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[1,5]. Imaging appearances of primary CNS lymphoma are highly variable. Findings on conventional CT scan and MRI are a function of its hypercellularity, high nuclear/cytoplasmic ratio, its predilection to perivascular and leptomeningeal regions, and disruption of the blood–brain barrier. Often CT scan findings are negative or non-diagnostic [6,7] and MRI remains the main method of diagnosis. Lymphomas are typically T1 hypointense or isointense and T2 isointense to hyperintense. Moderate to marked enhancement is shown following gadolinium contrast administration. Perilesional oedema is usually seen but is much less pronounced compared to malignant gliomas or metastases [8,9]. Calcification or haemorrhage within these lesions are exceptionally rare findings [10]. In the patient described here, linear enhancement radiating from the ependymal surface of the ventricle to the subcortical region proved to represent a subtype of lymphoma, namely primary CNS marginal zone B cell lymphoma. We suggest an imaging appearance of this ‘‘brain on fire’’ sign should alert clinicians to this potential diagnosis. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

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References [1] Park I, Huh J, Kim JH, et al. Primary central nervous system marginal zone B-cell lymphoma of the Basal Ganglia mimicking low-grade glioma: a case report and review of the literature. Clin Lymphoma Myeloma 2008;8:305–8. [2] King A, Wilson H, Penney C, et al. An unusual case of primary leptomeningeal marginal zone B cell lymphoma. Clin Neuropathol 1998;17:326–9. [3] Razaq W, Goel A, Amin A, et al. Primary central nervous system mucosaassociated lymphoid tissue lymphoma: case report and literature review. Clin Lymphoma Myeloma 2009;9:E5–9. [4] George AC, Ozsahin M, Janzer R, et al. Primary intracranial dural lymphoma of mucosa-associated lymphoid tissue (MALT) type: report of one case and review of the literature. Bull Cancer 2005;92:E51–6. [5] Papanicolau-Sengos A, Wang-Rodriguez J, Wang HY, et al. Rare case of a primary non dural central nervous system low grade B- cell lymphoma and literature review. Int J Clin Exp Pathol 2012;5:89–95. [6] Go JL, Lee SC, Kim PE. Imaging of primary central nervous system lymphoma. Neurosurg Focus 2006;21:E4. [7] Haldorsen IS, Krakenes J, Krossnes BK, et al. CT and MR imaging features of primary central system lymphoma in Norway, 1989–2003. AJNR Am J Neuroradiol 2009;30:744–51. [8] Haldorsen IS, Espeland A, Larson EM. Central nervous system lymphoma: characteristic findings on traditional and advanced imaging. AJNR Am J Neuroradiol 2011;32:984–92. [9] Johnson BA, Fram EK, Johnson PC, et al. The variable MR appearance of primary lymphoma of the central nervous system: comparison with histopathologic features. Am J Neuroradiol 1997;18:563–72. [10] Jahnke K, Thiel E, Schilling A, et al. Low-grade primary central nervous system lymphoma in immunocompetent patients. Br J Haematol 2005;128:616–24.

http://dx.doi.org/10.1016/j.jocn.2014.04.013

Superficial siderosis of the central nervous system secondary to spinal ependymoma Stylianos Pikis a, José E. Cohen a,b, Andres A. Vargas a, J. Moshe Gomori b, Sagi Harnof c, Eyal Itshayek a,⇑ a

Department of Neurosurgery, Hadassah-Hebrew University Medical Center, P.O. Box 12000, Jerusalem 91120, Israel Department of Radiology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel c Department of Neurosurgery, Sheba Medical Center, Tel Aviv University, Tel Hashomer, Israel b

a r t i c l e

i n f o

Article history: Received 26 May 2014 Accepted 30 May 2014

Keywords: Central nervous system Spinal ependymoma Superficial siderosis

a b s t r a c t Superficial siderosis of the central nervous system is a syndrome caused by deposition of hemosiderin in the subpial layers of the central nervous system, occurring as a result of recurrent asymptomatic or symptomatic bleeding into the subarachnoid space. We report a rare case of superficial siderosis in a 33-year-old man who presented with sensorineural hearing loss. The diagnosis of superficial siderosis on MRI brain studies led to further investigations with detection of a spinal ependymoma at L1–L2, compressing the cauda equina. Gross total resection of the tumor arrested the progression of the neurological deterioration. Our report underlies the importance of early diagnosis and surgical management, with imaging examination of the full neuroaxis to identify the source of bleeding, to halt disease progression and improve prognosis. Ó 2014 Elsevier Ltd. All rights reserved.

