DOCSLIB.ORG

Demyelinating Disorders

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Demyelinating Disorders 4 Demyelinating Disorders MULTIPLE SCLEROSIS Perhaps no neurological condition better illustrates the evolution of diagnostic criteria over time or has better known criteria than multiple sclerosis (MS). The criteria have evolved with technological advances in neuroimaging and diagnostic testing, so-called paraclinical data, as well as increased knowledge about the natural history of MS. However, despite the apparent increasing rate of diagnostic acumen and technical sophistication, we have had revisions 18 years, respectively, after each set of published criteria—Schumacher, 1965; Poser, 1983; and McDonald, 2001. Because of the increased reliance on magnetic resonance imaging (MRI) in the McDonald criteria, and the changes in terminology, many studies have looked at both the sensitivity and specificity of MRI in predicting MS after a single event, and comparisons with the Poser criteria. In light of the development of new therapies for MS and the realization that axonal loss may occur with demyelina- tion, even with a clinically isolated syndrome, there has been increased emphasis on early diagnosis by whatever criteria the clinician chooses. Tintore et al. examined individuals with a clinically isolated syndrome (that is, patients with a single episode) and MRI findings suggestive of MS. At a 1-year follow-up, 11% had clinically def- inite MS by Poser criteria and 37% by McDonald criteria. Eighty percent of those who fulfilled the McDonald criteria had a second event over the follow-up period (mean: 49 months). In another study, Fangerau et al. found that all patients with definite MS by Poser criteria met criteria by McDonald criteria. However, many of the patients labeled as laboratory-supported MS by Poser cri- teria would be classified as possible MS because of the inability to meet the additional data or sec- ond attack criteria. The MRI itself has been investigated in terms of interrater reliability. Even within this narrower field, there exist several criteria for what constitutes a positive MRI scan (for current criteria, see Tables 4 and 5). In general,there was poor agreement for the total number of lesions, and better agree- ment for the presence of a lesion. Interestingly, lesion location influenced agreement rates, and using a dichotomous lesion vs no lesion on T2-weighted images produced good agreement. NEUROMYELITIS OPTICA Also known as Devic’s disease, neuromyelitis optica (NMO) has been considered a syndrome in its own right or a variant of MS. It has generally been considered a rare disease, but it is unclear if this is because of true rarity vs poor ascertainment methods, or whether its incidence varies by population. It has been estimated to cause about 5% of demyelinating disorders in Japan and India. All of the criteria listed in Table 6 agree that NMO must present with optic neuritis and myelitis, but the relative timing is not always specified. The addition of neuroimaging criteria, especially the From: Current Clinical Neurology: Diagnostic Criteria in Neurology Edited by: A. J. Lerner © Humana Press Inc., Totowa, NJ 63 64 Diagnostic Criteria in Neurology Table 1 Schumacher Criteria for the Diagnosis of Multiple Sclerosis 1. Neurological examination reveals objective abnormalities of central nervous system (CNS) function. 2. History indicates involvement of two or more parts of the CNS. 3. CNS disease predominately reflects white matter involvement. 4. Involvement of CNS follows one of two patterns: a. Two or more episodes, each lasting at least 24 hours and at least 1 month apart. b. Slow or stepwise progression of signs and symptoms over at least 6 months. 5. Patient aged 10–50 years old at onset. 