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Published in: Acta Neurochirurgica 10/2011

01-10-2011 | Review Article

Three cases of superficial siderosis of the central nervous system and review of the literature

Authors: Jussi P. Posti, Seppo Juvela, Riitta Parkkola, Susanna Roine

Published in: Acta Neurochirurgica | Issue 10/2011

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Abstract

Superficial siderosis of the central nervous system results from chronic or intermittent hemorrhage into the subarachnoid space that causes hemosiderin deposition in subpial layers of the brain and the spinal cord leading to neuronal damage. Patients present with progressive and debilitating symptoms that typically include adult-onset slowly progressive cerebellar gait ataxia and sensorineural hearing impairment. Regardless of extensive investigations, the origin of the hemorrhage is often not clear. Because of the good availability of magnetic resonance imaging, asymptomatic cases of superficial siderosis of the central nervous system are increasingly discovered. SS cases are increasingly reported in the literature. We present three new cases. The etiology, pathogenesis, clinical features, and treatment options of SS are reviewed.
Literature
1.
go back to reference Anderson NE, Sheffield S, Hope JK (1999) Superficial siderosis of the central nervous system: a late complication of cerebellar tumors. Neurology 52:163–169PubMed Anderson NE, Sheffield S, Hope JK (1999) Superficial siderosis of the central nervous system: a late complication of cerebellar tumors. Neurology 52:163–169PubMed
2.
go back to reference Angstwurm K, Schielke E, Zimmer C, Kivelitz D, Weber JR (2002) Superficial siderosis of the central nervous system: response to steroid therapy. J Neurol 249:1223–1225PubMedCrossRef Angstwurm K, Schielke E, Zimmer C, Kivelitz D, Weber JR (2002) Superficial siderosis of the central nervous system: response to steroid therapy. J Neurol 249:1223–1225PubMedCrossRef
3.
go back to reference Aoyama T, Hida K, Yano S, Kawabori M, Iwasaki M, Iwasaki Y (2009) Malpositioned spinal instrument as a possible cause of superficial siderosis. Spinal Cord 47:826–828PubMedCrossRef Aoyama T, Hida K, Yano S, Kawabori M, Iwasaki M, Iwasaki Y (2009) Malpositioned spinal instrument as a possible cause of superficial siderosis. Spinal Cord 47:826–828PubMedCrossRef
4.
go back to reference Aquilina K, Kumar R, Lu J, Rawluk D (2005) Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery. Acta Neurochir (Wien) 147:291–297, discussion 297CrossRef Aquilina K, Kumar R, Lu J, Rawluk D (2005) Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery. Acta Neurochir (Wien) 147:291–297, discussion 297CrossRef
5.
go back to reference Bonito V, Agostinis C, Ferraresi S, Defanti CA (1994) Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg 80:931–934PubMedCrossRef Bonito V, Agostinis C, Ferraresi S, Defanti CA (1994) Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg 80:931–934PubMedCrossRef
6.
go back to reference Bracchi M, Savoiardo M, Triulzi F, Daniele D, Grisoli M, Bradac GB, Agostinis C, Pelucchetti D, Scotti G (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR Am J Neuroradiol 14:227–236PubMed Bracchi M, Savoiardo M, Triulzi F, Daniele D, Grisoli M, Bradac GB, Agostinis C, Pelucchetti D, Scotti G (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR Am J Neuroradiol 14:227–236PubMed
7.
go back to reference Cheng CY, Chen MH, Wang SJ, Lin KP (2011) A proposed mechanism of superficial siderosis supported by surgical and neuroimaging findings. Med Hypotheses 76:823–826PubMedCrossRef Cheng CY, Chen MH, Wang SJ, Lin KP (2011) A proposed mechanism of superficial siderosis supported by surgical and neuroimaging findings. Med Hypotheses 76:823–826PubMedCrossRef
8.
go back to reference Cohen-Gadol AA, Krauss WE, Spinner RJ (2004) Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases. Neurosurg Focus 16:E10PubMedCrossRef Cohen-Gadol AA, Krauss WE, Spinner RJ (2004) Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases. Neurosurg Focus 16:E10PubMedCrossRef
9.
go back to reference Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118(Pt 4):1051–1066PubMedCrossRef Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118(Pt 4):1051–1066PubMedCrossRef