1. Introduction Superficial siderosis (SS) of the central nervous system (CNS) is a rare, slowly progressing, and potentially devastating syndrome occurring as a result of continuous or recurrent bleeding into the subarachnoid space, which may be asymptomatic. Repeated subarachnoid hemorrhage results in deposition of hemosiderin on layers of the CNS in close proximity to the cerebrospinal fluid. Hemosiderin is toxic to neural tissue and may cause parenchymal damage.

SSCNS affects males more often than females, and is typically characterized by sensorineural hearing loss, ataxia [1,2] and pyramidal signs [3]. Despite extensive laboratory, imaging, and clinical evaluations, the bleeding site remains unidentified in approximately half of patients [1–3]. We report a patient with SSCNS caused by clinically asymptomatic subarachnoid hemorrhage from a myxopapillary spinal ependymoma.

2. Case report ⇑ Corresponding author. Tel.: +972 2 677 7092; fax: +972 2 643 1740. E-mail address: [email protected] (E. Itshayek).

A 33-year-old previously healthy man experienced sudden onset of deafness in the left ear. An otorhinolaryngologic hearing

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Fig. 1. Axial T2⁄-weighted MRI in a 33-year-old man revealed hypo-intense rings in the cerebellar folds, the classic appearance of hemosiderin deposition.

examination was diagnostic for sensorineural hearing loss. Brain MRI was then performed, and was significant for linear hypointensities in the cerebellar folds on T2-weighted images, suggestive for SSCNS; however, no bleeding source was detected. The patient was subsequently referred to our outpatient neurosurgical clinic for further management. On direct questioning the patient reported an 18 month history of nocturnal back pain, worse in the morning, radiating to the lower limbs. His past medical and family history was otherwise unremarkable. His physical and neurological examination were significant only for left ear hearing loss and positive straight legraising test bilaterally. Review of the patient’s T2-weighted brain MRI confirmed significant areas of SS in the cerebellar folds (Fig. 1). Additional spinal T1-weighted MRI with gadolinium revealed a well-circumscribed, homogenously enhancing intradural space-occupying lesion at the L1–L2 levels compressing the cauda equina (Fig. 2). The patient underwent L1–L2 laminectomy with complete resection of the spinal tumor. The postoperative course was uneventful and he was discharged 4 days after surgery. Histopathologic examination of the resected tumor was diagnostic for a World Health Organization (WHO) grade I myxopapillary ependymoma. Following surgery, he had complete resolution of his back pain. At 2-year follow-up the patient remained stable neurologically. Follow-up otorhinolaryngologic evaluation showed arrest of the sensorineural hearing loss progression.

3. Discussion SSCNS is a rare, slowly progressing, and potentially devastating syndrome occurring as a result of continuous or recurrent bleeding into the subarachnoid space. The most common causes include ruptured aneurysms, bleeding of unknown etiology, arteriovenous malformation and traumatic injury; however, more unusual sources of chronic bleeding include dural pathologies and tumors [2–4]. Resulting deposition of hemosiderin in the subpial layers of the CNS may cause parenchymal damage. We report an unusual

Fig. 2. Sagittal T1-weighted spine MRI with gadolinium revealed a homogenously enhancing space-occupying lesion with spinal cord compression at L1–L2, characteristic for ependymoma.

case of SSCNS resulting from a previously undiagnosed spinal ependymoma. To the best of our knowledge, only six cases of SSCNS associated with spinal ependymoma have been reported [5–10].

