Review
Diagnostic criteria for multiple sclerosis: an historical review

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Abstract

Starting with Charcot, diagnostic criteria for multiple sclerosis (MS) have evolved to reflect advances in our understanding of the disease and the development of new diagnostic techniques, and from purely clinical considerations to increasing dependency upon imaging of the central nervous system. The MS diagnostic process was revolutionized by the 1981 introduction of magnetic resonance imaging (MRI), but the increasing reliance upon this technique has led to a surge in erroneous diagnoses, mostly because of the failure to distinguish between MS and disseminated encephalomyelitis (DEM), as well as mounting disregard for the data obtained from the traditional history and physical examination. The most recent scheme of McDonald et al. incorporated quantitative MRI criteria of dubious origin and reliability, but failed to provide qualitative, illustrative ones that would help differentiate between MS and DEM. The choice will have to be made by the neurological community between basing the diagnosis of MS on the MRI alone, or to use it as one aspect of a comprehensive clinical diagnostic algorithm. There will never be a substitute for the experienced and astute clinician’s ‘feel’ for the patient.

Introduction

Multiple sclerosis (MS) is the most common neurological disease of young adults, and despite the fact that the disease was described over 130 years ago, problems with its exact diagnosis remain. The recent introduction of long-term therapeutic agents makes the accuracy of the diagnosis more imperative than ever. Its diagnosis has been, paradoxically, either very easy or very difficult. The clinical symptomatology, the typical transient character of the complaints and of abnormal neurological signs, are most often sufficient. On the other hand, the short and sometimes bizarre nature of the complaints may lead the clinician astray.

The evolution of diagnostic criteria reflects the hope that the use of laboratory examinations of various kinds would facilitate the diagnosis and make it more reliable. This review of the historical development of diagnostic criteria, which demonstrates the progressive incorporation of such technological aids, reveals that this goal remains to be achieved. Nevertheless, the increasing reliance of neurological practitioners on laboratory procedures, in particular on imaging, threatens to dominate the diagnostic process and overtake the long standing, more reliable tradition of the detailed history and careful neurological examination.

The distinctive geographic and ethnic distribution of the disease seems to play an important role in influencing the diagnosis. In areas where the disease is very rare, such as sub-Saharan Africa, neurologists are reluctant to consider the diagnosis, thus making MS appear even rarer than it actually is.

The various diagnostic schemes that have been published over the years will be dealt with in chronological order.

Section snippets

The development of diagnostic criteria for MS

Jean-Martin Charcot deserves credit for being the first to delineate diagnostic criteria for MS: the triad of nystagmus, intention tremor and scanning speech that appeared in one of his Leçons du mardi in 1868 that is said to have been the symptoms exhibited by his housemaid. Undocumented rumor is that she actually suffered from Friedreich’s ataxia rather than MS. He did not propose them as formal criteria, but rather as characteristics of the disease. Clearly, they are far from specific.

No

The Schumacher et al. criteria

In 1961 concern for the problems inherent in the conduct of therapeutic trials for MS led the National Institute of Neurological Diseases and Blindness of the United States National Institutes of Health to form a panel charged with the task of “achieving definition and uniformity in the use of terms in order to provide a common ground of acceptable terminology among different investigators and writers. The overall aim was to relate established principles of scientific investigation and control

The McAlpine, Lumsden, and Acheson criteria

In 1972, in their influential book, McAlpine (Fig. 6), Lumsden (Fig. 7), and Acheson (Fig. 8) [7] avoided the classification of definite MS, listing only latent probable, probable, and possible MS. A history of remitting symptoms was accepted, but the presence of physical signs and “evidence of MS in the past history”—a rather vague feature were required. The statement in the probable category that “allowance must be made for those from whom it is impossible to obtain a reliable history” is

The Poser et al. criteria

One problem with the various published diagnostic classifications is their discrepant terminology, what is considered probable in one is called definite in another. Another problem is that all the proposed schemes require subjective judgment. Although this may be desirable and inescapable in clinical practice, it proves to be an obstacle in choosing cases and controls for therapeutic trials and epidemiological studies. The problem cannot be completely overcome but can be diminished in the

Onset symptoms

One problem that emerges from the results of epidemiological studies is that there is little agreement on which symptoms and signs can be used for dating the clinical onset of the disease. To remedy this problem a list of such criteria was published in 1995 [15] (Table 6).

Pseudo-exacerbations

Little attention has been paid to the possible confounding effect of counting pseudo-exacerbations in the course of therapeutic trials. Donald Paty and George Ebers were the first to call attention to this problem in 1997 [16]. They listed the following features as useful in identifying pseudo-relapses (recurrence of old symptoms, short duration of symptoms, definable associated toxic or metabolic changes such as fever, and disappearance of symptoms and signs when the metabolic change has been

Magnetic resonance imaging

The emphasis on clinical history and examination, which had been the foundations of the diagnosis of MS for over 100 years, changed dramatically with the introduction of MRI by Young et al. in 1981 [17]. It took less than a decade for this technique to become almost universally adopted, and unfortunately, to become the exclusive basis for diagnosis by many clinical neurologists all over the world. This has occurred despite the repeated assertion that the abnormalities were non-specific and

Accuracy of diagnosis

The questionnaire survey described above, which was published in 1965 [4], showed that for a group of patients, neurologists were correct in 60% of the cases, although they were not the same cases. In 1987, Hankey and Stewart-Wynne [24] reclassified the cases of MS used in the 1981 Western Australia survey according to the Rose et al. criteria [8]. Only 68% of the 318 cases said to have MS were then called definite or probable. The influence of the MRI and its misinterpretation was apparent in

The McDonald et al. diagnostic criteria

It was inevitable that the rapidly growing importance of MRI in the diagnostic process would lead to recommendations for new diagnostic criteria for MS. They were developed by an international committee of neurologists and published in 2001 [26] (Table 7). Two major changes were introduced, MRI criteria were incorporated into the scheme, and long-needed guidelines for the diagnosis of primary progressive MS were defined. While there have been some improvements on the Poser et al. criteria,

Conclusion

The face of medicine has been rapidly changing for the past 60 years. Neurology, like other medical disciplines, has become increasingly dominated by reliance on sophisticated and expensive technology, replacing intuitive, astute anamnestic investigation. We are no longer teaching our students and trainees the art of obtaining a history. Landau expressed this very well [31]: “Our ‘non system’ is pushing neurological practice away from true physician/doctor skills toward predominant occupation

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