Refractory Status Epilepticus as an Initial Manifestation of IgA Nephropathy

Dear Editor,The kidney-brain axis is a bidirectional communication network between the kidneys and brain, influenced by inflammation, uremic toxins, vascular damage, neuron degeneration, and blood-brain barrier (BBB) permeability.1 Patients with renal failure are at increased risk of seizures because of uremic toxins associated with uremic encephalopathy, electrolyte disturbances, posterior reversible encephalopathy syndrome (PRES), nephrotoxic drugs, 2 and even dialysis disequilibrium syndrome.3 We recently encountered a patient with IgA nephropathy who initially presented with status epilepticus.A 26-year-old woman without prior comorbidities presented with sudden generalized tonic-clonic seizures accompanied by right upper eyeball deviation and altered mental status.She had no history of hypertension, though her blood pressure was rarely monitored.Her sister reported that she had experienced visual disturbances and severe headache two days before admission.On arrival, she was stuporous with a Glasgow Coma Scale score of 7 and a blood pressure of 164/100 mm Hg.Laboratory tests showed markedly elevated levels of blood urea nitrogen (78.0 mg/dL), creatinine (6.25 mg/dL), creatine kinase (5,697 U/L), lactate (5.41 mmol/L), and uric acid (23.2 mg/dL), along with proteinuria (3+, 300-650 mg/ dL).Despite lorazepam, seizures continued after administration of multiple antiseizure medications (ASMs), including levetiracetam (60 mg/kg intravenously), lacosamide (400 mg intravenously), valproic acid (40 mg/kg intravenously), and perampanel (12 mg orally).Owing to recurrent seizures without recovery of consciousness and the development of aspiration pneumonia, the patient was intubated and managed with continuous midazolam infusion.Electroencephalogram revealed diffuse background slowing without epileptiform discharges, and after the initiation of continuous midazolam infusion, certain findings, such as generalized delta slowing and increased fast activities, were considered partially attributable to sedative effects.Brain MRI, performed on hospital day 2, demonstrated diffuse cortical and subcortical T2 hyperintensities with swelling in the bilateral parieto-occipital regions and right temporal lobes, asymmetrical T2 and diffusion-weighted imaging hyperintensities in the bilateral basal ganglia, and T2 hyperintensity in the right thalamus (Fig. 1A), suggestive of atypical severe PRES.Despite combination therapy of ASMs, consciousness did not recover, although clinical seizures ceased by the hospital day 3. On the same day, her systolic blood pressure exceeded 160 mm Hg, and continuous intravenous nicardipine was initiated, maintaining systolic blood pressure below 130 mm Hg thereafter.Cerebrospinal fluid analysis revealed no evidence of central nervous system infection, and serologic tests for glomerulonephritis (anti-Pr3 Ab, anti-MPO Ab, anti-GBM Ab, C3, C4, CH50, and ANA) were negative.Serial laboratory tests showed progressive renal dysfunction, with creatinine rising from 5.8 mg/dL at admission to 6.7 mg/dL on hospital day 6, and blood urea nitrogen increasing to 120 mg/dL.Urine output also declined from over 3-4 L/day to approximately 1 L/day during the first week.Based on these trends, hemodialysis was initiated on