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Reversible Cerebral Vasoconstriction Syndrome and Multisystem Inflammatory Syndrome in Children With COVID-19
Atefeh Sadeghizadeh1, Zahra Pourmoghaddas2, Alireza Zandifar3
1Department of Pediatric Intensive Care, Isfahan University of Medical Sciences, Isfahan, Iran.
Insights
Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 can present with reversible cerebral vasoconstriction syndrome (RCVS). Early recognition and imaging are crucial for diagnosing RCVS in pediatric MIS-C patients with severe neurological symptoms.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Multisystem inflammatory syndrome in children (MIS-C) is a severe condition linked to COVID-19.
- Reversible cerebral vasoconstriction syndrome (RCVS) is rare in children and characterized by thunderclap headaches and reversible vasoconstriction on imaging.
Observation:
- Two pediatric patients with severe MIS-C presented with thunderclap headaches and neurological deficits.
- Both patients received intensive treatments including vasoactive agents, IV immunoglobulin, and immunosuppressants.
Findings:
- Imaging revealed multifocal cerebral vasoconstriction in both cases, with one experiencing infarcts.
- Cerebral vasoconstriction resolved completely on follow-up imaging after inflammation control.
Implications:
- RCVS should be considered in pediatric MIS-C patients presenting with thunderclap headaches and neurological signs.
- Prompt diagnosis through clinical and imaging findings is essential for effective management and recovery.
Background:
Multisystem inflammatory syndrome in children (MIS-C) involves multiple organs and shows increased inflammatory markers. Since the onset of the coronavirus disease 2019 (COVID-19) pandemic, several studies have reported the association between severe COVID-19 and MIS-C. Reversible cerebral vasoconstriction syndrome (RCVS) presents with thunderclap headaches and multifocal reversible vasoconstriction on imaging. RCVS is very rare in children. This article reports two cases of pediatric COVID-19 with severe MIS-C and clinical and imaging features indicative of RCVS.
Methods:
Clinical, laboratory, and imaging data of the patients were reviewed. The diagnosis of RCVS was confirmed based on clinical symptomatology and brain magnetic resonance imaging findings.
Results:
Two pediatric patients with clinical findings compatible with severe MIS-C and hemodynamic compromise presented to the hospital. During their hospitalization course, they developed thunderclap headaches and neurological deficits. Both were receiving vasoactive agents, intravenous immunoglobulin, and immunosuppressants. Imaging studies showed marked multifocal cerebral vasoconstriction in both cases and infarcts in one. The course and management of the patients will be presented. After controlling inflammation and elimination of triggers, both patients were ultimately symptom free upon discharge. Cerebral vasoconstriction had completely resolved on follow-up imaging.
Conclusions:
Although a variety of symptoms including headaches may be seen in pediatric COVID-19 patients with MIS-C, RCVS should be considered as a differential diagnosis in cases of thunderclap headache accompanied by neurological signs in these patients. Imaging findings and follow-up are also key in establishing the diagnosis.

