Reversible cerebral vasoconstriction mimicking posterior reversible encephalopathy syndrome in an infant with end-stage renal disease

Report

A.S.M. and G.M. contributed equally to this work.

A 4-month-old infant with end-stage renal disease presented with irritability, crying, and focal status epilepticus (Figure 1). Posterior reversible encephalopathy syndrome (PRES) was suspected. However, magnetic resonance imaging (MRI) (Figure 2) revealed extensively restricted brain diffusivity and circle of Willis vasoconstriction which resolved on follow-up, suggesting reversible cerebral vasoconstriction syndrome (RCVS). Furthermore, transient worsening of diffusion abnormalities during the first MRI suggested worsening hypoperfusion from declining blood pressure during inhalatory sedation with sevoflurane.

Figure 1.

EEG performed at admission (SI 10–20, eight electrodes, Sens 10 uV/mm, HF 15 Hz, LF 1.000 Hz).

Figure 2.

Neuroimaging findings.

RCVS is rare in children (1–3). Since thunderclap headache cannot be proven in infants, adapted diagnostic criteria including clinical correlates of headache and epileptic status should be used.