10.
go back to reference Gomori JM, Grossman RI, Bilaniuk LT, Zimmerman RA, Goldberg HI (1985) High-field MR imaging of superficial siderosis of the central nervous system. J Comput Assist Tomogr 9:972–975PubMedCrossRef Gomori JM, Grossman RI, Bilaniuk LT, Zimmerman RA, Goldberg HI (1985) High-field MR imaging of superficial siderosis of the central nervous system. J Comput Assist Tomogr 9:972–975PubMedCrossRef
11.
go back to reference Hamill RC (1908) Report of a case of melanosis of the brain, cord, and meninges. J Nerv Ment Dis 35:594CrossRef Hamill RC (1908) Report of a case of melanosis of the brain, cord, and meninges. J Nerv Ment Dis 35:594CrossRef
12.
go back to reference Hardy PA, Kucharczyk W, Henkelman RM (1990) Cause of signal loss in MR images of old hemorrhagic lesions. Radiology 174:549–555PubMed Hardy PA, Kucharczyk W, Henkelman RM (1990) Cause of signal loss in MR images of old hemorrhagic lesions. Radiology 174:549–555PubMed
13.
go back to reference Haroun RI, Li KW, Rigamonti D (2000) Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurol 53:554–558PubMedCrossRef Haroun RI, Li KW, Rigamonti D (2000) Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurol 53:554–558PubMedCrossRef
14.
go back to reference Hoxworth JM, Patel AC, Bosch EP, Nelson KD (2009) Localization of a rapid CSF leak with digital subtraction myelography. AJNR Am J Neuroradiol 30:516–519PubMedCrossRef Hoxworth JM, Patel AC, Bosch EP, Nelson KD (2009) Localization of a rapid CSF leak with digital subtraction myelography. AJNR Am J Neuroradiol 30:516–519PubMedCrossRef
15.
go back to reference Ikeda T, Noto D, Noguchi-Shinohara M, Ono K, Takahashi K, Ishida C, Yoshita M, Kawaguchi M, Kawahara N, Iwasa K, Tomita K, Yamada M (2010) CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: two case reports. Clin Neurol Neurosurg 112:62–64PubMedCrossRef Ikeda T, Noto D, Noguchi-Shinohara M, Ono K, Takahashi K, Ishida C, Yoshita M, Kawaguchi M, Kawahara N, Iwasa K, Tomita K, Yamada M (2010) CSF tau protein is a useful marker for effective treatment of superficial siderosis of the central nervous system: two case reports. Clin Neurol Neurosurg 112:62–64PubMedCrossRef
16.
go back to reference Janss AJ, Galetta SL, Freese A, Raps EC, Curtis MT, Grossman RI, Gomori JM, Duhaime AC (1993) Superficial siderosis of the central nervous system: magnetic resonance imaging and pathological correlation. Case report. J Neurosurg 79:756–760PubMedCrossRef Janss AJ, Galetta SL, Freese A, Raps EC, Curtis MT, Grossman RI, Gomori JM, Duhaime AC (1993) Superficial siderosis of the central nervous system: magnetic resonance imaging and pathological correlation. Case report. J Neurosurg 79:756–760PubMedCrossRef
17.
go back to reference Koeppen AH, Dentinger MP (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47:249–270PubMedCrossRef Koeppen AH, Dentinger MP (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47:249–270PubMedCrossRef
18.
go back to reference Koeppen AH, Dickson AC, Chu RC, Thach RE (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34:646–653PubMedCrossRef Koeppen AH, Dickson AC, Chu RC, Thach RE (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34:646–653PubMedCrossRef
19.
go back to reference Koeppen AH, Michael SC, Li D, Chen Z, Cusack MJ, Gibson WM, Petrocine SV, Qian J (2008) The pathology of superficial siderosis of the central nervous system. Acta Neuropathol 116:371–382PubMedCrossRef Koeppen AH, Michael SC, Li D, Chen Z, Cusack MJ, Gibson WM, Petrocine SV, Qian J (2008) The pathology of superficial siderosis of the central nervous system. Acta Neuropathol 116:371–382PubMedCrossRef
20.
go back to reference Kole MK, Steven D, Kirk A, Lownie SP (2004) Superficial siderosis of the central nervous system from a bleeding pseudomeningocele. Case illustration. J Neurosurg 100:718PubMedCrossRef Kole MK, Steven D, Kirk A, Lownie SP (2004) Superficial siderosis of the central nervous system from a bleeding pseudomeningocele. Case illustration. J Neurosurg 100:718PubMedCrossRef
21.
go back to reference Konya D, Peker S, Ozgen S, Kurtkaya O, Necmettin PM (2006) Superficial siderosis due to papillary glioneuronal tumor. J Clin Neurosci 13:950–952PubMedCrossRef Konya D, Peker S, Ozgen S, Kurtkaya O, Necmettin PM (2006) Superficial siderosis due to papillary glioneuronal tumor. J Clin Neurosci 13:950–952PubMedCrossRef
