Mathon B, Ducros A, Bresson D, Herbrecht A, Mirone G, Houdart E, Saint-Maurice J-P, Di Emidio P, George B, Chibbaro S
Service de neurochirurgie, CHU Lariboisière, 2, rue Ambroise-Paré, 75475 Paris cedex 10, France.
Service de neurologie, CHU Lariboisière, 2, rue Ambroise-Paré, 75475 Paris cedex 10, France.
Rev Neurol (Paris). 2014 Feb;170(2):110-8. doi: 10.1016/j.neurol.2013.07.032. Epub 2014 Jan 8.
Convexity subarachnoid and intra-cerebral hemorrhages, in patients aged<50 years, are always a diagnostic challenge. This condition is characterized by acute headaches with or without neurological symptoms and/or seizures, and by the radiological demonstration of subarachnoid and/or intra-cerebral hemorrhages and, more rarely, by the association of ischemic events.
In a prospective series of 30 consecutive patients (median age 31 years; 22 women) with a subarachnoid and intra-cerebral hemorrhages, 19 were diagnosed with reversible cerebral vasoconstriction syndrome (RCVS), 7 with cerebral venous sinus thrombosis (CVST), and 4 with a bleeding mycotic aneurysm (MA).
RCVS appeared spontaneously in 16 patients and was related to the postpartum period in three cases. Subarachnoid hemorrhage (SAH) was demonstrated in 24 patients as follows: 18 cases were in cortical areas, 4 were in the polygon of Willis, one was inter-hemispheric, and one was inter-hemispheric/intra-cerebral. A convexity pure intra-cerebral hemorrhage (ICH) was recorded in 6 cases. Among the 7 patients suffering from CVST, the superior sagittal sinus was involved in 4 cases, the transverse sinuses (TS) in 2, and the TS plus sigmoid sinus (SS) in one.
The three most common causes in this series were RCVS, followed by CVST and bleeding from MA. Because of atypical clinical or radiological presentations, this large spectrum of etiologies can cause diagnostic difficulties. Therefore, careful analysis is needed to ensure correct and prompt diagnosis and to avoid any dangerous delays in management.
50岁以下患者的凸面蛛网膜下腔出血和脑内出血一直是诊断难题。这种情况的特征是伴有或不伴有神经症状和/或癫痫发作的急性头痛,以及蛛网膜下腔和/或脑内出血的影像学表现,更罕见的是伴有缺血性事件。
在一项前瞻性研究中,连续纳入30例蛛网膜下腔出血和脑内出血患者(中位年龄31岁;22例女性),其中19例诊断为可逆性脑血管收缩综合征(RCVS),7例为脑静脉窦血栓形成(CVST),4例为出血性霉菌性动脉瘤(MA)。
16例患者的RCVS为自发出现,3例与产后时期有关。24例患者出现蛛网膜下腔出血(SAH),情况如下:18例位于皮质区域,4例位于Willis环,1例位于半球间,1例位于半球间/脑内。记录到6例凸面单纯脑内出血(ICH)。在7例CVST患者中,上矢状窦受累4例,横窦(TS)受累2例,横窦加乙状窦(SS)受累1例。
本系列中三种最常见的病因是RCVS,其次是CVST和MA出血。由于临床表现或影像学表现不典型,这种广泛的病因可导致诊断困难。因此,需要仔细分析以确保正确、及时的诊断,并避免治疗中出现任何危险的延误。