可逆性脑血管收缩综合征继发脑出血:1例报告及文献复习

F. Dantas, Eustáquio Claret dos Santos Júnior, Tiago Silva e Carvalho, Róberti Uili Rodrigues Firmino, Romulo Mourao, M. C. Fonseca, F. R. Dantas, R. Botelho, R. Darwich
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摘要

可逆性脑血管收缩综合征是一种罕见的疾病,其特征是反复发作的雷击性头痛和脑动脉节段性狭窄,通常在3个月内缓解。自20世纪60年代以来,它被描述为几个名称,包括Call-Fleming综合征。文献中已经报道了500多例,但病理生理学仍未得到很好的理解。缺血性或出血性中风是该综合征的主要可能并发症,导致一小部分患者出现永久性神经功能缺损或死亡。我们报告一例48岁的女性患者,没有已知的危险因素,表现出两个病灶的脑出血,与最大的脑内病变同侧偏瘫。mri示锥体对话正常,一周内神经功能缺损完全恢复。作者认为,在本病例中,神经功能障碍可能与对侧脑动脉狭窄和中枢神经系统弥漫性损害有关,而不是脑出血本身。RCVS是复发性雷击型头痛患者应考虑的罕见情况。需要进一步的前瞻性和随机化研究来改善对该综合征患者的管理和治疗。
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Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review
Reversible cerebral vasoconstriction syndrome is an unusual entity, characterized by recurrent thunderclap headache and segmental narrowing of the cerebral arteries, typically with remission within three months. It has been described since the 1960s with several names, including Call-Fleming syndrome. More than 500 cases have been described in the literature, yet the pathophysiology remains not well understood. Ischemic or hemorrhagic strokes are the major possible complications of the syndrome, leading to permanent neurological deficits or death in a small percentage of patients. We report a case of a 48-year-old female patient without known risk factors that presented two foci of intracerebral hemorrhages, with hemiparesis ipsilateral to the biggest intracerebral lesion. Magnetic resonance imaging tractography revealed normal pyramidal decussation, and the patient evolved with completely recover of the neurological deficit within a week. The authors believe that in the present case neurological deficit may be related to contralateral narrowing of the cerebral arteries and diffuse impairment of the central nervous system instead of intracerebral hemorrhage itself. RCVS is a rare condition that should be considered in patients with recurrent thunderclap type headache. Further prospective and randomized studies are still necessary to improve the management and treatment of patients with the syndrome.
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