococcus can exist elsewhere in the respiratory tract such as the sinuses, adenoids and tonsils Therefore, a negative throat culture DOES NOT rule out infection or exclude the diagnosis. Group A beta-hemolytic streptococcus (GABS) is believed to be the pathogen in the instigating immune response for many of the cases. ASO and anti-Dnase B titers will generally but not always follow waxing and waning of symptoms.  ASO is affected by many factors and in one study over 46% of children did not have a rising ASO titer despite having colonized strep [Shet2003].

 

  • Typical clinical presentation is one of abrupt onset of symptoms. Parents will frequently recall the exact date when symptoms appeared following an infectious episode(usually GABS). Other infections, bacterial, viral, or vaccinations will usher subsequent attacks.  Symptoms will wax and wane and subsequent attacks may not be related to GABS.  The most common precipitating infectious event is a sore throat or sinus infection. Even exposure to GABS (without clinical symptoms) from school or a family member can cause a recrudescence of symptoms.  Remember that normal ASO and anti-Dnase B titers DO NOT exclude the diagnosis, although in many cases rising and falling titers will parallel symptomatology.


Antibiotic therapy should be started immediately after the diagnosis of PANDAS. There is evidence that antibiotics can be curative if started immediately after the initial diagnosis.There are however four stages of a bacterial illness - exposure, adhesion, colonization and infection.  Once a child’s immune system is exposed, full infection may not be necessary to elicit a PANDAS exacerbation.  Exposure, adhesion or colonization may be sufficient to trigger the autoimmune response.  PANDAS children may suffer from strep carriage or repeated exposure to an individual who is a strept carrier.  However, treatment dose antibiotic therapy for the “initial presentation”  and subsequent  exacerbations appear to be temporarily effective in most cases, since most cases are not the true initial presentation.  Steroid treatment  as a concomitant  form of therapy is effective  and a response can be expected between day 3 and 10 of treatment. Temporary improvement has been reported following tonsillo-adenoidectomy and results are similar to antibiotic therapy. The use of plasmapheresis has resulted in significant improvement in many cases although resurgence of symptoms occurs in most patients. The mainstay of therapy remains  the use of Intravenous gamma globulin therapy. Response to therapy is reportedly greater than 80%. Extinction of symptoms can occur as early as the first day of therapy and as late as 7 to 8 weeks post IVIG infusion. Degree of response and the length of time between IVIG treatments depends on both patient’s age and the duration of clinical symptoms.  Recurrence rate is approximately 10 to 20%.  A second treatment appears to be effective in the remaining nonresponders. Post IVIG chronic antibiotic therapy is common.

 

PANDAS:  A Historical Perspective

 

Since PANDAS was first proposed as a distinct diagnosis in the 1990’s, conventional wisdom had held that it is a rare disease. No research has been done to survey diagnosed cases or make any estimates of how large or small the PANDAS population may be.  However, given the span of symptoms, it is conceivable that at least some children affected by PANDAS have been mis-diagnosed with such conditions as traditional OCD, Tourette’s Syndrome, ADHD or PDD-NOS, among others.

 

In research examining strep infections in the pediatric community, 63% of children have shown elevated titers in response to a GABHS infection.  However, clinicians should be advised that not all children produce elevated titer levels in response to a confirmed infection.  For any child, a series of titer levels should be required before determining what is “normal” for that particular individual.  Moreover, more than a third of children may not produce an elevated response to strep infections.  (Shet A, Kaplan EL, Johnson DR, Cleary PP, “Immune response to group A streptococcal C5a peptidase in children: implications for vaccine development", J Infect Dis. 2003 Sep 15;188(6):809-17.).

 

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