Kim Seung Ae, Kim Eung Yeop, Wang Shuu-Jiun, Lee Mi Ji
Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea.
Graduate School of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
J Headache Pain. 2025 Apr 28;26(1):89. doi: 10.1186/s10194-025-01978-5.
The diagnosis of reversible cerebral vasoconstriction syndrome (RCVS) is challenging due to its varied clinical manifestations and imaging findings. While it typically presents with a sudden, severe thunderclap headache and multifocal constriction of the cerebral arteries, the wide spectrum of radiological presentations may complicate the diagnosis.
This review presents a series of cases that show both typical and atypical presentations of RCVS. Typical cases show the characteristic "string of beads" pattern on angiography, which usually resolves within 3-6 months. However, diagnostic challenges arise when angiography appears normal in the early stages or when imaging artifacts obscure the findings. In addition, the variability in vasoconstriction patterns and the need for a differential diagnosis further complicate the accurate identification. These cases highlight the importance of considering RCVS in patients with recurrent thunderclap headaches, even when the initial imaging is inconclusive. Recognizing these challenges and the variability in presentation, along with the use of high-resolution vessel wall MRI and blood-brain barrier imaging, can improve diagnostic accuracy and improve patient outcomes.
The diagnosis of RCVS requires careful integration of clinical evaluation and advanced imaging techniques, with particular attention to radiological findings that can guide accurate diagnosis and management. Despite challenges, such as normal early stage angiography and imaging variability, maintaining a high suspicion of RCVS is essential, especially in patients with recurrent thunderclap headaches.
可逆性脑血管收缩综合征(RCVS)的诊断具有挑战性,因其临床表现和影像学表现多样。虽然它通常表现为突发的、严重的霹雳样头痛和脑动脉的多灶性收缩,但广泛的放射学表现可能使诊断复杂化。
本综述展示了一系列显示RCVS典型和非典型表现的病例。典型病例在血管造影上显示出特征性的“串珠”模式,通常在3至6个月内消退。然而,当血管造影在早期看起来正常或成像伪影掩盖了结果时,诊断就会出现挑战。此外,血管收缩模式的变异性以及鉴别诊断的必要性进一步使准确识别变得复杂。这些病例强调了在复发性霹雳样头痛患者中考虑RCVS的重要性,即使初始影像学检查结果不明确。认识到这些挑战和表现的变异性,以及使用高分辨率血管壁磁共振成像和血脑屏障成像,可以提高诊断准确性并改善患者预后。
RCVS的诊断需要仔细整合临床评估和先进的成像技术,特别关注能够指导准确诊断和管理的放射学表现。尽管存在诸如早期血管造影正常和成像变异性等挑战,但对RCVS保持高度怀疑至关重要,尤其是在复发性霹雳样头痛患者中。