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Nervenheilkunde 2011; 30(07): 480-486
DOI: 10.1055/s-0038-1628383
Multiple Sklerose
Schattauer GmbH

Neuromyelitis optika und Susac-Syndrom

Seltene, aber wichtige Differenzialdiagnosen der MS Article in several languages: deutsch | English
J. Dörr
1   NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin
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Publication History

eingegangen am: 07 January 2011

angenommen am: 02 March 2011

Publication Date:
23 January 2018 (online)

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Zusammenfassung

Der Ausschluss von Differenzialdiagnosen ist elementarer Bestandteil der zeitgemäßen Diagnosestellung der Multiplen Sklerose (MS). Zwei seltene, aufgrund unterschiedlicher Therapiestrategien und Prognosen aber wichtige Differenzialdiagnosen der MS sind die Neuromyelitis optika und das Susac-Syndrom. Während erstere in den letzten Jahren zunehmend als eigenständige Erkrankung, denn als Variante der MS angesehen wird, wird letzteres in vielen Fällen gar nicht erst erkannt. In dieser Übersicht werden beide Erkrankungen vorgestellt, wobei der Schwerpunkt auf der differenzialdiagnostischen Abgrenzung von der MS liegt.

Schlüsselwörter

Multiple Sklerose - Neuromyelitis optika - Susac-Syndrom - Differenzialdiagnose
 

  • Literatur

  • 1 Compston A, Coles A. Multiple sclerosis. Lancet 2008; 372 9648 1502-17.
    CrossrefPubMedGoogle Scholar
  • 2 McDonald WI. et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001; 50 (01) 121-7.
    CrossrefGoogle Scholar
  • 3 Polman CH. et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the “McDonald Criteria”. Ann Neurol 2005; 58 (06) 840-6.
    CrossrefGoogle Scholar
  • 4 Wingerchuk DM. et al. The spectrum of neuromyelitis optica. Lancet Neurol 2007; 06 (09) 805-15.
    CrossrefGoogle Scholar
  • 5 Weinshenker BG. et al. OSMS is NMO, but not MS: proven clinically and pathologically. Lancet Neurol 2006; 05 (02) 110-1.
    CrossrefGoogle Scholar
  • 6 Lucchinetti C. et al. A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain 2002; 125 (07) 1450-1.
    CrossrefGoogle Scholar
  • 7 Lennon VA. et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004; 364 9451 2106-12.
    CrossrefGoogle Scholar
  • 8 Lennon VA. et al. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 2005; 202 (04) 473-7.
    CrossrefGoogle Scholar
  • 9 Paul F. et al. Antibody to aquaporin 4 in the diagnosis of neuromyelitis optica. PLoS Med 2007; 04 (04) e133.
    CrossrefGoogle Scholar
  • 10 Jarius S. et al. NMO-IgG in the diagnosis of neuromyelitis optica. Neurology 2007; 68 (13) 1076-7.
    CrossrefGoogle Scholar
  • 11 Jarius S, Wildemann B. AQP4 antibodies in neuromyelitis optica: diagnostic and pathogenetic relevance. Nat Rev Neurol 2010; 06 (07) 383-92.
    Google Scholar
  • 12 Graber DJ, Levy M, Kerr D, Wade WF. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation 2008; 05: 22.
    CrossrefGoogle Scholar
  • 13 Wingerchuk DM. et al. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology 1999; 53 (05) 1107-14.
    CrossrefGoogle Scholar
  • 14 Poppe AY. et al. Neuromyelitis optica with hypothalamic involvement. Mult Scler 2005; 11 (05) 617-21.
    CrossrefGoogle Scholar
  • 15 Magaña SM. et al. Posterior reversible encephalopathy syndrome in neuromyelitis optica spectrum disorders. Neurology 2009; 72 (08) 712-7.
    CrossrefPubMedGoogle Scholar
  • 16 Wingerchuk DM. et al. Revised diagnostic criteria for neuromyelitis optica. Neurology 2006; 66 (10) 1485-9.
    CrossrefGoogle Scholar
  • 17 Pittock SJ. et al. Brain abnormalities in neuromyelitis optica. Arch Neurol 2006; 63 (03) 390-6.
    CrossrefGoogle Scholar
  • 18 Sellner J. et al. EFNS guidelines on diagnosis and management of neuromyelitis optica. Eur J Neurol 2010; 17 (08) 1019-32.
