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CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 4  |  Page : 894-896

Superficial Siderosis of the Central Nervous System Caused by Glioneuronal Tumor: A Case Report and Literature Review


1 Department of Neurosurgery, Fujita Health University, Aichi, Japan
2 Department of Neurosurgery, Fujita Health University, Toyoake, Aichi, Japan
3 Department of Diagnostic Radiology, Fujita Health University, Toyoake, Aichi, Japan
4 Department of Diagnostic Pathology, Fujita Health University, Toyoake, Aichi, Japan
5 Department of Pathology, School of Health Sciences, Fujita Health University, Toyoake, Aichi, Japan

Date of Web Publication26-Aug-2020

Correspondence Address:
Dr. Shigeo Ohba
Department of Neurosurgery, Fujita Health University School of Medicine, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.293458

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 » Abstract 


Superficial siderosis is a rare disease resulting from the deposited hemosiderin owing to repeated subarachnoid hemorrhage. It has been reported that hemosiderin deposits on the brain surface and brain parenchyma causes nerve disorder, resulting in progressive and irreversible hearing loss, cerebellar ataxia and pyramidal disorder. The brain tumor is one of the cause of superficial siderosis. A 16-year-old female present a nearby hospital with complaints of absence seizure. A magnetic resonance imaging (MRI) revealed a heterogeneously enhanced mass at the right temporal lobe. Susceptibility-weighted imaging revealed diffuse and extensive superficial siderosis on the brain surface. The tumor was gross totally removed and diagnosed as glioneuronal tumor. The patient had been well, although susceptibility-weighted imaging performed one year after the surgery showed superficial siderosis remained. Early diagnosis and prevention of bleeding sources are recommended to prevent symptom progression associated with superficial siderosis. Susceptibility-weighted imaging is considered useful for early detection of superficial siderosis.


Keywords: Glioneuronal tumor, hemosiderin, magnetic resonance imaging, superficial siderosis, susceptibility-weighted image
Key Messages: Superficial siderosis (SS) is a rare entity and brain tumors are commonly reported etiology for SS. The patients report irreversible hearing loss, cerebellar ataxia and Pyramidal symptoms. Ependymomas are among the commonest tumors causing SS. Susceptibility weighted imaging (SWI) is essential in detecting the iron deposits and diagnosis of SS. Early recognition and prompt treatment of the primary cause leads to a better outcome.


How to cite this article:
Teranishi T, Ohba S, Kawazoe Y, Adachi K, Murayama K, Yamada S, Abe M, Hasegawa M, Hirose Y. Superficial Siderosis of the Central Nervous System Caused by Glioneuronal Tumor: A Case Report and Literature Review. Neurol India 2020;68:894-6

How to cite this URL:
Teranishi T, Ohba S, Kawazoe Y, Adachi K, Murayama K, Yamada S, Abe M, Hasegawa M, Hirose Y. Superficial Siderosis of the Central Nervous System Caused by Glioneuronal Tumor: A Case Report and Literature Review. Neurol India [serial online] 2020 [cited 2020 Oct 26];68:894-6. Available from: https://www.neurologyindia.com/text.asp?2020/68/4/894/293458




Superficial siderosis is a rare disease resulting from hemosiderin deposition owing to repeated subarachnoid hemorrhage.[1],[2] It has been reported that excess intrathecal iron due to hemosiderin stored in the brain surface or brain parenchyma may overload ferritin synthesis. The subsequent free radicals and lipid peroxidation cause neuronal injury.[2] Progressive and irreversible sensorineural hearing loss, cerebellar ataxia, and pyramidal disorder are the characteristic symptoms of superficial siderosis.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11] Tumor, vascular malformation, trauma, subarachnoid hemorrhage, and amyloid angiopathy have been reported as the causes of superficial siderosis.[2],[5],[6],[7],[10],[11] Susceptibility-weighted imaging (SWI), which utilizes magnetic susceptibility artifact owing to the magnetic body components, such as iron and blood products in the tissues, has been useful in detecting hemorrhage.[12] We present a case of glioneuronal tumor with superficial siderosis and a review of literature and discuss the usefulness of SWI.


