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The PANDAS Subgroup: Recognition and Treatment

Published online by Cambridge University Press:  07 November 2014

Susan E. Swedo ,
Marjorie Garvey ,
Lisa Snider ,
Charlotte Hamilton  and
Henrietta L. Leonard
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Abstract

A subgroup of patients with childhood-onset obsessive-compulsive disorder (OCD) has been identified who share a common clinical course characterized by dramatic symptom exacerbations following Group A beta-hemolytic streptococcal (GABHS) infections. The term PANDAS has been applied to the subgroup, to indicate the postulated etiology of their symptoms: Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. Five clinical characteristics define the PANDAS subgroup: presence of OCD and/or tic disorder, prepubertal symptom onset, sudden onset or abrupt exacerbations (sawtooth course), association with neurological abnormalities (presence of adventitious movements or motoric hyperactivity during exacerbations), and temporal association between symptom exacerbations and GABHS infections. Post-streptococcal symptom exacerbations are typically quite dramatic, with patients reporting that their symptoms “…came on overnight” or “…appeared all of a sudden a few days after I had a sore throat.” The post-streptococcal inflammatory nature of the neuropsychiatric symptoms provides novel opportunities for treatment and prevention, including immunomodulatory therapies such as therapeutic plasma exchange (TPE) and intravenous immunoglobulin (IVIG). A recently completed placebo-controlled trial revealed that both IVIG and TPE were effective in reducing neuropsychiatric symptom severity (40% to 55% reductions, respectively) for a group of severely ill children with OCD and/or tic disorders. Further research is required to determine why the treatments are helpful, as well as to ascertain whether or not antibiotic prophylaxis can help prevent post-streptococcal symptom exacerbations.

Type
Feature Article
Information
CNS Spectrums , Volume 6 , Issue 5: Neuroimmunology , May 2001 , pp. 419 - 426
Copyright
Copyright © Cambridge University Press 2001

