McDonald criteria

Animation showing dissemination of multiple sclerosis lesions in time and space as demonstrated by monthly MRI studies along a year

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 discourage the previously used terms such as "clinically definite" and "probable MS", and propose as diagnostic either "MS", "possible MS", or "not MS".[1] They have undergone revisions in 2005[2] and 2010.[3]

They make use of advances in magnetic resonance imaging (MRI) techniques and are intended to replace the Poser criteria and the older Schumacher criteria. The new criteria facilitate the diagnosis of MS in patients who present with signs and symptoms suggestive of the disease. These include monosymptomatic disease, disease with a typical relapsing-remitting course or insidious progression but no clear attacks and remissions.

The McDonald criteria for the diagnosis of multiple sclerosis were revised in 2005 to clarify exactly what is meant by an "attack", "dissemination" and a "positive MRI", etc.[2]

For some people McDonald's criteria are a clinical definition, being therefore 100% sensitive respect themselves. For others, they are just some diagnosis criteria and therefore have to be evaluated against other more basic criteria, like biopsies or autopsies. Currently sensitivity and specificity of McDonald's criteria are 46% and 63% respect the criterium "conversion to definite in 40 months".[4] and the sensitivity can be around 25% slower respect pathological MS because of silent MS cases[5]

There is an ongoing discusion about whether MS should be considered a pathological or clinical entity. The original article of McDonald states that "MS is a clinical entity and therefore should be diagnosized [sic] with clinical and paraclinical criteria".[1] Nevertheless, they acknowledge the existence of lesion-based MS definition, saying that some other groups consider that "the only proved diagnosis of MS can be made upon autopsy, or occasionally upon biopsy, where lesions typical of MS can be directly detected through standard histopathological techniques".[1]

The 2010 McDonald criteria are regarded as the gold standard 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:

a. Positive brain MRI (nine T2 lesions or four or more T2 lesions with positive VEP)
b. Positive spinal cord MRI (two focal T2 lesions)
c. Positive CSF

Criticism

McDonald criteria have been shown to have a low sensitivity and specificity (with respect to the presence of lesions) in Asiatic populations.[6][7] They present instead a good behaviour (respect CIS to CDMS conversion) when evaluated in non-selected populations.[8]

Sensitivity and specificity respect autopsy confirmation depend strongly from the taken sample, ranging from 0% sensitivity in CIS cases (McDonald positive is incompatible with CIS definition) to nearly 100% in clearly confirmed cases. Therefore, the low sensitivity in early cases hinders the use of McDonalds criteria for research in pathology.

Some authors consider McDonald criteria as an attempt for a clinical definition and propose a pathological definition instead. According to Hans Lassmann, the pathological definition should be preferred because the clinical definitions have problems with differential diagnosis.[9] Besides, subclinical MS cases would not be accepted as MS cases by these criteria.[10]

Finally, McDonald also states "objective evidence of dissemination in time and space of lesions typical of MS is essential in making a secure diagnosis, as is the exclusion of other, better explanations for the clinical features". Therefore, when used as a definition, the McDonald criteria are defining MS just by exclusion of other diseases.

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.[3] 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.[3]

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 a positive CSF and 2 or more MRI lesions consistent with MS
* 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:
1. Evidence for DIS in the brain based on 1 or more T2 lesions in the MS-characteristic (periventricular, juxtacortical, or infratentorial) regions
2. Evidence for DIS in the spinal cord based on 2 or more T2 lesions in the cord
3. Positive CSF (isoelectric focusing evidence of oligoclonal bands and/or elevated IgG index)

References

  1. ↑ 1.0 1.1 1.2 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". Ann. Neurol. 50 (1): 121–7. doi:10.1002/ana.1032. PMID 11456302.
  2. ↑ 2.0 2.1 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.
  3. ↑ 3.0 3.1 3.2 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.
  4. ↑ J K Swanton et al. Modification of MRI criteria for multiple sclerosis in patients with clinically isolated syndromes, J Neurol Neurosurg Psychiatry. Jul 2006; 77(7): 830–833. doi:10.1136/jnnp.2005.073247
  5. ↑ 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.
  6. ↑ HT Chong et al (2006) Proposed modifications to McDonald diagnostic criteria for Asians with multiple sclerosis, Neurology Asia 11:87–90
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ Lassmann, H (2 February 2010). "Acute disseminated encephalomyelitis and multiple sclerosis". Brain 133 (2): 317–319. doi:10.1093/brain/awp342. PMID 20129937.
  10. ↑ McDonnell, G.V.; Cabrera-Gomez, J; Calne, D.B.; Li, D.K.B.; Oger, J (1 March 2003). "Clinical presentation of primary progressive multiple sclerosis 10 years after the incidental finding of typical magnetic resonance imaging brain lesions: The subclinical stage of primary progressive multiple sclerosis may last 10 years". Multiple Sclerosis 9 (2): 204–209. doi:10.1191/1352458503ms890cr. PMID 12708816.
  11. ↑ 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.