Clinical Characterization and Ancillary Tests in Susac Syndrome: A Systematic Review.
Wilf-Yarkoni A, Zmira O, Tolkovsky A, Pflantzer B, Gofrit SG, Kleffner I, Paul F, Dörr J.Neurol Neuroimmunol Neuroinflamm. 2024 May;11(3):e200209. doi: 10.1212/NXI.0000000000200209. Epub 2024 Feb 16.PMID: 38364193

Central intra-lesional iron deposits as a possible novel imaging marker at 7 Tesla MRI in Susac Syndrome - an exploratory study.
Strunk D, Sinnecker T, Kleffner I, Doerr J, Ringelstein M, Gross CC, Deuschl C, Maderwald S, Quick HH, Yamac E, Wrede KH, Kraemer M.BMC Med Imaging. 2024 Jan 2;24(1):4. doi: 10.1186/s12880-023-01171-7.PMID: 38166655

Susac syndrome and pregnancy: a review of published cases and considerations for patient management.
Willekens B, Kleffner I.Ther Adv Neurol Disord. 2021 Jan 30;14:1756286420981352. doi: 10.1177/1756286420981352. eCollection 2021.PMID: 33796140

Fundamental mechanistic insights from rare but paradigmatic neuroimmunological diseases.
Wiendl H, Gross CC, Bauer J, Merkler D, Prat A, Liblau R.
Nat Rev Neurol. 2021 Jul;17(7):433-447. doi: 10.1038/s41582-021-00496-7. Epub 2021 May 28.
PMID: 34050331

CD8⁺ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome.
Gross CC, Meyer C, Bhatia U, Yshii L, Kleffner I, Bauer J, Tröscher AR, Schulte-Mecklenbeck A, Herich S, Schneider-Hohendorf T, Plate H, Kuhlmann T, Schwaninger M, Brück W, Pawlitzki M, Laplaud DA, Loussouarn D, Parratt J, Barnett M, Buckland ME, Hardy TA, Reddel SW, Ringelstein M, Dörr J, Wildemann B, Kraemer M, Lassmann H, Höftberger R, Beltrán E, Dornmair K, Schwab N, Klotz L, Meuth SG, Martin-Blondel G, Wiendl H, Liblau R.Nat Commun. 2019 Dec 18;10(1):5779. doi: 10.1038/s41467-019-13593-5.PMID: 31852955

Diagnostic criteria for Susac syndrome.
Kleffner I, Dörr J, Ringelstein M, Gross CC, Böckenfeld Y, Schwindt W, Sundermann B, Lohmann H, Wersching H, Promesberger J, von Königsmarck N, Alex A, Guthoff R, Frijns CJ, Kappelle LJ, Jarius S, Wildemann B, Aktas O, Paul F, Wiendl H, Duning T; European Susac Consortium (EuSaC).J Neurol Neurosurg Psychiatry. 2016 Dec;87(12):1287-1295. doi: 10.1136/jnnp-2016-314295. Epub 2016 Oct 25.PMID: 28103199

Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography.
Ringelstein M, Albrecht P, Kleffner I, Bühn B, Harmel J, Müller AK, Finis D, Guthoff R, Bergholz R, Duning T, Krämer M, Paul F, Brandt A, Oberwahrenbrock T, Mikolajczak J, Wildemann B, Jarius S, Hartung HP, Aktas O, Dörr J.Neurology. 2015 Aug 18;85(7):610-8. doi: 10.1212/WNL.0000000000001852. Epub 2015 Jul 22.PMID: 26203089

RETINAL LESION EVOLUTION IN SUSAC SYNDROME.
Brandt AU, Oberwahrenbrock T, Costello F, Fielden M, Gertz K, Kleffner I, Paul F, Bergholz R, Dörr J.Retina. 2016 Feb;36(2):366-74. doi: 10.1097/IAE.0000000000000700.PMID: 26200513

Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study.
Jarius S, Kleffner I, Dörr JM, Sastre-Garriga J, Illes Z, Eggenberger E, Chalk C, Ringelstein M, Aktas O, Montalban X, Fechner K, Stöcker W, Ringelstein EB, Paul F, Wildemann B.J Neuroinflammation. 2014 Mar 8;11:46. doi: 10.1186/1742-2094-11-46.PMID: 24606999

Update on Susac syndrome: new insights in brain and retinal imaging and treatment options.
Dörr J, Ringelstein M, Duning T, Kleffner I.
J Alzheimers Dis. 2014;42 Suppl 3:S99-108. doi: 10.3233/JAD-132519.
PMID: 24662104

Characteristics of Susac syndrome: a review of all reported cases.
Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, Aktas O, Ringelstein EB, Paul F, Kleffner I.Nat Rev Neurol. 2013 Jun;9(6):307-16. doi: 10.1038/nrneurol.2013.82. Epub 2013 Apr 30.PMID: 23628737

