Elsevier

The Lancet Neurology

Volume 17, Issue 2, February 2018, Pages 162-173
The Lancet Neurology

Position Paper
Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria

https://doi.org/10.1016/S1474-4422(17)30470-2Get rights and content

Summary

The 2010 McDonald criteria for the diagnosis of multiple sclerosis are widely used in research and clinical practice. Scientific advances in the past 7 years suggest that they might no longer provide the most up-to-date guidance for clinicians and researchers. The International Panel on Diagnosis of Multiple Sclerosis reviewed the 2010 McDonald criteria and recommended revisions. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical clinically isolated syndrome, define what is needed to fulfil dissemination in time and space of lesions in the CNS, and stress the need for no better explanation for the presentation. The following changes were made: in patients with a typical clinically isolated syndrome and clinical or MRI demonstration of dissemination in space, the presence of CSF-specific oligoclonal bands allows a diagnosis of multiple sclerosis; symptomatic lesions can be used to demonstrate dissemination in space or time in patients with supratentorial, infratentorial, or spinal cord syndrome; and cortical lesions can be used to demonstrate dissemination in space. Research to further refine the criteria should focus on optic nerve involvement, validation in diverse populations, and incorporation of advanced imaging, neurophysiological, and body fluid markers.

Introduction

Diagnostic criteria for multiple sclerosis combining clinical, imaging, and laboratory evidence have evolved over time, with the most recent being the 2010 McDonald criteria from the International Panel on Diagnosis of Multiple Sclerosis (referred to as the Panel from here on).1, 2, 3, 4, 5 The increasing incorporation of paraclinical assessments, especially imaging, to supplement clinical findings has allowed earlier, more sensitive, and more specific diagnosis.6 New data, emerging technology, and evolving consensus necessitate a periodic re-examination of diagnostic criteria and their usefulness. The Panel reconvened under the auspices of the International Advisory Committee on Clinical Trials in Multiple Sclerosis (sponsored by the US National Multiple Sclerosis Society and the European Committee for Treatment and Research in Multiple Sclerosis) for two meetings (Nov 2–5, 2016, in Philadelphia, PA, USA, and May 20–21, 2017, in Berlin, Germany). In this Position Paper, we discuss issues related to misdiagnosis, differential diagnosis, and appropriate application of the McDonald criteria, with a particular emphasis on diagnosis in diverse populations and in patients with atypical presentations. With the 2017 McDonald criteria, we present recommendations concerning the diagnostic process for multiple sclerosis, make specific revisions to the 2010 McDonald criteria, and outline research that should be done to inform future refinements of the criteria.

Section snippets

Rationale and methods for the 2017 revisions

The Panel meetings to consider revisions to the 2010 McDonald criteria were motivated by new data in several areas: the performance of the 2010 McDonald criteria in diverse populations; the distinction between multiple sclerosis and other diseases with potentially overlapping clinical and imaging features, such as neuromyelitis optica spectrum disorders (NMOSDs); challenges in making the diagnosis in individuals with presentations other than a typical clinically isolated syndrome; the frequency

Misdiagnosis and differential diagnosis

Before considering potential revisions of the 2010 McDonald criteria, the Panel reviewed issues related to the diagnosis of multiple sclerosis, appropriate use of the McDonald criteria, and performance of the criteria across patient populations.

Misdiagnosis of multiple sclerosis remains an issue in clinical practice,15, 16, 17, 18 and the Panel identified several factors that potentially increase this risk. Multiple sclerosis has heterogeneous clinical and imaging manifestations, which differ

Role of MRI in diagnosis of multiple sclerosis

MRI has been increasingly used to support the diagnosis of multiple sclerosis and to look for atypical radiological features arguing against this diagnosis. MAGNIMS and the Consortium of Multiple Sclerosis Centers recently proposed standardised MRI protocols for the diagnostic process, to determine prognosis, and for follow-up.14, 59, 60 Brain and spinal cord MRI remain the most useful paraclinical tests to aid the diagnosis of multiple sclerosis and can substitute for clinical findings in the

Role of CSF examination in diagnosis of multiple sclerosis

Although CSF examination has been de-emphasised in successive iterations of the McDonald criteria, it remains a valuable diagnostic test.61 In the appropriate clinical setting, evidence of intrathecal antibody synthesis, although not specific for multiple sclerosis, supports the diagnosis.62 Conversely, CSF findings atypical of multiple sclerosis (eg, an elevated protein concentration of >100 mg/dL, pleocytosis with >50 cells per mm3, or the presence of neutrophils, eosinophils, or atypical

CSF oligoclonal bands

Numerous studies65, 66, 67, 68, 69, 70, 71, 72, 73 have provided evidence that, in adult patients with a clinically isolated syndrome, CSF oligoclonal bands are an independent predictor of the risk of a second attack when controlling for demographic, clinical, treatment, and MRI variables. After considering these data, the Panel recommended that with a typical clinically isolated syndrome, fulfilment of clinical or MRI criteria for DIS, and no better explanation for the clinical presentation,

Number of periventricular lesions

The 20013 and 20054 McDonald criteria required three or more periventricular lesions as one of the anatomical locations that could fulfil MRI criteria for DIS. In the 2010 McDonald criteria,5 this requirement was changed to one or more periventricular lesions as one of the four anatomical locations (periventricular, juxtacortical, and infratentorial brain regions, and the spinal cord). However, non-specific white matter lesions are common in older individuals and in those with vascular risk

Conclusions

Early diagnostic criteria for multiple sclerosis were based primarily on clinical evidence.1 Subsequent criteria incorporated imaging and other paraclinical markers in response to technological advances and new data.2, 3, 4, 5 The 2017 revisions to the well established 2010 McDonald criteria revitalise the role of CSF analysis, reconsider the value of imaging findings previously not included (symptomatic and cortical lesions), and articulate more clearly cautions about misdiagnosis and

Search strategy and selection criteria

In preparation for the meetings, the Panel conducted literature searches (completed April 15, 2017) in PubMed (English language, using search terms “multiple sclerosis” and “diagnosis” with a focus on publications since 2010 but also including earlier publications as appropriate). It reviewed papers on topics including, but not limited to: the role in diagnosis of MRI, optical coherence tomography, evoked potentials, and CSF analysis; diagnosis in diverse populations (paediatric, Asian, and

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