Research

Diagnostics, Differential Diagnostics And The Treatment Of Pandas Syndrome – Case Description Mukhtorjonova Khusnigul Nodirbekovna, Tukhtabayeva Kamila Abrarovna, Tashkent Padiatric Medical Institute. World Bulletin of Public Health (WBPH). Volume-6, January 2022. ISSN: 2749-3644

This article gives a case of PANDAS syndrome in an 11-year-old boy. The
course and clinic of the disease in the form of prolonged sneezing attacks
made it difficult to diagnose this syndrome. Conducting biochemical blood
tests, daily video electroencephalographic monitoring (EEG monitoring) and
magnetic resonance imaging of the brain (MRI) allowed to determine the
condition. The appointment of antibiotic therapy and intravenous
administration of immunoglobulin led to remission of the disease. It is
necessary to conduct further studies to determine the effectiveness of the
above drugs for this syndrome.

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McHugh A, Chan A, Herrera C, Park JM, Balboni I, Gerstbacher D, Hsu JJ, Lee T, Thienemann M, Frankovich J. Profiling Behavioral and Psychological Symptoms in Children undergoing treatment for Spondyloarthritis and Polyarthritis. J Rheumatol. 2022 Feb 1:jrheum.210489. doi: 10.3899/jrheum.210489. Epub ahead of print. PMID: 35105715.

Results: There were 111 patients and 1753 healthy controls (HCs). Compared to HCs, patients with SpA or PolyA had worse total competence and internalizing scores. Higher cJADAS10 scores were associated with worse total competence, worse internalizing, and higher total problems scores. Most of these differences reached statistical significance (P < 0.01). Self-harm/suicidality was almost 4-fold higher in patients with PolyA than HCs (OR 3.6, 95% CI 1.3-9.6, P = 0.011).

Conclusion: Our study shows that patients with SpA and PolyA with more active disease have worse psychological functioning in activities, school, and social arenas, and more internalized emotional disturbances, suggesting the need for regular mental health screening by rheumatologists.

M. Della Corte, C. Delehaye, E. Savastano, M.F. De Leva, P. Bernardo, A. Varone, Neuropsychiatric syndrome with myoclonus after SARS-CoV-2 infection in a paediatric patient, Clinical Neurology and Neurosurgery, Volume 213, 2022. DOI: 10.1016/j.clineuro.2022.107121

• 12 Year old subacutely developed a positive and negative myoclonus of limbs and face, drowsiness and memory deficits after getting infected by SARS-CoV-2.
• Neuropsychological examination showed an impairment in memory, visual-motor coordination, inductive reasoning skills, attention, and concentration
• First treated with clonazepam and then with intravenous methylprednisolone for five days, with poor response.
  • Then received a cycle of IVIG, thus reaching a gradual and complete recovery.
• To date, this is the first case of a COVID-19 associated myoclonus affecting a paediatric patient.

Letter to the Edito. Antistreptolysin-O Titers: Implications for Adult PANDAS. ANDREW J. CHURCH, B.SC., and RUSSELL C. DALE, M.B.CH.B., M.R.C.P., London, U.K. Published Online:1 Feb 2002. https://doi.org/10.1176/appi.ajp.159.2.320

To the Editor: A diagnosis of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is made when neuropsychiatric disease is precipitated by streptococcal infection (1). Antistreptolysin-O titers are an important tool for diagnosing recent streptococcal infection in patients with Sydenham’s chorea and PANDAS, since throat cultures are usually negative because of the latent onset of the neuropsychiatric disease. The upper limit for normal antistreptolysin-O titers is 200 IU/ml in children, but no normal upper limit exists for healthy adults. We propose that an antistreptolysin-O titer of 270 IU/ml is the significant upper limit for healthy adults. This finding will aid in the investigation and diagnosis of new cases of adult PANDAS.

Kumaraswamy MS, Cornelius LP, Shanthi B. PANDAS: Paediatric Autoimmune Neuropsychiatric Disorder Associated with Group A Streptococci and its Intricacy with Rheumatic Heart Disease – Case Report. Postgrad J Pediatr Adol Med. 2022;1(2):30-32. Link

“Here, we discuss about a 9-year-old boy who presented with choreoathetoid movements and mitral valve involvement. He was treated for neuropsychiatric as well as cardiac complications following streptococcal infection under the suspicion of RHD. Due to poor compliance, the child had relapses of involuntary movements in the intermittent period which is consistent with the criteria of PANDAS. Hence high index of suspicion should be considered for all the children with features of insignificant valvular abnormalities in endemic populations during their subsequent follow-up for signs/symptoms of PANDAS.”

Endres, D., Pollak, T.A., Bechter, K. et al. Immunological causes of obsessive-compulsive disorder: is it time for the concept of an “autoimmune OCD” subtype?. Transl Psychiatry 12, 5 (2022). https://doi.org/10.1038/s41398-021-01700-4

Pathophysiologically, the following subtypes should currently be distinguished:

1. 1. OCD with PANDAS/PANS,
2. 2. OCD with neuronal antibodies: a. against well-characterized cell surface antigens (such as NMDA-R), b. against well-characterized paraneoplastic, intracellular antigens (such as Ma2), and c. against non-well-characterized and novel neuronal autoantibodies,
3. 3. OCD in the context of systemic autoimmune diseases with potential brain involvement (such as systematic lupus erythematosus),
4. 4. OCD in the context of established autoimmune CNS disorders (such as multiple sclerosis).

