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Diagnose der Multiplen Sklerose: Revision der McDonald-Kriterien 2017

Diagnosis of multiple sclerosis: revision of the McDonald criteria 2017

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Zusammenfassung

Die Diagnose der Multiplen Sklerose (MS) stützt sich auf zwei Pfeiler: 1) den Nachweis einer örtlichen und zeitlichen Dissemination der fokal-neurologischen Defizite, 2) den Ausschluss wichtiger Differenzialdiagnosen. Auch die aktuelle Revision der Diagnosekriterien – McDonald 2017 – folgt diesen Prinzipien, berücksichtigt neue Daten zur MRT-Bildgebung und stärkt die (zuletzt eingeschränkte) Rolle der Liquordiagnostik bei der schubförmigen Verlaufsform. Übergeordnetes Ziel bleibt die möglichst frühe zuverlässige Diagnosestellung zwecks zeitnahem Start einer verlaufsmodifizierenden Therapie. Zu den konkreten Neuerungen gehören die Berücksichtigung kortikaler MRT-Läsionen (äquivalent zu juxtakortikalen Herden), die aufgehobene Differenzierung zwischen asymptomatischen und symptomatischen MRT-Läsionen und die Berücksichtigung charakteristischer Liquorbefunde für das Kriterium der zeitlichen Dissemination. So lässt sich bereits bei einem ersten Schub mit der Detektion liquorspezifischer oligoklonaler Bande sowie dem MRT-Nachweis einer MS-typischen örtlichen Läsionsverteilung (auch ohne Schrankenstörung) eine MS diagnostizieren. Für die primär-progrediente Verlaufsform, für die mittlerweile auch eine erste Therapieoption existiert, bleibt die bekannte Definition bestehen. Hinsichtlich der Differenzialdiagnostik erfolgt eine klare Abgrenzung gegenüber den mittlerweile als NMO-Spektrum-Erkrankungen (NMOSD) bezeichneten, meist durch Anti-Aquaporin-4-Antikörper charakterisierten Devic-Syndrom. Die Zuordnung des sog. radiologisch isolierten Syndroms (RIS, inzidentell gefundene MS-typische MRT-Läsionen ohne klinisches Korrelat), der Stellenwert von Erkrankungen mit Nachweis von Myelin-Oligodendrozyten-Glykoprotein(MOG)-Antikörpern wie auch eine einheitliche Definition für den sekundär chronisch-progredienten Verlauf bleiben offen. Zusammengefasst steht damit McDonald 2017 im konzeptuellen Gerüst der Vorgänger und vereinfacht die Frühdiagnose.

Abstract

Multiple sclerosis (MS) is the most common chronic autoimmune disorder of the central nervous system (CNS) largely affecting young adults. The diagnosis of MS is based on two pillars: 1) detection of the spatial and temporal dissemination of focal neurological deficits and 2) exclusion of important differential diagnoses. The current revision of the diagnostic criteria (McDonald 2017) also follows these principles, takes new data on magnetic resonance imaging (MRI) into account and reintroduces the role of cerebrospinal fluid (CSF) diagnostics for relapsing-remitting forms. The main priority is a reliable diagnosis as early as possible with the aim of a timely initiation of course-adapted treatment. Some of the concrete innovations are the consideration of cortical MRI lesions (equivalent to juxtacortical foci), the elimination of a distinction between asymptomatic and symptomatic MRI lesions and consideration of characteristic CSF findings for the criterion of temporal dissemination. Relapsing MS can be diagnosed at the time of the first attack by the detection of CSF-specific oligoclonal bands and the MRI detection of a typical local lesion distribution (even without simultaneous detection of a contrast-enhancing lesion). For the primary progressive course, for which a first treatment option has recently been approved, the known definition remains unaltered. With respect to the differential diagnosis there is a clear demarcation from Devic’s syndrome, now known as neuromyelitis optica spectrum disorders (NMOSD), as recent insights indicate a separate disease entity caused by an autoimmune response against the astrocytic aquaporin 4 (AQP4) water channel. Finally, future studies will have to provide a definition for secondary progressive MS courses and clarify how to handle diseases characterized by antibodies against myelin oligodendrocyte glycoprotein (MOG) or patients with radiologically isolated syndrome (RIS), i. e. incidental MRI-based detection of CNS lesions in the absence of any clinical event. In summary, McDonald 2017 is within the conceptual structure of its predecessor and simplifies an early diagnosis, thus paving the way to early treatment of MS.

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Abbreviations

AQP4:

Aquaporin 4

CIS:

Klinisch isoliertes Syndrom

DIS:

Örtliche Dissemination

DIT:

Zeitliche Dissemination

FLAIR:

„fluid attenuated inversion recovery“

MOG:

Myelin-Oligodendrozyten-Glykoprotein

MRT:

Magnetresonanztomographie

MS:

Multiple Sklerose

NMO:

Neuromyelitis optica

NMOSD:

NMO-Spektrum-Erkrankung

VEP:

Visuell evozierte Potenziale

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Authors and Affiliations

  1. Klinik für Neurologie, Medizinische Fakultät, und Zentrum für Neurologie und Neuropsychiatrie, LVR Klinikum Heinrich-Heine-Universität Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland

    O. Aktas & H.-P. Hartung

  2. Institut für Diagnostische und Interventionelle Radiologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    M. P. Wattjes

  3. Klinische Neuroimmunologie und Neurochemie, Klinik für Neurologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    M. Stangel

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O. Aktas erhielt Forschungsförderung durch Bayer Healthcare, Biogen, Novartis, Sanofi-Genzyme, und Teva, sowie Honorare und Reisekostenunterstützung durch Almirall, Bayer Healthcare, Biogen, Medimmune, Merck, Novartis, Roche, Sanofi-Genzyme und Teva, mit Genehmigung der Rektorin der Heinrich-Heine-Universität Düsseldorf. M.P. Wattjes erhielt Vortrags- und Beratungshonorare von Biogen, Novartis, IXICO, Janssen, Roche, Sanofi-Genzyme und Springer mit Genehmigung des Präsidiums der Medizinischen Hochschule Hannover. M. Stangel erhielt Referentenhonorare, Reisekostenzuschüsse und Beraterhonorare von Biogen, Baxalta/Shire, Bayer Vital, CSL Behring, Grifols, MedDay, Merck-Serono, Novartis, Roche, Sanofi-Genzyme und Teva. Seine Institution erhielt Forschungsunterstützung von Bayer Healthcare, Biogen, Merck-Serono, Novartis, Sanofi-Genzyme und Teva. H.-P. Hartung erhielt Honorare für Beratungs- und Vortragstätigkeiten und Mitarbeit in steering committees von Bayer Healthcare, Biogen, GeNeuro, MedImmune, Merck, Novartis, Receptos Celgene, Roche, Sanofi-Genzyme und Teva, mit Genehmigung der Rektorin der Heinrich-Heine-Universität Düsseldorf.

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Aktas, O., Wattjes, M.P., Stangel, M. et al. Diagnose der Multiplen Sklerose: Revision der McDonald-Kriterien 2017. Nervenarzt 89, 1344–1354 (2018). https://doi.org/10.1007/s00115-018-0550-0

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