Lesion morphology at 7 Tesla MRI differentiates Susac syndrome from multiple sclerosis.
Wuerfel J, Sinnecker T, Ringelstein EB, Jarius S, Schwindt W, Niendorf T, Paul F, Kleffner I, Dörr J.Mult Scler. 2012 Nov;18(11):1592-9. doi: 10.1177/1352458512441270. Epub 2012 Jun 18.PMID: 22711711

Neurological picture. Susac's syndrome: effective combination of immunosuppression and antiplatelet treatment.
Kleffner I, Ringelstein EB, Stupp N, Niederstadt TU, Young P.J Neurol Neurosurg Psychiatry. 2006 Dec;77(12):1335. doi: 10.1136/jnnp.2006.095596.PMID: 17110748

(Brandt, Zimmermann et al. 2012, Dorr, Krautwald et al. 2013, Engeholm, Leo-Kottler et al. 2013, Dorr, Ringelstein et al. 2014, Kleffner, Dorr et al. 2014, Ringelstein, Albrecht et al. 2015, Brandt, Oberwahrenbrock et al. 2016, Kleffner, Dorr et al. 2016, Gross, Meyer et al. 2019) Brandt, A. U., T. Oberwahrenbrock, F. Costello, M. Fielden, K. Gertz, I. Kleffner, F. Paul, R. Bergholz and J. Dorr (2016). "Retinal Lesion Evolution in Susac Syndrome." Retina 36(2): 366-374.

(Gross 2019) Gross, C. C., C. Meyer, U. Bhatia, L. Yshii, I. Kleffner, J. Bauer, A. R. Troscher, A. Schulte-Mecklenbeck, S. Herich, T. Schneider-Hohendorf, H. Plate, T. Kuhlmann, M. Schwaninger, W. Bruck, M. Pawlitzki, D. A. Laplaud, D. Loussouarn, J. Parratt, M. Barnett, M. E. Buckland, T. A. Hardy, S. W. Reddel, M. Ringelstein, J. Dorr, B. Wildemann, M. Kraemer, H. Lassmann, R. Hoftberger, E. Beltran, K. Dornmair, N. Schwab, L. Klotz, S. G. Meuth, G. Martin-Blondel, H. Wiendl and R. Liblau (2019). "CD8(+) T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome." Nat Commun 10(1): 5779.

(Kleffner 2016) Kleffner, I., J. Dorr, M. Ringelstein, C. C. Gross, Y. Bockenfeld, W. Schwindt, B. Sundermann, H. Lohmann, H. Wersching, J. Promesberger, N. von Konigsmarck, A. Alex, R. Guthoff, C. J. Frijns, L. J. Kappelle, S. Jarius, B. Wildemann, O. Aktas, F. Paul, H. Wiendl, T. Duning and C. European Susac (2016). "Diagnostic criteria for Susac syndrome." J Neurol Neurosurg Psychiatry 87(12): 1287-1295.

(Ringelstein 2015) Ringelstein, M., P. Albrecht, I. Kleffner, B. Buhn, J. Harmel, A. K. Muller, D. Finis, R. Guthoff, R. Bergholz, T. Duning, M. Kramer, F. Paul, A. Brandt, T. Oberwahrenbrock, J. Mikolajczak, B. Wildemann, S. Jarius, H. P. Hartung, O. Aktas and J. Dorr (2015). "Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography." Neurology 85(7): 610-618.

(Dörr 2014) Dorr, J., M. Ringelstein, T. Duning and I. Kleffner (2014). "Update on Susac syndrome: new insights in brain and retinal imaging and treatment options." J Alzheimers Dis 42 Suppl 3: S99-108.

(Jarius 2014) Jarius S, Kleffner I, Dörr JM, Sastre-Garriga J, Illes Z, Eggenberger E, et al. Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study.

(Kleffner 2014) Kleffner, I., J. Dorr, T. Duning, P. Young, E. B. Ringelstein and M. Schilling (2014). "Susac syndrome treated with subcutaneous immunoglobulin." Eur Neurol 71(1-2): 89-92.

(Dörr 2013) Dörr J, Krautwald S, Wildemann B, Jarius S, Ringelstein M, Duning T, et al. Characteristics of Susac Syndrome: Systematic Analysis of all Reported Cases. Nat Rev Neurol. 2013 Apr 30. doi: 10.1038/nrneurol.2013.82.

(Engeholm 2013) Engeholm, M., B. Leo-Kottler, H. Rempp, T. Lindig, H. Lerche, I. Kleffner, M. Henes and M. Dihne (2013). "Encephalopathic Susac's Syndrome associated with livedo racemosa in a young woman before the completion of family planning." BMC Neurol 13: 185.

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