Case Reports / Journal of Clinical Neuroscience 21 (2014) 2017–2019

SSCNS is typically characterized by sensorineural hearing loss, ataxia [1,2], and pyramidal signs [3] mimicking spinocerebellar degeneration. Other SSCNS manifestations may include sensory and cognitive disturbances, seizures, headache, urinary symptoms, visual and olfactory complaints [2], anisocoria, lumbar backache, bilateral sciatica (10%) and neck pain, extra-oculomotor nerve palsy, and lower motor neuron signs with muscle wasting [3]. In their 1995 review, Fearnley et al. [3] described SSCNS secondary to a variety of dural pathologies, CNS tumors, and vascular anomalies. Kumar et al. [2] identified a history of trauma as a possible cause of SSCN in 17 of 22 patients in their series. MRI evaluation of the entire neuraxis to diagnose SSCNS and to identify a potential source of bleeding is recommended [5]. The characteristic presentation of hemosiderin deposits are best seen on T2-weighted and T2-gradient echo imaging, where they appear as marginal hypointense signal around the cerebellum, brain stem, the Sylvian and interhemispheric fissures, and cortical sulci [1,2]. The hypointensity results from magnetic susceptibility effects of the blood degradation products ferritin and hemosiderin. Cerebellar and spinal cord atrophy may be seen; however, the peripheral nervous system is not affected [1]. Optimal management of SS remains to be determined. Successful surgical management depends on early diagnosis of SSCNS with identification and ablation of the bleeding source before permanent CNS damage has occurred. In accordance with our report, arrest of SS symptoms following surgical excision of a spinal ependymoma has been described [9]. Myxopapillary ependymoma is a rare, WHO grade I ependymoma occurring predominantly in the lumbosacral region, particularly the filum terminale. On MRI, ependymomas without capsular rupture appear as well-circumscribed masses, which are hypointense on T1-weighted and hyperintense on T2-weighted MRI. Homogenous enhancement following gadolinium administration distinguishes ependymomas from neurinomas of the cauda equina, which enhance heterogeneously [11]. 4. Conclusion SS is a rare, slowly progressive condition with potentially severe clinical sequelae. SSCNS manifestations vary and presentation may mimic a variety of neurological and otorhinolaryngological diseases; thus, clinicians should be aware of and include SSCNS in the differential diagnosis of cerebellar-pyramidal syndromes. Optihttp://dx.doi.org/10.1016/j.jocn.2014.05.020

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mal management remains to be determined. However, early diagnosis with identification and surgical ablation of the bleeding source appears to halt disease progression, thus improving the prognosis of the patient.

Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Acknowledgements The authors wish to thank Shifra Fraifeld, a research associate and senior medical writer at the Hadassah-Hebrew University Medical Center, for her careful review and editing of this case report. References [1] Kumar N. Superficial siderosis: associations and therapeutic implications. Arch Neurol 2007;64:491–6. [2] Kumar N, Cohen-Gadol AA, Wright RA, et al. Superficial siderosis. Neurology 2006;66:1144–52. [3] Fearnley JM, Stevens JM, Rudge P. Superficial siderosis of the central nervous system. Brain 1995;118:1051–66. [4] Steinberg J, Cohen JE, Gomori JM, et al. Superficial siderosis of the central nervous system due to chronic hemorrhage from a giant invasive prolactinoma. J Clin Neurosci 2013;20:1032–4. [5] Friedman DP, Hollander MD. Neuroradiology case of the day. Myxopapillary ependymoma of the conus medullaris or filum terminale resulting in superficial siderosis and dissemination of tumor along CSF pathways. Radiographics 1998;18:794–8. [6] Grech R, Galvin L, Looby S, et al. Spinal ependymoma complicated by superficial siderosis. BMJ Case Rep 2013;2013. [7] Lemmerling M, De Praeter G, Mollet P, et al. Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. Neuroradiology 1998;40:312–4. [8] Messori A, Di Bella P, Herber N, et al. The importance of suspecting superficial siderosis of the central nervous system in clinical practice. J Neurol Neurosurg Psychiatry 2004;75:188–90. [9] Spengos K, Vassilopoulou S, Tsivgoulis G, et al. Superficial siderosis due to a lumbar ependymoma mimicking adult-onset spinocerebellar ataxia. Clin Neurol Neurosurg 2007;109:705–7. [10] Vibert D, Häusler R, Lövblad KO, et al. Hearing loss and vertigo in superficial siderosis of the central nervous system. Am J Otolaryngol 2004;25:142–9. [11] Nakamura M, Ishii K, Watanabe K, et al. Long-term surgical outcomes for myxopapillary ependymomas of the cauda equina. Spine (Phila Pa 1976) 2009;34:E756–60.

Superficial siderosis of the central nervous system secondary to spinal ependymoma.

Superficial siderosis of the central nervous system is a syndrome caused by deposition of hemosiderin in the subpial layers of the central nervous sys...
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