McDonald criteria
The McDonald criteria are diagnostic criteria for multiple sclerosis (MS). These criteria are named after neurologist W. Ian McDonald. In April 2001, an international panel in association with the National Multiple Sclerosis Society (NMSS) of America recommended revised diagnostic criteria for MS. They have undergone revisions in 2005[1] and 2010.[2]
McDonald criteria are intended to replace the Poser criteria and the older Schumacher criteria. They discourage the previously used Poser terms such as "clinically definite" and "probable MS", and propose as diagnostic either "MS", "possible MS", or "not MS".[3]
The criteria try to prove the existence of demyelinating lesions, by image or by their effects, showing their dissemination in time (DIT) and space (DIS) as characteristics for MS. They rely only in clinical and paraclinical techniques, including advances in magnetic resonance imaging (MRI). These criteria facilitate the diagnosis of MS in patients who present early signs and symptoms suggestive of the disease but with no sufficient clinical information.
The McDonald criteria for the diagnosis of multiple sclerosis were revised first in 2005 to clarify exactly what is meant by an "attack", "dissemination" and a "positive MRI", etc.[1] Later they were revised again in 2010.
McDonald's criteria are the standard clinical case definition for MS and the 2010 version is regarded as the gold standard test for MS diagnosis.
Diagnostic Criteria
| Clinical Presentation | Additional Data Needed |
|---|---|
| * 2 or more attacks (relapses) * 2 or more objective clinical lesions |
None; clinical evidence will suffice (additional evidence desirable but must be consistent with MS) |
| * 2 or more attacks * 1 objective clinical lesion |
Dissemination in space, demonstrated by: * MRI * or a positive (cerebrospinal fluid) CSF and 2 or more MRI lesions consistent with MS * or further clinical attack involving different site |
| * 1 attack * 2 or more objective clinical lesions |
Dissemination in time, demonstrated by: * MRI * or second clinical attack |
| * 1 attack * 1 objective clinical lesion (monosymptomatic presentation) |
Dissemination in space demonstrated by: * MRI * or positive CSF and 2 or more MRI lesions consistent with MS and Dissemination in time demonstrated by: * MRI * or second clinical attack |
| Insidious neurological progression suggestive of MS (primary progressive MS) |
One year of disease progression (retrospectively or prospectively determined) and
Two of the following:
|
They discourage the previously used terms such as "clinically definite" and "probable MS", and propose as diagnostic either "MS", "possible MS", or "not MS".[3]
Criticism
The McDonald criteria seem to rely on a definition of MS based solely in the presence of demyelinating lesions and its dissemination in time and space, regardless of the nature of the lesions, being therefore non-specific. They address the problem of specificity requiring that "no better explanation" is found for the lesions. This converts MS into some kind of default diagnosis for demyelinating diseases that produce lesions. Besides, they consider as MS just the presence of the lesions, and not the underlying condition that produces them.
Other problem is the lack of publications respect the sensitivity and specificity of the criteria. See below for more information.
Sensitivity and specificity
Pathology is generally regarded as the gold standard in defining different forms of inflammatory demyelinating diseases (Adams and Kubik, 1952; Alvord, 1985; Wegner, 2005)[4] Even being a gold standard for diagnosis, McDonald criteria are not perfect and its parameters should be evaluated.
Even being a clinical definition McDonald criteria rely on a pathological basis trying to demonstrate lesion dissemination in space and time.
Problems for the evaluation
Sensitivity and specificity of the criteria (respect autopsy confirmation) cannot be evaluated during the initial presentations. When autopsy is available, normally the lesions of the patient are very old for evaluation of the initial diagnosis. In the few cases of people dying during their initial stages, the disease is normally too strong to be considered typical. On the other hand, the more time that a patient is followed, the most certain the diagnosis is (being 100% after death).
Therefore sensitivity and specificity are normally evaluated retrospectively by stages, following the patients during the course of the disease's evolution. A normal milestone is to check if the CIS converts to CDMS in a given number of years.
Estimations
In any case, the sensitivity of McDonald criteria is low respect pathologically defined MS because around 25% of MS cases are silent silent MS cases[5] even with two or more pathologically disseminated lesions. Of course, anyway McDonalds perform much better than older Poser criteria, which were not using MRI. Positives during the CIS are rare (McDonald positive is nearly incompatible with CIS definition) but retrospective MRI shows that there is normally more than one pathological lesion when the CIS appears. Therefore, sensitivity is expected to be low in the initial stages.