 » Case Report Top


A 16-year-old female with no past medical history presented in a nearby medical clinic with complaints of absence seizure. Magnetic resonance imaging (MRI) revealed intracranial tumor. She was referred to our hospital for further examinations. The patient did not present with any symptoms at the admission. Computed tomography revealed a lesion of approximately 4 cm with calcification in the right temporal lobe. The lesion showed low intensity on T1-weighted imaging and high intensity on T2-weighted imaging. Moreover, there was no signal change suggesting superficial siderosis on T2-weighted imaging [Figure 1]a and b]. T1-weighted gadolinium-enhanced MRI revealed that the mass was heterogeneously enhanced [Figure 1]c. SWI revealed a low-intensity signal area on the cerebellar and cerebral hemispheric surfaces, indicating extensive hemosiderin deposition [Figure 1]d and [Figure 1]e.
Figure 1: (a and b) T2-weighted imaging revealed a low intensity mass. and there was no signal change suggesting superficial siderosis. (c) T1-weighted gadolinium-enhanced magnetic resonance imaging revealed a heterogeneously enhanced mass on the right temporal lobe. (d and e) Susceptibility-weighted imaging revealed low-intensity signal areas in the cerebellar and cerebral hemispheric brain surfaces, indicating extensive hemosiderin deposition

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The patient underwent operation for the removal of the tumor. The tumor had yellowish and brownish pigmentation under the arachnoid membrane, where hemosiderin deposition was suspected. The tumor was immunopositive for glial fibrillary acidic protein. Neuropil cores were positive for synaptophysin. The histopathological diagnosis was glioneuronal tumor. Furthermore, Berlin blue staining revealed iron deposition [Figure 2].
Figure 2: Berlin blue staining revealed iron deposition mainly in the brain surface

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Post-operative MRI revealed that the tumor was grossly and totally removed. MRI performed one year after the surgery did not reveal tumor recurrence. However, compared with preoperative MRI, MRI performed one year after the surgery revealed that superficial siderosis remained unchanged [Figure 3]a and [Figure 3]b. The patient did not experience any symptoms one year after the surgery.
Figure 3: (a and b) Compared with the preoperative susceptibility-weighted imaging (SWI), SWI performed 1 year after surgery revealed that superficial siderosis remained unchanged

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 » Discussion Top


As shown in our case, brain tumor is a cause of superficial siderosis. A total of 46 cases of superficial siderosis associated with tumors have been reported,[1],[7] Among them, 21 cases were ependymoma, accounting for approximately half of the cases; only one case related to glioneuronal tumor has been reported.[5] The common symptoms of superficial siderosis were as follows: progressive and irreversible sensorineural hearing loss, cerebellar ataxia, and pyramidal disorder.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11] However, our case did not present any symptoms of superficial siderosis.

We reviewed a total of 11 cases, whose outcomes have been described in the literature [Table 1]. Gross total resection (GTR) was performed in 8 cases, whereas subtotal resection (STR) was performed in 3 cases. Considering the correlation between the extent of resection and clinical outcome, symptoms of superficial siderosis did not worsen in all cases who underwent GTR, whereas the symptoms progressed in 2 cases who underwent STR without additional radiotherapy. Superficial siderosis due to a tumor is considered to be caused by hemosiderin deposition due to repeated hemorrhage from the tumor; therefore, the presence of residual tumor was suggested to affect the progression of symptoms.[1],[10] Even in cases who underwent GTR, symptoms were not improved in most cases. Because the symptoms caused by superficial siderosis are irreversible, in case where superficial siderosis is due to a tumor, it is important to detect and eliminate bleeding sources as soon as possible.[2],[5],[9],[13]
Table 1: Outcome of superficial siderosis caused by tumors

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In previous reports, T2-weighted imaging was used to diagnose superficial siderosis.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] However, in our case, there was no signal change suggesting superficial siderosis in the preoperative T2-weighted imaging. However, preoperative SWI revealed low signal intensity on the cerebellar and cerebral hemispheric brain surfaces, suggesting extensive hemosiderin deposition. SWI could detect minor superficial siderosis at an early stage, which could not be detected using conventional T2-weighted imaging. This may be the reason why the patient was asymptomatic although she still had superficial siderosis.

