ber of studies have looked at the validity of this test , including a recent one by Hesselmark and Bejero . All Swedish patients who had the Cunningham panel drawn prior to 2014 were eligible to participate . Of the 154 eligible patients , 53 chose to participate . The prior results were collected , along with new blood samples . As control , the panel was drawn on 21 healthy subjects . Compared to a clinical diagnosis of PANS , the panel had a sensitivity of 15-60 %, a specificity of 28-92 %, and a positive predictive value of 17-40 %. The majority of healthy controls had a pathological Cunningham Panel . 9 Therefore , the significance of such testing is uncertain .
Treatment
A number of case reports have evaluated antibiotics as treatment for PANS / PANDAS , showing an overall effectiveness of 8-52 %. A prospective study by Garvey and Swedo does not support antibiotic / antimicrobial prophylactic treatment . 10 Plasma exchange ( PLEX ) has been reported to have mixed results , ranging from dramatic improvement in symptoms to , more commonly , the treatment being ineffective . Similarly , there are some case reports of successful treatment with corticosteroids , but the majority of the evidence is inconclusive . There are numerous studies evaluating intravenous immunoglobulin ( IVIG ) use for PANS / PANDAS patients . Hoekstra et al ,
Dr . Swedo stressed that the episodic course of this syndrome was characterized by an abrupt onset or by dramatic symptom exacerbations , with the symptoms diminishing significantly or resolving completely within 6-8 weeks of onset or exacerbation . 3 studying the efficacy of IVIG compared to placebo in a group of PANDAS patients , reported no significant symptomatic improvement in the group treated with IVIG . 11 Another study collated a series of six case reports reviewing 19 different patients with a diagnosis of either PANS , PITAND , or PANDAS . Eleven of the 19 patients were reported to have full remission of symptoms following IVIG . 3 A randomized , double-blind , placebo-controlled study of IVIG use in patients with PANS by Williams et al showed no significant improvement in the IVIG treated patients following a six-week trial of IVIG . 12
Conclusion
In conclusion , PANS may be a definite entity ; however , such a diagnosis should be made with caution and only for those patients who meet the clinical criteria detailed in Table 2 . At present , there is no convincing evidence of a serologic marker for this diagnosis . Some patients may benefit from treatment with IVIG , steroid , PLEX , or a combination thereof ; however , exact specifics regarding dose and duration of treatment , as well as efficacy of such treatment , remains largely anecdotal and not conclusive . Therefore , each case should be considered on an individual basis and treatment should be tailored accordingly .
References
1 . Dr . Susan E . Swedo ’ s bio . Available online at https :// neuroscience . nih . gov / Faculty / Profile / susan-swedo . aspx
2 . Swedo SE , Leonard HL , Garvey M , Mittleman B , Allen AJ , Perlmutter S , et al . Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections : clinical description of the first 50 cases . Am J Psychiatry 1998 ; 155 ( 2 ): 264-71 .
3 . Sigra S , Hesselmark E , Bejerot S . Treatment of PANDAS and PANS : a systemic review . Neurosci Biobehav Rev . 2018 ; 86:51-65 .
4 . Kawikova I , Leckman JF , Kronig H , Katsovich L , Bessen DE , Ghebremichael M , et al . Decreased Numbers of Regulatory T Cells Suggests Impaired Immune Tolerance in Children with Tourette Syndrome : A Preliminary Study . Biol Psychiatry 2007 ; 61 ( 3 ): 273-8 .
5 . Martin J . The Streptococcus pyogenes Carrier State . 2016 Jul 25 . In : Ferretti JJ , Stevens DL , Fischetti VA , editors . Streptococcus pyogenes : Basic Biology to Clinical Manifestations [ Internet ]. Oklahoma City ( OK ): University of Oklahoma Health Sciences Center ; 2016- . Available from : https :// www . ncbi . nlm . nih . gov / books / NBK374206 /
6 . Kurlan R , Johnson D , Kaplan EL ; Tourette Syndrome Study Group . Streptococcal infection and exacerbations of childhood tics and obsessive-compulsive symptoms : a prospective blinded cohort study . Pediatrics 2008 ; 121 ( 6 ): 1188-1197 .
7 . Shulman ST . Pediatric autoimmune neuropsychiatric disorders associated with streptococci ( PANDAS ). Curr Opin Ped . 2009 ; 21:127 – 130 .
8 . Swedo SE , Leckman JF , Rose NR . From Research Subgroup to Clinical Syndrome : Modifying the PANDAS Criteria to Describe PANS ( Pediatric Acute-onset Neuropsychiatric Syndrome ). Pediatr Therapeut 2012 , 2:2 .
9 . Hesselmark E , Bejerot S . Biomarkers for diagnosis of Pediatric Acute Neruopsychiatric Syndrome ( PANS ) – Sensitivity and specificity of the Cunningham Panel J Neuroimmunol . 2017 Nov 15 ; 312:31- 37 .
10 . Garvey MA , Perlmutter SJ , Allen AJ , Hamburger S , Lougee L , Leonard HL , et al . A pilot study of penicillin prophylaxis for neuropsychiatric exacerbations triggered by streptococcal infections . Biol Psychiatry . 1999 , 45:1564-1571 .
11 . Hoekstra PJ , Minderaa RB , Kallenberg CGM . Lack of effect of intravenous immunoglobulin on tics : a double-blind placebo-controlled study . J Clin Psychiatry 2004 ; 65 : 537 – 542 .
12 . Williams KA , Swedo SE , Farmer CA , Grantz H , Grant PJ , D ’ Souza P , et al . Randomized , controlled trial of intravenous immunoglobulin for pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections . J Am Acad Child Adolesc Psychiatry . 2016 Oct ; 55 ( 10 ): 860-867 .
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