6.Signs and symptoms cannot be better explained by other disease process. Adapted from Schumacher FA, Beeve GW, Kibler RF, et al. Problems of experimental trials of multiple sclerosis. Ann NY Acad Sci 1965;122:552–568. Table 2 Poser Criteria for the Diagnosis of Multiple Sclerosis Clinically definite multiple sclerosis (MS) • Two attacks and clinical evidence of two separate lesions. • Two attacks, clinical evidence of one, and paraclinical evidencea of another separate lesion. Laboratory-supported definite MS • Two attacks, either clinical or paraclinical evidence of one lesion, and cerebrospinal fluid (CSF) immunological abnormalities. • One attack, clinical evidence of two separate lesions, and CSF abnormalities. • One attack, clinical evidence of one, and paraclinical evidence of another separate lesion, and CSF abnormalities. Clinically probable MS •Two attacks and clinical evidence of one lesion. • One attack and clinical evidence of two separate lesions. • One attack, clinical evidence of one lesion, and paraclinical evidence of another separate lesion. Laboratory-supported probable MS • Two attacks and CSF abnormalities. aParaclinical evidence includes the results of magnetic resonance imaging, evoked potentials, or other diagnostic tests of central nervous system dysfunction. (Adapted with permission from Poser CM, Paty DW, Scheinberg L, et al. New diagnostic criteria for multiple scle- rosis: guidelines for research protocols Ann Neurol 1983;13:227–231, and from John Wiley and Sons.) requirement that the spinal lesion span several vertebral levels, has helped in excluding cases of MS that clinically present as NMO. The differential diagnosis must also include individuals with a his- tory of isolated optic neuritis who later develop a myelopathy. Care must be taken to ascertain that mass lesions, cervical spondylosis, or other pathological entities have been excluded. Conversely, there may be individuals with incomplete syndromes, such as recurrent optic neuritis or recurrent “transverse myelitis,” without other extensive disease who should be considered at-risk for develop- ing NMO. This is important, because the clinical course of NMO is often more virulent than typical MS. The current standard therapy for attack prevention combines oral corticosteroids with immuno- suppressive agents, such as azathioprine. Early initiation of therapy is recommended to prevent attack-related disability. TRANSVERSE MYELITIS An inflammatory disease of the spinal cord, transverse myelitis is a rare but often devastating syn- drome. Its incidence is about one to four individuals per million per year. It may be associated with Demyelinating Disorders 65 Table 3 McDonald Criteria for the Diagnosis of Multiple Sclerosis Clinical presentation Additional data needed for MS diagnosis Two or more attacks, objective clinical Nonea evidence of two or more lesions Two or more attacks, objective clinical Dissemination in space, demonstrated by: evidence of one lesion • MRI, or • Two or more MRI-detected lesions consistent with MS plus positive CSF, or • Further clinical attack, implicating a different site One attack; objective clinical evidence • Dissemination in time (demonstrated by MRI), or of two or more lesions • Second clinical attack One attack; objective clinical evidence Dissemination in space, demonstrated by: of one lesion (monosymptomatic • MRI, or presentation, clinically isolated • Two or more MRI-detected lesions consistent with syndrome) MS plus positive CSF, and Dissemination in time, demonstrated by: • MRI, or • Second clinical attack Insidious neurological progression Positive CSF, and suggestive of MS Dissemination in space, demonstrated by: •Nine or more T2 lesions in brain, or • Two or more lesions in spinal cord, or • Four to eight brain lesions plus one spinal cord lesion, or • Abnormal VEP associated with four to eight brain lesions, or • Abnormal VEP with fewer than four brain lesions plus one spinal cord lesion; and Dissemination in time, demonstrated by: • MRI, or • Continued progression for 1 year aCaution urged if all tests are negative. MS, multiple sclerosis; MRI, magnetic resonance imaging; CSF, cerebrospinal fluid (oligoclonal bands or raised immunoglobulin G index); VEP, visually evoked potential. (Adapted with permission from McDonald WI, Compton A, Edan G, et al. Recommended diagnostic criteria for mul- tiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001; 50:121–127, and from John Wiley and Sons.) Table 4 Guidelines for Magnetic Resonance Imaging Criteria or Brain Abnormality Three of the four following guidelines: 1. One gadolinium-enhancing lesion or nine T2-hyperintense lesions if there is no gadolinium-enhancing lesions. 2. At least one infratentorial lesion. 3. At least one juxtacortical lesion. 