【双语病例】系统性红斑狼疮伴颅内钙化1例

6 9 月
Description
A 34-year-old man with dysarthria and lower-limb weakness

34岁男性,构音困难,双下肢无力。

【双语病例】系统性红斑狼疮伴颅内钙化1例

【双语病例】系统性红斑狼疮伴颅内钙化1例

【双语病例】系统性红斑狼疮伴颅内钙化1例

【双语病例】系统性红斑狼疮伴颅内钙化1例

【双语病例】系统性红斑狼疮伴颅内钙化1例

LegendsAxial noncontrast CT scans (A, B) demonstrate bilateral, nearly symmetrical calcifications of the caudate, lentiform nuclei, and periventricular white matter. The dentate nuclei are spared (C). There is also focal atrophy and subcortical hypodensity within the right parietal lobe (D), suggesting prior infarct.

横断位CT平扫(图A,B)示双侧豆状核、尾状核及室旁白质区对称性钙化。齿状核无明显钙化(图C)。右顶叶皮层下低密度、局部脑萎缩(图D)提示陈旧性脑梗塞。

【双语病例】系统性红斑狼疮伴颅内钙化1例

Brain Calcification in Systemic Lupus Erythematosus (SLE)

系统性红斑狼疮伴颅内钙化

Background

  • Brain calcifications have been reported in SLE with neuropsychiatric manifestations. The most frequent location involved is the basal ganglia, with less frequent involvement of the cerebral white matter and cerebellum.

    有文献报道,有神经系统症状的SLE患者,可发生颅内钙化。最常发生的部位为基底节区,其次是大脑白质区和小脑。

  • The underlying mechanism of calcification is unknown, but may be dystrophic following microinfarctions due to primary vascular damage and ongoing venous inflammation.

    钙化发生的机制尚完全不清楚,可能与微小梗死所致的营养不良或持续静脉炎症有关。

Clinical PresentationCNS manifestations of SLE including seizures or focal neurological deficits, occurring in ~20% of patients.

SLE的中枢神经系统症状包括:癫痫或局灶性神经功能异常。但出血神经系统症状的SLE患者不足20%。

Key Diagnostic Feature

  • Cerebral atrophy is the most common finding on cranial CT. Basal ganglia calcification is present in up to 25% of cases.

    头颅CT最常见的表现是脑萎缩,约25%的患者可见基底节区钙化。

  • MRI shows cerebral involvement in approximately 70% of patients with SLE. The most frequent imaging finding in SLE is multifocal high-signal intensities on FLAIR and T2WI in the subcortical white matter. SLE also can present with diffuse white matter abnormality, infarcts, cortical atrophy, cerebral edema, intracranial hemorrhage, and calcifications.

    MRI示,约70%的SLE患者可见大脑受累,常表现为FLAIR或T2WI序列皮层下白质区内的多发局灶性高信号,也可表现为弥漫性脑白质异常、梗死、皮质萎缩、脑水肿、颅内出血、钙化等。

DDx

  • Fahr disease Fahr病
  • Hyperparathyroidism 甲状旁腺功能亢进
  • (Pseudo)hypoparathyroidism 假性甲旁亢
  • lead intoxication 铅中毒
  • AIDS 艾滋病
  • Radiation therapy 放疗后改变
RxMainstay of treatment for SLE is immunosuppressive agents, including steroids.

SLE主要治疗方法是免疫抑制剂治疗,包括激素治疗。

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