Benign Multiple Sclerosis

The clinical data of 309 patients with definite Multiple Sclerosis were recorded in the European Data Base for Multiple Sclerosis (EDMUS) to determine the prognostic significance of several Demographic and Clinical variables.

An interview with closed questions structured according to standardized criteria of disease phases and courses was used to assess the clinical course. The reliability was evaluated by four trained Neurologists in a sample of 33 patients with Multiple Sclerosis.

Both the within and between rater agreement on data collection was fair to high for the historical variables (K = 0.33-1). Between rater agreement was more variable for the evaluation of 12 different EDMUS event categories (K = 0.3-0.95).

The predictive model for the time to reach a Secondary/Progression, showed that an age at Onset older than 25 (p = 0.006) and an event at Onset followed by disability > or = 3 on the Kurtzke Expanded Disability Status Scale (EDSS; p = 0.004) were the most unfavorable clinical variables in 249 patients with Relapsing/Remitting (180) or Relapsing/Progressive (69) courses.

In the 69 patients with Relapsing/Progressive disease, the time to reach severe disability (EDSS > or = 6) was negatively influenced by a first interval between attacks shorter than one year, a number of bouts with EDSS > 2 in the first two years of the disease, and involvement of the Pyramidal System at Onset (p < 0.05).

In 60 patients with Chronic/Progressive Disease this outcome was negatively influenced by Pyramidal, BrainStem, and Sensory involvement at Onset (p < 0.01).

In a retrospective study we analyzed clinical features and their prognostic significance in 72 patients with Onset of Multiple Sclerosis by the age of 21 years.

In juvenile Multiple Sclerosis disease progression does not depend on age of Onset, Severity of Neurologic Involvement, or PolySymptomatic or MonoSymptomatic Involvement at presentation.

In a hospital-based study of 400 patients with Multiple Sclerosis (MS), 42 per cent of patients who had had MS for 10 years or more had Benign disease. Early age of Onset and a long first remission were significantly associated with a good prognosis.

There was a suggestion that initial presentation with Paresthesia and possibly Optic Neuritis were associated with a Benign prognosis, but the only significant finding was the association between Limb Weakness and a poor outcome (p less than 0.05).

Fewer patients with Benign disease had a Progressive element to their disease than those in the more disabled group (p less than 0.001).

The only laboratory test which was associated with a Benign prognosis was the absence of CSF Myelin Basic Protein in remission.

Abnormalities of Visual Evoked Responses, CSF IgG and peripheral blood T-Lymphocytes appeared to have no value in assessing prognosis in the patients studied.

Objective
To examine the natural history, survival, and prognostic factors in a sample of Turkish MS patients.

Method
This multicenter study included 1,259 definite MS patients diagnosed according to the criteria of Poser et al.

Actuarial analysis of selected disability levels of 3, 6, 8, and 10 achieved with the Expanded Disability Status Scale (EDSS); a multivariate Cox regression analysis for prognostic factors related to time to reach EDSS > or = 6; and Pearson's correlation coefficient for individual factors were performed.

Results
The survival (+/- SE) at 15 years from Onset was 94.6 +/- 2.9%, and at 25 years was 89.0 +/- 5.8%. The disability reached by 15 years was EDSS > or = 3 in 66.4%, EDSS > or = 6 in 41.2%, EDSS > or = 8 in 10.5%, and EDSS = 10 in 5.4%.

The most significant unfavorable prognostic factors were Progressive course (relative risk [RR], 3.73; CI, 2.71 to 5.13) and Sphincter Symptoms at Onset (RR, 1.86; CI, 1.23 to 2.82), followed by male sex, motor symptoms at Onset, and a high attack frequency within the first 5 years.

Primary/Progressive disease was correlated positively with male sex (r = 0.0895, p = 0.001), older age (r = 0.1807, p = 0.000), and Motor (r = 0.1433, p = 0.000) or Sphincter Symptoms (r = 0.1001, p = 0.000) at Onset, unlike Relapsing/Remitting and Secondary/Progressive disease.

Conclusions
Although a slightly better prognosis is observed in the Turkish MS population, early prognostic factors are similar to most of the previous Western series.

Primary/Progressive disease, mostly seen in older men with Motor and Sphincter involvement at Onset, has a worse prognosis and may represent a distinct behavioral variant of MS.

We report a case of Multiple Sclerosis which began at the age of 12 years. Clinical symptoms at Onset were acute, regressive CerebelloVestibular Ataxia and Optic Neuritis.

Twenty-four years later Vertigo, Motor and Sensory deficit of the right lower limb and Grand Mal Seizures developed. CSF and MRI were suggestive of Multiple Sclerosis. The patient is now free of Neurological symptoms with an 8 years' follow-up.

941 cases of Definite/Probable Multiple Sclerosis living in Los Angeles County, California and King and Pierce Counties, Washington in 1970 who had Onset between 1960 and 1969 were followed for mortality and disability through 1980.

Early age of Onset and residence in Washington State were predictors of less rapid and severe subsequent course.

Coordination symptoms at Onset were prognostic of rapid progression to disability and/or early death, whereas early motor weakness was significantly predictive only for Disability.

The presence of Sensory symptoms in addition to Motor and/or coordination symptoms at Onset, however, indicated a better prognosis than coordination and/or Motor symptoms alone.

This observation and the results of regression analyzes indicated that specific groupings of symptoms at Onset were more important for predicting course than the number of symptoms present at Onset.

In 61 of 300 patients suffering from Multiple Sclerosis, disease Onset was RetroBulbar Optical Neuritis.

Comparing the clinical data of these patients with those of the remaining 239 cases, the following typical features were observed: in patients with Optical Neuropathy, Disease Onset is more frequently acute and course, in the early years of the disease, is in fits and starts.

Recurrence frequency is higher in these patients but not significantly so. Prognosis, deduced from the analysis of degrees of invalidity, does not differ substantially between the two groups.

Current techniques of diagnosing Multiple Sclerosis in patients with RetroBulbar Optical Neuritis are also discussed.

The information of 349 cases of Multiple Sclerosis, seen in a Neurological department over a twenty-year period and followed up for a mean of nine years, was analyzed by computerized data processing.

The mean age at Onset was 30.0 years for the Remittent Onset types (82 per cent cases) and 37.3 years for the Progressive Onset types (18 per cent cases).

During the course of the disease:
1. The age of the 'Pure Relapse' stage was 29.2 years
2. The relapse with sequelae stage 33.9 years
3. The Progressive Phase 38.0 years

---

#9

---

#10

---

#11

Clinically Benign Multiple Sclerosis Despite Large T2 Lesion Load: Can We Explain This Paradox?

---

#12

Long Term Clinical Relevance Of Criteria For Designating Multiple Sclerosis As Benign After Ten Years Of Disease

Conclusions
Although designating patients as having a Benign course after 10 years has a poor predictive value; three factors at presentation indicate a better prognosis.