OT: Measures of Disease Progression

[Guest guest]

In response to the recent post on the LDN board about charting disease

progression. I felt the material would be good for MScured also.

Charting / journaling our disease progression is a critical part of

managing-fixing our condition. Without adequate journaling, out

decisions will be made on a purely emotional basis. Also, human

memory is one of the most error prone methods of storing data. (papers

available on request)

An example of my Excel journal may be found in the " pics " section of

my MySpace profile. The Excel journal allows for data to be

categorized, searched for, and reports made. Look for the picture of

the cover of my hand written journal. A copy of my stool test may

also be found in the " pics " section. Look for the picture of me

throwing a toilet.

http://www.myspace.com/alrightguy123

Now for a copy of an article from www.uptodate.com. This is a website

for physicians and has a subscription fee of about $500 per year.

Frequently university libraries will have access to this website.

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Measures of disease progression: The Kurtzke disability score (DSS)

and the expanded version (expanded disability status score, or EDSS)

are commonly used indices of clinical disability in MS. These indices

use numbers ranging from 0 for normal examination and function to 10

for death due to MS. The scales are nonlinear, with great emphasis on

ambulation capabilities with scores above 4.

Most MS " populations " have bimodal distributions of EDSS scores, with

peaks at values of 1 (no disability with minimal neuralgic signs) and

6 (cane needed for walking). The time spent by a patient at a given

level of disability varies with the score. The median time spent with

a DSS score of 4 or 5 is 1.2 years, while the median time spent at DSS

1 is four years and at DSS 6 three years. These results have powerful

implications for the conduct of clinical studies with respect to

patient selections, stratification, and duration of follow-up: if many

patients of DSS 1 or 6 are included, little movement will be seen in a

group followed for a year or two.

The EDSS is universally used in clinical trails, but it has a number

of serious limitations. Inter-and-intrarater variations in scoring

are common. EDSS scores of 4 and higher depend almost entirely on the

ability to walk. Problems such as the development of dementia, visual

loss, or hand weakness may pass undetected by the scoring. This,

other outcome measures should be also used, and minor changes in the

EDSS alone should not be over interpreted.

Disability progression - Progression of disability due to MS is highly

variable, but accumulating evidence suggests that progression in most

patients with MS is slow. On of the largest longitudinal studies

followed 2319 patients from British Columbia for 22,723 patient years.

Disability scores were prospectively assigned in greater than 95

percent of the patients.

The following observations were reported

• The median time from disease onset to EDSS 6 (cane needed form

walking) was 27.9 years; the median age from birth to EDSS 6 was 59 years.

• A primary progressive course was associated with more rapid disease

progression than a relapsing course, and was a risk factor in

multivariate analysis for time to use a cane (EDSS 6) from both MS

onset (hazard ration

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2.90, 95% CI 2.39 – 3.52) and from birth )

HR 2.68, 95% CI 2.20-3.26)

• Although men progressed more quickly than women from onset, both men

and women required a cane at similar ages (58.8 and 60.1 yrs), and

male sex was not associated with a worse outcome after controlling for

other factors.

• The type of onset symptoms (eg, motor, sensory, optic neuritis,

cerebella, ataxia, or brainstem did not predict disease progression

after controlling for other factors.

• A younger age at onset was associated with slower progression, but

patients older at onset were consistently older when they progressed

to EDSS 6 than patients younger at onset. Similar results were found

in a large epidemiology study from France.

Some earlier studies suggest that MS progressed more rapidly. As an

example, a 25-year follow-up study of 308 patients with MS found that

50 percent of the patients reached EDSS 6 within 16 years of onset.

Benign MS – Benign forms of MS do occur. Approximately 15 percent of

patients will never experience a second relapse, although the exact

frequency of this benign form of disease is unknown since many of

these individuals never come to medical attention. Among patients in

a population –based cohort study who had MS for 10 years or more,

about 17 percent had minimal or no disability.

Autopsy studies have found a significant number of cases with CNS

pathology consistent with MS but no documented clinical evidence of

disease. MRI studies of asymptomatic relative of MS patients have

discovered lesions consistent with demyelination in up to 15 percent

of these relatives. The use of MRI may expand the spectrum of MS by

detecting milder cases that previously were not included in prognosis

studies.

In our experience and that of others, patients who have had a known

benign course for 15 years will only rarely develop a more severe

course. In one long-term cohort, for example, only 7.5 percent of

patients with mild disease (EDSS score of 0 to 3) five years after

diagnosis progressed to severe disease (EDSS 6) by 10 years, and only

11.5 percent by 15 years. Similar results were noted in a second

cohort, 17 percent of whom had minimal or no disability (EDSS score of

2 or lower) at study onset despite a 10-year or longer history of MS.

The longer the duration of MS and the lower the disability, the more

likely the patient was to remain stable and not progress.