The McDonald Criteria for Multiple Sclerosis: time for clarification

2010 ◽  
Vol 16 (5) ◽  
pp. 566-575 ◽  
Author(s):  
Christopher H Hawkes ◽  
Gavin Giovannoni

The McDonald Criteria for Multiple Sclerosis in general have replaced the Poser criteria. Despite this, many consider that there are still areas of ambiguity. In this study, neurologists completed a questionnaire about familiarity with, usefulness and applicability of the McDonald Criteria for Multiple Sclerosis. Understanding of ‘attack’; objective clinical evidence; ‘two or more lesions’ and their ability to interpret case scenarios was evaluated. Responses were analysed overall and by skill group. Ninety-seven consultants and 30 trainees responded, of whom 37 operated a multiple sclerosis clinic (‘experts’). Some (62%) thought the McDonald Criteria for Multiple Sclerosis were useful, and 31% found them confusing or difficult to apply; 38% thought the criteria should be applied universally, others (14—28%) favoured their use for drug trials/research, and 17% rarely used them. Thirty-six (29.1%) thought the McDonald Criteria for Multiple Sclerosis specified two categories: ‘MS and not MS’, but others considered ‘possible’ or ‘probable’ multiple sclerosis were permitted. Experts understood better ‘an attack’ and ‘objective clinical evidence’. All skill groups comprehended poorly what constituted an episode of demyelination, and whether Lhermitte’s phenomenon was acceptable as evidence for cervical cord demyelination. A consistent response was given by 44—50% to ‘two or more lesions’, although this is not well defined. Criteria for primary progressive multiple sclerosis were understood well. We conclude that the McDonald Criteria for Multiple Sclerosis have improved diagnosis but areas of misinterpretation remain, particularly the definition of ‘an attack’, ‘objective clinical evidence’ and ‘two or more lesions’. There was uncertainty about how many multiple sclerosis categories were permitted and whether the terms ‘possible’ and ‘probable’ were allowable. Further clarification might allow the criteria to be applied more consistently.

2021 ◽  
pp. 62-64
Author(s):  
I. Vanessa Marin Collazo

A 58-year-old, right-handed man with a medical history of nephrolithiasis, essential hypertension, and type 2 diabetes sought care for a 6-year history of gait impairment. Initially, he noted subtle left foot and ankle weakness with associated falls that progressed over time. Two to 3 years later he again noted progressive left leg weakness and new arm weakness. Subsequently, progressive pain developed on the soles of his feet in addition to edema with erythematous discoloration around the left ankle and foot. On neurologic examination, he was found to have mild upper motor neuron pattern weakness in the left arm and leg, most pronounced in the left hand finger extensor and left hip flexion and abduction. Left patellar reflex was brisk, and there was an extensor Babinski sign on the left. There was mild reduction in pinprick sensation in both feet. His gait was spastic with left leg circumduction. Magnetic resonance imaging of the brain showed left-sided predominant periventricular and subcortical T2 fluid-attenuated inversion recovery hyperintensities. Magnetic resonance imaging of the cervical and thoracic spinal cord showed intramedullary cord T2 signal hyperintensities, eccentrically located on the left at C3, C5, C6, on the right at C7 to T1, and centrally at T4/T5 and T8/T9. A diagnosis of primary progressive multiple sclerosis was made. The patient met the 2017 McDonald criteria for primary progressive multiple sclerosis. After the diagnosis was confirmed and comprehensive education about the disease and the role of disease-modifying therapy was discussed with the patient, he was started on ocrelizumab. Gabapentin was started for management of painful foot paresthesias. Vitamin D3 supplementation was started. Physical therapy was also initiated. Multiple sclerosis is a chronic immune-mediated demyelinating disease of the central nervous system and is the leading cause of disability in the young population. Approximately 1 million people in the United States currently have multiple sclerosis.


2004 ◽  
Vol 10 (3_suppl) ◽  
pp. S36-S45 ◽  
Author(s):  
Massimo Filippi ◽  
Marco Rovaris ◽  
Maria A Rocca

Patients with primary progressive multiple sclerosis (PPMS) typically experience a progressive disease course from onset, leading to the accumulation of severe neurological disability. This is in contrast with the observation that the burden and activity of lesions on conventional magnetic resonance imaging (MRI) scans of the brain are much lower in patients with PPMS than in those with other less disabling forms of the disease. Studies with structural and functional MRI techniques are providing relevant contributions to the understanding of the mechanisms underlying the accumulatio n of irreversible neurological deficits in patients with PPMS. The results of these studies underpin that the main factors possibly explaining the clinical/MRI discrepancy observed in patients with PPMS include the presence of a diffuse tissue damage that is beyond the resolution of conventional imaging, the extent of cervical cord damage, and the impairment of the adaptive capacity of the cortex to limit the functional consequences of subcortical pathology.


Author(s):  
Loren A. Rolak

Because no laboratory test or imaging study is both highly sensitive and highly specific for multiple sclerosis, some uncertainty often accompanies the diagnosis. Various guidelines have been developed to assist the clinician, including the much-modified and commonly used McDonald criteria. Typical features of the history, the physical examination, and magnetic resonance imaging findings usually allow for a secure diagnosis. However, limitations inherent in any testing modality still pose challenges. This is especially true for diagnosis of primary progressive multiple sclerosis because the sensitivity and specificity of the evaluations are lower.


2020 ◽  
Vol 27 (1) ◽  
pp. 28-38 ◽  
Author(s):  
Rosa Cortese ◽  
Carmen Tur ◽  
Ferran Prados ◽  
Torben Schneider ◽  
Baris Kanber ◽  
...  

