Skip to main content
Erschienen in: Der Nervenarzt 6/2004

01.06.2004 | Ergebnisse und Kasuistik

Superfizielle Siderose des ZNS—2 Kasuistiken und eine Übersicht über die Literatur

verfasst von: Dr. A. Hug, M. Hartmann, A. Grau

Erschienen in: Der Nervenarzt | Ausgabe 6/2004

Einloggen, um Zugang zu erhalten

Zusammenfassung

Die superfizielle Siderose des ZNS ist ein seltenes Krankheitsbild, bei dem es durch chronisch rezidivierende Subarachnoidalblutungen (SAB) zu oberflächlichen Ablagerungen von Hämosiderin an Großhirn, Kleinhirn und Rückenmark kommt. Bekannte Blutungsquellen sind ZNS-Tumoren, Gefäßmalformationen, Nervenwurzelläsionen und neurochirurgische Eingriffe. Der Blutungsquellennachweis gelingt nur in etwa 50% der Fälle. Klinisch kommt es zu progredientem sensoneuralem Hörverlust, zerebellärer Ataxie und Pyramidenbahnläsionen. Im Liquor können Ferritin und freies Eisen erhöht sein. Die kraniale und spinale Kernspintomographie erlaubt den sensitiven und spezifischen Nachweis der Hämosiderinablagerungen. Einzig die Elimination der Blutungsquelle scheint die Progression der meist irreversiblen Symptomatik verhindern zu können. Daher sollte frühzeitig und gründlich nach der Blutungsquelle gesucht werden. Es werden 2 Kasuistiken vorgestellt und eine Übersicht über die Literatur gegeben.
Literatur
1.
Zurück zum Zitat Iwanowski L, Olszewski J (1960) The effects of subarachnoid injections of iron-containing substances on the central nervous system. J Neuropathol Exp Neurol 19: 433–448 Iwanowski L, Olszewski J (1960) The effects of subarachnoid injections of iron-containing substances on the central nervous system. J Neuropathol Exp Neurol 19: 433–448
2.
Zurück zum Zitat Koeppen AH, Dickson AC, Chu RC, Thach RE. (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34: 646–653PubMed Koeppen AH, Dickson AC, Chu RC, Thach RE. (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34: 646–653PubMed
3.
Zurück zum Zitat Koeppen AH, Hurwitz CG, Dearborn RE et al. (1992) Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 112: 38–45CrossRefPubMed Koeppen AH, Hurwitz CG, Dearborn RE et al. (1992) Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 112: 38–45CrossRefPubMed
4.
Zurück zum Zitat Koeppen AH, Borke RC (1991) Experimental superficial siderosis of the central nervous system. I. Morphological observations. J Neuropathol Exp Neurol 50: 579–594PubMed Koeppen AH, Borke RC (1991) Experimental superficial siderosis of the central nervous system. I. Morphological observations. J Neuropathol Exp Neurol 50: 579–594PubMed
5.
Zurück zum Zitat Koeppen AH, Dentinger MP (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47: 249–270PubMed Koeppen AH, Dentinger MP (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47: 249–270PubMed
6.
Zurück zum Zitat 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
7.
Zurück zum Zitat Hamill RC (1908) Report of a case of melanosis of the brain, cord and meninges. J Nerv Ment Dis 35: 594 Hamill RC (1908) Report of a case of melanosis of the brain, cord and meninges. J Nerv Ment Dis 35: 594
8.
Zurück zum Zitat Tomlinson BE, Walton JN (1964) Superficial haemosiderosis of the central nervous system. J Neurol Neurosurg Psychiatry 27: 332–339 Tomlinson BE, Walton JN (1964) Superficial haemosiderosis of the central nervous system. J Neurol Neurosurg Psychiatry 27: 332–339
9.
Zurück zum Zitat 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: S51–56 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: S51–56
10.
Zurück zum Zitat Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118: 1051–1066PubMed Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118: 1051–1066PubMed
11.
Zurück zum Zitat Schievink WI, Apostolides PJ, Spetzler RF (1998) Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg 89: 1029–1031PubMed Schievink WI, Apostolides PJ, Spetzler RF (1998) Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg 89: 1029–1031PubMed
12.
Zurück zum Zitat Matsumoto S, Kang Y, Sato S et al. (1998) Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 88: 890–894PubMed Matsumoto S, Kang Y, Sato S et al. (1998) Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 88: 890–894PubMed
13.
Zurück zum Zitat Haroun RI, Li KW, Rigamonti D (2000) Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurol 53: 554–558CrossRefPubMed Haroun RI, Li KW, Rigamonti D (2000) Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurol 53: 554–558CrossRefPubMed
14.
Zurück zum Zitat Offenbacher H, Fazekas F, Reisecker F et al. (1991) Superficial siderosis of the spinal cord: a rare cause of myelopathy diagnosed by MRI. Neurology 41: 1987–1989PubMed Offenbacher H, Fazekas F, Reisecker F et al. (1991) Superficial siderosis of the spinal cord: a rare cause of myelopathy diagnosed by MRI. Neurology 41: 1987–1989PubMed
15.
