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Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections: Clinical Description of the First 50 Cases

Link to Journal Article

Abstract

OBJECTIVE: The purpose of this study was to describe the clinical characteristics of a novel group of patients with obsessive-compulsive disorder (OCD) and tic disorders, designated as pediatric autoimmune neuropsychiatric disorders associated with streptococcal (group A ß-hemolytic streptococcal [GABHS]) infections (PANDAS).

METHOD: The authors conducted a systematic clinical evaluation of 50 children who met all of the following five working diagnostic criteria: presence of OCD and/or a tic disorder, prepubertal symptom onset, episodic course of symptom severity, association with GABHS infections, and association with neurological abnormalities.

RESULTS: The children’s symptom onset was acute and dramatic, typically triggered by GABHS infections at a very early age (mean=6.3 years, SD=2.7, for tics; mean=7.4 years, SD=2.7, for OCD). The PANDAS clinical course was characterized by a relapsing-remitting symptom pattern with significant psychiatric comorbidity accompanying the exacerbations; emotional lability, separation anxiety, nighttime fears and bedtime rituals, cognitive deficits, oppositional behaviors, and motoric hyperactivity were particularly common. Symptom onset was triggered by GABHS infection for 22 (44%) of the children and by pharyngitis (no throat culture obtained) for 14 others (28%). Among the 50 children, there were 144 separate episodes of symptom exacerbation; 45 (31%) were associated with documented GABHS infection, 60 (42%) with symptoms of pharyngitis or upper respiratory infection (no throat culture obtained), and six (4%) with GABHS exposure.

CONCLUSIONS: The working diagnostic criteria appear to accurately characterize a homogeneous patient group in which symptom exacerbations are triggered by GABHS infections. The identification of such a subgroup will allow for testing of models of pathogenesis, as well as the development of novel treatment and prevention strategies. (Am J Psychiatry 1998; 155:264–271)

Paediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS)

Source: Behavioural Neurotherapy Clinic

Have you noticed how different your child has been acting ever since he had that sore throat? He seems hyperactive, moody and keeps blinking his eyes. He also has become very particular about the way he does certain things. His teachers say that he’s not paying attention in class and they’re having trouble reading his handwriting.

Your child may have developed what the medical community has named PANDAS. Although rare, PANDAS stands for Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus. What does all that mean? Basically, it means that when the body’s defences are trying to attack the Streptococcal bacteria causing the sore throat, there is some degree of mistaken identity and it also attacks some parts of the brain.

The autoimmune attack is thought to occur on closely related parts of the brain, causing a range of behavioural and emotional problems. When first discovered PANDAS was linked to obsessive-compulsive disorder, Tics and Tourettes syndrome. Mostly because these abnormal behaviours are overt and easily recognised.

Tics can be uncontrollable movements, such as eye-blinking or shoulder-shrugging, or automatic noises such as throat clearing, grunting or saying certain words repeatedly. More recently PANDAS has been associated with a wider range of related behaviours. Affected children can have any combination of the following symptoms:

* Cognitive inflexibility, difficult to reason with, as if stuck on an idea,

* Obsessive/repetitive/compulsive argumentative behaviours,

* TICS (repetitive vocalisations of body movements),

* Tourettes Syndrome,

* Attention deficits and oppositional/defiant behaviours.

The bacteria associated with this disorder are known as Group A Beta-Haemolytic Streptococcus (GABHS). They are also the bacteria associated with rheumatic fever, a disease characterised by heart and joint inflammation that can occur after an untreated strep throat. A type of rheumatic fever with mostly neurological symptoms is Sydenham’s chorea (also known as St. Vitus Dance). Symptoms of Sydenham chorea may occur several weeks to months after the infection and may include poor or diminished muscle control and tone, poor coordination and awkward movements of the face, body, arms and legs.

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