    CrossrefGoogle Scholar
  • 19 de Seze J. et al. Is Devic’s neuromyelitis optica a separate disease? A comparative study with multiple sclerosis. Mult Scler 2003; 09 (05) 521-5.
    CrossrefGoogle Scholar
  • 20 McLean BN, Luxton RW, Thompson EJ. A study of immunoglobulin G in the cerebrospinal fluid of 1007 patients with suspected neurological disease using isoelectric focusing and the Log IgG-Index. A comparison and diagnostic applications. Brain 1990; 113 (05) 1269-89.
    CrossrefGoogle Scholar
  • 21 Waters P. et al. Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis. Arch Neurol 2008; 65 (07) 913-9.
    Google Scholar
  • 22 Jarius S. et al. Standardized method for the detection of antibodies to aquaporin-4 based on a highly sensitive immunofluorescence assay employing recombinant target antigen. J Neurol Sci 2010; 291 (1–2) 52-6.
    CrossrefGoogle Scholar
  • 23 Kalluri SR. et al. Quantification and functional characterization of antibodies to native aquaporin 4 in neuromyelitis optica. Arch Neurol 2010; 67 (10) 1201-8.
    Google Scholar
  • 24 Wingerchuk DM, Weinshenker BG. Neuromyelitis optica: Clinical predictors of a relapsing course and survival. Neurology 2003; 60 (05) 848-53.
    CrossrefPubMedGoogle Scholar
  • 25 Watanabe S. et al. Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica. Mult Scler 2007; 13 (01) 128-32.
    CrossrefGoogle Scholar
  • 26 Cree BAC. et al. An open label study of the effects of rituximab in neuromyelitis optica. Neurology 2005; 64 (07) 1270-2.
    CrossrefGoogle Scholar
  • 27 Trebst C. [Diagnosis and treatment of neuromyelitis optica: Consensus recommendations of the Neuromyelitis Optica Study Group.]. Nervenarzt. 2010 Dez 22 www.ncbi.nlm.nih.gov/pubmed/21174070
    Google Scholar
  • 28 Bot JCJ. et al. Spinal cord abnormalities in recently diagnosed MS patients: added value of spinal MRI examination. Neurology 2004; 62 (02) 226-33.
    CrossrefGoogle Scholar
  • 29 Weinshenker BG. et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006; 59 (03) 566-9.
    CrossrefGoogle Scholar
  • 30 Jarius S. et al. Polyspecific, antiviral immune response distinguishes multiple sclerosis and neuromyelitis optica. J Neurol Neurosurg Psychiatr 2008; 79 (10) 1134-6.
    CrossrefGoogle Scholar
  • 31 Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979; 29 (03) 313-6.
    CrossrefGoogle Scholar
  • 32 Eluvathingal TJMuttikkal, Vattoth S, Keluth VNChavan. Susac syndrome in a young child. Pediatr Radiol 2007; 37 (07) 710-3.
    CrossrefGoogle Scholar
  • 33 McLeod DS. et al. Retinal and optic nerve head pathology in Susac’s Syndrome. Ophthalmology. 2010 Okt 2 www.ncbi.nlm.nih.gov/pubmed/20920828
    Google Scholar
  • 34 O’Halloran HS. et al. Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. Ophthalmology 1998; 105 (06) 1038-44.
    Google Scholar
  • 35 Petty GW. et al. Retinocochleocerebral vasculopathy. Medicine (Baltimore) 1998; 77 (01) 12-40.
    CrossrefGoogle Scholar
  • 36 Heiskala H. et al. Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: two new cases. J Neurol Sci 1988; 86 (2–3) 239-50.
    CrossrefGoogle Scholar
  • 37 Barker R. et al. Microangiopathy of the brain and retina with hearing loss in a 50 year old woman: extending the spectrum of Susac’s syndrome. J Neurol Neurosurg Psychiatr 1999; 66 (05) 641-3.
    CrossrefGoogle Scholar
  • 38 Ballard E, Butzer JF, Donders J. Susac’s syndrome: neuropsychological characteristics in a young man. Neurology 1996; 47 (01) 266-8.
    CrossrefGoogle Scholar
  • 39 Bogousslavsky J. et al. Encephalopathy, deafness and blindness in young women: a distinct retinocochleocerebral arteriolopathy?. J Neurol Neurosurg Psychiatry 1989; 52 (01) 43-6.
    CrossrefGoogle Scholar
