Figure 2- Optic nerve head (ONH) and retinal nerve fiber layer (RNFL) - signs of papilledema
As the result, high dose methylprednisolone for 3 days and acetazolamide were started for the patient. Due to loss of vision, high opening pressure in LP, eye problems, papilledema, and ataxia treatment with high-dose methylprednisolone 30 mg/kg three times a day and Intravenous Immunoglobulin (IVIG) IV stat in 10-12 hours (2gr/kg), ciprofloxacin eye drop, ceftriaxone 1gr Intravenous (IV) per 12 hours, intravenous acetaminophen (Apotel) 650 mg IV stat, were started for the patient. After these chain of therapies 10 days after the admission, the headache and ataxia of the patient were disappeared, the vision became better than before and the ophthalmology reports were shown no signs of papilledema. He was discharged with the treatment of prednisolone 15mg four times a day for three days, acetazolamide 250mg, and betamethasone, and ciprofloxacin. At the day of discharge ESR and CRP were reduced but the count of WBC was elevated.
Discussion and Conclusions:
The prevalence of neurologic symptoms in MIS-C is not completely clear in the past articles, but it may be about 20% in complicated cases (5, 6). MIS-C has some neurological manifestations like encephalopathy, reduced reflexes, muscle weakness, and headaches; (7). In a study showing neuroimaging presentations in children, encephalopathy with SARS-CoV-2, acute encephalomyelitis-like changes of the brain, and myelitis were also the common presentations. Corpus callosum reversible changes was observed in MIS-C patients in some cases, but cerebrovascular disorders in children were less than adults (8). Ataxia is not common in the presentation of MIS-C. Even though acute ataxia is commonly presented in children, it is rare in SARS-COV2 patients. This rare happening can be related to children’s relative resistance to the severe types of COVID-19. Cerebellar ataxia is common in children 2-5. 9 years old as an uncommon age range for COVID-19 infection. It might take place because of immunosuppression or chronic diseases conditions (9). Further investigations are needed to investigate the presence of ataxia in MIS-C children after COVID-19.
As far as we know, we reported the first case of ataxia in a MIS-C patient with the presentation of other rare manifestations like papilledema. Akçay et al. reported a rare case of cerebellitis in a MIS-C patient. The signs of nystagmus, ataxia, and dysarthria were observed in the child. But the patient wasn’t complicated with the signs of eye problems (10).
Due to ataxia and headache in our case, the patient underwent brain MRI and CSF assessment by LP. Papilledema were observed in MRI of our patient as a rare presentation of MIS-C. LP was abnormal in about one fourth of the past studies’ investigations (11). CSF assessment revealed high opening pressure (40 cmH2O) and elevation of the pressure of CSF (30 cmH2O). The etiology of this high pressure in MIS-C patients is not still completely clear (12). Additionally, high opening pressure caused by immunomodulatory treatment earlier than LP could be mistaken as a result of MIS-C instead of aseptic meningitis (13). But because of starting pulse therapy with methylprednisolone after LP, one of the reasons for high pressure could be because of the presence of MIS-C. In our patient, abnormal circulation of CSF might lead to papilledema and ataxia, simultaneously.
The results of kidney sonography revealed marked parenchymal thinning and cortical damage. The level of ferritin was 506.2 at the fourth day of admission. High level of ferritin is significantly associated with acute kidney injury. As an inflammatory marker it is also observed in hepatitis. The levels of ALT and AST were significantly high at the fifth day of admission. Due to statistical analysis, the prevalence of hepatitis and acute kidney injury were 49% and 25%, respectively (14).
In spite of reduction of neutrophil, ESR and CRP after the treatment, the WBC count increased significantly. It might be because of the role of methylprednisolone as a glucocorticoid in the elevation of WBC count after the treatment (15).
Due to rare presence of ataxia and papilledema in MIS-C after COVID-19, as far as we know, we presented the first study of this presentation. Neurologic symptoms, especially Pseudotumor cerebri, should be kept in mind in MIS-C patients, and for prevention of vision loss even in mild cases of MIS-C treatment also should be started immediately.