22.
go back to reference Kumar A, Aggarwal S, Willinsky R, TerBrugge KG (1993) Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery 32:455–457, discussion 457PubMedCrossRef Kumar A, Aggarwal S, Willinsky R, TerBrugge KG (1993) Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery 32:455–457, discussion 457PubMedCrossRef
23.
go back to reference Kumar N (2007) Superficial siderosis: associations and therapeutic implications. Arch Neurol 64:491–496PubMedCrossRef Kumar N (2007) Superficial siderosis: associations and therapeutic implications. Arch Neurol 64:491–496PubMedCrossRef
24.
go back to reference Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE (2006) Superficial siderosis. Neurology 66:1144–1152PubMedCrossRef Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE (2006) Superficial siderosis. Neurology 66:1144–1152PubMedCrossRef
25.
go back to reference Kumar N, Lane JI, Piepgras DG (2009) Superficial siderosis: sealing the defect. Neurology 72:671–673PubMedCrossRef Kumar N, Lane JI, Piepgras DG (2009) Superficial siderosis: sealing the defect. Neurology 72:671–673PubMedCrossRef
26.
go back to reference Kumar N, Miller GM, Piepgras DG, Mokri B (2010) A unifying hypothesis for a patient with superficial siderosis, low-pressure headache, intraspinal cyst, back pain, and prominent vascularity. J Neurosurg 113:97–101PubMedCrossRef Kumar N, Miller GM, Piepgras DG, Mokri B (2010) A unifying hypothesis for a patient with superficial siderosis, low-pressure headache, intraspinal cyst, back pain, and prominent vascularity. J Neurosurg 113:97–101PubMedCrossRef
27.
go back to reference Levy M, Llinas RH (2011) Deferiprone reduces hemosiderin deposits in the brain of a patient with superficial siderosis. AJNR, Am J Neuroradiol 32:E1CrossRef Levy M, Llinas RH (2011) Deferiprone reduces hemosiderin deposits in the brain of a patient with superficial siderosis. AJNR, Am J Neuroradiol 32:E1CrossRef
28.
go back to reference Levy M, Turtzo C, Llinas RH (2007) Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 3:54–58, quiz 59PubMedCrossRef Levy M, Turtzo C, Llinas RH (2007) Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 3:54–58, quiz 59PubMedCrossRef
29.
go back to reference Lewey FH, Govons SR (1942) Hemochromatotic pigmentation of the central nervous system. J Neuropathol Exp Neurol 129–138 Lewey FH, Govons SR (1942) Hemochromatotic pigmentation of the central nervous system. J Neuropathol Exp Neurol 129–138
30.
go back to reference Matsumoto S, Kang Y, Sato S, Kawakami Y, Oda Y, Araki M, Kawamura J, Uchida H (1998) Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 88:890–894PubMedCrossRef Matsumoto S, Kang Y, Sato S, Kawakami Y, Oda Y, Araki M, Kawamura J, Uchida H (1998) Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 88:890–894PubMedCrossRef
31.
go back to reference Messori A, Di Bella P, Herber N, Logullo F, Ruggiero M, Salvolini U (2004) The importance of suspecting superficial siderosis of the central nervous system in clinical practice. J Neurol Neurosurg Psychiatry 75:188–190PubMedCrossRef Messori A, Di Bella P, Herber N, Logullo F, Ruggiero M, Salvolini U (2004) The importance of suspecting superficial siderosis of the central nervous system in clinical practice. J Neurol Neurosurg Psychiatry 75:188–190PubMedCrossRef
32.
go back to reference Miliaras G, Bostantjopoulou S, Argyropoulou M, Kyritsis A, Polyzoidis K (2006) Superficial siderosis of the CNS: report of three cases and review of the literature. Clin Neurol Neurosurg 108:499–502PubMedCrossRef Miliaras G, Bostantjopoulou S, Argyropoulou M, Kyritsis A, Polyzoidis K (2006) Superficial siderosis of the CNS: report of three cases and review of the literature. Clin Neurol Neurosurg 108:499–502PubMedCrossRef
33.
go back to reference Noguchi K, Seto H, Kamisaki Y, Tomizawa G, Toyoshima S, Watanabe N (2000) Comparison of fluid-attenuated inversion-recovery MR imaging with CT in a simulated model of acute subarachnoid hemorrhage. AJNR Am J Neuroradiol 21:923–927PubMed Noguchi K, Seto H, Kamisaki Y, Tomizawa G, Toyoshima S, Watanabe N (2000) Comparison of fluid-attenuated inversion-recovery MR imaging with CT in a simulated model of acute subarachnoid hemorrhage. AJNR Am J Neuroradiol 21:923–927PubMed