4. At least three periventricular lesions. Note: One spinal cord lesion can be substituted for one brain lesion. (Adapted with permission from McDonald WI, Compton A, Edan G, et al. Recommended diagnostic criteria for multi- ple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121–127.) numerous viral, bacterial, parasitic (e.g., schistosomiasis), or autoimmune collagen vascular diseases. Transverse myelitis may be the presenting symptom of MS, and is an integral part of NMO. 66 Diagnostic Criteria in Neurology Table 5 Magnetic Imaging Criteria for Dissemination of Lesions in Time 1. If a first scan occurs 3 months or more after the onset of the clinical event, the presence of a gadolinium- enhancing lesion is sufficient to demonstrate dissemination in time, provided that it is not at the site implicated in the original clinical event. If there is no enhancing lesion at this time, a follow-up scan is required. The timing of this scan is not crucial, but 3 months
Load more

Recommended publications
  • ANKRD55 and DHCR7 Are Novel Multiple Sclerosis Risk Loci
    Genes and Immunity (2012) 13, 253 --257 & 2012 Macmillan Publishers Limited All rights reserved 1466-4879/12 www.nature.com/gene ORIGINAL ARTICLE ANKRD55 and DHCR7 are novel multiple sclerosis risk loci I Alloza1,14, D Otaegui2,14, A Lopez de Lapuente1, A Antigu¨ edad3, J Varade´ 4,CNu´n˜ez4, R Arroyo5, E Urcelay4, O Fernandez6, L Leyva6, M Fedetz7, G Izquierdo8, M Lucas9, B Oliver-Martos6, A Alcina7, A Saiz10, Y Blanco10, M Comabella11, X Montalban11, J Olascoaga12, F Matesanz7,8,14 and K Vandenbroeck1,13,14 Multiple sclerosis (MS) shares some risk genes with other disorders hallmarked by an autoimmune pathogenesis, most notably IL2RA and CLEC16A. We analyzed 10 single-nucleotide polymorphisms (SNPs) in nine risk genes, which recently emerged from a series of non-MS genome-wide association studies (GWAS), in a Spanish cohort comprising 2895 MS patients and 2942 controls. We identified two SNPs associated with MS. The first SNP, rs6859219, located in ANKRD55 (Chr5), was recently discovered in a meta-analysis of GWAS on rheumatoid arthritis (RA), and emerged from this study with genome-wide significance (odds ratio (OR) ¼ 1.35; P ¼ 2.3 Â 10À9). The second SNP, rs12785878, is located near DHCR7 (Chr11), a genetic determinant of vitamin D insufficiency, and showed a size effect in MS similar to that recently observed in Type 1 diabetes (T1D; OR ¼ 1.10; P ¼ 0.009). ANKRD55 is a gene of unknown function, and is flanked proximally by the IL6ST-- IL31RA gene cluster. However, rs6859219 did not show correlation with a series of haplotype-tagging SNPs covering IL6ST--IL31RA, analyzed in a subset of our dataset (D0o 0.31; r2o 0.011).
    [Show full text]
  • Research Article Increasing Incidence in Relapsing-Remitting MS and High Rates Among Young Women in Finland: a Thirty-Year Follow-Up
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Crossref Hindawi Publishing Corporation Multiple Sclerosis International Volume 2014, Article ID 186950, 8 pages http://dx.doi.org/10.1155/2014/186950 Research Article Increasing Incidence in Relapsing-Remitting MS and High Rates among Young Women in Finland: A Thirty-Year Follow-Up Marja-Liisa Sumelahti,1,2 Markus H. A. Holmberg,1 Annukka Murtonen,1 Heini Huhtala,3 and Irina Elovaara1,2 1 School of Medicine, University of Tampere, Arvo 312, 33014 Tampere, Finland 2 Tampere University Hospital, P.O. Box 2000, 33521 Tampere, Finland 3 School of Health Sciences, University of Tampere, 33014 Tampere, Finland Correspondence should be addressed to Marja-Liisa Sumelahti; [email protected] Received 28 May 2014; Revised 23 September 2014; Accepted 9 October 2014; Published 9 November 2014 Academic Editor: Bianca Weinstock-Guttman Copyright © 2014 Marja-Liisa Sumelahti et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Object. Gender and disease course specific incidences were studied in high- and medium-risk regions of MS in Finland. Methods. Age- and gender-specific incidences with 95% CIs were calculated in 10-year periods from 1981 to 2010. Poser diagnostic criteria were used and compared with the McDonald criteria from 2001 to 2010. Association between age and diagnostic delay over time was assessed by using the Kruskal-Wallis test. Results. 1419 (89%) RRMS and 198 (11%) PPMS cases were included.
