Pohjola Anni, Asikainen Aleksanteri, Kaprio Jaakko, Rautalin Ilari Matias, Niemelä Mika, Laakso Aki, Korja Miikka
Department of Neurosurgery, Helsinki University Hospital, Finland; and.
Institute for Molecular Medicine FIMM, University of Helsinki, Finland.
Neurology. 2025 Jul 22;105(2):e213818. doi: 10.1212/WNL.0000000000213818. Epub 2025 Jun 23.
The number of unexpected prehospital deaths from brain arteriovenous malformations (AVMs) is unknown because existing mortality data are derived from hospital-based cohorts. In this study, we aimed to determine the rate and causes of AVM-related deaths in a large population-based cohort, both in and outside hospitals. We hypothesized that AVMs infrequently cause unexpected prehospital deaths, and when they do, it is typically due to hemorrhages rather than epileptic seizures.
In this population-based cross-sectional study, we examined the AVM-related deaths within the Helsinki University Hospital (HUH) region during 1998-2015, including both diagnosed patients and undiagnosed individuals with prehospital deaths identified at autopsy. Autopsies are legally mandatory in Finland for all unexpected and sudden deaths. Prehospital deaths were identified through autopsy reports, which were obtained from the National Cause of Death Register (using the ICD-10 codes I60.8, I61.0-I61.9, or Q28.0-Q28.3), and the patients who were already diagnosed were identified from the Helsinki AVM Register. Age-standardized detection rates were calculated using the 2013 European Standard Population (ESP)-derived age-specific weights, with 95% CIs derived from the normal approximation of binomial distribution.
Between 1998 and 2015, we identified 4 unexpected prehospital AVM deaths in undiagnosed individuals (median age 41 years, 50% female) in the HUH region from the National Cause of Death Register data. For the same period, the HUH AVM database revealed 284 newly diagnosed patients with AVM (mean age at diagnosis 41.1 years [SD 18.0 years], 48% female). Altogether, there were 6 AVM-related prehospital deaths, 2 in previously diagnosed patients. Four of the 6 prehospital deaths were caused by AVM-related epileptic seizures and 2 by AVM hemorrhage. Among individuals experiencing their first AVM-related hemorrhage (n = 168), 1.2% experienced prehospital deaths due to hemorrhage. Similarly, of those diagnosed with AVM-related epileptic seizures (n = 69), 5.8% experienced prehospital deaths from seizures. For people with unruptured and ruptured AVMs, 6-month case fatality rates (CIs) from detection were 5.8% (1.7%-10.8%) and 11.9% (7.1%-17.3%), respectively. The crude and ESP-standardized detection rates of AVMs (including prehospital deaths) were 0.80 (0.71-0.89) and 0.78 (0.69-0.87) per 100,000 person-years.
AVM-related prehospital deaths are rare and often caused by epileptic seizures. Most AVM-related deaths occur in hospitals.
由于现有死亡率数据来自医院队列，脑动静脉畸形（AVM）导致的院外意外死亡人数尚不清楚。在本研究中，我们旨在确定一个基于大样本人群队列中AVM相关死亡的发生率及原因，包括院内和院外情况。我们假设AVM很少导致院外意外死亡，即便发生，通常也是由于出血而非癫痫发作。
在这项基于人群的横断面研究中，我们调查了1998 - 2015年期间赫尔辛基大学医院（HUH）区域内与AVM相关的死亡情况，包括已确诊患者以及尸检时发现的院外死亡的未确诊个体。在芬兰，所有意外和突然死亡依法必须进行尸检。通过尸检报告确定院外死亡病例，这些报告来自国家死亡原因登记处（使用国际疾病分类第十版代码I60.8、I61.0 - I61.9或Q28.0 - Q28.3），而已确诊患者则从赫尔辛基AVM登记处确定。使用2013年欧洲标准人口（ESP）得出的年龄特异性权重计算年龄标准化检出率，95%置信区间由二项分布的正态近似得出。
1998年至2015年期间，我们从国家死亡原因登记处数据中确定了HUH区域内4例未确诊个体的院外AVM意外死亡（中位年龄41岁，50%为女性）。同一时期，HUH的AVM数据库显示有284例新确诊的AVM患者（诊断时平均年龄41.1岁[标准差18.0岁]，48%为女性）。总共有6例与AVM相关的院外死亡，其中2例发生在先前已确诊的患者中。6例院外死亡中有4例由与AVM相关的癫痫发作导致，2例由AVM出血导致。在首次发生与AVM相关出血的个体（n = 168）中，1.2%因出血在院外死亡。同样，在被诊断为与AVM相关癫痫发作的个体（n = 69）中，5.8%因癫痫发作在院外死亡。对于未破裂和破裂AVM的患者，从发现起6个月的病死率（置信区间）分别为5.8%（1.7% - 10.8%）和11.9%（7.1% - 17.3%）。AVM（包括院外死亡）的粗检出率和ESP标准化检出率分别为每10万人年0.80（0.71 - 0.89）和0.78（0.69 - 0.87）。
与AVM相关的院外死亡很少见，且通常由癫痫发作导致。大多数与AVM相关的死亡发生在医院。
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