OCD and beta haemolytic Streptococcus: a nasty association

Authors: A. Mazzola1, G. Mazzola2

1 Infectious diseasesspecialist, ARNAS Civico; Palermo, Italy

2 Psychiatric specialist, Mental Health Department; Palermo, Italy

 

Key words: Streptococcal infection, autoimmunity, OCD, PANDAS.

Summary

The precise cause of paediatric obsessive-compulsive disorder (OCD) is unknown. In addition to genetic factors, autoimmunity may play a role, possibly as a sequel of preceding streptococcal infections. Although PANDAS patients have been reported to improve after therapeutic plasma exchange, and antibiotics may prevent symptom exacerbations, immune-based treatments should not be routinely given.

To remarking the post-infective nature of this disorder, in this brief note we report a case of a 10-year-old girl who presented with tic and hemi-chorea associated with group A beta haemolytic Streptococcus (GABHS) throat infection. She had a recurrence of her symptoms associated with a further GABHS infection, but has had no further symptoms following tonsillectomy.

Introduction

Just as medical sleuths have discovered there is an association between Helicobacter pylori infections and peptic ulcers and that Chlamydia pneumoniae has suspected role in atherosclerosis, streptococcal infections (group A beta haemolytic streptococcus) now have been implicated in worsening some cases of obsessive-compulsive disorders (OCD).

Obsessive-compulsive disorders (OCD) in children has many clinical features similar the form encountered in adults. The symptoms experienced by the subject, like the products of his own thoughts, a compulsive behaviour, agitation and abnormal movements, are repetitive, stereotyped, unpleasant, and recognised as excessive, aimless or absurd. These signs are thought to involve corticostriato-thalamocortical circuits and dysregulation of their component neurotransmitter systems1.

As regards diagnosis, the criteria proposed by DSM-IV require the presence of obsessions and/or compulsions almost every day, over a period of at least 2 weeks. Symptoms are associated with the subject's efforts at resistance and involve a momentary reduction in anxiety. These manifestations cause significant distress and/or are time-consuming (take more than 1 hour per day), significantly interfere with the patient's functioning.

An history of infections from group A beta hemolytic Streptococcus (GABHS) is frequently found in children affected by OCD. This correlation would be common to different clinical pictures classified as PANDAS (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections)2.

The initial diagnostic difficulty, the clinical implications of the disorder, and the plurality of the mechanisms involved, justify a careful evaluation of children with OCD. Diagnosis remains a clinical procedure (Yale-Brown obsessive compulsive scale for children could be useful), because of, at the date, any laboratory test  can simplify the diagnosis.

The PANDAS is identified by five clinical criteria: presence of OCD and/or tic disorder, prepuberal onset, episodic course characterized by acute, severe onset and dramatic symptom exacerbations, neurological abnormalities (e.g., choreiform movements) present during exacerbations and temporal relation between GABHS infections and symptom exacerbations 2,3. The major distinctive feature of the PANDAS is the temporal association between neuropsychiatric symptom exacerbations and GABHS infections.

Recent evidence suggests that the pathogenesis of Sydenham's chorea following group A streptococcal infection is due to antibodies which develop due to the infection and infiltrate the brain and basal ganglia. These antibodies react with the surface of neuronal cells with an elevation of tyrosine hydroxylase and subsequent dopamine release which may lead to the movement disorder. Monoclonal antibodies from Sydenham's chorea demonstrated the mimicry between lysoganglioside and the group A streptococcal carbohydrate epitope. Reports have suggested an analogy between PANDAS and rheumatic fever immunopathogenesis: patient with D8/17 lymphocyte overexpression could have an higher risk of developing disorders after a streptococcal infection4.

We report a case of a 10-year-old girl who presented with tic and hemi-chorea associated with group A beta haemolytic Streptococcus (GABHS) throat infection. 

Case  Report

A 10-year-old  girl referred to us with a short history of left-sided facial weakness, facial tic and irregular left upper and lower limb movements, aggravated by exertion consistent with hemi-chorea. She didn’t recall any particular problem, apart from a history of sore throat 8 days before the onset of symptoms. A MR of the brain didn’t showed any particular picture. Anti- streptolysin O titres and anti- DNAse B levels were significantly elevated at 750 (N<200) and 500 (N >240) respectively. In throat culture developed a beta-haemolytic Group A streptococci. We started a treatment by oral penicillin and in this way we obtained an absolute resolution of her symptoms. Four weeks later she had a further episode of facial tics associated with a confirmed streptococcal throat infection. Another course of oral penicillin determined a complete resolution of neurological symptoms.

