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FRI0516 The Largest Cohort of Children and Adolescents with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus Infection (Pandas): Preliminary Descriptive Analysis
  1. F. Falcini1,
  2. A. Meini2,
  3. G. Lepri1,
  4. D. Rigante3,
  5. A. Ferrari2,
  6. E. Casalini1,
  7. M. Matucci-Cerinic1
  1. 1Department of Internal Medicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence
  2. 2Pediatric Clinic, University of Brescia and Spedali Civili di Brescia, Brescia
  3. 3Institute of Pediatrics, University Cattolica del Sacro Cuore, Roma, Italy

Abstract

Background PANDAS is defined by neuropsychiatric symptoms, mainly obsessive-compulsive disorder (OCD) or tics, temporally associated with group A β-hemolytic Streptococcus (GAS) infections, showing acute prepubertal onset and relapsing/remitting course.

Objectives To describe the clinical features of a cohort of 242 Italian pts with OCD and/or tics related to a previously confirmed GAS infection.

Methods Between May 2009 and January 2015 we recruited 242 pts (185 males, 76.45%) with OCD and/or tics starting before puberty associated with a previous upper airway infection (impetigo in 2 cases). GAS infection was confirmed in all patients. Their mean age at onset was 77.64 months ±30.1 SD.

Results Two hundred and twenty-three children (93.31%) were full-term born after a regular pregnancy, and 52 (37.68%) from Caesarean section (though for 4 adopted children there were no information); 101/236 (42.8%) had familiars with OCD/tics or other neurologic diseases (for 6 patients there were no familial history data). Sport activity was evaluated in only 149 out of 242 patients, and was performed by 122/149 (81.88%). Clinical signs were motor tics in 109/242 (45.04%), vocal tics in 6/242 (2.48%), motor/vocal tics in 28/242 (3.31%), motor/vocal tics and OCD in 18/242 (7.44%), OCD in 16/242 (6.61%), OCD and motor tics in 61/242 (25.21%), OCD and vocal tics in 4/242 (1.65%). At the first evaluation the mean age was 102.70 months ±35.12 SD. Brain MRI was performed resulting within normal limits in 110/242 patients. Pharyngeal swab was positive in 34/242 (14.05%). Anti-streptolysin O titer was <250 IU in 19/242 (7.85%), 250-550 in 66/242 (27.28%), >550 in 129/242 (53.31%), while in the remaining 28/242 (11.57%) this titer was in progress or not evaluated. Anti-DNase B titer was >650 IU/ml in 76/242 (31.4%) and <650 in 42/242 (17.36%); in the remaining 118 (48.76%) this titer was not evaluated. Anti-nuclear antibodies were studied in 142/242 patients, resulting positive in 7/142 (4.93%). All patients received benzathine benzylpenicillin; neuroleptic drugs were used in 27/242 (11.16%). Therapeutic efficacy was null in 5/242 (2.07%), <25% in 7/242 (2.89%), 25-75% in 125/242 (51.65%), >75% in 58/242 (23.97%), and not-valuable in 47/242 (19.42%).

Conclusions Our preliminary data in the largest pediatric population with PANDAS ever studied until now show male predominance and familiar recurrence rate over 40%. Clinical onset occurs at a mean age of 8.5±2.9 years with motor tics in 45% of cases. Increased anti-DNase B titer is found in over 30% of patients. Benzathine benzylpenicillin has resulted effective in 3/4 of cases with complete or partial remission of symptoms.

Disclosure of Interest None declared

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