Infratentorial superficial siderosis of central nervous system: three cases report and literature review

Abstract

Objective To report 3 cases of infratentorial superficial siderosis of central nervous system and analyze its etiology and clinical manifestations. Methods and Results The main clinical symptoms were recurrent headache or epilepsy (3 cases) with obvious hearing loss (2 cases); the appearance of cerebrospinal fluid of lumbar puncture was cloudy and bloody fluid, with increased red blood cell count and protein quantity (2 cases). MRI scan of head or cervical spine showed siderosis deposition on the surface of cerebellum hemisphere, pons and midbrain without enhancement, and occupying lesion at jugular foramen was seen at the same time (Case 1); tortuous vessels on the surface of cervical spinal cord, epidural effusion at the level of C6⁃7 vertebral body, C7 dural defect (Case 2) or paraventricular hemorrhage (Case 3). There were no abnormal findings in angiography of the whole brain. The clinical diagnosis was infratentorial superficial siderosis of central nervous system (type Ⅰ 2 cases, type Ⅱ 1 case). The causes were neurilemmoma, traumatic dural defect or cerebrovascular malformation. According to the cause of the disease, the left C7 dural defect repair (Case 2) or left occipital lobe hematoma removal (Case 3) were performed. The patients' condition improved significantly and the headache disappeared. Conclusions The etiology of infratentorial superficial siderosis of central nervous system is heterogeneous. Detailed auxiliary examination is of great significance in avoiding misdiagnosis or missed diagnosis.

Keywords

• hemosiderosis
• central nervous system
• dura mater
• cerebrospinal fluid
• magnetic resonance imaging