Multiple Sclerosis and Related Disorders (2015) 4, 93–94
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/msard
EDITORIAL
Editors’ welcome We are delighted to inform readers that the National Library of Medicine has approved our application. From now on MS and Related Disorders will be cited in PubMed. We regard this as recognition of the high quality of our journal, enabled by the efforts of our many authors, reviewers and Editorial Board members. Also we are pleased to report that MSARD is now recognized by Thomson-Reuters Web of Science (formerly ISI Web of Knowledge). Exactly 60 years ago John Kurtzke published his first article that for the first time ascribed a numerical scale to mainly physical disabilities associated with MS. It contained two complementary components, the summation of the neurologic examination into mutually exclusive Functional Systems and their integration into a Disability Status Scale (DSS), with the latter amplified in 1983 into the Expanded Disability Status Scale (EDSS), a system that is still in use today. Few other rating scales have withstood so well the erosion by time and rigors of peer review. About 10 years ago, Ludwig Kappos addressed some of the weaknesses of the original scale, renamed it Neurostatus, a scoring system that has gained near universal use for MS clinical trials. In our first article. Dr Kurtzke addresses the evolution of the EDSS from its inception to the present day. The autonomic nervous system is often damaged in MS and this is the basis of a meta-analysis by Racosta and colleagues. Prevalence estimates of autonomic dysfunction ranged from 10% to 80% with an average of about 42% (for just one abnormal test) likely reflecting the considerable variation in diagnostic criteria and variety of tests undertaken. The autonomic system in MS deserves more attention, especially given the cardiac side effects of some newer disease-modifying and symptomatic therapies. The potential role of melatonin and N-acetylserotonin in MS is another area in need of further appraisal. Melatonin is a powerful antioxidant with anti-inflammatory anti-nociceptive actions; it inhibits demyelination and increases remyelination. As mentioned by the authors (Anderson and Rodrigues) the latitude effects of sunshine on MS susceptibility that are often attributed to vitamin D level may have important effects on melatonin levels, which also vary by latitude. Could melatonin be a key player in MS
http://dx.doi.org/10.1016/j.msard.2015.03.001 2211-0348/& 2015 Published by Elsevier B.V.
susceptibility? A pilot trial of melatonin supplementation in MS might be worthwhile. The CombiRx investigator group describe their large cohort of 1008 relapsing-remitting patients who were enrolled in a phase 3 clinical trial of beta-interferon or Glatiramer acetate. They applied tensor based morphometry to estimate the degree of atrophy in gray matter compared to healthy controls. Thalamic atrophy was the most consistent observation and this correlated with EDSS and disease duration. In another MRIbased study, Damasceno and co-workers emphasize the importance of subclinical MRI activity that may impair cognitive function and demonstrate a correlation between a high burden of cortical lesions and subclinical disease activity. Unmet patient need is a recurrent theme in all countries, no less so in the Irish Republic where over half of the MS sample described by Lonergan et al reported problems. These included access to health care providers, physiotherapy, occupational therapy, social isolation, financial, employment and equipment requirements. Not surprisingly unmet needs were more prevalent with increasing disease severity. Kjolhede and colleagues highlight the relevance of maximal muscle strength (MMS) in prediction of functional capacity in MS patients. Rate of force development (RFD) in knee movement is a novel parameter that they investigated. It was found that RFD and MMS both correlated with functional capacity although MMS of the weaker leg was the better predictor of walking performance. Biotin has received scant attention in the MS literature. This vitamin acts as a co-enzyme for carboxylases in energy metabolism and fatty acid synthesis. It also activates acetylCoA carboxylase, which is probably involved in myelin production. Thus, Sedel and co-workers administered high doses of biotin to patients with progressive MS in a nonblinded proof of concept study. There was some improvement in vision or disability measurements which intriguingly, did not appear for several months. Double blind trials are underway, the results of which we await with considerable interest. Now, take a look at the MRI scans in the report of two cases of Fabry’s disease by Shribman and colleagues. You would be forgiven if you confused these with MS – just like the
94 neurologists who were involved initially. Suspicion of Fabry’s disease should be aroused in anyone with a provisional diagnosis of relapsing MS who is troubled by deafness, retinal vasculopathy, stroke-like episodes or painful sensory symptoms. The classic bathing trunk rash may be absent or just confined to the perineum. The most deceptive cases are late presenting, middle-aged female heterozygotes whose symptoms may be mild. Reduced alpha-galactosidase A level, the key measurement, may be normal as in their case 2, where the diagnosis was substantiated finally by gene sequencing. Accurate diagnosis is now crucial given the opportunity for enzyme replacement therapy.
B. Banwell et al. Finally Dr. Toro and co-workers present a teaching case to remind us of the difficulties in diagnosis and treatment of primary progressive MS.
