Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections
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|Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections
๐ง Introduction
- PANDAS describes a group of acute-onset neuropsychiatric disorders in children triggered by an autoimmune response to Group A ฮฒ-haemolytic streptococcal (GAS) infection.
- Proposed mechanism: antibodies generated against streptococcal antigens cross-react with basal ganglia neurons (molecular mimicry), leading to abrupt psychiatric and neurological symptoms.
- First described in the late 1990s, PANDAS remains controversial and is considered a subtype of Paediatric Acute-onset Neuropsychiatric Syndrome (PANS).
โ ๏ธ Key Clinical Features
- ObsessiveโCompulsive Disorder (OCD): Rapid, dramatic onset of intrusive thoughts, compulsions, rituals.
- Tics: Motor or vocal tics resembling Touretteโs syndrome; often emerge suddenly after infection.
- Other Neuropsychiatric Symptoms: Emotional lability, anxiety, separation anxiety, irritability, behavioural regression.
- Somatic symptoms: Sleep disturbances, enuresis, handwriting deterioration, and in some cases food restriction or anorexia-like symptoms.
๐งช Diagnosis
- Diagnosis is clinical, supported by history of a recent streptococcal infection.
- Proposed criteria:
- Presence of OCD and/or tic disorder.
- Prepubertal onset (usually 3โ12 years).
- Abrupt onset with episodic course.
- Temporal association with streptococcal infection.
- Neurological abnormalities (e.g., hyperactivity, choreiform movements).
- Investigations: ASO titres or anti-DNase B may suggest recent strep exposure, but not diagnostic.
- Exclusion of other causes (e.g., Sydenhamโs chorea, Touretteโs, primary OCD).
๐ Management
- Eradication of Streptococcus:
- First-line: Antibiotics (penicillin V, amoxicillin, or azithromycin if penicillin-allergic).
- Long-term prophylaxis sometimes considered in recurrent cases (though evidence mixed).
- Neuropsychiatric Symptom Management:
- CBT and SSRIs for OCD (standard of care, as in non-PANDAS OCD).
- Risperidone or other atypical antipsychotics for disabling tics/behavioural symptoms (specialist-led).
- Immunomodulation (specialist use only):
- IVIG or plasmapheresis may benefit severe, refractory cases, but evidence remains limited and controversial.
- Supportive care: Multidisciplinary approach โ child psychiatry, paediatrics, psychology, education support.
๐ Prognosis
- Symptoms often improve with antibiotics and psychiatric support, but relapses are common with new infections.
- Some children progress to chronic OCD or tic disorders despite treatment.
- Early recognition is essential to reduce long-term psychological and functional impact.
๐ Key Exam Pearls
- PANDAS is a clinical diagnosis โ no specific lab test confirms it.
- Always rule out Sydenhamโs chorea in post-strep neuropsychiatric presentations (part of rheumatic fever spectrum).
- Antibiotics may improve psychiatric symptoms โ unique compared to primary OCD/Touretteโs.
- Still controversial โ some guidelines (esp. in UK) prefer term PANS for broader differential.
๐ References
- Elia J. et al, Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, J Child Neurol.
- Oxford Handbook of Clinical Medicine, p.692 โ Tic Disorders.
- Royal College of Psychiatrists: Guidance on PANS/PANDAS (2020).