Case Report Spinal Ependymoma Presenting As Subarachnoid Hemorrhage and Leading to Superficial Siderosis of the Central Nervous System: a Case Report
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Int J Clin Exp Med 2017;10(8):12712-12716 www.ijcem.com /ISSN:1940-5901/IJCEM0056596 Case Report Spinal ependymoma presenting as subarachnoid hemorrhage and leading to superficial siderosis of the central nervous system: a case report Jingzhe Han1, Ye Ji1, Duanhua Cao1, Zhilei Kang2, Jianguo Zhu1 Departments of 1Neurology, 2MRI, Harrison International Peace Hospital, Hengshui, Hebei, China Received May 2, 2017; Accepted July 20, 2017; Epub August 15, 2017; Published August 30, 2017 Abstract: Superficial siderosis of the central nervous system is a very uncommon disorder. We report a rare case of spinal ependymoma in a 58-year-old man whose main complaints at presentation were recent lumbago and head- ache, and he was evaluated to confirm suspected spinal subarachnoid hemorrhage (SAH). After cerebrospinal fluid analysis and imaging studies the patient was diagnosed with SAH and superficial siderosis of the central nervous system (SSCNS). Following enhanced MRI scans and surgery the final diagnosis was spinal ependymoma with SAH and SSCNS. This is unusual case of SSCNS due to repeated small hemorrhage of a spinal ependymoma presenting as acute SAH. This case report provides a new factual basis for the pathogenesis of SSCNS. Keywords: Subarachnoid hemorrhage, siderosis, ependymoma, magnetic resonance imaging, case report Introduction nerve root irritation. The rates of missed and misdiagnosed spinal SAH are high, especially Superficial siderosis of the central nervous sys- for patients showing no symptoms of spinal tem (SSCNS) is a rare and slowly progressing cord damage or patients testing positive only disease which result by the hemosiderin depo- for meningeal irritation, both of which could be sition derived from continuous or recurrent easily confused with intracranial SAH. It is bleeding into the subarachnoid space [1, 2]. reported that about 80% of spinal SAH patients Hemoglobin iron is deposited on the subpial had cerebral symptoms, with symptoms of layers which results in an increase of microglia headache accounting for 70% of these symp- and injury to nerve cells which can lead to cer- toms, and about 22% of patients showing ebellar ataxia, sensorineural deafness, dem- symptoms of consciousness change [8]. entia, pyramidal signs, bladder disturbance, seizures, anosmia, and aniscoria [1-5]. The rup- Spinal ependymoma is a rare ependymoma tured aneurysms, arteriovenous malformation, occurring predominantly in the lumbosacral and traumatic injury are the main causes of region, particularly the filum terminale. In rare SSCNS; In addition, there are more unusual cases spinal ependymoma has been found to sources of chronic bleeding, such as dural be the cause of SAH [9-11]. We report here an pathologies, tumors, and surgery [1, 4, 5]. unusual case of a patient with spinal ependy- moma presenting as SAH which led to SSCNS. Subarachnoid hemorrhage (SAH) is a clinical Case report syndrome that can be caused by bleeding from an arteriovenous malformation or bleeding This study was approved by Harrison Inter- from the rupture of a cerebral aneurysm direct- national Peace Hospital. Informed consent was ly into the subarachnoid cavity [6]. SAH of spi- obtained from all individual participants includ- nal origin is a rare clinical entity, occurring in ed in the study. only 0.05%-0.6% cases [7]. It has characteristic clinical manifestations such as headaches, The patient who had experienced lumbago for 7 unconsciousness, back pain, and symptoms of days was accompanied by headache, nausea, Spinal ependymoma leading to superficial siderosis of CNS fold were symmetrical, and his tongue was in the center. Mu- scle strength and muscular tension of the limbs were nor- mal. The bilateral tendon re- flexes existed. The bilateral Babinski sign was negative, and neck resistance was sus- pected to be positive. Body examination revealed bilateral mild ataxia. Brain CT indicated that the intensity of the ante- rior median fissure was incr- eased, and SAH could not be excluded. There was no abnor- mality found based on the result of laboratory tests of blood, urine, and feces. Lum- bar puncture was performed and it was found that unifo- rmly and consistently bloody cerebrospinal fluid (CSF) was discharged, and the measured pressure of CSF was greater than 300 mm H2O. Furtherm- ore, measurements of pres- Figure 1. Brain MRI on T2W1 demonstrated short T2 signals in the bilateral sure in the neck and abdomen dentate nuclei, midbrain roof, and lateral fissure (A-C); SWI showed low sig- were negative. The biochemis- nal in the bilateral dentate nuclei, midbrain roof, and lateral fissure. There- try of CSF was as follows: total fore, it was considered to be the deposition of hemosiderin (D-F). There was protein more than 3358 mg/L; no evidence of acute SAH found on FLAIR (G-I). total number of erythrocytes 188.0×1012/L. MRI, magnet- and vomiting for 4 days. Seven