    [Show full text]
  • Efficacy and Safety of Mitoxantrone, As an Initial Therapy, in Multiple Sclerosis: Experience in an Indian Tertiary Care Setting
    Original Article Efficacy and safety of mitoxantrone, as an initial therapy, in multiple sclerosis: Experience in an Indian tertiary care setting B. S. Singhal, Sheth Geeta, Shilpa G. Hundalani, Suresh Menon Department of Neurology, Bombay Hospital Institute of Medical Sciences, India Abstract Background and Purpose: Mitoxantrone is an approved disease modifying agent for treatment of multiple sclerosis (MS). The aim of the study was to assess its efficacy and safety in Indian MS patients. Materials and Methods: A total of 23 patients with clinically definite MS (Poser criteria) were enrolled in an open label study. Of which, 21 satisfied the McDonald’s criteria for MS and two satisfied the diagnostic criteria of neuromyelitis optica (NMO). The numbers of relapses and expanded disability status scale (EDSS) score were used as primary and secondary outcome measures. The patients were monitored for the adverse effects. Results: In 17 (15 MS and two NMO) patients who completed one year of therapy, there was significant difference in the mean annual relapse rates [before 0.879 6 0.58; on mitoxantrone 0.091 6 0.17, (P 5 0.003)]. Of the 17 patients, ten (MS 9 and NMO 1) completed therapy for two years. Annual relapse rates [before (1.024 6 0.59), on therapy (0.155 6 0.21), (P 5 0.0054)] and EDSS score [before start of therapy 5.3, at the end of therapy 2.4, (P 5 0.001)] showed significant benefit in the ten patients who completed Address for correspondence: Dr. B. S. Singhal, two years therapy. This benefit persisted during the mean follow-up period of two and a Bombay Hospital Institute of half years after completion of therapy.
    [Show full text]
  • Remitting and Primary-Progressive Multiple Sclerosis: Effectiveness and Value
    Disease-Modifying Therapies for Relapsing- Remitting and Primary-Progressive Multiple Sclerosis: Effectiveness and Value Draft Evidence Report November 22, 2016 Prepared for ©Institute for Clinical and Economic Review, 2016 ICER Staff and Consultants University of Washington School of Pharmacy Modeling Group Jeffrey A. Tice, MD Marita Zimmermann, MPH, PhD Associate Professor of Medicine Research Fellow University of California, San Francisco Pharmaceutical Outcomes Research and Policy Program, Department of Pharmacy Rick Chapman, PhD, MS University of Washington Director of Health Economics Institute for Clinical and Economic Review Elizabeth Brouwer, MPH Graduate Student Varun Kumar, MBBS, MPH, MSc Pharmaceutical Outcomes Research and Policy Health Economist Program, Department of Pharmacy Institute for Clinical and Economic Review University of Washington Anne M. Loos, MA Josh J. Carlson, PhD, MPH Senior Research Associate Associate Professor Institute for Clinical and Economic Review Pharmaceutical Outcomes Research and Policy Program, Department of Pharmacy Shanshan Liu, MS, MPH University of Washington Research Associate Institute for Clinical and Economic Review Matt Seidner, BS Program Manager The role of the University of Washington (UW) School of Institute for Clinical and Economic Review Pharmacy Modeling Group is limited to the development of the cost-effectiveness model, and the resulting ICER reports do not necessarily represent the views of the UW. Daniel A. Ollendorf, PhD Chief Scientific Officer Institute for Clinical and Economic Review David Rind, MD Chief Medical Officer Institute for Clinical and Economic Review Steven D. Pearson, MD, MSc President Institute for Clinical and Economic Review DATE OF PUBLICATION: November 22, 2016 We would also like to thank Margaret Webb for her contributions to this report.
