BY ED SUSMAN
PARIS—A high percentage of patients diagnosed with multiple sclerosis (MS) did not progress and may fit into a subgroup with a "benign" form of the disease, researchers reported here at the joint meeting of the European Committee for Treatment and Research in Multiple Sclerosis and the Americas Committee for Treatment and Research in Multiple Sclerosis.
Of 77 patients for whom the research team had consistent follow-up, 31 individuals or 41 percent had normal disability scores, reported Karen Chung. MBBS, BSc (Hons), a clinical research fellow at University College London Institute of Neurology.
"Very stable 'benign' multiple sclerosis does exist," she said, in her oral presentation. "An Expanded Disability Status Scale threshold score of 3.5 showed no significant occupational or cognitive impairment. All these patients remained in the relapsing-remitting phase of MS for at least 30 years."
In the initial cohort, Dr. Chung and colleagues enrolled and followed prospectively 132 patients in the 1984-1987 period. About 61 percent of the group were women; the mean age of the cohort was 31.5 years. About 52 percent presented with optic neuritis; 27 percent had a spinal cord syndrome, and 20 percent were diagnosed with brainstem syndrome. They were assessed at multiple intervals — at five, 10, 14, 20, and 30 years.
During the 30-year period, nine patients were lost to follow-up, and 29 died. Of the remaining 94 patients, 63 underwent scanning and medical assessment; three just had clinical assessment without scanning; 25 underwent an Expanded Disability Status Scale test via telephone using a validated questionnaire, and three of the patients declined to participate, Dr. Chung said.
The 30 patients who only demonstrated a clinically isolated syndrome were dropped from the updated analysis, she said, so that only patients with diagnosed MS who had a history of relapse were evaluated. While 44 percent have continued to be diagnosed with relapsing-remitting MS, 33 percent of the patients are now diagnosed with progressive disease, and 20 percent have died.
All of the study participants with relapsing-remitting disease and Expanded Disability Status Scale scores less than 4 are still working full-time or part-time or retired at pension age, Dr. Chung said.
This study was funded by a grant from the MS Society of Great Britain and Northern Ireland. Dr. Chung has received funding for travel to scientific meetings from Teva and has received honoraria for speaking at scientific and patient-education meetings for Biogen-Idec and Roche.
In a second study presented at the meeting, Ali Manouchehrinia, PhD, a postdoctoral fellow in neuroepidemiology at the Karolinska Institute, Solna, Sweden, presented similar findings in his study of benign multiple sclerosis.
For his study, he considered a case of benign MS meant a patient had the disease for at least 15 years and had an Expanded Disability Status Score of 3 or less. He noted that in the Swedish Gothenburg cohort after 50 years, 10 percent of patients had minimal neurological and neuropsychiatric disability.
Dr. Manouchehrinia evaluated patients in the Swedish national multiple sclerosis registry, identifying 11,222 relapse onset patients with available data – 2,420 individuals diagnosed with benign MS and 8,802 with non-benign disease. He found that the benign patients tended to be younger at disease onset – 28 years versus 32 years; and that benign patients were more likely to be female –75 percent versus 70 percent. Complete recovery from the first attack occurred more often with the patients who later had a benign course.
"Multiple sclerosis is not a homogeneous disease," he said. "A subset of patients with MS accumulate physical disability at much lower rates, are cognitively less impaired, have a better general state of health, and survive significantly longer."
But, Dr. Manouchehrinia advised in his oral presentation, "Benign multiple sclerosis is a retrospective diagnosis and can by no means be used to predict the future of a MS patients."
Dr. Manouchehrinia had no financial disclosures.
Commenting on the study, Patrick Vermersch, MD, professor of neurology, head of the Multiple Sclerosis Clinic, and vice dean of the faculty of medicine at the University of Lille, France, said: “I am not completely sure if benign multiple sclerosis really does exist.”
“As Professor McDonald once said, 'We know only that MS is benign when the patient is 80 years old.' The major issue is that there is no way to predict who has benign multiple sclerosis. We think that if there is no disability and no MRI lesions after 20 years then statistically it is a good prognosis,” Dr. Vermersch told the Neurology Today Conference Reporter. “But if we look at these patients with low disability, half of them have quite severe episodes of depression, and half of them have some degree of cognitive dysfunction and usually quite severe fatigue syndromes.”
“These people may look normal and be able to walk,” he continued. “They still may have relapses, but it is just the emerged part of the iceberg. Even if they have no clinical relapses, they continue to accumulate lesions in the brain, and continue to degenerate,” he said.
LINK UP FOR RELATED INFORMATION:
ECTRIMS Abstract 199: Kung KK, Barkhof F, Altmann DR, et al. Does 'benign' multiple sclerosis exist? A 30-year follow-up study of people presenting with clinically isolated syndrome.
ECTRIMS Abstract 200: Crielaard L, Kavalimuas A, Ramanujam R, et al. Course of disease of initially benign multiple sclerosis patients: Long-term follow-up results of physical disability and cognitive impairment.