Tore Broman

Clinical studies on multiple sclerosis: I. PRESENTATION OF AN INCIDENCE MATERIAL FROM GOTHENBURG

Probable and possible MS cases with a debut during the years 1950–1964 within the city of Gothenburg were identified (312 cases). This corresponds to an incidence of 5.3 per 100,000. The material was stratified according to diagnostic probability into three categories. For the final analysis cases with the lowest diagnostic probability were omitted (about 9 %). The follow‐up was completed during 1977, i.e. 13–27 years from onset. A longitudinal analysis of each case was based upon a mixed prospective/retrospective study in which the authors personally examined the majority of the cases during most of the years. The female/male ratio was 1.5–1.6. The mortality rate was higher for males due in part to an earlier progressive development of multiple sclerosis and in part to a higher trend for acquisition of other mortal diseases. Bouts, as the first manifestation of the disease, were more frequent in young ages than in old, and more frequent among females than in males. The average bout frequency decreased significantly with the duration of the disease as well as with the age of onset. The opposite trend was characteristic for development of a progressive course. Among symptoms at onset, those indicating lesions of long sensory and/or motor tracts (particularly the sensory) dominated. Such initial symptoms were seen in 30–40 % of the younger patients and 70 % of the older patients. The rest was divided between cases with optic nerve lesions (20–30 % among younger, 12–15 % among older patients) and brain stem lesions (approx. 25 % among younger, 10–19 % among older patients). The occurrence of various symptoms during the first two decades of the disease was also analyzed and the pattern of symptoms presented graphically. These results will be treated further in subsequent studies.

Differentiation between spasticity and rigidity.

The clinical aspects on spasticity are not only as complicated as the neurophysiological aspects; they also seem to be less exact. Spasticity and rigidity are clinical conditions which may be differentiated from a phenomenological point of view, although mixed forms are common. By definition pure spasticity appears as a pathologically exaggerated resistance of special character to passive movements if these are performed with a certain minimal speed. The pathological resistance is characterized by a swift increase of an elastic brake with a final rebound phenomenon. Clonus is a succession of such rebounds with regular intervals, and a state of pure spasticity will never show a continuous activity upon stretch. A pure rigidity of the lead-pipe type, on the other hand, appears as a constant resistance to passive movements, independent of the speed and manifest from the start of the movements. During rest a spastic muscle is inactive as distinguished from a muscle in rigidity. In several cases of combined spasticity and rigidity the joints are locked by the rigidity to such a degree that the component of spasticity may be difficult to demonstrate. In clinical literature, the definition of spasticity has become so comprehensive that it now seems to be rather obscure. I should like to illustrate this by some examples. Sometimes the Babinski phenomenon is considered to be a sign of spasticity, in spite of the fact that spasticity is the result only of exaggerated muscle-stretch reflexes. In analogy, the concept of central motor-neuron paralysis is erroneously designated as spastic paralysis. I t is true that in most instances it is spastic, but it is not always so. I t is understandable that a simple label is needed to distinguish these conditions from those concerned with peripheral paralysis, and the word “spastics” is short and simple. I t would, however, be desirable that the term be used within established limitations. This is certainly not always the case. The most common misuse of the term “spasticity” seems nowadays to be the characterization of all kinds of rigidity, with the exception of Parkinsonism, as spasticity. In this way the term “rigidity” is prejudicially coupled only with Parkinsonism, in spite of the fact that rigidity is a manifest symptom in many different neurological diseases. Sometimes the term “spasms” is also used instead of rigidity. As examples the following current expressions may be mentioned. Adductor rigidity is often denominated as “adductor spasms” or “spasticity of the adductor muscles.” And the flexion contracture in some paraplegics is designated as

