19/1/16PANDAS

ETIOLOGY

OutlinePATHO

GENESISCLINICAL

FEATURES

DIAGNOSTICCRITERIA

TREATMENT PROGNOSIS

PSYCHIATRICCOMORBIDITY

INTRODUCTION- Neuropsychiatric disorders

- Autoimmune basis

- Related to infection with group A beta-hemolytic streptococci (GAS)

- OCD, Tic disorder and Tourette’s syndrome

- Genetic predisposition: Tic disorders and OCD in first-degree relatives --> higher

GAS OCD Tics Tourette

ETIOLOGY

True incidence/prevalance not known, rare disorder, 10 cases wereidentified among 30,000 throat cultures positive for GAS

M:F = 2.6:1 SYMPTOMS Prepubertal 3-11 yo.

PATHOGENESIS

MRI scans- enlargements of the caudate, putamen, and globus pallidus,

which points to regional inflammatory changes

Clinical features

Tics- Classic form, appear for the first time - Abrupt onset- Dramatic exacerbations- Occur during or after a strep infection

abnormal results of neurologicalexamination (hyperactivity, choreiformmovements, or tics) during an exacerbation

OCD

OCD symptoms of Children with PANDASand Primary OCD or Tic Disorder

Some or all of the following symptoms:

depend on clinicalhistory and

severity

Blood testfor ASOtiters

45 MIN

at symptom onset of <2 weeks duration and again4-8 weeks laterThroat swab

(rapid andculture)

Rule outOtherdiseases

Lyme Disease, ThyroidDisease, Celiac Disease,Lupus, SydenhamChorea, Kawasaki’sDisease, and acuteRheumatic Fever

Investigations

Presence ofOCD and/or Tic

GABHS

Pediatric onset

Episodic course

Diagnostic criteria

NeurologicalAbnormalities

SUB HEADERPsychiatric comorbidity

ADHD Mood&Affective Anxiety

Mood

Anxiety

ADHD

Therapy for GAS infection

Therapy for OCD/Tic disorder

Immunomodulatory therapy

Prophylactic therapy

TREATMENT

No approved evaluation and treatment protocols

↓Incidencesuppurative

complications

↓nonsuppurativecomplications

↓ Risk oftransmission

Therapy for GAS infection

Antistreptococcal therapy

positive culture orrapid antigen detection test for GAS

Standard pharmacologic and behaviourtherapies

Pharmacological; Childhood OCD- SSRIs -->first-line tx.Tic disorder/Touretteʼs syndrome-- α adrenergic agonists - clonidine, guanfacine- atypical neuroleptic agents - risperidone- typical neuroleptic agents- haloperidol,pimozideNonpharmacological interventions;CBT-cognitive behavioral therapy (i.e., ERP-exposure and responseprevention)

Consider evaluated for fine motor deficitsby an occupational therapist if handwritingor coordination skills have deteriorated

Therapy for OCD/Tic disorder

Immunomodulatory therapy

- Not recommended outside of the research setting - May be considered an alternative for severely ill patients who have not respondedto standard therapies

Glucocorticoids

Plasma exchange

IVIG

Prophylactic therapy

Prophylactic antibiotics to prevent recurrences- not recommended

Future studies are required to develop guidelines

Prospective case-control study - continuous prophylaxis against GAS mightprevent an average of 0.06 exacerbations perpatient-year

Prognosis- Assuming the infection is adequately treated,the first symptoms generally improve within 4-6weeks

- The next OCD episode - may last longer - may be triggered by a variety of immunologicalchallenges such as another strep infection, or byother bacterial or viral infections (ear infections,sinusitis, pneumonia, meningitis, impetigo)- Long-term outcome of children who meetcriteria for PANDAS is not known

- Unrecognized PANDAS and untreated PANDASmay result in an increased risk of progression tolifelong OCD and tic disorders

4-6wks