Journal of Neurology

, Volume 261, Issue 2, pp 277–282 | Cite as

Prevalence of cortical superficial siderosis in patients with cognitive impairment

  • Frank Arne WollenweberEmail author
  • Katharina Buerger
  • Claudia Mueller
  • Birgit Ertl-Wagner
  • Rainer Malik
  • Martin Dichgans
  • Jennifer Linn
  • Christian Opherk
Original Communication


Cortical superficial siderosis (cSS) is a magnetic resonance imaging marker of cerebral amyloid angiopathy (CAA) and can be its sole imaging sign. cSS has further been identified as a risk marker for future intracranial hemorrhage. Although uncommon in the general population, cSS may be much more prevalent in high risk populations for amyloid pathology. We aimed to determine the frequency of cSS in patients with cognitive impairment presenting to a memory clinic. We prospectively evaluated consecutive patients presenting to our memory clinic between April 2011 and April 2013. Subjects received neuropsychological testing using the Consortium to Establish a Registry for Alzheimer’s Disease battery (CERAD-NP). Two hundred and twelve patients with documented cognitive impairment further underwent a standardized 3T-MR-imaging protocol with T2*-weighted gradient-echo sequences for detection of cSS. Thirteen of 212 patients (6.1 %) displayed cSS. In seven of them (54 %) cSS was the only imaging sign of CAA. Patients with cSS did not differ from patients without cSS with regard to medical history, age or cardiovascular risk profile. Subjects with cSS performed worse in the mini-mental state examination (p = 0.001), showed more white matter hyperintensities (p = 0.005) and more often had microbleeds (p = 0.001) compared to those without cSS. cSS is common in patients with cognitive impairment. It is associated with lower cognitive scores, white matter hyperintensities and microbleeds and can be the only imaging sign for CAA in this patient group.


