Diagnostics, Differential Diagnostics And The Treatment Of Pandas Syndrome – Case Description

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.

Read case study.

Status Of Vitamin D In Children With Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS)
McNally JD, Menon K, Chakraborty P, Fisher L, Williams KA, Al-Dirbashi OY, Doherty DR; Canadian Critical Care Trials Group. The association of vitamin D status with pediatric critical illness. Pediatrics. 2012 Sep;130(3):429-36. doi: 10.1542/peds.2011-3059. Epub 2012 Aug 6. PMID: 22869837.
“Numerous studies have indicated an association between vitamin D deficiency and the immune dysregulation and the pathogenesis of autoimmunity. An increased prevalence of vitamin D deficiency has been demonstrated in several pediatric autoimmune diseases, including autoimmune thyroid disease and Type-1 diabetes mellitus. This study was undertaken to determine the prevalence of vitamin D deficiency in children with PANS, an autoimmune disorder characterized by abrupt-onset neuropsychiatric symptoms.”
Neuropsychiatric syndrome with myoclonus after SARS-CoV-2 infection in a paediatric patient

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.
Immunological causes of obsessive-compulsive disorder: is it time for the concept of an “autoimmune OCD” subtype?
Endres D, Pollak TA, Bechter K, Denzel D, Pitsch K, Nickel K, Runge K, Pankratz B, Klatzmann D, Tamouza R, Mallet L, Leboyer M, Prüss H, Voderholzer U, Cunningham JL; ECNP Network Immuno-NeuroPsychiatry, Domschke K, Tebartz van Elst L, Schiele MA. Immunological causes of obsessive-compulsive disorder: is it time for the concept of an “autoimmune OCD” subtype? Transl Psychiatry. 2022 Jan 10;12(1):5. doi: 10.1038/s41398-021-01700-4. PMID: 35013105; PMCID: PMC8744027.
  • There is increasing evidence for secondary immune-mediated forms of OCD.
  • The DSM-5 and novel ICD-11 criteria include the category of secondary OCD, without, however, providing guidelines according to which such a diagnosis should be established.
  • The authors have drafted a first proposal of clinical criteria for the definition of secondary autoimmune OCD.
  • Recognizing the autoimmune causes of OCD could inform additional therapeutic options for the affected patients to promote treatment response and reduce chronicity.”
‘These doctors don’t believe in PANS’: Confronting uncertainty and a collapsing model of medical care

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.”

The relationship between allergic diseases and tic disorders: A systematic review and meta-analysis

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.
Modulating neuroinflammation in COVID-19 patients with obsessive-compulsive disorder

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.

 

 

SARS-CoV-2 as a Trigger in the Development of Tourette’s-Like Symptoms: A Case Report

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.

 

Treatment barriers in PANS/PANDAS: Observations from eleven health care provider families

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%).
Mycoplasma pneumoniae IgG positivity is associated with tic severity in chronic tic disorders

J Schnell, M Bond, N Moll, E Weidinger, B Burger, R Bond, A Dietrich, PJ. Hoekstra, A Schrag, D Martino, M Schwarz, U-X Meier, N Müller. Brain, Behavior, and Immunity. Vol 99, 2022. DOI: 10.1016/j.bbi.2021.10.012

  • M. pneumoniae IgG positivity was not associated with the presence of CTD.
  • M. pneumoniae IgG positivity was not associated with the first onset of tics.
  • M. pneumoniae IgG positivity was significantly associated with higher tic severity.

“It is possible that M. pneumoniae infection influences tic severity in CTD or, that having more severe tics, increases the risk of infection. However, it is more likely that the association observed in this study reflects a propensity toward enhanced immune responses in people with CTD and that, rather than a causal relationship, infection and greater tic severity are indirectly linked via shared underlying immune mechanisms.”

Clinical Manifestations of Patients with PANDAS in Patients Followed Up at Srebrnjak Children’s Hospital in 5-Year Period

Sizgoric MK, Kovac Sizgoric M, Miculinic A, et al. Clinical manifestations of patients with PANDAS in patients followed up at Srebrnjak children’s hospital in 5-year period. Archives of Disease in Childhood 2021;106:A169. Article

Clinical:
– 20 children, 16 (80%) male, 4 (20%) female, aged 3 to 11 years (median age 6.5)
-Elevated levels of antistreptolysin titers 19 of 20 (95%). Anti-DNAse-B levels were tested in 7 patients and was elevated in 4 (57%)
-Neuroimaging studies (N=12 or 60%) – no specific findings found
-Family history of tics and autoimmune disorders was positive in N=5
(25%)

Symptoms:
-All patients had OCD and tics with acute onset of symptoms
-Attention disorders N=17 (85%)
-Sleeping difficulties N=4 (20%)
-Speech difficulties N=5 (25%)
-Decline in school performance N=10 (71%)

Treatment:
-15 (75%) patients given first-line antibiotics. In 5 (25%) patients a full regression of symptoms was registered, 10 (50%) had partial improvement, 5 (25%) had no response.
-7 (35%) were given IVIG after initial antibiotic treatment with incomplete regression. Tics persisted in 11 patients and 4 had tics with behavioral difficulties. In 7 patients treated with IVIG we proceeded with parenteral antibiotics because of partial symptom regression and 2 were given corticosteroids (still followed).

Plasmapheresis, Rituximab, and Ceftriaxone Provided Lasting Improvement for a 27-Year-Old Adult Male with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS)

A Krouse, H Li, JA Krenzer, WN Rose. Plasmapheresis, Rituximab, and Ceftriaxone Provided Lasting Improvement for a 27-Year-Old Adult Male with PANDAS. Dept of Pathology, University of Wisconsin Hospital. Article

  • At 17 – developed sudden psychosis and confusion after GAS infection. Had elevated anti-streptolysin O (ASO) titers. Did not present with typical symptoms of PANDAS – OCD and/or tic-like symptoms were not observed. A tentative diagnosis of PANDAS was given. Treated with antipsychotics, antibiotics, tonsillectomy, and IVIG which resulted in remissions and relapses of his neuropsychiatric symptoms.
  • At age 27 – received a trial of therapeutic plasma exchange (TPE), rituximab, and ceftriaxone. Eventually resulted in sustained benefit and minimal fluctuations of his clinical symptoms.