34.
go back to reference Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G (1996) Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 38(Suppl 1):S51–S56PubMedCrossRef Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G (1996) Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 38(Suppl 1):S51–S56PubMedCrossRef
35.
go back to reference Payer M, Sottas C, Bonvin C (2010) Superficial siderosis of the central nervous system: secondary progression despite successful surgical treatment, mimicking amyotrophic lateral sclerosis. Case report and review. Acta Neurochir (Wien) 152:1411–1416CrossRef Payer M, Sottas C, Bonvin C (2010) Superficial siderosis of the central nervous system: secondary progression despite successful surgical treatment, mimicking amyotrophic lateral sclerosis. Case report and review. Acta Neurochir (Wien) 152:1411–1416CrossRef
36.
go back to reference Revesz T, Earl CJ, Barnard RO (1988) Superficial siderosis of the central nervous system presenting with longstanding deafness. J R Soc Med 81:479–481PubMed Revesz T, Earl CJ, Barnard RO (1988) Superficial siderosis of the central nervous system presenting with longstanding deafness. J R Soc Med 81:479–481PubMed
37.
go back to reference River Y, Honigman S, Gomori JM, Reches A (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9:559–562PubMedCrossRef River Y, Honigman S, Gomori JM, Reches A (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9:559–562PubMedCrossRef
38.
go back to reference Schievink WI, Apostolides PJ, Spetzler RF (1998) Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg 89:1029–1031PubMedCrossRef Schievink WI, Apostolides PJ, Spetzler RF (1998) Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg 89:1029–1031PubMedCrossRef
39.
go back to reference Shih P, Yang BP, Batjer HH, Liu JC (2009) Surgical management of superficial siderosis. Spine J 9:e16–e19PubMedCrossRef Shih P, Yang BP, Batjer HH, Liu JC (2009) Surgical management of superficial siderosis. Spine J 9:e16–e19PubMedCrossRef
40.
go back to reference Tacconi L, Marinella T (1999) Superficial siderosis of the central nervous system secondary to a thalamic hamartoma. J Clin Neurosci 6:532–535PubMedCrossRef Tacconi L, Marinella T (1999) Superficial siderosis of the central nervous system secondary to a thalamic hamartoma. J Clin Neurosci 6:532–535PubMedCrossRef
41.
go back to reference Tapscott SJ, Eskridge J, Kliot M (1996) Surgical management of superficial siderosis following cervical nerve root avulsion. Ann Neurol 40:936–940PubMedCrossRef Tapscott SJ, Eskridge J, Kliot M (1996) Surgical management of superficial siderosis following cervical nerve root avulsion. Ann Neurol 40:936–940PubMedCrossRef
42.
go back to reference van Harskamp NJ, Rudge P, Cipolotti L (2005) Cognitive and social impairments in patients with superficial siderosis. Brain 128:1082–1092PubMedCrossRef van Harskamp NJ, Rudge P, Cipolotti L (2005) Cognitive and social impairments in patients with superficial siderosis. Brain 128:1082–1092PubMedCrossRef
43.
go back to reference Vernooij MW, Ikram MA, Hofman A, Krestin GP, Breteler MM, van der Lugt A (2009) Superficial siderosis in the general population. Neurology 73:202–205PubMedCrossRef Vernooij MW, Ikram MA, Hofman A, Krestin GP, Breteler MM, van der Lugt A (2009) Superficial siderosis in the general population. Neurology 73:202–205PubMedCrossRef
44.
go back to reference Wang K, Xu Z, Xiong G, Benyan L (2010) Superficial siderosis of the central nervous system manifested with seizures. J Clin Neurosci 17:277–278PubMedCrossRef Wang K, Xu Z, Xiong G, Benyan L (2010) Superficial siderosis of the central nervous system manifested with seizures. J Clin Neurosci 17:277–278PubMedCrossRef
Metadata
Title
Three cases of superficial siderosis of the central nervous system and review of the literature
Authors
Jussi P. Posti
Seppo Juvela
Riitta Parkkola
Susanna Roine
Publication date
01-10-2011
Publisher
Springer Vienna
Published in
Acta Neurochirurgica / Issue 10/2011
Print ISSN: 0001-6268
Electronic ISSN: 0942-0940
DOI
https://doi.org/10.1007/s00701-011-1116-0

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