Unveiling the hyperperfusion syndrome in cerebral proliferative angiopathy: A novel clinical presentation. A case report and evidence-based review.

INTRODUCTION AND

IMPORTANCE

Cerebral proliferative angiopathy (CPA) is a rare vascular disorder distinct from arteriovenous malformations (AVMs), marked by diffuse transdural supply, arterial stenosis, and angiectasia. While CPA typically causes headaches or seizures, its association with cerebral hyperperfusion syndrome (CHS) has never been reported. This case highlights a novel CPA complication, underscoring the need for prompt recognition and tailored management.

CASE PRESENTATION: A 21-year-old woman presented with status epilepticus and progressive right-sided headaches. Imaging revealed CPA with perfusion studies showing elevated cerebral blood flow (CBF) and volume (CBV) in the right frontal lobe, consistent with CHS. Symptoms persisted despite medical therapy but resolved after a calvarial burr hole procedure, with sustained improvement at three-year follow-up.

CLINICAL DISCUSSION: This is the first report of CPA complicated by CHS, challenging the view of CPA as purely ischemic. Pathophysiology likely involves disrupted autoregulation from aberrant angiogenesis and stenosis. Perfusion imaging was pivotal for diagnosis. Decompressive surgery (e.g., burr holes) may alleviate refractory symptoms by modulating intracranial pressure and hemodynamics.

CONCLUSION

CPA can present with hyperperfusion syndrome, a previously unrecognized complication. Early diagnosis via advanced imaging and individualized treatment, including surgical decompression, is critical. Further research is needed to explore CPA's hemodynamic complexities and optimal management strategies.