IVIG May Improve Neurological Symptoms in MIS-C

Recent case series findings, as well as previous studies, show that children with multisystem inflammatory syndrome (MIS-C) who present with signs of acute neurological symptoms may show improvement with intravenous immune globulin (IVIG) and corticosteroids.

In the recent case series findings, 12 children were diagnosed with acute neurological symptoms and MIS-C in two hospitals in the Isfahan province in Iran between March 1, 2020, and Dec. 28, 2021. Diagnosis of MIS-C was made according to World Health Organization criteria and recommendations from the American College of Rheumatology, in addition to prior detection of SARS-CoV-2. The median age of the patients was 4.5 years, and three patients were aged 1 year or younger. Underlying diseases included chronic neurological disorders (neurodegenerative disease and seizure disorder), cardiac disease and malignancy. Immunodeficiency was not observed in any patient.

All patients were treated per the Iranian treatment protocol for children with MIS-C. Two patients were admitted to the intensive care unit during their hospitalization and one patient experienced myocarditis and vegetation-like lesions in the endocardium. Eleven patients were ultimately discharged in good condition, and in four-week follow-ups, 10 of them showed no complaints. One patient suffered from short-term memory loss.

Notably, relapses of MIS-C occurred in two patients due to rapid tapering of corticosteroids at two weeks instead of the standard protocol of four to six weeks. One patient who suffered from cobalamin deficiency died after full recovery from acute neurological symptoms, attributed to irreversible shock due to cobalamin deficiency.

Based on their observations, the investigators said central nervous system involvement is a non-rare associating condition with MIS-C. Acute neurological symptoms are life-threatening, although the epidemiology, pathophysiology and prognosis of acute neurological symptoms in children with MIS-C are still unclear. Cerebrospinal fluid (CSF) analysis of the cases was almost insignificant. However, one case had pleocytosis in their CSF analysis, which has been noted previously in other patients with COVID-19. Importantly, however, lumbar puncture of cases was performed within 24 hours of admission, which may not be enough time for white blood cells to migrate to the CSF.

According to the investigators, limitations included 1) the retrospective nature of the study meant that investigations of patients were dissimilar and dependent on the current guidelines at the time, although neurologic evaluation was performed for all patients in the same way and 2) because MIS-C is a clinical diagnosis and diagnostic tests are lacking, it is not possible to distinguish between acute neurological symptoms as part of MIS-C and as a coincidence. Yet, despite these limitations and the remaining gaps in knowledge about MIS-C, the investigators concluded that immunomodulatory agents such as IVIG and corticosteroids can significantly improve patients’ prognosis.