Discussion

A diagnosis of PANDAS was made in this girl as she fulfilled the diagnostic: presence of OCD and/or tic disorder, prepubertal onset, episodic course characterized by acute, severe onset and dramatic symptom exacerbations, neurological abnormalities (e.g., choreiform movements) present during exacerbations and temporal relation between GABHS infections and symptom exacerbations 2,3. The pathogenesis of PANDAS is thought to be due to the presence of anti-brain antibodies, and in particular, anti-basal ganglia antibodies in certain patients with GABHS infection.  A recent study has shown a significant difference in the presence of anti-neuronal antibodies of children with GABHS infection and PANDAS when compared with those who have GABHS infection, but no symptoms of PANDAS.  Patients with OCD, Tourette Syndrome (TS) or tic disorder are more likely than controls to have had prior streptococcal infection in the 3 months before onset date. The risk is higher among children with multiple streptococcal infections within 12 months. Having multiple infections with group A beta-haemolytic streptococcus within a 12-month period is associated with an increased risk for TS5.

The role of penicillin prophylaxis for PANDAS is doubtful6  and immunomodulatory therapy such as plasma exchange or intravenous immunoglobulin  have been criticised for having a high side effect profile and unproven efficacy7. Significant improvements in OCD and tics have been reported in PANDAS patients after tonsillectomy for recurrent tonsillitis8,9. The recognition that some paediatric neurobehavioral syndromes have infectious and/or immunologic triggers points to important new avenues of disease treatment10.

These findings lend epidemiologic evidence that PANDAS may arise as a result of a post infectious autoimmune phenomenon induced by childhood streptococcal infection.

Overall, studies performed in recent years underline the relative frequency and the short- and long-term effects of this disorder in children. If importance is to be ascribed to age-related features of the condition, the relevant studies now underline the developmental consistency of this disorder.

Future studies are needed to assessing the precise relationship between symptom exacerbations, infections, and immune parameters, possibly along with gene expression profiles.

 

References

 

1. Harris K, Singer HS. Tic disorders: neural circuits, neurochemistry, and neuroimmunology. (2006) J Child Neurol; 21(8):678-89.
2. Swedo S.E., Leonard H.L., Garvey M. et Al."Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases". (1998) Am. J. Psychiatry; 155:264-71
3. Snider LA, Swedo SE. PANDAS: current status and directions for research. (2004) Mol Psychiatry; 9: 900-907.
4. Murphy TK, Goodman WK, Fudge MW, et al. B lymphocyte antigen D8/17: A peripheral marker or childhood-onset obsessive-compulsive disorder and Tourette's syndrome. (1997) Am J Psych; 154: 125-34.
5. Mell L K, Davis RL, Owens D Association between streptococcal infection and obsessive-compulsive disorder, Tourette's syndrome and tic disorder. (2005 Jul)
Pediatrics.; 116(1):56-60)
6. Garvey MA, Perlmutter SJ, Allen AJ. A pilot study of penicillin prophylaxis for neuropsychiatric exacerbations triggered by streptococcal infections. (1999) Biol Psychiatry;45(12):1564-71
7. Perlmutter SJ et al. Therapeutic plasma exchange and intravenous immunoglobulin for obsessive compulsive disorder and tic disorders in childhood. (1999) Oct Lancet 2;354(9185):1153-8.
8. Orvidas LJ, Slattery MJ. Pediatric autoimmune neuropsychiatric disorders and streptococcal infections: role of otolaryngologist. (2001) Laryngoscope; 111: 1515-1519.
9. Heubi C, Shott SR. PANDAS: pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections - an uncommon, but important indication for tonsillectomy. (2003) Int J Pediatr Otorhinolaryngol; 67: 837-840.
10. Pavone P, Parano E, Rizzo R, Trifiletti RR Autoimmune neuropsychiatric disorders associated with streptococcal infection: sydenham chorea, pandas, and pandas variant (2006) J Child Neurol; 21(9):727-36.
First Published September 2006

Corresponding author: Angela MazzolaEmail: ang.mazzola@libero.it

Address: Via Cimbali n.41 Palermo, Italy


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