In conclusion, to prevent the exacerbation of symptoms associated with superficial siderosis, early diagnosis and elimination of bleeding sources are recommended. SWI was useful for the diagnosis of superficial siderosis at an early stage.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Espinosa Rodríguez EE, Moro RC, Martínez San Millán JS, Pian Arias HG. Rare association of secondary superficial siderosis caused by a fourth ventricle hemorrhage ependymoma mimicking a cavernoma: Case report and literature review. Surg Neurol Int 2017;68:14.  Back to cited text no. 1
    
2.
Kumar N Superficial siderosis: Associations and therapeutic implications. Arch Neurol 2007;64:491-6.  Back to cited text no. 2
    
3.
Elalaoui A, Bozorg Grayeli A, Bouccara D, Ambert-Dahan E, Cyna-Gorse F, Krainik A, et al. Progressive bilateral hearing loss with superficial hemosiderosis of the central nervous system: Contribution of cochlear implantation. Ann Otolaryngol Chir Cervicofac 2003;120:225-30.  Back to cited text no. 3
    
4.
Grech R, Galvin L, Looby S, Thornton J. Spinal ependymoma complicated by superficial siderosis. BMJ Case Rep pii: bcr 2013; 2013-201036.  Back to cited text no. 4
    
5.
Konya D, Peker S, Ozgen S, Kurtkaya O, Necmettin Pamir M. Superficial siderosis due to papillary glioneuronal tumor. J Clin Neurosci 2006;13:950-2.  Back to cited text no. 5
    
6.
Matsumoto S, Kang Y, Sato S, Kawakami Y, Oda Y, Araki M, et al. Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 1998;88:890-4.  Back to cited text no. 6
    
7.
Nogales Romo MT, Cuadrado ML, Matamoros P, Porta-Etessam J. Superficial siderosis of the central nervous system as a presentation of stage IV melanoma. Neurologia 2016;31:132-4.  Back to cited text no. 7
    
8.
Pikis S, Cohen JE, Vargas AA, Gomori JM, Harnof S, Itshayek E. Superficial siderosis of the central nervous system secondary to spinal ependymoma. J Clin Neurosci 2014;21:2017-9.  Back to cited text no. 8
    
9.
Spengos K, Vassilopoulou S, Tsivgoulis G, Karachalios G, Vassilopoulos D. Superficial siderosis due to a lumbar ependymoma mimicking adult-onset spinocerebellar ataxia. Clin Neurol Neurosurg 2007;109:705-7.  Back to cited text no. 9
    
10.
Tosaka M, Sato K, Amanuma M, Higuchi T, Arai M, Aishima K, et al. Superficial siderosis of the central nervous system caused by hemorrhagic intraventricular craniopharyngioma: Case report and literature review. Neurol Med Chir 2015;55:89-94.  Back to cited text no. 10
    
11.
Vibert D, Häusler R, Lövblad KO, Schroth G. Hearing loss and vertigo in superficial siderosis of the central nervous system. Am J Otolaryngol 2004;25:142-9.  Back to cited text no. 11
    
12.
Schelhorn J, Gramsch C, Deuschl C, Quick HH, Nensa F, Moenninghoff C, et al. Intracranial hemorrhage detection over time using susceptibility-weighted magnetic resonance imaging. Acta Radiologica 2015;56:1501-7.  Back to cited text no. 12
    
13.
Lévêque M, Mc Laughlin N, Bojanowski MW. Secondary superficial siderosis of the central nervous system: Report of three cases. Neurochirurgie 2009;55:315-21.  Back to cited text no. 13
    


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