Specificity is also very low due to the fact that the nature of the lesions is not considered, but only their dissemination. None of the criteria are MS-specific. In order to reduce false positives, McDonald et al propose that their criteria should be applied only after any other disease has been ruled out.[3] Currently, sensitivity and specificity are estimated at around 46% and 63% in respect of the criterion "conversion to definite in 40 months".[6] Currently, the best predictor of future development of clinical multiple sclerosis is the number of T2 lesions visualized by magnetic resonance imaging after the CIS.[7]
A more optimistic estimation was given indirectly in a study reported validating clinical criteria against the pathological test "perivenous vs. confluent demyelination".[4] The study used the clinical criteria of McDonald for MS and International Paediatric Multiple Sclerosis Study Group for ADEM, and tried to stablish these parameters for ADEM. It showed that the diagnostic criteria for ADEM matched the pathological diagnosis with a sensitivity of 80% and a specificity of 91%, but applying Bayesian inference also the parameters for McDonald can be estimated, being around 85% for both parameters (result valid when restricted to the sample population, of course).
Asiatic populations
McDonald criteria have been shown to have a low sensitivity and specificity (with respect to the pathological presence of lesions) in Asiatic populations.[8][9] They have good predictive quality (with respect to CIS [clinically isolated syndrome] to CDMS [Clinically Definite Multiple Sclerosis] conversion) when evaluated in non-selected populations.[10]
2010 Revisions
In 2010, the International Panel on Diagnosis of MS met in Dublin, Ireland for a third time to discuss and revise the McDonald diagnostic criteria above.[2] Reasons for revisions to the criteria included the simplification of demonstration of CNS lesions in space and time via imaging, and the above criticisms that the previous criteria did not appropriately apply to non-Western Caucasian populations.[2]
One study has suggested that the new criteria allow a faster diagnosis, but with slight sacrifice in accuracy.[11]
Revised Diagnostic Criteria (2010)
| Clinical Presentation | Additional Data Needed |
|---|---|
| * 2 or more attacks (relapses) * 2 or more objective clinical lesions |
None; clinical evidence will suffice (additional evidence desirable but must be consistent with MS) |
| * 2 or more attacks * 1 objective clinical lesion |
Dissemination in space, demonstrated by: * MRI * or further clinical attack involving different site. New criteria: Dissemination in Space (DIS) can be demonstrated by the presence of 1 or more T2 lesions in at least 2 of 4 of the following areas of the CNS: Periventricular, Juxtacortical, Infratentorial, or Spinal Cord. |
| * 1 attack * 2 or more objective clinical lesions |
Dissemination in time (DIT), demonstrated by: * MRI * or second clinical attack New criteria: No longer a need to have separate MRIs run; Dissemination in time, demonstrated by: Simultaneous presence of asymptomatic gadolinium-enhancing and nonenhancing lesions at any time; or A new T2 and/or gadolinium-enhancing lesion(s) on follow-up MRI, irrespective of its timing with reference to a baseline scan; or Await a second clinical attack. [This allows for quicker diagnosis without sacrificing specificity, while improving sensitivity.] |
| * 1 attack * 1 objective clinical lesion (clinically isolated syndrome) |
New criteria: Dissemination in space and time, demonstrated by:
For DIS: 1 or more T2 lesion in at least 2 of 4 MS-typical regions of the CNS (periventricular, juxtacortical, infratentorial, or spinal cord); or Await a second clinical attack implicating a different CNS site; and For DIT: Simultaneous presence of asymptomatic gadolinium-enhancing and nonenhancing lesions at any time; or A new T2 and/or gadolinium-enhancing lesion(s) on follow-up MRI, irrespective of its timing with reference to a baseline scan; or Await a second clinical attack. |
| Insidious neurological progression suggestive of MS (primary progressive MS) |
New criteria: One year of disease progression (retrospectively or prospectively determined) and
two or three of the following: |
Future directions
The 2010 revision of McDonald criteria explicitly cite two future directions: The use of biomarkers and the improvements in imaging technology.[2]
The European group MAGNIMS periodically publishes guidelines for using MRI in the diagnosis of MS that are updated as MRI technology evolves.[12]
About the use of biomarkers they cite four open ways: The CSF,[13] the serum anti-GAGA4[14] and protein signatures[15] and finally the circulating microRNA[16]
References
- 1 2 Polman, CH; Reingold, SC; Edan, G; Filippi, M; Hartung, HP; Kappos, L; Lublin, FD; Metz, LM; McFarland, HF; O'Connor, PW; Sandberg-Wollheim, M; Thompson, AJ; Weinshenker, BG; Wolinsky, JS (December 2005). "Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria".". Annals of Neurology 58 (6): 840–6. doi:10.1002/ana.20703. PMID 16283615.