Background: Pathology in the spinal cord of patients with primary progressive multiple sclerosis (PPMS) contributes to disability progression. We previously reported abnormal Q-space imaging (QSI)-derived indices in the spinal cord at baseline in patients with early PPMS, suggesting early neurodegeneration. Objective: The aim was to investigate whether changes in spinal cord QSI over 3 years in the same cohort are associated with disability progression and if baseline QSI metrics predict clinical outcome. Methods: Twenty-three PPMS patients and 23 healthy controls recruited at baseline were invited for follow-up cervical cord 3T magnetic resonance imaging (MRI) and clinical assessment after 1 year and 3 years. Cord cross-sectional area (CSA) and QSI measures were obtained, together with standard brain MRI measures. Mixed-effect models assessed MRI changes over time and their association with clinical changes. Linear regression identified baseline MRI indices associated with disability at 3 years. Results: Over time, patients deteriorated clinically and showed an increase in cord QSI indices of perpendicular diffusivity that was associated with disability worsening, independently of the decrease in CSA. Higher perpendicular diffusivity and lower CSA at baseline predicted worse disability at 3 years. Conclusion: Increasing spinal cord perpendicular diffusivity may indicate ongoing neurodegeneration, which underpins disability progression in PPMS, independently of the development of spinal cord atrophy.


2012 ◽  
Vol 19 (8) ◽  
pp. 1095-1100 ◽  
Author(s):  
SB Kelly ◽  
K Kinsella ◽  
M Duggan ◽  
N Tubridy ◽  
C McGuigan ◽  
...  

Background: The diagnostic criteria for primary–progressive multiple sclerosis (PPMS) have undergone revision over the last 20 years. Cerebrospinal fluid oligoclonal bands (CSFOBs) have received less emphasis in recent revisions of the McDonald criteria. The aim of this study was to examine the sensitivity of the diagnostic criteria for PPMS with particular reference to spinal cord criteria and examine the utility of CSFOBs in a cohort of PPMS patients. Methods: All new PPMS diagnoses between 1990 and 2011 were identified. Baseline clinical details and paraclinical evaluations including MRI of the brain, spinal cord, CSF and visually evoked responses (VERs) were assessed. The proportion of patients who met the requirements for diagnosis of PPMS on the basis of Thompson’s and the McDonald Criteria (2001, 2005, 2010) were determined. Results: There were 88/95 PPMS patients who had at least two diagnostic investigations. The sensitivity of Thompson’s and the McDonald 2001 criteria was 64%; the McDonald 2010 revisions gave the highest sensitivity (77%); the McDonald 2005 criteria had intermediate sensitivity (74%). The combination of CSFOBs and MRI of the brain yielded the greatest number of patients demonstrating dissemination in space (DIS) on only two investigations. VERs did not aid diagnosis. Reducing requirements for the number of spinal cord lesions (symptomatic or not) to one increased diagnostic sensitivity to 84%. Conclusion: An alternative criterion requiring two of: i) MRI of the brain with one or more lesions in two of three regions typical for demyelination; ii) the presence of one T2-weighted spinal cord plaque (typical for demyelination); iii) CSFOBs; would increase the diagnostic sensitivity for PPMS.


2009 ◽  
Vol 15 (12) ◽  
pp. 1459-1465 ◽  
Author(s):  
X. Montalban ◽  
J. Sastre-Garriga ◽  
M. Filippi ◽  
Z. Khaleeli ◽  
N. Téllez ◽  
...  

The diagnostic criteria used in primary progressive (PP) and relapsing—remitting (RR) multiple sclerosis (MS) show substantial differences. This introduces complexity in the diagnosis of MS which could be resolved if these criteria could be unified in terms of the requirements for dissemination in space (DIS). The aim of this study was to assess whether a single algorithm may be used to demonstrate DIS in all forms of MS. Five sets of RRMS criteria for DIS were applied to a cohort of 145 patients with established PPMS (mean disease duration: 11 years — PPMS-1): C1: Barkhof—Tintoré (as in 2005 McDonald’s criteria); C2: Swanton et al. (as in JNNP 2006); C3: presence of oligoclonal bands plus two lesions (as in McDonald’s criteria); C4 and C5: a two-step approach was also followed (patients not fulfilling C1 or C2 were then assessed for C3). Two sets of PPMS criteria for DIS were applied: C6: Thompson et al. (as in 2001 McDonald’s criteria); C7: 2005 McDonald criteria. A second sample of 55 patients with less than 5 years of disease duration (PPMS-2) was also analysed using an identical approach. For PPMS-1/PPMS-2, fulfilment was: C1:73.8%/66.7%; C2:72.1%/59.3%; C3:89%/79.2%; C4:96%/92.3%; C5:96%/85.7%; C6:85.8%/78.7%; C7:91%/80.4%. Levels of fulfilment suggest that the use of a single set of criteria for DIS in RRMS and PPMS might be feasible, and reinforce the added value of cerebrospinal fluid (CSF) findings to increase fulfilment in PPMS. Unification of the DIS criteria for both RRMS and PPMS could be considered in further revisions of the MS diagnostic criteria.


2002 ◽  
Vol 8 (6) ◽  
pp. 532-533 ◽  
Author(s):  
N F Kalkers ◽  
F Barkhof ◽  
E Bergers ◽  
R van Schijndel ◽  
C H Polman

Progressive axonal loss is the most likely pathologic correlate of irreversible neurologic impairment in primary progressive multiple sclerosis. In a run-in versus treatment trial, we show that the neuroprotective agent riluzole seems to reduce the rate of cervical cord atrophy and the development of hypointense T1 brain lesions on magnetic resonance imaging.


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