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An adolescent with a history of autism and focal segmental glomerulosclerosis type nephrotic syndrome who was non-compliant with therapy for the past 5 years presented with increasing lethargy, horizontal beating nystagmus and a generalised seizure with right gaze deviation. The patient was hypertensive, with a systolic blood pressure of 220 mm Hg, but otherwise unremarkable vital signs and demonstrated moderate periorbital and lower extremity pitting oedema. CT following emergent intubation for airway protection showed calcifications of the basal ganglia without acute findings ( figure 1A). MRI (figure 1B–H) demonstrated left posterior parietal hyperintensity on diffusion-weighted sequences without abnormalities on apparent diffusion coefficient sequences consistent with T2 shine through. Fluid-attenuated inversion recovery sequences revealed hyperintensities in the posterior parietal, temporal, cerebellar hemispheres and pulvinar regions. Overall, neuroimaging was consistent with posterior reversible encephalopathy syndrome (PRES). Video telemetry monitoring showed diffuse slowing and left temporal periodic lateralised epileptiform discharges (figure 2). The patient was started on prednisone and continued on levetiracetam without seizures prior to discharge on day 7, when neurologic examination was normal. At 1-year follow-up, the nephrotic syndrome had remitted completely following treatment with steroids and mycophenolate mofetil. Hypertension was well-controlled on clonidine and lisinopril, and anti-seizure activity was discontinued after 6 months. The patient remains seizure-free and is followed quarterly with nephrology.