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References

REFERENCES

1.Swedo, SE. Sydenham's chorea: a model for childhood autoimmune neuropsychiatric disorders. JAMA. 1994;272(22):1788–1791.CrossRefGoogle Scholar
2.Swedo, SE, Rapoport, JL, Cheslow, DL, et al.High prevalence of obsessive-compulsive symptoms in patients with sydenham's chorea. Am J Psychiatry. 1989;46:335–341.Google Scholar
3.Swedo, SE, Leonard, HL, Schapiro, MB, et al.Sydenham's chorea: physical and psychological symptoms of st. vitus' dance. Pediatrics. 1993;91:706–713.Google ScholarPubMed
4.Husby, G, van de Rijn, I, Zabriskie, JB, et al.Antibodies reacting with cytoplasm of subthalamic and caudate nuclei neurons in chorea and acute rheumatic fever. J Exp Med. 1976;144:1094–1110.CrossRefGoogle ScholarPubMed
5.Bronze, MS, Dale, JB. Re-emergence of serious group a streptococcal infections and acute rheumatic fever. Am J Med Sci. 1996; 311:41–54.CrossRefGoogle Scholar
6.Garvey, MA, Swedo, SE. Sydenham's chorea: clinical and therapeutic update. Adv Exp Med Biol. 1997;418:115–120.CrossRefGoogle ScholarPubMed
7.Swedo, SE, Leonard, HL, Kiessling, LS. Speculations on antineuronal antibody-mediated neuropsychiatric disorders of childhood. Pediatrics. 1994; 93:323–326.CrossRefGoogle ScholarPubMed
8.Swedo, SE, Rapoport, JL, Leonard, HL, et al.Obsessive-compulsive disorder in children and adolescents: clinical phenomenology of 70 consecutive cases. Arch Gen Psychiatry. 1989;46:335–341.CrossRefGoogle ScholarPubMed
9.Allen, AJ, Leonard, HL, Swedo, SE. Case study: a new infection-triggered, autoimmune subgroup of pediatric OCD and tourette's syndrome. J Am Acad Child Adolesc Psychiatry. 1995;34(3):307–311.CrossRefGoogle Scholar
10.Swedo, SE, Leonard, HL, Garvey, M, et al.Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS): a clinical description of the first fifty cases. Am J Psychiatry. 1998;155:264–271.CrossRefGoogle Scholar
11.Perlmutter, SJ, Garvey, MA, Castellanos, X, et al.A case of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. Am J Psychiatry. 1998;155(11):1592–1598.CrossRefGoogle ScholarPubMed
12.Kaplan, EL. Recent epidemiology of group a streptococcal infections in North America and abroad: an overview. Pediatrics. 1996;97:945–948.CrossRefGoogle ScholarPubMed
13.Wannamaker, L. Perplexity and precision in the diagnosis of streptococcal pharyngitis. Am J Dis Child. 1972;124:352–358.Google ScholarPubMed
14.Veasy, LG. Rheumatic fever: t. duckett jones and the rest of the story. Cardiol Young. 1995;5:293–301.CrossRefGoogle Scholar
15.Asbahr, FR, Ramos, RT, Negrao, AB, et al.Case series: increased vulnerability to obsessive-compulsive symptoms with repeated episodes of sydenham chorea. J Am Acad Child Adolesc Psychiatry. 1999;38(12):1522–1525.CrossRefGoogle ScholarPubMed
16.Leonard, HL, Swedo, SE, Lenane, MC, et al.A 2- to 7-year follow-up study of 54 obsessive-compulsive children and adolescents. Arch Gen Psychiatry. 1993;50:429–439.CrossRefGoogle ScholarPubMed
17.Leonard, HL, Lenane, MC, Swedo, SE, et al.Tics and tourette's syndrome: a 2- to 7- year follow-up of 54 obsessive-compulsive children. Am J Psychiatry. 1992;149:1244–1251.Google ScholarPubMed
18.Luxenberg, JS, Swedo, SE, Flament, M, et al.Neuroanatomic abnormalities in obsessive-compulsive disorder detected with quantitative x-ray computed tomography. Am J Psychiatry. 1988;145(9):1089–1094.Google ScholarPubMed
19.Rapoport, JL, Swedo, SE. Obsessive-compulsive disorder. In: Rutter, M, Taylor, E (eds): Child and Adolescent Psychiatry Modern Approaches, 4th ed. In press.Google Scholar
20.Giedd, JN, Rapoport, JL, Leonard, HL, et al.Case study: acute basal ganglia enlargement and obsessive-compulsive symptoms in an adolescent boy. J Am Acad Child Adolesc Psychiatry. 1996;35(7):913–915.CrossRefGoogle Scholar
21.Giedd, JN, Rapoport, JL, Garvey, MA, et al.MRI assessment of children with obsessive-compulsive disorder or tics associated with streptococcal infection. Am J Psychiatry. 2000;157(2):281–283.CrossRefGoogle ScholarPubMed
22.Garvey, MA, Perlmutter, SJ, Allen, AJ, et al.A pilot study of penicillin prophylaxis for neuropsychiatric exacerbations triggered by streptococcal infections. Biol Psychiatry. 1999;45:1564–1571.CrossRefGoogle ScholarPubMed
23.Garvey, MA, Swedo, SE, Shapiro, MB, et al.Intravenous immunoglobulin and plasmapheresis as effective treatments of sydenham's chorea. Neurology 1996;46:A147.Google Scholar
24.Barron, KS, Sher, MR, Silverman, ED. Intravenous immunoglobulin therapy: magic or black magic. J Rheumatol. 1992;19:94–97.Google Scholar
25.Plasmapheresis and acute Guillain-Barre syndrome. The Guillain-Barre syndrome study group. Neurology 1985;35:1096–1104.CrossRefGoogle Scholar
26.Tucker, DM, Leckman, JF, Scahill, L, et al.A putative poststreptococcal case of OCD with chronic tic disorder, not otherwise specified. J Am Acad Child Adolesc Psychiatry. 1996;35(12): 1684–1691.CrossRefGoogle Scholar
27.Jonasson, G, Wilkinson, SR. Prednisolone-induced obsessive-compulsive behavior in a child. Tidsskr Nor Laegeforen. 1993;113:3162–3166.Google ScholarPubMed
28.Perlmutter, SJ, Leitman, SF, Garvey, MA, et al.Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood. Lancet. 1999;354:1153–1158.CrossRefGoogle ScholarPubMed
29.Leonard, HL, Swedo, SE, Lenane, MC, et al.A 2- to 7-year follow-up study of 54 obsessive-compulsive children and adolescents. Arch Gen Psychiatry. 1993;50(6):429–439.CrossRefGoogle ScholarPubMed
30.Nicolson, R, Swedo, SE, Lenane, M, et al.An open trial of plasma exchange in childhood-onset obsessive-compulsive disorder without post-streptococcal exacerbations. J Am Acad Child Adol Psychiatry. 2000;39:1313–1315.CrossRefGoogle Scholar
31.Kiessling, LS, Marcotte, AC, Culpepper, L. Antineuronal antibodies in movement disorders. Pediatrics. 1993;92:39–43.CrossRefGoogle ScholarPubMed
32.Singer, HS, Giuliano, JD, Hansen, BH, et al.Antibodies against human putamen in children with tourette syndrome. Neurology. 1998;50(6):1618–1624.sπCrossRefGoogle ScholarPubMed