Objective:

Background:

Reversible cerebral vasoconstriction syndrome (RCVS) is marked by sudden constriction of cerebral vessels characterized by thunderclap headache with or without neurological deficits or seizures.  Pediatric RCVS is rare and requires a high index of suspicion for diagnosis. Pathophysiology remains poorly understood. Precipitant factors identified include vasoactive medications, postpartum state and immunosuppressant medication; cases without a trigger are reported. 

Design/Methods:

Medical chart and literature review 

Results:

A typically developing 10-year-old female was admitted for new-onset headaches and pharyngitis. Headaches were sudden-onset, episodic, band-like, 10/10 intensity, and lasted about 45 minutes without associated neurological deficits, treated following institutional migraine algorithm. CTA and CTV head were unremarkable. After tonsilloadenoidectomy she was discharged with as-needed dexamethasone, requiring two doses at home. Five days later, she presented with altered mental status, intermittent mild headaches, unsteady gait, and lower extremity weakness. MRI brain revealed multiple small acute infarcts in a watershed distribution. CTA brain showed areas of vessel narrowing and irregularity in the anterior and posterior circulation. She was started on high-dose methylprednisolone due to concern for post-infectious focal arteriopathy. Three days later, she developed new left lower extremity weakness and numbness. MRI brain demonstrated a new right subcortical frontal infarct. Further serologic and CSF workup remained unrevealing. DSA revealed mild, resolved RCVS. She was started on nimodipine and aspirin, and steroids were discontinued. Mental status and focal neurologic deficits gradually improved to baseline prior to discharge.   

Conclusions:

RCVS must be considered a potential diagnosis in pediatric patients with sudden onset of severe headaches. Corticosteroids often used in managing various acute neurological conditions are known to trigger RCVS. Care is required when starting patients with RCVS on new medications. Similar to adults, pediatric patients can be treated with calcium channel blockers, which have been associated with good outcomes.