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Published on: January 27, 2015
Cerebral venous thrombosis in an adult with relapsing minimal change disease
Matthias Bergmann1, Thanh N Nguyen2, Christine C Segal3
1Division of Nephrology, Department of Medicine, St. Elizabeth's Medical Center, Boston, MA, USA; Department of Medicine, Tufts University School of Medicine, Boston, MA, USA.
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.
Insights
Minimal change disease (MCD) can cause nephrotic syndrome (NS) with serious blood clot risks, like cerebral venous thrombosis (CVT). Early recognition of neurological symptoms in NS patients is crucial for timely intervention and improved outcomes.
Area of Science:
- Nephrology
- Neurology
- Hematology
Background:
- Minimal change disease (MCD) is a primary cause of nephrotic syndrome (NS), often linked to thrombotic events.
- Cerebral venous thrombosis (CVT) is a rare but severe complication of NS, potentially leading to significant morbidity and mortality.
Approach:
- A case report of a patient with MCD-associated NS and CVT is presented.
- A systematic literature review identified 33 adult cases of NS-associated CVT to analyze clinical features, management, and outcomes.
Key Points:
- Headache, nausea, vomiting, and altered mental status are common symptoms of NS-associated CVT.
- CVT can occur at initial NS diagnosis or during relapse; MCD is the most frequent kidney biopsy finding.
- While anticoagulation is standard, outcomes vary, with a notable mortality rate, underscoring the need for prompt diagnosis and management.
Conclusions:
- The high prevalence of MCD in NS-associated CVT cases suggests a potential link between acute NS onset and thrombotic risk.
- Clinicians must maintain a high index of suspicion for CVT in NS patients presenting with neurological symptoms.
- Prompt recognition and management of CVT in NS patients are critical for improving patient outcomes.
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