Purpose or Case Report: Reversible cerebral vasoconstriction syndrome (RCVS) is commonly seen in middle-aged patients. This syndrome is characterized by thunderclap headaches which may not be apparent or recognized in pediatric patients, posing a challenge in diagnosis. Several medications have been associated with the development of RCVS, though they are not reliably known to cause it: highlighting the potential influence of other underlying predisposing factors. RCVS is self-limiting with treatment focused on reducing the degree of vasoconstriction.
A 7-year-old male patient with a medical history of B-cell acute lymphoblastic lymphoma, currently undergoing treatment with intrathecal methotrexate, cytarabine, and IV vincristine was admitted to our institution for acute perforated appendicitis. On the second day of hospitalization, the patient developed left arm weakness which prompted evaluation with MRI/MRA brain. Imaging revealed restricted diffusion in the left superior frontal gyrus with corresponding low ADC values and mild T2/FLAIR signal suggestive of an acute ischemic infarct. Vascular imaging showed a beaded appearance of the supraclinoid ICA, ACA and MCA bilaterally. Transcranial doppler study of the arteries confirmed elevated velocities consistent with multifocal stenoses. Initial differential diagnoses included acute ischemic infarct secondary to vasculitis with infectious causes considered due to the patient’s neutropenic state. Although, there was no evidence of vasculitis in other regions of the body and a primary CNS vasculitis typically presents with bilateral infarcts. Methotrexate leukoencephalopathy was also considered, although it typically affects white matter, primarily in the centrum semiovale. Posterior reversible encephalopathy syndrome was another possibility, but the absence of cerebral microhemorrhages argued against this. Given the vascular and ischemic findings, RCVS was ultimately suspected. A follow-up MRI performed several days later revealed a new infarct in the right frontal lobe. Notably, repeat CTA imaging showed complete resolution of the arterial stenoses, further supporting the diagnosis of RCVS.
This case illustrates the diagnostic challenge of RCVS in a pediatric patient with comorbidities that initially pointed towards a neoplastic or infectious etiology. Although not performed in the presented case, vessel wall MRI may be a useful tool in differentiating between arterial narrowing from vasculitis versus RCVS or atherosclerosis.
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