可逆性脑血管收缩综合征伴自发性脊髓硬膜下出血——一个令人困惑的难题!!

Harini Pavuluri, S. M. Krishna Mohan Mavuru, D. Sharma, S. Sreedharan, P. Sylaja
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摘要

一位中年女士,以头痛、呕吐为主诉,检查无异常。她的大脑成像包括MR和CT血管造影正常,但腰椎穿刺(LP)显示脑脊液均匀血染,提示SAH。随后的脊柱成像显示自发性脊髓硬膜下出血(SSDH)在背部和腰骶索。凝血功能和其他基本检查都正常。痉挛3周后的数字减影血管造影(DSA)显示广泛的脑血管痉挛,呈肠状珠状外观,类似于脊髓血管正常的可逆性脑血管收缩综合征(RCVS)。尼莫地平治疗6周。她的头痛好转了,没有复发。重复DSA和脊柱成像正常,血管痉挛和SSDH均得到缓解。该患者有两种明显的病理,SSDH和可能的RCVS,确定其诱发事件是一项复杂的任务,也是讨论的中心思想。我们试图通过为这两种临床情况提供合理的论据,来解决这个“先有鸡还是先有蛋”的悖论,即SSDH导致颅内血管痉挛vs RCVS导致SSDH。我们也想强调对雷击性头痛患者进行脊柱成像的必要性。
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Reversible Cerebral Vasoconstriction Syndrome with Spontaneous Spinal Subdural Hemorrhage—a Perplexing Conundrum!!!
A middle aged lady presented with thunderclap headache and vomiting without any deficits on examination. Her cerebral imaging including MR and CT angiogram were normal but lumbar puncture (LP) revealed uniformly blood stained CSF suggestive of SAH. Subsequent spine imaging revealed spontaneous spinal subdural hemorrhages (SSDH) in the dorsal and lumbosacral cord. Coagulopathy and other essential work up was normal. Digital subtraction angiography (DSA) done 3 weeks into ictus revealed extensive cerebral vasospasm with sausage like beaded appearance akin to reversible cerebral vasoconstriction syndrome (RCVS) with normal spinal vasculature. She was treated with Nimodipine for 6 weeks. Her headache improved without any recurrence. Repeat DSA and spine imaging was normal with resolution of both vasospasm and SSDH. Establishing the inciting event in this patient with 2 obvious pathologies, SSDH and possible RCVS, is a perplexing task and forms the central idea of discussion. We made an attempt to resolve this chicken egg paradox, ie, SSDH leading to intracranial vasospasm vs RCVS leading SSDH, by providing rational arguments for both the clinical scenarios. We would also like to highlight the necessity of spinal imaging in patients with thunderclap headache.
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