Ten percent of patients with MS have a Progressive course from onset with no history of relapses or remissions.
A smaller subgroup follow a similar Progressive course but have a single relapse at some point (Transitional/Progressive [TP] MS). To date these patients have been excluded from receiving licensed treatments for MS and from most therapeutic trials.
To document the clinical and MRI characteristics of a large cohort of Progressive patients, including 158 with Primary/Progressive (PP) MS and 33 with TPMS.
Data from a small reference group of 20 patients with Secondary/Progressive (SP) MS are also presented for reference.
Patients were recruited from six European centers. All underwent a clinical assessment including scoring on the Expanded Disability Status Scale (EDSS) and MRI of the Brain and Spinal Cord.
The men-to-women ratio was 81:77 (51% men) in the PP group, 14:19 (42% men) in the TP group, and 5:15 (25% men) in the SP group.
The mean age at disease onset was significantly higher in the PP group than it was in the other two groups (PP 40.2 years, TP 34.9 years, SP 28.7 years).
On MRI the PP group had lower mean Brain T2 and T1 HypoIntensity lesion loads than the SP group (T2 12.02 versus 27.74 cm3, p = 0.001; T1 4.34 versus 7.04 cm3, p = 0.015).
The SP and TP cohorts had significantly more T2-weighted lesions in the Spinal Cord than the PP patients, and the SP cohort had the greatest degree of Atrophy.
There was a correlation in the PP and TP patients between EDSS score and Brain and Spinal Cord Atrophy (r = 0.3, 0.2, p < or = 0.006) but not with Brain Lesion Load.
The PP and TP patients who presented with Spinal Cord pathology had significantly lower Brain T2 and T1 Lesion loads than those with non-Spinal Cord presentations (p = 0.002).
The monitoring of disease progression in PPMS is difficult, although measures of Atrophy correlate with the EDSS and appear most promising.
This study increases our understanding of this unique patient group, which will be further expanded with the acquisition of serial data.
To study the prevalence and the natural course of Transitional/Progressive Multiple Sclerosis (TPMS). This clinical form is defined by a Progressive course beginning many years after an isolated bout.
The distinction between clinical stages of Multiple Sclerosis has gained attention with the evidence that different PathoPhysiological mechanisms are involved at different stages of the disease, and therefore that different therapeutic goals have to be achieved.
214 consecutive outpatients with Definite or Probable Multiple Sclerosis were studied. The prevalence of TPMS was established.
Patients with TPMS were compared with patients with other Progressive forms of Multiple Sclerosis according to the clinical course.
In a population of 214 MS outpatients
- 55 had Secondary/Progressive disease (SPMS)
- 38 had Primary/Progressive disease (PPMS)
- 12 were identified as having TPMS
Retrospective analysis of the clinical data of these patients shows that TPMS is very similar to SPMS at the beginning of the disease (age at onset, time before progression, clinical symptoms at onset, progression index).
In addition a cohort of patients was prospectively followed up clinically and by MRI for one year.
The results did not show any significant differences between the three forms during this follow up.
However, all data showed a concordant trend suggesting that at this progressive stage, TPMS is closer to PPMS in terms of progression of Disability and new MRI lesions.
Tullman MJ, Oshinsky RJ, Lublin FD, Cutter GR
Mult Scler 2004 Aug;10(4):451-4
The Corinne Goldsmith Dickinson Center for Multiple Sclerosis, Department of Neurology, The Mount Sinai Hospital, New York, NY 10029, USA
Patients with Progressive/Relapsing (PR) Multiple Sclerosis (MS) may accrue disability by incomplete recovery from acute exacerbations and by ongoing deterioration.
In Primary/Progressive (PP) MS, disability accumulates solely by continuous decline.
Because it is the least common form of MS, there is scant information regarding the clinical characteristics of PRMS, but relapses are reportedly uncommon.
The purpose of this study is to describe the clinical features of a cohort of patients with PRMS.
A retrospective chart review of 16 patients diagnosed with PRMS at two academic MS centres over a four-year period.
Nine men and seven women had PRMS. The mean age at onset was 35.1+/-11.2 years. The most common presenting symptom was a progressive myelopathy.
The mean disease duration was 10.1+/-8.5 years and the average time to first exacerbation was 4.1+/-3.7years.
Patients had an average of 2.8+/-2.3 relapses with an annualized relapse rate of 0.6+/-0.8. Time to Expanded Disability Status Scale (EDSS) 6.0 was strongly associated with time to first exacerbation.
Although there was no correlation between the number of relapses and time to EDSS 6.0, there was a modest inverse relation between time to EDSS 6.0 and annualized relapse rate.
Relapses in PRMS may occur more often than previously described and disability may accumulate more rapidly in PRMS than in PPMS.
We suggest differentiating between these two forms of MS.