1 - Unidade de Infecciologia, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
2 - Serviço de Neuropediatria, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
- Reunião internacional (11th Excellence in Pediatrics Conference)
Introduction Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci (PANDAS) is an ill-defined condition, with little consensus over its definition, epidemiology, pathogenesis and treatment.
Case description We report the case of a 6 year old boy with known medical history of migraines who presented with fever, fatigue and refusal to eat. Antigen screening for group A streptococcal (GAS) was positive and he was treated with amoxicilin and clavulanic acid for five days, with prompt resolution of symptoms. After two months, during which his mother considered his behavior as lethargic and apathetic, the patient started to display symptoms of impulsiveness, restlessness, trouble finishing tasks, facial tics, speech impairment (trouble starting sentences, often repeating the first words) and repetitive behavior (such as washing hands). This deeply contrasted with his previous demeanor, which had been described as “exceptional” by his school teacher. The symptoms progressed over three months. Blood tests revealed a 1.5-fold increase of anti-DNase B titters (301 IU/mL), with a normal anti-streptolysin O value. A bacterial culture from an oropharynx specimen was negative for streptococci. After a traumatic lumbar puncture, the cerebrospinal fluid analysis revealed 9cells/mm3 (monocytes) with normal protein levels. There was no evidence of intrathecal IgG synthesis or oligoclonal bands. The MRI was normal. Throughout this period, the patient displayed the aforementioned symptoms, with apparent critique but little control over his conduct. He was medicated with risperidone and subsequently discharged. Since his release from the hospital, he began follow up with a child psychiatrist, maintaining a sometimes impulsive and hyperkinetic behavior, having started to successfully obey rules.
Conclusion While there’s little agreement over vital matters, such as the definition of the temporal relation between onset of neuropsychiatric symptoms and GAS infection, this case seems to meet all of the working criteria over which PANDAS is currently diagnosed. Albeit general acceptance that GAS infections are one of many factors that can exacerbate symptoms of some neuropsychiatric disorders, causality and the role of autoimmunity are yet to be confirmed.
Palavras Chave: PANDAS, SGA