    [Show full text]
  • Perspectives on Multiple Sclerosis
    Review Article Perspectives on Multiple Sclerosis Robert I. Grossman and Joseph C. McGowan There is no question that MR imaging is presently the sensitivity of MR imaging, as the study does not best method for imaging multiple sclerosis (MS); yet, include state-of-the art spinal cord imaging. Com- even today, MS remains a clinical diagnosis, with the bined brain and spinal cord imaging can increase the relationship between clinical course and MR findings sensitivity to almost 100% (19). still problematic (1, 2). MR imaging appears to be the MS is a disease characterized by a variety of clinical most important test performed in the diagnostic courses. Terminology regarding clinical classification workup of MS; it has much greater impact than CSF can be confusing and even contradictory (20). Relaps- analysis and evoked potential testing (3). It is far ing-remitting MS is the most common course of the more sensitive than a clinical examination in revealing disease, initially occurring in up to 85% of patients. disease activity, as MR abnormalities are found five Exacerbations are, at the beginning, followed by re- to 10 times more often than clinical abnormalities missions, but over a period of years, additional exac- detected by standard neurologic examination (4–8). erbations result in incomplete recovery. Within 10 In this review, we briefly summarize the clinical and years, 50% (and within 25 years, 90%) of these pa- pathologic hallmarks of MS and discuss the MR find- tients enter a progressive phase, termed secondary- ings in this disease, examining the strengths and progressive (or relapsing-progressive) MS, in which weakness of conventional imaging protocols in rela- deficits are progressive without much remission in the tion to their clinical utility.
    [Show full text]
  • Solution for Injection, Pre-Filled Syringe>
    Common Technical Document Glatiramer acetate 20 mg/mL Solution for injection in pre-filled syringe 1 NAME OF THE MEDICINAL PRODUCT [Product name]<20 mg/mL><Solution for Injection, Pre-filled Syringe> 2 QUALITATIVE AND QUANTITATIVE COMPOSITION 1 ml of solution for injection contains 20 mg glatiramer acetate*, equivalent to 18 mg of glatiramer base per pre-filled syringe. * The average molecular weight of glatiramer acetate is in the range of 5,000-9,000 daltons. Due to its compositional complexity, no specified polypeptide can be fully characterized in terms of amino acid sequence, although the final glatiramer acetate composition is not entirely random. For the full list of excipients, see section 6.1. 3 PHARMACEUTICAL FORM Solution for Injection, Pre-filled Syringe [Solution for Injection] Clear, colourless to slightly yellow/brownish solution free from visible particles. The solution for injection has a pH of 5.5 - 7.0 and an osmolarity of about 265 mOsmol/L. 4 CLINICAL PARTICULARS 4.1 Therapeutic indications Glatiramer acetate is indicated for the treatment of relapsing forms of multiple sclerosis (MS) (see Section 5.1 for important information on the population for which efficacy has been established). Glatiramer acetate is not indicated in primary or secondary progressive MS. 4.2 Posology and method of administration Posology The recommended dosage in adults is 20 mg of glatiramer acetate (one pre-filled syringe), administered as a subcutaneous injection once daily. At the present time, it is not known for how long the patient should be treated. A decision concerning long term treatment should be made on an individual basis by the treating physician.
    [Show full text]
  • Primary Progressive Multiple Sclerosis
    Brain (1997), 120, 1085–1096 REVIEW ARTICLE Primary progressive multiple sclerosis A. J. Thompson,1 C. H. Polman,2 D. H. Miller,1 W. I. McDonald,1 B. Brochet,3 M. Filippi,4 X. Montalban5 and J. De Sa´6 1Department of Clinical Neurology, Institute of Neurology, Correspondence to: Alan J. Thompson, The National London, UK, 2Department of Neurology, Vrije Universiteit Hospital, Queen Square, London WC1N 3BG, UK Hospital, Amsterdam, The Netherlands, 3Departement de Neurologie, Hoˆpital Pellegrin, Centre Hospitalier Universitaire, Bordeaux, France, 4Department of Neurology, Scientific Institute, Ospedale San Raffaele, University of Milan, Italy, 5Department of Neurology, Multiple Sclerosis Unit, Hospital General Universitari Vall d’Hebron, Barcelona, Spain and 6Servic¸o de Neurologia, Hospital de Santa Maria, Lisboa, Portugal Summary Patients with multiple sclerosis who develop progressive disability and the role of MRI in monitoring disease activity disability from onset without relapses or remissions pose in this clinical subgroup require elucidation, particularly in difficulties in diagnosis, monitoring of disease activity and view of the lack of change on conventional imaging in the treatment. There is a need to define the diagnostic criteria presence of continuing clinical deterioration. The prognosis for this group more precisely and, in particular, to describe is poor and there are currently no treatment trials for this a comprehensive battery of investigations to exclude other form of the disease. conditions. The mechanisms underlying the development of Keywords: multiple sclerosis; primary progressive; diagnosis Abbreviation: HLA 5 human lymphocyte antigen Introduction Multiple sclerosis is classically characterized by a relapsing/ disease both immunologically and pathologically. Indeed the remitting course due to lesions in many parts of the CNS, question arises as to whether this may be a separate disease with the majority of patients developing progressive disability entity or part of the broad spectrum of multiple sclerosis.