days previously, ic resonance angiography (MRA), susceptibility- the lumbago occurred when the patient worked weighted imaging (SWI) and magnetic reso- in a field, but he had no headache, nausea, nance venongraphy (MRV) of the brain showed: vomiting, or limb movement disorder. Thus, his no acute SA H, widening of the longitudinal backache did not receive special attention. fissure, schizencephaly remove and subarach- However, three days later the backache was noid space of the cerebellum with blurred exacerbated and headache occurred, mainly at edges. Therefore, the findings were considered the bilateral orbital and temporal sites. Then, to indicate the deposition of hemosiderin; no the headache was gradually aggravated and abnormality was found on MRA and MRV was accompanied by nausea and vomiting, (Figure 1). Spinal MRI showed an occupying which led him to visit our hospital. The patient lesion in the cone region at the L2 level. MRI had a 20-year history of waist placeholder sur- after contrast agent injection showed the gery and often suffered from lumbago. He also abnormal enhanced shadow of a strip in L1-2 had a medical history of 5-6 years of walking vertebral canals, but the boundary was clear instability and hearing loss. The results of phys- (Figure 2A-C). The imaging diagnosis was spi- ical examination showed that the patient was nal ependymoma associated to spinal SAH and conscious and had fluent speech. However, the SSCNS. Occupying lesions were removed by patient had hearing loss. His bilateral pupils surgery. During surgery, it was found that the were equally round and normal in size, were blood supply in the tumor was rich and closely sensitive to light reflex, and his eyes moved attached to nerves. Histopathologic examina- freely. Horizontal and small nystagmus was vis- tion of the resected tumor was diagnostic for a ible. The bilateral frontal lines and nasolabial World Health Organization (WHO) grade II ep- 12713 Int J Clin Exp Med 2017;10(8):12712-12716 Spinal ependymoma leading to superficial siderosis of CNS discharged. The patient was then diagnosed with SAH. In order to further investigate the etiology of SAH, MRI+ MRA+SWI examination of the brain was performed. The im- ages obtained showed the low signal of T2-weighted imaging (T2WI) in the bilateral dentate nuclei of the cerebellum, lon- gitudinal fissure, and lateral fissure, as well as the SWI sig- nal. Hence, it was considered to indicate the deposition of hemosiderin. MRI is an effec- tive method for diagnosis of SSCNS [2]: T2WI reveals a ch- aracteristic linear hypointen- sity due to hemosiderin de- position that is in contrast to Figure 2. Lumbar vertebrae MRI shows occupying lesions occurred in the cone region of L2 level spinal cord, with low signal of T1 (A) and mixed T2 the hyperintense signals of signal (B), while the enhanced scanning showed the abnormal enhanced the CSF [12]. Compared with shadow of a strip in L1-2 vertebrae , and the boundary was clear (red arrow) T2WI, SWI is more sensitive (C). Histopathologic examination of the specimen confirmed a World Health to hemosiderin and can more Organization (WHO) grade II ependymoma (magnification ×200, D). accurately detect hypointense signals in the brain [4], which endymoma (Figure 2D). Following surgery, the provides more valuable imaging information patient’s back pain completely resolved. At 6 for the diagnosis of SSCNS. Therefore, SWI and month follow-up he remained stable neurologi- T2WI gradient echo are often used to diagnose cally. Follow-up otorhinolaryngologic evaluation SSCNS. This is consistent with the results we showed arrest of the sensorineural hearing loss observed by MRI, and combined with the symp- progression. toms and signs of the patient, the diagnosis of SSCNS was clear. Most studies have shown Discussion that for the diagnosis of acute SAH, MRI fluid attenuation inversion recovery (FLAIR) and SWI In this case report we described a patient with sequences are more sensitive than CT for lumbar ependymoma which not only led to clini- detecting traces of bleeding, and can be used cally asymptomatic spinal SAH but also result- as a supplementary means of diagnosis in ed in SSCNS. CT-based examination for acute SAH [13]. Although the patient had complained of head- The main clinical presentation of SSCNS is ache, nausea, vomiting, and meningismus, SWI including sensorineural hearing loss, pyramidal and FLAIR did not provide the evidence for signs, cerebella ataxia, and sensory and cogni- acute SAH. Head CT showing high density in the tive disturbances, seizures, headache, urinary anterior median fissure indicated the deposi- symptoms, visual and olfactory complaints. Ex- tion of hemosiderin, so the patient was con- cept above, anisocoria, lumbar backache, bilat- firmed to have SAH. Cerebral screening could eral sciatica and neck pain, and extra-oculomo- not demonstrate the particular cause of SAH. tor nerve palsy, are also typically clinical feature Since our patient reported that he had back of SSCNS [1-5]. pain before the onset, we were alert to the pos- sibility of spinal SAH.