LaRusso, M. & Abadía-Barrero, C. (2021). “These doctors don’t believe in PANS”: Confronting Uncertainty and a Collapsing Model of Medical Care. Chapter 9, pp. 197-216. In Montesi, Laura and Calestani, Melania (Eds.) Managing Chronicity in Unequal States. Ethnographic perspectives on caring. University College of London Press, ISBN. 9781800080287.

“This chapter presents ethnographic research in the United States with families with children affected by PANS, a relatively new condition that is defying the disciplinary borders between infectious diseases, environmental causes of immune dysregulation, neurological problems and developmental psychopathology in paediatrics care. PANS opens a window to examine critically why the hegemony of a biomedical model of care structured around sub- specialties is collapsing. In facing the uncertainty of the new condition, families’ approach to care emphasises the need for comprehensiveness and immediacy; however, biomedicine seems to be ill- equipped to meet those needs. Rather than offering support and facilitating a path to recovery, clinicians often challenge the knowledge of families, negate PANS as a viable diagnosis, and delay adequate care, which results in increased harm to both the child and the family. Furthermore, the chapter shows how families incur significant debt by trying several therapeutic options that are not covered by insurance. This signals how the inadequacy of the social welfare and broade rsafety nets in the United States further magnify children’s and parents’ suffering.”

J Huang, R Li, L Li, Y Song, L Jin. The relationship between allergic diseases and tic disorders: A systematic review and meta-analysis. Neuroscience & Biobehavioral Reviews, Vol 132, 2022, DOI: 10.1016/j.neubiorev.2021.12.004.

• Asthma, allergic rhinitis and allergic conjunctivitis are associated with tic disorders.
• Among subtypes of tic disorders, patients with provisional tic disorder would be more likely to have allergic disease.
• Shared genetic and etiological factors might account for the association between tic disorders and allergic illness.

V Nezgovorova, CJ Ferretti, S Pallanti, E Hollander. Modulating neuroinflammation in COVID-19 patients with obsessive-compulsive disorder. Journal of Psychiatric Research, 2021, DOI: 10.1016/j.jpsychires.2021.11.025.

Abstract: Exacerbation of symptoms of obsessive-compulsive disorder (OCD) during COVID-19 or new onset of the OCD symptoms resulting from COVID-19 infection is an understudied area of research. It is possible that increased proinflammatory immune status is associated with the onset of obsessive-compulsive symptoms in patients with COVID-19 and that targeted anti-inflammatory treatments for COVID-19 infection can mitigate the new onset of Obsessive-Compulsive (OC) spectrum symptoms. In this review, we cover OCD pathogenesis as related to COVID-19, summarize the impact of cytokines on behavior, and suggest that anti-cytokine treatments can help mitigate post-COVID-19 and new onset of the OC symptoms.

Sabine Hazan, MD, Sheldon Jordan, MD. SARS-CoV-2 as a Trigger in the Development of Tourette’s-Like Symptoms: A Case Report, 15 November 2021, PREPRINT (Version 1) available at Research Square DOI: 10.21203/rs.3.rs-1079406/v1  This case study has not been peer-reviewed.

• Case report describe Tourette’s-like symptoms resulting from SARS-CoV-2 disrupting gut microbiota
• SARS-CoV-2 could cause significant havoc in the enteric flora, damaging intestinal barrier, disrupting communication along the GMBA, imparing immune function, leading to neuropsychiatric symptoms
• Several possible therapeutic approaches change the microbiome including prebiotics, postbiotics, synbiotics, and fecal transplantation
• Using these therapies to modulate bacterial composition and enhance microbiota diversity is a pragmatic approach for enhanced protection against the acute morbidities associated with viral illnesses including COVID-19.

Tang, A. W., Appel, H. J., Bennett, S. C., Forsyth, L. H., Glasser, S. K., Jarka, M. A., Kory, P. D., Malik, A. N., Martonoffy, A. I., Wahlin, L. K., Williams, T. T., Woodin, N. A., Woodin, L. C., Miller, I. K. T., & Miller, L. G. (2021). Treatment barriers in PANS/PANDAS: Observations from eleven health care provider families. Families, Systems, & Health. Advance online publication. DOI: 10.1037/fsh0000602

Despite published diagnostic and treatment guidelines for this condition, there are long delays in obtaining appropriate care. We sought to identify health care system barriers to timely treatment by examining cases of PANDAS/PANS occurring in children of health care professionals.

• Parents included 11 physicians, 2 mental health professionals, 2 nurses, and a PharmD.
  Nine cases (82%) had “very delayed” diagnosis and treatment (>4 weeks after onset).
• The most commonly encountered causes for treatment delay were clinician lack of awareness (82%), clinician skepticism (82%), overdependence on diagnostic testing (91%), and out-of-pocket expenses >$100 US (82%).
• Other common challenges included difficulties finding a provider to spearhead care (64%), psychological misdiagnosis (55%), and children’s suppression of behaviors during assessments (55%).