    [Show full text]
  • Evidence Tables
    Drug Class Review on Disease-modifying drugs for Multiple Sclerosis Final Report Evidence Tables July 2007 The Agency for Healthcare Research and Quality has not yet seen or approved this report A scan of the medical literature is done periodically The purpose of this report is to make available information regarding the comparative effectiveness and safety profiles of different drugs within pharmaceutical classes. Reports are not usage guidelines, nor should they be read as an endorsement of, or recommendation for, any particular drug, use or approach. Oregon Health & Science University does not recommend or endorse any guideline or recommendation developed by users of these reports. Marian McDonagh, PharmD Tracy Dana, MLS Benjamin KS Chan, MS Sujata Thakurta, MPA-HA Andrew Gibler Oregon Evidence-based Practice Center Oregon Health & Science University Mark Helfand, MD, MPH, Director Copyright © 2007 by Oregon Health & Science University Portland, Oregon 97239. All rights reserved. Note: A scan of the medical literature relating to the topic is done periodically (see http://www.ohsu.edu/ohsuedu/research/policycenter/DERP/about/methods.cfm for scanning process description). Upon review of the last scan, the Drug Effectiveness Review Project governance group elected not to proceed with another full update of this report. Some portions of the report may not be up to date. Final Report Evidence Tables Drug Effectiveness Review Project Table of Contents Evidence Table 1. Systematic reviews of disease-modifying drugs................................... 3 Evidence Table 2. Head-to-head trials of β interferons .................................................... 18 Evidence Table 3. Effectiveness and adverse events in head-to-head trials of β interferons ........................................................................................................................
    [Show full text]
  • A Randomized, Double-Blind, Placebo-Controlled Trial
    CLINICAL TRIALS SECTION EDITOR: IRA SHOULSON, MD Intravenous Immunoglobulin Treatment Following the First Demyelinating Event Suggestive of Multiple Sclerosis A Randomized, Double-blind, Placebo-Controlled Trial Anat Achiron, MD, PhD; Irena Kishner, MD; Ida Sarova-Pinhas, MD; Havi Raz, BA; Meir Faibel, MD; Yael Stern, MD; Mor Lavie, MA; M. Gurevich, PhD; Mark Dolev, MD; David Magalashvili, MD; Yoram Barak, MD Background: Intravenous immunoglobulin (IVIg) has cally definite multiple sclerosis was significantly lower been reported to reduce disease activity in patients with in the IVIg treatment group compared with the placebo relapsing-remitting multiple sclerosis. We assessed the group (rate ratio, 0.36 [95% confidence interval, 0.15- effect of IVIg treatment in patients after the first neuro- 0.88]; P=.03). Patients in the IVIg treatment group had logical event suggestive of demyelinative disease and a significant reduction in the volume and number of T2- evaluated the occurrence of a second attack and dissemi- weighted lesions and in the volume of gadolinium- nation in time demonstrated by brain magnetic reso- enhancing lesions as compared with the placebo group nance imaging within the first year from onset. (P=.01, P=.01, and P=.03, respectively). Treatment was well tolerated, compliance was high, and incidence of ad- Methods: We conducted a randomized, placebo- verse effects did not differ significantly between groups. controlled, double-blind study in 91 eligible patients en- rolled within the first 6 weeks of neurological symp- Conclusions: Intravenous immunoglobulin treatment toms. Patients were randomly assigned to receive IVIg for the first year from onset of the first neurological event treatment (2-g/kg loading dose) or placebo, with boost- suggestive of demyelinative disease significantly lowers ers (0.4 g/kg) given once every 6 weeks for 1 year.
