PANDAS

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections

• Prepubertal onset
• Presence of OCD and/or tics
• Abrupt onset or explosive course
• Associated with Group A Strep
• Abnormal exam with choreiform movements, deterioration in handwriting, tics, or hyperactivity
• No association with rheumatic fever or cardiac involvement
• Treatment:
  • OCD with SSRIs or Risperidone
  • Tics with Clonidine, Haldol, Risperidone
  • Immunomodulation with IVIG or plasma exchange
  • Penicillin ?

Discussion

Clinical observations and systematic study have demonstrated a subgroup of children with obsessive-compulsive disorder (OCD) and/or tic disorder that experience symptom exacerbations after group A ß-hemolytic streptococcal (GAS) infections. This subgroup of children has been designated by the acronym PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections). The recognition of the 5 criteria for PANDAS by Swedo et al and colleagues established a homogenous subgroup of children with childhood-onset OCD and/or tic disorders.

The 5 clinical characteristics that define the PANDAS subgroup are:

1. the presence of OCD and/or tic disorder
2. association with neurologic abnormalities during exacerbations (adventitious movements or motoric hyperactivity)
3. a temporal association between symptom exacerbations and a GAS infection.
4. prepubertal age of onset
5. abrupt onset and relapsing-remitting symptom course

Since the PANDAS subgroup was first proposed in 1998, Sydenham's chorea (SC) has served as a model of etiology. Sydenham's chorea is the neurologic manifestation of rheumatic fever and is a diagnosis of exclusion requiring only the presence of frank chorea in the absence of another neurologic disorder.  Both SC and PANDAS are neurologic autoimmune disorders resulting from GAS infections, and the high proportion of obsessive-compulsive symptoms among children with SC is well documented.  However, the absence of frank chorea and unique relapsing-remitting symptom pattern in children presenting with sudden-onset OCD and tic disorders suggests that PANDAS has a unique pathology of its own. The previous diagnosis of SC has remained an exclusionary criterion for the PANDAS subgroup by definition

Intense interest in the idea of PANDAS has led to small experimental trials of antibiotic prophylaxis and immunomodulation with intravenous immunoglobulin (IVIG) with or without plasma exchange. At present, these interventions have not been proven effective. Indeed, in July 2000, the National Institute of Mental Health released a statement (http://www.nimh.nih.gov/events/pandaalert.cfm) that treatment of PANDAS, TS, or OCD with IVIG or plasma exchange is considered experimental and that these interventions should be used only in NIH-approved research protocols. Therefore, for patients whose tics appear to be exacerbated by GABHS infections, we recommend that:

• Standard pharmacologic and nonpharmacologic approaches be used to treat tics and exacerbations
• Until evidence supports their clinical implementation, the use of immune modulation, including plasma exchange and IVIG, be reserved for clinical studies
• Antibiotics not be used to treat tic exacerbations

Antibiotic prophylaxis be reserved for patients who have ARF

PEDIATRICS Vol. 114 No. 6 December 2004, pp. e748-e751 (doi:10.1542/peds.2004-0308)
Echocardiographic Findings in the PANDAS Subgroup

Bradley L. Schlaggar and Jonathan W. Mink
Movement Disorders in Children
Pediatr. Rev., Feb 2003; 24: 39 - 51.