  • 40 MacFadyen DJ, Schneider RJ, Chisholm IA. A syndrome of brain, inner ear and retinal microangiopathy. Can J Neurol Sci 1987; 14 (03) 315-8.
    CrossrefGoogle Scholar
  • 41 Monteiro ML. et al. A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology 1985; 35 (08) 1113-21.
    CrossrefGoogle Scholar
  • 42 Papeix C. et al. [Susac’s syndrome: improvement with combined cyclophosphamide and intravenous immunoglobulin therapy]. Rev Neurol (Paris) 2000; 156 (8–9) 783-5.
    Google Scholar
  • 43 Susac JO. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994; 44 (04) 591-3.
    CrossrefPubMedGoogle Scholar
  • 44 Tashima K. et al. Susac’s syndrome: beneficial effects of corticosteroid therapy in a Japanese case. Intern Med 2001; 40 (02) 135-9.
    CrossrefGoogle Scholar
  • 45 Magro C. Susac’s syndrome: an autoimmune endotheliopathy. Abstract presented at 1st Susac’s Syndrome Symposium, held at Ohio State University, Columbus. 2005
    Google Scholar
  • 46 Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac’s syndrome: 1975–2005 microangiopathy/ autoimmune endotheliopathy. J Neurol Sci 2007; 257 (1–2) 270-2.
    CrossrefGoogle Scholar
  • 47 Waldman J, Knight D. Antiendothelial cell antibodies in Susac’s syndrome. Abstract presented at the 1st Susac’s Syndrome Symposium, held at Ohio State University, Columbus. 2005
    Google Scholar
  • 48 Jarius S. et al. Anti-endothelial serum antibodies in a patient with Susac’s syndrome. J Neurol Sci 2009; 285 (1–2) 259-61.
    CrossrefGoogle Scholar
  • 49 Aubart-Cohen F. et al. Long-term outcome in Susac syndrome. Medicine (Baltimore) 2007; 86 (02) 93-102.
    CrossrefGoogle Scholar
  • 50 Petty GW. et al. Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years. Mayo Clin Proc 2001; 76 (09) 958-60.
    CrossrefGoogle Scholar
  • 51 Dörr J. et al. Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. Nat Rev Neurol 2009; 05 (12) 683-8.
    CrossrefGoogle Scholar
  • 52 Susac JO. et al. MRI findings in Susac’s syndrome. Neurology 2003; 61 (12) 1783-7.
    CrossrefGoogle Scholar
  • 53 White ML, Zhang Y, Smoker WR. Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. AJNR Am J Neuroradiol 2004; 25 (05) 706-13.
    Google Scholar
  • 54 Kleffner I. et al. Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology 2008; 70 (19 Pt 2) 1867-9.
    CrossrefGoogle Scholar
  • 55 Egan RA, Hills WL, Susac JO. Gass plaques and fluorescein leakage in Susac Syndrome. J Neurol Sci 2010; 299 (1–2) 97-100.
    CrossrefGoogle Scholar
  • 56 Hilgert E, Harrèus U, Kramer MF, Matthias C. [Susac’s syndrome. A rare microangiopathy of cochlea, retina, and brain]. HNO 2006; 54 (04) 303-6.
    Google Scholar
  • 57 Papo T. et al. Susac syndrome. Medicine (Baltimore) 1998; 77 (01) 3-11.
    CrossrefGoogle Scholar
  • 58 Pawate S, Agarwal A, Moses H, Sriram S. The spectrum of Susac’s syndrome. Neurol Sci 2009; 30 (01) 59-64.
    CrossrefPubMedGoogle Scholar
  • 59 Mallam B, Damato EM, Scolding NJ, Bailey C. Serial retinal fluorescein angiography and immune therapy in Susac’s syndrome. J Neurol Sci 2009; 285 (1–2) 230-4.
    CrossrefGoogle Scholar
  • 60 Rennebohm RM, Egan RA, Susac JO. Treatment of Susac’s Syndrome. Curr Treat Options Neurol 2008; 10 (01) 67-74.
    CrossrefGoogle Scholar
  • 61 Fox RJ. et al. Treatment of Susac syndrome with gamma globulin and corticosteroids. J Neurol Sci 2006; 251 (1–2) 17-22.
    CrossrefGoogle Scholar
  • 62 Gean-Marton AD. et al. Abnormal corpus callosum: a sensitive and specific indicator of multiple sclerosis. Radiology 1991; 180 (01) 215-21.
    CrossrefGoogle Scholar
  • 63 Paty DW. et al. MRI in the diagnosis of MS: a prospective study with comparison of clinical evaluation, evoked potentials, oligoclonal banding, and CT. Neurology 1988; 38 (02) 180-5.
    CrossrefGoogle Scholar