    [Show full text]
  • MAKING the DIAGNOSIS of MULTIPLE SCLEROSIS J Palace *Ii3
    J Neurol Neurosurg Psychiatry 2001;71(suppl II):ii3–ii8 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.71.suppl_2.ii3 on 1 December 2001. Downloaded from MAKING THE DIAGNOSIS OF MULTIPLE SCLEROSIS J Palace *ii3 uspected cases of multiple sclerosis (MS) are usually young adults attending the neurology outpatient clinic. The onset of symptoms is rare before puberty or after the age of 60 years; Showever, being a relatively common neurological disease (1:800 in UK), both situations may be familiar to practising neurologists. MS will usually present with either a history of acute relapse(s) or with progressive neurological impairment c RELAPSING REMITTING DISEASE The most common presentation of MS is following a relapse. The peak age of the first episode is in the third decade; however, patients often present to the neurologist later with their second or third relapse or less commonly in the secondary progressive phase of illness. This form of MS is twice as common in women as in men. The most common scenario is a patient of 20–40 years old referred fairly urgently to the outpatient clinic with a subacute onset of neurological disturbance, most commonly sensory symptoms, often resolving by the time they are seen. Because sensory symptoms may occur in the absence of clinical signs the neurological examination may be normal or near normal even when seen during relapse. The distribution of sensory loss is extremely variable but most commonly involves one or more limbs and usually represents a partial transverse myelitis with an evolving copyright. sensory level (which may be patchy or unilateral).
    [Show full text]
  • Updated Criteria for Diagnosing Multiple Sclerosis
    r e v i e w a r t i c l e Updated criteria for diagnosing Multiple Sclerosis or in the case of progressive MS, a slow or step- Key take home messages wise progression of disability over a period of • The diagnostic criteria for MS have at least six months been recently updated • and objective clinical evidence of lesions in • These criteria should only be applied two or more distinct sites in the white matter to populations in whom MS is of the central nervous system common and patients who present • with no more satisfactory explanation. Peter Brex with typical symptoms for which no The Schumacher criteria were purely clinical, MB BS, MD, FRCP is a Consultant Neurologist at King’s College Hospital better explanation can be found although investigations were encouraged (blood, NHS Foundation Trust. He trained in • All patients with suspected MS should urine, chest X-ray, CSF analysis) to exclude London and was appointed to his current have an MRI brain scan; spinal cord alternative conditions. role in 2005. He specialises in multiple imaging is not mandatory Over the next few years modifications to the sclerosis, with a particular interest in early 2,3 prognostic markers and the management • Unmatched oligoclonal bands in the Schumacher criteria were published but in 1983 of MS during pregnancy. CSF can be used as a substitute for the Schumacher criteria were replaced by criteria demonstrating dissemination in time, developed by a committee chaired by Charles allowing an earlier diagnosis than was Poser (1923 – 2010).4 The Poser criteria incorpor- previously possible ated laboratory and clinical tests developed in the previous decade to support the diagnosis with ‘paraclinical evidence’ of lesions.
    [Show full text]
  • And Multiple Sclerosis Particular Problem, I Wish to Make the Follow
    524 Maters arising prolong the lives of those suffering, thereby Most of these problems are not taken into 6 Poser C, Paty D, Scheinberg L, et al. New J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.6.524-b on 1 June 1992. Downloaded from providing the necessity for self-action by the consideration in Sibley's article. diagnostic criteria for multiple sclerosis. Ann patient. It is germane to point out that the "scien- Neurol 1983;13:227-31. Perhaps when we advise patients and tific" evidence of epidemiology and biostatis- families who face difficult decisions, we tics overlooks the fact that such studies can at Sibley et al reply: would be better to heed the available evi- best only be estimates within a cohort and dence about who will do well and who will do cannot be generalised; epidemiological stud- We age- and sex-matched MS patients with badly rather than withholding recommenda- ies are most useful in providing aetiological healthy controls solely to establish the degree tions because unduly cautious statisticians clues but cannot be used to deny possible of accident-proneness of MS patients. It choose to neglect the downside effects of causal relationships.2 As was pointed out by probably needs to be clearly restated that the their theoretical arguments. Neurologists are Schoenberg, "Statistical significance (or lack patients were used as their own controls in an especially favourable position to under- thereof) does not equal biological when they were not "at risk". stand the misery that accompanies chronic, significance". Physical trauma had no positive influence overwhelming brain damage.
    [Show full text]