Cerebral amyloid angiopathy Superficial siderosis MRI Cognitive decline 


Conflicts of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Knudsen KA, Rosand J, Karluk D, Greenberg SM (2001) Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 56:537–539. doi: 10.1212/WNL.56.4.537 CrossRefPubMedGoogle Scholar
  2. 2.
    Linn J, Bruckmann H (2010) Superficial siderosis in cerebral amyloid angiopathy. AJNR Am J Neuroradiol 31:E29. doi: 10.3174/ajnr.A1913 CrossRefPubMedGoogle Scholar
  3. 3.
    Charidimou A, Jager RH, Fox Z, Peeters A, Vandermeeren Y, Laloux P, Baron JC, Werring DJ (2013) Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology 81:626–632. doi: 10.1212/WNL.0b013e3182a08f2c CrossRefPubMedGoogle Scholar
  4. 4.
    Linn J, Halpin A, Demaerel P, Ruhland J, Giese AD, Dichgans M, van Buchem MA, Bruckmann H, Greenberg SM (2010) Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology 74:1346–1350. doi: 10.1212/WNL.0b013e3181dad605 PubMedCentralCrossRefPubMedGoogle Scholar
  5. 5.
    Vernooij MW, Ikram MA, Hofman A, Krestin GP, Breteler MM, van der Lugt A (2009) Superficial siderosis in the general population. Neurology 73:202–205. doi: 10.1212/WNL.0b013e3181ae7c5e CrossRefPubMedGoogle Scholar
  6. 6.
    Linn J, Wollenweber FA, Lummel N, Bochmann K, Pfefferkorn T, Gschwendtner A, Bruckmann H, Dichgans M, Opherk C (2013) Superficial siderosis is a warning sign for future intracranial hemorrhage. J Neurol 260:176–181. doi: 10.1007/s00415-012-6610-7 CrossRefPubMedGoogle Scholar
  7. 7.
    Petersen RC (2004) Mild cognitive impairment as a diagnostic entity. J Intern Med 256:183–194. doi: 10.1111/j.1365-2796.2004.01388.x CrossRefPubMedGoogle Scholar
  8. 8.
    Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198. doi: 10.1016/0022-3956(75)90026-6 CrossRefPubMedGoogle Scholar
  9. 9.
    Morris JC, Heyman A, Mohs RC, Hughes JP, van Belle G, Fillenbaum G, Mellits ED, Clark C (1989) The consortium to establish a registry for Alzheimer’s disease (CERAD). Part I. Clinical and neuropsychological assessment of Alzheimer’s disease. Neurology 39:1159–1165. doi: 10.1212/WNL.39.9.1159 CrossRefPubMedGoogle Scholar
  10. 10.
    Yesavage JA, Brink TL, Rose TL, Lum O, Huang V, Adey M, Leirer VO (1982) Development and validation of a geriatric depression screening scale: a preliminary report. J Psychiatr Res 17:37–49. doi: 10.1016/0022-3956(82)90033-4 CrossRefPubMedGoogle Scholar
  11. 11.
    Wardlaw JM, Smith EE, Biessels GJ, Cordonnier C, Fazekas F, Frayne R, Lindley RI, O’Brien JT, Barkhof F, Benavente OR, Black SE, Brayne C, Breteler M, Chabriat H, Decarli C, de Leeuw FE, Doubal F, Duering M, Fox NC, Greenberg S, Hachinski V, Kilimann I, Mok V, Oostenbrugge R, Pantoni L, Speck O, Stephan BC, Teipel S, Viswanathan A, Werring D, Chen C, Smith C, van Buchem M, Norrving B, Gorelick PB, Dichgans M, nEuroimaging STfRVco (2013) Neuroimaging standards for research into small vessel disease and its contribution to ageing and neurodegeneration. Lan Neurol 12:822–838. doi: 10.1016/S1474-4422(13)70124-8 CrossRefGoogle Scholar
  12. 12.
    Wahlund LO, Agartz I, Almqvist O, Basun H, Forssell L, Saaf J, Wetterberg L (1990) The brain in healthy aged individuals: MR imaging. Radiology 174:675–679. doi: 10.1148/radiology.174.3.2305048 CrossRefPubMedGoogle Scholar
  13. 13.
    Charidimou A, Gang Q, Werring DJ (2012) Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatr 83:124–137. doi: 10.1136/jnnp-2011-301308 CrossRefPubMedGoogle Scholar
  14. 14.
    Ellis RJ, Olichney JM, Thal LJ, Mirra SS, Morris JC, Beekly D, Heyman A (1996) Cerebral amyloid angiopathy in the brains of patients with Alzheimer’s disease: the CERAD experience, part XV. Neurology 46:1592–1596. doi: 10.1212/WNL.46.6.1592 CrossRefPubMedGoogle Scholar
  15. 15.
    Arvanitakis Z, Leurgans SE, Wang Z, Wilson RS, Bennett DA, Schneider JA (2011) Cerebral amyloid angiopathy pathology and cognitive domains in older persons. Ann Neurol 69:320–327. doi: 10.1002/ana.22112 PubMedCentralCrossRefPubMedGoogle Scholar
  16. 16.
    O’Donnell HC, Rosand J, Knudsen KA, Furie KL, Segal AZ, Chiu RI, Ikeda D, Greenberg SM (2000) Apolipoprotein E genotype and the risk of recurrent lobar intracerebral hemorrhage. New Engl J Med 342:240–245. doi: 10.1056/NEJM200001273420403 CrossRefPubMedGoogle Scholar
  17. 17.
    Rosand J, Hylek EM, O’Donnell HC, Greenberg SM (2000) Warfarin-associated hemorrhage and cerebral amyloid angiopathy: a genetic and pathologic study. Neurology 55:947–951. doi: 10.1212/WNL.55.7.947 CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Frank Arne Wollenweber
    • 1
    Email author
  • Katharina Buerger
    • 1
  • Claudia Mueller
    • 1
  • Birgit Ertl-Wagner
    • 2
  • Rainer Malik
    • 1
  • Martin Dichgans
    • 1
    • 3
  • Jennifer Linn
    • 4
  • Christian Opherk
    • 1
    • 5
  1. 1.Institute for Stroke and Dementia ResearchLudwig-Maximilians-UniversityMunichGermany
  2. 2.Institute of Clinical RadiologyLudwig-Maximilians-UniversityMunichGermany
  3. 3.Munich Cluster for Systems Neurology (SyNergy)Ludwig-Maximilians-UniversityMunichGermany
  4. 4.Department of NeuroradiologyLudwig-Maximilians-UniversityMunichGermany
  5. 5.Department of NeurologySLK-Kliniken HeilbronnHeilbronnGermany

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