Cyclophosphamide

Abstract

Posterior reversible encephalopathy syndrome: case report A 73-year-old man developed posterior reversible encephalopathy syndrome during treatment with cyclophosphamide for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The man presented to the hospital with persistent oliguric azotaemia along with sepsis due to an acute cholangitis. He had initially received endoscopic retrograde biliary drainage state and an antibiotic treatment for sepsis due to common biliary duct cancer for a month. One month ago, he had undergone haemodialysis for 2 weeks due to septic acute kidney injury with oliguria and uraemic symptoms. Following the resolution of sepsis, his azotaemia and oliguria showed no improvement and haemodialysis was continued for another one month. Upon presentation, his BP was high and pretibial pitting oedema was observed on both sides. Laboratory tests revealed elevated levels of blood urea nitrogen and serum creatinine. Additionally, total protein, serum albumin and total cholesterol levels were observed to be low. Urinalysis indicated a microscopic haematuria and proteinuria with an elevated protein/creatinine ratio. His anti-neutrophil cytoplasmic antibody was also detected positive. A kidney needle biopsy revealed crescentic glomerulonephritis and a bronchoscopy showed diffuse alveolar haemorrhage. He was diagnosed with ANCA-associated vasculitis along with a pulmonary renal syndrome. He received initial treatment with plasma exchange and methylprednisolone for a week. One week later, he was additionally treated with oral cyclophosphamide 100mg every day. Following the second dose of cyclophosphamide on day 3 of treatment, he suddenly developed generalised tonic-clonic seizures along with 30 seconds deviation of both eyeballs for five consecutive times. At the time of seizure attack, his BP was 175/76mm Hg. At the end of his seizure attack, he exhibited a stuporous mental status. A brain MRI was performed at once, which indicated multifocal high signal intensity in both frontoparietal lobes, occipital lobes, temporal lobes, pons and cerebellum on T2-fluid attenuated inversion recovery (FLAIR). An electroencephalography revealed frequent short bursts of bilateral polymorphic δ to slow waves with an amplitude of medium to high along with short-attenuated periods. Thus, a final diagnosis of posterior reversible encephalopathy syndrome secondary to cyclophosphamide was confirmed. The man received treatment with fosphenytoin for seizure control. His treatment with methylprednisolone was continued but cyclophosphamide was withdrawn. After 7 days of seizure attack, his mentality fully recovered. Two weeks later, a repeat brain MRI was performed, which indicated resolution of disseminated enhancing lesions on T2-FLAIR. Hence, at the time, he showed no neurologic symptoms and indicated a normal condition. After 10 days of seizure attack, his oliguria had resolved and serum creatinine had reduced. Haemodialysis was stopped and he was discharged with prednisolone. Four months later, a follow-up showed that he was ongoing maintenance treatment with methylprednisolone with gradual tapering. His renal function was under stable condition with a chronic kidney disease stage G4A2. Eventually, relapse of any other neurologic symptoms was not seen.