Cerebral venous thrombosis in an adult with relapsing minimal change disease

Abstract

Minimal change disease (MCD) is a well-known cause of fulminant acute nephrotic syndrome (NS) and has been associated with thrombotic complications. We report the case of a 51-year-old woman with previous biopsy-proven MCD in remission who presented with worsening headache and acute confusion shortly after a relapse of the NS and was diagnosed with cerebral venous thrombosis (CVT) complicated by intracranial hemorrhage and midline shift. One month prior, she had been initiated on an oral contraceptive agent during remission of the NS. After initiation of systemic anticoagulation, her condition rapidly deteriorated, and she passed away before being able to undergo catheter-based venous thrombectomy. We conducted a systematic literature review and identified 33 case reports of adults with NS-associated CVT. The most common symptoms were headache (83%), nausea or vomiting (47%), and altered mental status (30%). 64% of patients presented at time of initial diagnosis of the NS and 32% during a relapse. Mean urinary protein excretion was 9.32 g/day and mean serum albumin was 1.8 g/dL. 91% of patients received systemic anticoagulation, and 19% died. The outcome in the remaining cases was favorable with only one report (5%) of residual neurological deficit. Of the available kidney biopsy results, MCD was the most common diagnosis (70%), raising the hypothesis that the fulminant acute onset of the NS might be a predisposing factor for this serious thrombotic complication. Clinicians should have a high index of suspicion for CVT in patients with the NS who present with new-onset neurological symptoms, including headache and nausea.

Keywords: Cerebral venous thrombosis; Intracranial thrombosis; Minimal change disease; Nephrotic syndrome; Oral contraceptive use.