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Characteristic imaging features of neurovascular involvement in primary Sneddon’s syndrome: an analysis of 12 cases
Neurological Sciences ( IF 3.3 ) Pub Date : 2020-10-13 , DOI: 10.1007/s10072-020-04621-0
Ezgi Yilmaz , Ethem Murat Arsava , Rahşan Gocmen , Kader Karli Oguz , Anil Arat , Mehmet Akif Topcuoglu

Objective

Sneddon’s syndrome is a cerebrocutaneous non-inflammatory progressive distal arteriopathy, characterized by livedo racemosa, stroke, and neuropsychiatric symptoms. Our aim was to highlight the characteristic neuroimaging features of Sneddon’s syndrome that might be helpful to clinicians in timely diagnosis of this entity.

Methods

Twelve patients (median age 49 years, 11 female) with primary Sneddon’s syndrome, diagnosed in last 10 years, were analyzed from the perspective of magnetic resonance imaging (MRI) features. In addition, a novel pseudoangiomatosis score was defined for grading angiographic abnormalities (range: 0 to 6).

Results

Median interval from the onset of neurological symptoms to diagnosis was 6 years. Presentation was with acute stroke in 5, seizures in 3, dementia/speech problems in 2, seizures plus cognitive dysfunction in 1, and chronic progressive hemiparesis in 1. All patients had a typical lesion pattern on MRI. This included multiple (median 3) cortical-subcortical supratentorial and cerebellar non-territorial infarcts, accompanied by multifocal cerebral atrophy. Of note, large territorial infarcts due to cerebral parent artery occlusion, an embolic pattern with multi-territorial involvement on diffusion-weighted imaging, small vessel disease features like severe white matter involvement or lacunar infarcts, and cerebral hemorrhage in the absence of anticoagulation were not observed. MRI lesion severity was not correlated with angiographic arteriopathy severity, clinical stage, or presentation symptoms.

Conclusion

Sneddon’s syndrome is characterized by highly typical clinico-radiological features. Brain MRI has diagnostic value. By knowing the characteristics of the syndrome, misdiagnosis and potentially harmful treatment can be prevented in this entity that might pose a diagnostic challenge.



中文翻译:

原发性Sneddon综合征神经血管受累特征影像学特征:12例分析

目的

斯内登氏综合症是一种脑皮非炎性进行性远端动脉病,其特征是消旋消旋,中风和神经精神症状。我们的目的是强调Sneddon综合征的特征性神经影像学特征,这可能有助于临床医生及时诊断该实体。

方法

从磁共振成像(MRI)的角度分析了12例最近10年诊断为原发性Sneddon综合征的患者(中位年龄49岁,女性11位)。此外,为血管造影异常分级定义了新的假血管瘤病评分(范围:0至6)。

结果

从神经系统症状发作到诊断的中位间隔为6年。表现为急性中风5例,癫痫3例,痴呆/语音障碍2例,癫痫加认知功能障碍1例,慢性进行性偏瘫1例。所有患者在MRI上均具有典型的病变模式。这包括多发(中位数3)皮层下皮质上上和小脑非领土梗塞,并伴有多灶性脑萎缩。值得注意的是,由于脑父母动脉闭塞引起的大区域梗塞,弥散加权成像中具有多区域累及的栓塞模式,严重的白质累及或腔隙性梗塞等小血管疾病特征以及没有抗凝治疗的脑出血都没有观测到的。

结论

Sneddon综合征的特征是高度典型的临床放射学特征。脑部MRI具有诊断价值。通过了解该综合征的特征,可以防止在该实体中可能引起诊断挑战的误诊和潜在的有害治疗。

更新日期:2020-10-13
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