Declarations
Ethics approval and consent to participate
The authors confirm that they have obtained all proper patient consent forms. The patient has consented for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed. Also, a signed and consent form was received from the patient in Persian. All methods were performed in accordance with the ethical standards in the Declaration of Helsinki.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and accompanying images
Availability of data and materials
All data an materials of the study are available by corresponding author
Competing interests
The authors declare that they have no competing interests
Funding:
No funding was received for this work
Authors’ contributions
ShN and RN saw the case for the first time and realized its specialness and completed its information with the cooperation of LJ. RM and HG did the work of writing and reviewing the literature and collecting information, also ShN managed all the steps as the professor and supervisor of team. All authors read and approved the final manuscript.
Acknowledgment:
The authors would like to thank all the persons and staff help us with this report.
References:
1. CDC Health Alert Network. Multisystem Inflammatory Syndrome in Children
(MIS-C) Associated With Coronavirus Disease 2019 (COVID-19). Available
online at: https://emergency.cdc.gov/han/2020/han00432.asp (accessed April
27, 2022)
2. Radia T, Williams N, Agrawal P, Harman K, Weale J, Cook J, et al. Multi-system inflammatory syndrome in children & adolescents (MIS-C): A systematic review of clinical features and presentation. Paediatric respiratory reviews. 2021;38:51-7.
3. Tomar LR, Shah DJ, Agarwal U, Batra A, Anand I. Acute Post-Infectious Cerebellar Ataxia Due to COVID-19. Mov Disord Clin Pract. 2021;8(4):610-2.
4. Becker AE, Chiotos K, McGuire JL, Bruins BB, Alcamo AM. Intracranial Hypertension in Multisystem Inflammatory Syndrome in Children. J Pediatr. 2021;233:263-7.
5. LaRovere KL, Riggs BJ, Poussaint TY, Young CC, Newhams MM, Maamari M, et al. Neurologic involvement in children and adolescents hospitalized in the United States for COVID-19 or multisystem inflammatory syndrome. JAMA neurology. 2021;78(5):536-47.
6. Son MBF, Friedman K. COVID-19: Multisystem inflammatory syndrome in children (MIS-C) clinical features, evaluation, and diagnosis. upToDate. Waltham.(Accessed on August 19, 2021); 2021.
7. Olivotto S, Basso E, Lavatelli R, Previtali R, Parenti L, Fiori L, et al. Acute encephalitis in pediatric multisystem inflammatory syndrome associated with COVID-19. Eur J Paediatr Neurol. 2021;34:84-90.
8. Lindan CE, Mankad K, Ram D, Kociolek LK, Silvera VM, Boddaert N, et al. Neuroimaging manifestations in children with SARS-CoV-2 infection: a multinational, multicentre collaborative study. The Lancet Child & Adolescent Health. 2021;5(3):167-77.
9. O’Neill KA, Polavarapu A. Acute Cerebellar Ataxia Associated with COVID-19 Infection in a 5-Year-Old Boy. Child Neurology Open. 2021;8:2329048X211066755.
10. Akçay N, Oğur M, Emin Menentoğlu M, İrem Sofuoğlu A, Boydağ Güvenç K, Bakirtaş Palabiyik F, et al. Acute Cerebellitis in MIS-C: A Case Report. Pediatr Infect Dis J. 2022;41(1):e16-e8.
11. Verdoni L, Mazza A, Gervasoni A, Martelli L, Ruggeri M, Ciuffreda M, et al. An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study. The Lancet. 2020;395(10239):1771-8.
12. Becker AE, Chiotos K, McGuire JL, Bruins BB, Alcamo AM. Intracranial hypertension in multisystem inflammatory syndrome in children. The Journal of pediatrics. 2021;233:263-7.
13. Kemmotsu Y, Nakayama T, Matsuura H, Saji T. Clinical characteristics of aseptic meningitis induced by intravenous immunoglobulin in patients with Kawasaki disease. Pediatric Rheumatology. 2011;9(1):1-5.
14. Guimarães D, Pissarra R, Reis‐Melo A, Guimarães H. Multisystem inflammatory syndrome in children (MISC): a systematic review. International Journal of Clinical Practice. 2021;75(11):e14450.
15. Shoenfeld Y, Gurewich Y, Gallant LA, Pinkhas J. Prednisone-induced leukocytosis. Influence of dosage, method and duration of administration on the degree of leukocytosis. Am J Med. 1981;71(5):773-8.