Zurück zum Zitat Bostantjopoulou S, Katsarou Z, Pigadas A, Kazis A (2000) Superficial CNS siderosis and spinal pilocytic astrocytoma. Neurology 55: 450PubMed Bostantjopoulou S, Katsarou Z, Pigadas A, Kazis A (2000) Superficial CNS siderosis and spinal pilocytic astrocytoma. Neurology 55: 450PubMed
16.
Zurück zum Zitat Straube A, Dudel C, Wekerle G, Klopstock T (2001) Polyradiculopathy in the course of superficial siderosis of the CNS. J Neurol 248: 63–64CrossRefPubMed Straube A, Dudel C, Wekerle G, Klopstock T (2001) Polyradiculopathy in the course of superficial siderosis of the CNS. J Neurol 248: 63–64CrossRefPubMed
17.
Zurück zum Zitat Manfredi M, De Togni L, Beltramello A (2000) Superficial siderosis of the central nervous system in a patient with neurofibromatosis type I. Eur Neurol 43: 121–122CrossRefPubMed Manfredi M, De Togni L, Beltramello A (2000) Superficial siderosis of the central nervous system in a patient with neurofibromatosis type I. Eur Neurol 43: 121–122CrossRefPubMed
18.
Zurück zum Zitat Mascalchi M, Salvi F, Pirini MG et al. (1999) Transthyretin amyloidosis and superficial siderosis of the CNS. Neurology 53: 1498–1503PubMed Mascalchi M, Salvi F, Pirini MG et al. (1999) Transthyretin amyloidosis and superficial siderosis of the CNS. Neurology 53: 1498–1503PubMed
19.
Zurück zum Zitat Kato K, Tomura N, Takahashi S et al. (1998) Superficial siderosis appeared in a case of suprasellar embryonal carcinoma. No To Shinkei 50: 936–940PubMed Kato K, Tomura N, Takahashi S et al. (1998) Superficial siderosis appeared in a case of suprasellar embryonal carcinoma. No To Shinkei 50: 936–940PubMed
20.
Zurück zum Zitat Manfredi M, Magni E, Gandolfini M et al. (1999) Superficial siderosis of the central nervous system and anticoagulant therapy: a case report. Ital J Neurol Sci 20: 247–249CrossRefPubMed Manfredi M, Magni E, Gandolfini M et al. (1999) Superficial siderosis of the central nervous system and anticoagulant therapy: a case report. Ital J Neurol Sci 20: 247–249CrossRefPubMed
21.
Zurück zum Zitat D'Antonio V, Brancatelli G, Banco A, Montalbano E (1999) Superficial siderosis of the central nervous system associated with Escherichia coli meningoencephalitis. A case report. Radiol Med (Torino) 98: 520–522 D'Antonio V, Brancatelli G, Banco A, Montalbano E (1999) Superficial siderosis of the central nervous system associated with Escherichia coli meningoencephalitis. A case report. Radiol Med (Torino) 98: 520–522
22.
Zurück zum Zitat Kumar A, Aggarwal S, Willinsky R, TerBrugge KG (1993) Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery 32: 455–457PubMed Kumar A, Aggarwal S, Willinsky R, TerBrugge KG (1993) Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery 32: 455–457PubMed
23.
Zurück zum Zitat Bracchi M, Savoiardo M, Triulzi F et al. (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. Am J Neuroradiol 14: 227–236PubMed Bracchi M, Savoiardo M, Triulzi F et al. (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. Am J Neuroradiol 14: 227–236PubMed
24.
Zurück zum Zitat Uchino A, Aibe H, Itoh H, Aiko Y, Tanaka M (1997) Superficial siderosis of the central nervous system. Its MRI manifestations. Clin Imaging 21: 241–245CrossRefPubMed Uchino A, Aibe H, Itoh H, Aiko Y, Tanaka M (1997) Superficial siderosis of the central nervous system. Its MRI manifestations. Clin Imaging 21: 241–245CrossRefPubMed
25.
Zurück zum Zitat Parnes SM, Weaver SA (1992) Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngol Head Neck Surg 107: 69–77PubMed Parnes SM, Weaver SA (1992) Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngol Head Neck Surg 107: 69–77PubMed
26.
Zurück zum Zitat River Y, Honigman S, Gomori JM, Reches A (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9: 559–562PubMed River Y, Honigman S, Gomori JM, Reches A (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9: 559–562PubMed
27.
Zurück zum Zitat Stevens I, Petersen D, Grodd W, Poremba M, Dichgans J (1991) Superficial siderosis of the central nervous system. A 37-year follow-up of a case and review of the literature. Eur Arch Psychiatry Clin Neurosci 241: 57–60PubMed Stevens I, Petersen D, Grodd W, Poremba M, Dichgans J (1991) Superficial siderosis of the central nervous system. A 37-year follow-up of a case and review of the literature. Eur Arch Psychiatry Clin Neurosci 241: 57–60PubMed
Metadaten
Titel
Superfizielle Siderose des ZNS—2 Kasuistiken und eine Übersicht über die Literatur
verfasst von
Dr. A. Hug
M. Hartmann
A. Grau
Publikationsdatum
01.06.2004
Verlag
Springer-Verlag
Erschienen in
Der Nervenarzt / Ausgabe 6/2004
Print ISSN: 0028-2804
Elektronische ISSN: 1433-0407
DOI
https://doi.org/10.1007/s00115-003-1559-5

Weitere Artikel der Ausgabe 6/2004

Der Nervenarzt 6/2004 Zur Ausgabe

Mitteilungen der DGPPN

Mitteilungen der DGPPN