Mansour Moustafa A, Mostafa Hamdi Nabawi
Department of Neurosurgery, Nasser Institute for Research and Treatment, Cairo, Egypt.
Department of Neurosurgery, Nasser Institute for Research and Treatment, Cairo, Egypt; Department of Neurosurgery, Faculty of Medicine, Misr University for Science and Technology, Giza, Egypt.
Int J Surg Case Rep. 2025 Aug;133:111673. doi: 10.1016/j.ijscr.2025.111673. Epub 2025 Jul 11.
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.
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.
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.
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.
脑增生性血管病（CPA）是一种罕见的血管疾病，与动静脉畸形（AVM）不同，其特征为弥漫性经硬膜供血、动脉狭窄和血管扩张。虽然CPA通常会引起头痛或癫痫发作，但其与脑高灌注综合征（CHS）的关联从未有过报道。本病例突出了一种新的CPA并发症，强调了及时识别和针对性管理的必要性。
一名21岁女性出现癫痫持续状态和进行性右侧头痛。影像学检查显示为CPA，灌注研究表明右侧额叶脑血流量（CBF）和脑血容量（CBV）升高，符合CHS。尽管进行了药物治疗，症状仍持续存在，但在颅骨钻孔手术后症状缓解，三年随访时持续改善。
这是首例CPA并发CHS的报告，挑战了CPA纯粹为缺血性疾病的观点。病理生理学可能涉及异常血管生成和狭窄导致的自动调节功能紊乱。灌注成像对诊断至关重要。减压手术（如钻孔）可通过调节颅内压和血流动力学来缓解难治性症状。
CPA可表现为高灌注综合征，这是一种先前未被认识的并发症。通过先进影像学进行早期诊断并采取包括手术减压在内的个体化治疗至关重要。需要进一步研究以探索CPA的血流动力学复杂性和最佳管理策略。
