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OP0118 Clinical Overview of A Cohort of 87 Italian Patients with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus Infection (PANDAS)
  1. F. Falcini1,
  2. G. Lepri2,
  3. F. Bertini2,
  4. G. Tarantino3,
  5. M. Matucci Crinic2,
  6. D. Rigante3
  1. 1University of Florence, Division of Rheumatology
  2. 2Department of Internal Medicine, Section of Rheumatology, Transition Clinic, University of Florence, AOU Careggi, Florence
  3. 3Institute of Paediatrics, Università Cattolica del Sacro Cuore, Rome, Italy

Abstract

Background PANDAS is a pathological condition, starting before puberty, characterized by rapid onset of OCD and/or tics, temporally related with immune-mediated response to GABHS infection

Objectives To describe the clinical features of 87 Italian patients with sudden occurrence of OCD and/or tics related to a previously GABHS infection

Methods Between May 2009- January 2014 we observed 87 patients (66 M, 21 F, mean age: 77.9±28.3 months) with OCD and/or tics associated with a previous GABHS infection. Demographic, familiar data, and specific laboratory data (thyroid function, ANA, anti-dsDNA, anti-ENA, anti-cardiolipin, LAC, and anti-tissue transglutaminase antibodies) were assessed. All underwent EEG, echocardiography and neuropsychiatric consultation; 55/87 brain MRI

Results 80/87 (93.0%) were full-term, 19/87 (22.1%) after Caesarean section, 41/86 (47,6%) had familiars with OCD/tics or other neurologic diseases. 74/87 (87.7%) did regular physical activity. In all, clinical onset was dramatically acute and occurred at a mean age of 77.9±28.3 months (range 24-160). 40/87 (46.0%) started with motor tics; 5 (5.7%) with OCD; 6 (6.9%) with motor/vocal tics; 29 (33,3%) with motor tics and OCD, and 6 (6.9%) with motor/vocal tics and OCD. At first evaluation 12/87 (13.8%) had motor/vocal tics, 35/87 (40.2%) only motor tics, 1/87 (1.1%) vocal tics, 23/87 (26.4%) motor tics and OCD, 8/87 (9.2%) motor/vocal tics and OCD, 4/87 (4.6%) OCD, and 3/87 (3.4%) had no apparent symptom. 33/87 had no clinically-evident infectious episode before clinical onset, 53/87 (61.3%) a previously-established pharyngitis, otitis and/or upper airway infection; 1/87 had a previous impetigo.Mean age at diagnosis was 104.8±32.5 months. Inflammatory parameters were negative in all, at onset and at our first clinical evaluation. Brain MRI, EEG, thyroid tests, and coeliac disease screening were normal in all. Their subsequent histories were mostly alternating between remission and exacerbation. Anti-streptolysin O titer was evaluated in 79 patients: it was less than 250 IU in 14 (17.7%), between 250 and 550 IU in 37 (46.8%), and over 550 IU in 28 (35.4%). Anti-DNase titre was evaluated in 56 patients, resulting increased (over 650 U) in 26 (46.4%). ANA was positive at low titre in 2 patients, while other autoimmunity tests were completely negative. All received benzathine benzylpenicillin while amoxicillin was given for 10 days in the case of a concurrent demonstrated streptococcal infection. Sertraline was used in 4, haloperidol in 6. Psychotherapy was required in most patients. 60/82 patients (73.2%) displayed complete/partial remission of neurological symptoms; the follow-up of 5 patients is in progress

Conclusions Our preliminary data show that PANDAS is mainly observed in males (75.9%) with familiar recurrence of different motor disturbances in 47.6% of cases, clinical onset occurs at a mean age of 6.4±2.3 years with the coexistence of OCD and motor tics in most cases, and an increased anti-DNase titre is found in 46.4% of patients. In addition, benzathine benzylpenicillin has resulted effective in all cases with complete/partial remission of symptoms

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.3560

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