A STUDY OF PROGRESS AND HANDICAP DEVELOPMENT IN MS

Our studies in Gothenburg have been concentrated upon a registration of an incidence material of MS patients with a longitudinal follow-up for some decades. The aim has been to contribute to the analysis of the course of the disease, i.e. its natural history. A survey of the material will be published this year. Most of our interest has been directed towards questions about the onset and the early manifestations of the disease, as they are available for a statistical analysis in our material. Various problems will be treated separately in the future. I originally considered dealing with questions concerning the appearance of haudicap later on as a secondary problem, but the announcement of this symposium has brought about a change in our program. I have therefore tried to prepare the analysis not only with the appearance of neurological symptoms in mind but also with regard to the regional ADL-impairment and the disability. This aim was met with in two different ways. On the one hand the case material was registered in new tabular forms for permanent or progressive handicap with additional notice of the various causes for the handicap. On the other hand. earlier principles for quantification of disability had to be revised and completed. This last-mentioned task was carried out in co-operation with our social worker hlu/gurcfu Svensson and my colleague Loreriz Berpur7r2, who will present it here as a proposal for discussion. The results of the handicap registrations, which are available in stenciles. were based upon our earlier scoring systems of impairment and disability from 1972. This was because the revision of principles for disability-scoring was not completed when I started the registration. These scoring systems are presented in separate stencils, connected with the results of my registrations. I have chosen 5 categories of regional impairment : deficiencies of mental functions, visual function, arm function, station and gait (predominantly the leg function), and bladder and bowel control. Disability was registered in a bipolar way separate for ability and dependence. However, in order to make it easier to compare with the other registrations, the ability scores were inverted into its negative counterpart ~~ reduced ability. The present handicap registrations are based upon a material of 272 cases of probable MS, after exclusion of 1 1 with sonie uncertainties about onset or development of the disease (in the original 283). The material has been subdivided according to sex and age a t onset, as i t was demonstrated earlier that these factors influence the course of the disease. With few exceptions three dominating trends are indicated. First oi. all there is an increase in handicap with time, which of course is a self-evident fact. Exceptions from this trend are explained by the deaths of some

A point scale for the diagnostic probability of MS. A short outline.

As the diagnosis of MS usually cannot be made with certainty in clinical work, a definition is necessary about the principles used in the limitation of the clientele. In scientific connections it would be an obvious advantage if such a definition could be made not only by a linguistic graduation as “probable”, “possible” and so on, but even with the use of numerical values, e.g. probability a t least 95% for diagnosis of MS. Fundamentally there should be an intimate relation between the value of words (the adjectives to the diagnosis) and the value of the corresponding figures. We have started to tackle this problem but found it to be extremely difficult, partly because several of the diagnostic parameters (cf. page 544) are mutually dependent. In any case it seems reasonable to connect the two parameters, age at onset of disease and the empirical significance of the symptoms for the MS diagnosis, as a basis for the whole calculation (“basic figure of the point scale”). As further parameters the least possible number of lesions and the occurrence of remissions and bouts must be closely related to this basic figure. The remaining parameters, the heredity and the CSF-findings (electrophoretic pattern, cytologic picture) may be added as more independent factors in the equation. A fundamental principle should always be the demand for a thorough elimination of cases with other possible diagnoses. When the aim is to determine a border of high degree probability for the MS diagnosis, calculations may be limited to the upper end of the point scale, which eliminates some of the difficulties. There remain, however, many other troubles and a lot of open questions. We therefore think it advisable to confine our position to-day to the following statements: I ) A point scale for the probability of MS diagnosis can only be elaborated as a working hypothesis and has to be checked by a thorough follow-up of the clientele.

NEUROLOGICAL REHABILITATION AND LONG‐TERM TREATMENT

An important consequence of the social cvolutioii in our countries is the expectation of complete care for the patients including adequate follow-up care, rehabilitation or differentiated long-term treatment. No patient should nowadays leave the hospital without a thorough exploration of such demands for further medical or social assistance, and measures should be started as early as possible for his adaptation to a future life worthy of a human being. As a large category of the neurological clientele is in manifest need of further rehabilitation and continued contact with the specialists it is natural that the neurologists in our countries have taken a strong initiative for development of the care for the sick and disabled along these lines. In Sweden this has resulted in a recommendation to a special neurological rehabilitation organization associated with every regional hospital (calculated as a differentiated hospital with all medical disciplines necessary for a population of 1-1+ million). The neurological clientele is numerous enough a t least in densely settled rural areas for motivating a separate organization, although a certain coordination with other rehabilitation activities is advisable, particularly with respect to certain treatment locales, workshops, laboratories and lecture rooms. In order to calculate the size and character of the clientele for estimation of the demand for rehabilitation and different kinds of long-term treatment it is necessary first to state certain definitions and premises. According to our experience we have based our calculations on the following premises: