Focus on Cardiologic Findings in 30 Children With PANS/PANDAS: An Italian Single-Center Observational Study

Abstract

Objective: Cardiac involvement in PANS has not been clarified relying on the scientific literature available until today. It is known that streptococcal infections play a role in the etiology of a great number of diseases including Sydenham chorea and rheumatic fever, among others. Based on the suspected pathogenesis of PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) reported in the medical literature, we decided to investigate the cardiologic involvement in children with a recent PANS/PANDAS diagnosis. Methods: The study population satisfies PANS (1) and PANDAS (2) criteria of diagnoses. Cardiologic assessment was performed through clinical examination, electrocardiography, and echocardiography. Results: In the selected pediatric population, a significant number of children presented mitral valve involvement, systolic murmurs and electrocardiographic abnormalities. High ASLOT levels did not seem to be associated to a cardiac involvement. Conclusions: Often PANS is difficult to diagnose because it is little known by physicians and most of the cardiologic findings described in this study are common among the healthy pediatric population. Also, ASLOT levels seems not to be predictive of cardiac involvement. Furthermore, the existence of PANDAS as a clinical entity is associated with a group of anti-neuronal autoantibodies found in Sydenham chorea is still controversial. We recommend a complete cardiologic evaluation in those children who meet the PANS/PANDAS diagnostic criteria.

Keywords: PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infection); PANS (Pediatric Acute-onset Neuropsychiatric Syndrome); Streptococcus beta hemolytic; cardiologic consultation; heart murmurs; mitral valve (MV); pediatry; tics.

Figure 1

Hierarchy of the Pediatric PANS, modified from Swedo et al. (1).

Figure 2

Map of the 20 Italian regions with number of patients per region (zero where not indicated). In accordance with the diagnostic criteria for PANDAS established by Swedo et al. (2), we selected children with the following characteristics:

1. - Presence of OCD and tics according to the diagnostic criteria for tics and OCD of the DSM-IV and DSM-V;

2. - Onset in childhood (from 3 years to puberty);

3. - Sudden onset and cyclic exacerbation of more severe symptoms, as the patient's parents consult doctors when the child begins to show tics, OCD and other pathological behaviors, or a return of past neuropsychiatric symptoms;

4. - Temporal connection between symptom onset and GAS infection [positive pharyngeal swab, high anti-streptolysin O titer; ASLOT (2)], as sometimes the infection has progressed by the time of disease onset (also 6–9 months), although most children experience symptom onset at 6 weeks;

5. - A connection with neurological abnormalities, including cognitive difficulties, worsening academic performance, dysgraphia, ADHD, depression, separation anxiety, irritability, emotional lability, oppositive behavior, sleeping difficulties, choreiform movements, and Romberg test abnormalities.

Figure 3

Alterations founded in 30 pediatric PANS/PANDAS patients. Percentage refers to the totality of the study population; the darker bars represent the percentage of positive patients for each test (the number inside indicate the absolute number of positive patients).