Background

Sturge-Weber syndrome (SWS) is a neurocutaneous disorder associated with epilepsy, glaucoma, cognitive impairments, and a port-wine birthmark. Although individuals with SWS are vulnerable to known risk factors for suicide, including chronic illness and physical differences (port-wine birthmark), frequency of suicidal ideation and attempts in youth and adults with SWS is unknown.

Purpose

We aimed to assess the frequency of positive screens for suicidal thoughts or actions, and the clinical factors associated with suicide risk, in patients with SWS.

Methods

As a part of routine hospital practice, all outpatients age 8 and older underwent suicide risk screening during nursing triage using a standardized suicide screening tool. Suicide risk screening results, demographic variables, and medical history (as available) for patients with SWS (N=34; Median age=15.5; Range=8-47yrs, 44% male) and other neurological conditions seen at the same institution (N=369; Median age=14; Range=8-78yrs, 66% male) were used for retrospective within- and between-group analysis.

Results

In the combined sample of SWS and neurologically involved patients, a positive suicide risk screen was related to SWS diagnosis (p=0.043); analysis by sex showed SWS diagnosis increased risk in males (p=0.008) but not females. Within the SWS group, use of a Selective Serotonin Reuptake Inhibitor (p=0.019) was related to a positive risk screen.

Conclusion

People with SWS may be at greater risk of suicidal thoughts or behaviors than those with other neurological conditions. Further study of suicide risk in patients with SWS is needed.

中文翻译：

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Sturge-Weber综合征的自杀筛查：一个需要进一步研究的重要问题。

背景

Sturge-Weber综合征（SWS）是与癫痫，青光眼，认知障碍和葡萄酒酒胎记相关的神经皮肤疾病。尽管具有SWS的个体容易受到已知的自杀风险因素的影响，包括慢性疾病和身体差异（葡萄酒中的胎记），但对于SWS的青年和成年人，自杀意念的发生频率和尝试次数尚不清楚。

目的

我们的目的是评估SWS患者自杀想法或行动的阳性筛查频率以及与自杀风险相关的临床因素。

方法

作为常规医院实践的一部分，所有8岁及以上的门诊患者在护理分流过程中均使用标准化的自杀筛查工具进行自杀风险筛查。SWS（N = 34；中位年龄= 15.5；范围= 8-47yrs，男性占44％）和在同一机构就诊的其他神经系统疾病患者的自杀风险筛查结果，人口统计学变量和病史（可用） = 369；中位年龄= 14；范围= 8-78岁，男性占66％）用于组内和组间分析。

结果

在SWS和神经系统受累患者的合并样本中，自杀风险筛查阳性与SWS诊断相关（p = 0.043）；性别分析显示SWS诊断增加了男性的风险（p = 0.008），而女性则没有。在SWS组中，使用选择性5-羟色胺再摄取抑制剂（p = 0.019）与阳性风险筛查有关。

结论

与其他神经系统疾病患者相比，SWS患者可能具有更大的自杀念头或行为风险。SWS患者的自杀风险有待进一步研究。