Editors in Chief Brenda Banwell Gavin Giovannoni Chris Hawkes Fred Lublin
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/msard
EDITORIAL
Editors’ welcome We are delighted to inform readers that the National Library of Medicine has approved our application. From now on MS and Related Disorders will be cited in PubMed. We regard this as recognition of the high quality of our journal, enabled by the efforts of our many authors, reviewers and Editorial Board members. Also we are pleased to report that MSARD is now recognized by Thomson-Reuters Web of Science (formerly ISI Web of Knowledge). Exactly 60 years ago John Kurtzke published his first article that for the first time ascribed a numerical scale to mainly physical disabilities associated with MS. It contained two complementary components, the summation of the neurologic examination into mutually exclusive Functional Systems and their integration into a Disability Status Scale (DSS), with the latter amplified in 1983 into the Expanded Disability Status Scale (EDSS), a system that is still in use today. Few other rating scales have withstood so well the erosion by time and rigors of peer review. About 10 years ago, Ludwig Kappos addressed some of the weaknesses of the original scale, renamed it Neurostatus, a scoring system that has gained near universal use for MS clinical trials. In our first article. Dr Kurtzke addresses the evolution of the EDSS from its inception to the present day. The autonomic nervous system is often damaged in MS and this is the basis of a meta-analysis by Racosta and colleagues. Prevalence estimates of autonomic dysfunction ranged from 10% to 80% with an average of about 42% (for just one abnormal test) likely reflecting the considerable variation in diagnostic criteria and variety of tests undertaken. The autonomic system in MS deserves more attention, especially given the cardiac side effects of some newer disease-modifying and symptomatic therapies. The potential role of melatonin and N-acetylserotonin in MS is another area in need of further appraisal. Melatonin is a powerful antioxidant with anti-inflammatory anti-nociceptive actions; it inhibits demyelination and increases remyelination. As mentioned by the authors (Anderson and Rodrigues) the latitude effects of sunshine on MS susceptibility that are often attributed to vitamin D level may have important effects on melatonin levels, which also vary by latitude. Could melatonin be a key player in MS
http://dx.doi.org/10.1016/j.msard.2015.03.001 2211-0348/& 2015 Published by Elsevier B.V.
susceptibility? A pilot trial of melatonin supplementation in MS might be worthwhile. The CombiRx investigator group describe their large cohort of 1008 relapsing-remitting patients who were enrolled in a phase 3 clinical trial of beta-interferon or Glatiramer acetate. They applied tensor based morphometry to estimate the degree of atrophy in gray matter compared to healthy controls. Thalamic atrophy was the most consistent observation and this correlated with EDSS and disease duration. In another MRIbased study, Damasceno and co-workers emphasize the importance of subclinical MRI activity that may impair cognitive function and demonstrate a correlation between a high burden of cortical lesions and subclinical disease activity. Unmet patient need is a recurrent theme in all countries, no less so in the Irish Republic where over half of the MS sample described by Lonergan et al reported problems. These included access to health care providers, physiotherapy, occupational therapy, social isolation, financial, employment and equipment requirements. Not surprisingly unmet needs were more prevalent with increasing disease severity. Kjolhede and colleagues highlight the relevance of maximal muscle strength (MMS) in prediction of functional capacity in MS patients. Rate of force development (RFD) in knee movement is a novel parameter that they investigated. It was found that RFD and MMS both correlated with functional capacity although MMS of the weaker leg was the better predictor of walking performance. Biotin has received scant attention in the MS literature. This vitamin acts as a co-enzyme for carboxylases in energy metabolism and fatty acid synthesis. It also activates acetylCoA carboxylase, which is probably involved in myelin production. Thus, Sedel and co-workers administered high doses of biotin to patients with progressive MS in a nonblinded proof of concept study. There was some improvement in vision or disability measurements which intriguingly, did not appear for several months. Double blind trials are underway, the results of which we await with considerable interest. Now, take a look at the MRI scans in the report of two cases of Fabry’s disease by Shribman and colleagues. You would be forgiven if you confused these with MS – just like the
94 neurologists who were involved initially. Suspicion of Fabry’s disease should be aroused in anyone with a provisional diagnosis of relapsing MS who is troubled by deafness, retinal vasculopathy, stroke-like episodes or painful sensory symptoms. The classic bathing trunk rash may be absent or just confined to the perineum. The most deceptive cases are late presenting, middle-aged female heterozygotes whose symptoms may be mild. Reduced alpha-galactosidase A level, the key measurement, may be normal as in their case 2, where the diagnosis was substantiated finally by gene sequencing. Accurate diagnosis is now crucial given the opportunity for enzyme replacement therapy.
B. Banwell et al. Finally Dr. Toro and co-workers present a teaching case to remind us of the difficulties in diagnosis and treatment of primary progressive MS.
Editors in Chief Brenda Banwell Gavin Giovannoni Chris Hawkes Fred Lublin