- 1 2 3 4 Polman, CH; Reingold, SC; Banwell, B; Clanet, M; Cohen, JA; Filippi, M; Fujihara, K; Havrdova, E; Hutchinson, M; Kappos, L; Lublin, FD; Montalban, X; O'Connor, P; Sandberg-Wollheim, M; Thompson, AJ; Waubant, E; Weinshenker, B; Wolinsky, JS (February 2011). "Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria.". Annals of Neurology 69 (2): 292–302. doi:10.1002/ana.22366. PMC 3084507. PMID 21387374. Cite uses deprecated parameter
|coauthors=(help) - 1 2 3 McDonald WI, Compston A, Edan G, et al. (2001). "Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis" (PDF). Ann. Neurol. 50 (1): 121–7. doi:10.1002/ana.1032. PMID 11456302.
- 1 2 Hans Lassmann, Acute disseminated encephalomyelitis and multiple sclerosis, Brain 2010, DOI: http://dx.doi.org/10.1093/brain/awp342 awp342
- ↑ Engell T (May 1989). "A clinical patho-anatomical study of clinically silent multiple sclerosis". Acta Neurol Scand 79 (5): 428–30. doi:10.1111/j.1600-0404.1989.tb03811.x. PMID 2741673.
- ↑ Swanton J K; et al. (2006). "Modification of MRI criteria for multiple sclerosis in patients with clinically isolated syndromes". J Neurol Neurosurg Psychiatry 77 (7): 830–833. doi:10.1136/jnnp.2005.073247.
- ↑ Cramer SP, Modvig S, Simonsen HJ, Frederiksen JL, Larsson HB (Jul 2015). "Permeability of the blood-brain barrier predicts conversion from optic neuritis to multiple sclerosis". Brain 138: 2571–83. doi:10.1093/brain/awv203. PMID 26187333.
- ↑ HT Chong et al (2006) Proposed modifications to McDonald diagnostic criteria for Asians with multiple sclerosis, Neurology Asia 11:87–90
- ↑ Chong, H; Kira, J; Tsai, C; Ong, B; Li, P; Kermode, A; Tan, C (19 June 2009). "Proposed modifications to the McDonald criteria for use in Asia". Multiple Sclerosis 15 (7): 887–888. doi:10.1177/1352458509104587.
- ↑ Fortini, Alexandre S.; Sanders, Elizabeth L.; Weinshenker, Brian G.; Katzmann, Jerry A. (1 November 2003). "Cerebrospinal Fluid Oligoclonal Bands in the Diagnosis of Multiple Sclerosis Isoelectric Focusing With IgG Immunoblotting Compared With High-Resolution Agarose Gel Electrophoresis and Cerebrospinal Fluid IgG Index". American Journal of Clinical Pathology 120 (5): 672–675. doi:10.1309/Y5VFF2UAW0RK5W63. PMID 14608891.
- ↑ Runia, TF; Jafari, N; Hintzen, RQ (Dec 2013). "Application of the 2010 revised criteria for the diagnosis of multiple sclerosis to patients with clinically isolated syndromes.". European journal of neurology : the official journal of the European Federation of Neurological Societies 20 (12): 1510–6. doi:10.1111/ene.12243. PMID 23906114.
- ↑ Massimo Filippi et al. MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines, The Lancet Neurology, Volume 15, Issue 3, March 2016, Pages 292–303
- ↑ Awad A, Hemmer B, Hartung HP, et al. Analysis of cerebrospinal fluid in the diagnosis and monitoring of multiple sclerosis. J Neuroimmunol. 2010;219:1–7.
- ↑ Brettschneider J, Jaskowski TD, Tumani H, et al. Serum anti-GAGA4 IgM antibodies differentiate relapsing remitting and secondary progressive multiple sclerosis from primary progressive multiple sclerosis and other neurological diseases. J Neuroimmunol. 2009;217:95–101.
- ↑ Quintana FJ, Farez MF, Viglietta V, et al. Antigen microarrays identify unique serum autoantibody signatures in clinical and pathologic subtypes of multiple sclerosis. Proc Natl Acad Sci U S A. 2008;105:18889–18894.
- ↑ Keller A, Leidinger P, Lange J, et al. Multiple sclerosis: microRNA expression profiles accurately differentiate patients with relapsing-remitting disease from healthy controls. PLoS One. 2009;4:e7440.
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