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PANDAS
Disease definition
PANDAS is an acronym for Pediatric Autoimmune Neuropsychiatric Disorders Associated with a group A beta-hemolytic Streptococcal infection and applied to a subgroup of children with obsessive-compulsive disorder (OCD) and/or tic disorders.
ORPHA:66624
Classification level: Disorder- Synonym(s):
- Pediatric autoimmune disorders associated with Streptococcus infections
- Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Childhood
- ICD-10: G96.8
- ICD-11: 8E4A.0
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: 7312
- MedDRA: -
Summary
Epidemiology
The prevalence is unknown but the boy-to-girl ratio is 2.6:1.
Clinical description
The current diagnostic criteria for the PANDAS are: presence of OCD and/or a tic disorder, very young age at onset (prepubertal), sudden and dramatic onset of symptoms, association between streptococcal infections and episodic relapsing-remitting exacerbations manifesting as neuropsychiatric symptoms (motor hyperactivity or adventitious movements including choreiform movements or tics). The increased severity of symptoms usually persists for at least several weeks, but may last for several months or longer, followed by a slow, gradual improvement. The major distinctive feature of PANDAS is the temporal association between neuropsychiatric symptom exacerbations and streptococcal infections. Additional neuropsychiatric symptoms occur frequently: emotional lability, separation anxiety, anorexia, impulsivity, distractibility and motor hyperactivity characteristic of attention deficit hyperactivity disorder (ADHD). Comorbid disorders include major depression (36%), major dysthymia (6%) and separation anxiety disorder (20%).
Etiology
The etiology is uncertain. One theory is that streptococcal infections trigger an antibody response in some children that causes changes in the basal ganglia. No specific genetic factors have been identified.
Diagnostic methods
Diagnosis of PANDAS is clinical. Neuroimaging studies may reveal increased basal ganglia volumes.
Management and treatment
Management includes standard interventions for obsessive-compulsive and tic disorders: cognitive-behavioural therapy, reversal therapy in the case of tic disorders and pharmacologic therapy (neuropsychiatric drugs, antibiotics to prevent infections and intravenous immunoglobulin therapy).
A summary on this disease is available in Deutsch (2006) Español (2006) Français (2006) Italiano (2006) Nederlands (2006)
Additional information