EDMUS, Europeandatabase for multiple sclerosis

Jrournal of Neurology, Neurosurgery, and Psychiatry 1992;55:671-676

EDMUS, a European database for multiplesclerosis

C Confavreux, D A S Compston, 0 R Hommes,W I McDonald, A J Thompson

AbstractEDMUS is a minimal descriptive recorddeveloped for research purposes to docu-ment clinical and laboratory data inpatients with multiple sclerosis (MS). Ithas been designed by a committee of theEuropean Concerted Action for MS, orga-nised under the auspices of the Commis-sion of the European Communities. Thesoftware is user-friendly and fast, with aminimal set of obligatory data. Priorityhas been given to analytical data and thesystem is capable of automatically gen-erating data, such as diagnosis classi-fication, using appropriate algorithms.This procedure saves time, ensures auniform approach to individual cases andallows automatic updating of theclassification whenever additional infor-mation becomes available. It is alsocompatible with future developments andrequirements since new algorithms can beentered in the programme when neces-sary. This system is flexible and may beadapted to the users needs. It is run onApple and IBM-PC personal microcom-puters. Great care has been taken topreserve confidentiality of the data. It isanticipated that this "common" languagewill enable the collection of appropriatecases for specific purposes, including pop-ulation-based studies of MS and will beparticularly useful in projects where thecollaboration of several centres is neededto recruit a critical number of patients.

(7 Neurol Neurosurg Psychiatry 1992;55:671-676)

There is a need to standardise morbidityrecords in patients with multiple sclerosis(MS). The use of a common language is anessential prerequisite in the establishment of adatabase which will provide descriptive detailsof the disease and allow rapid identification ofappropriate patients for cross-sectional studiesor therapeutic trials. The establishment of adatabase is one of the main themes of theEuropean Concerted Action for BasicResearch and Treatment in Multiple Sclerosisorganised under the auspices of the Commis-sion of the European Communities (CEC).The Lyon MS database system, which has

been developing since 1976, served as a basis.'A workshop, Towards a European DatabaseSystem for Multiple Sclerosis, was held in Lyon,France, 21-22 September 1990 to completethe design of standardised forms. An EDMUSSteering Committee, composed of members

from the various countries of the EuropeanEconomic Community (EEC), was alsonominated. In 1991 the software was refinedand tested by the EDMUS Steering Commit-tee members. We present the conceptual andtechnical design of the EDMUS system, withspecial emphasis on the Basic Version, whichcould be considered a minimal descriptiverecord for any patient with MS. This version isnow available and it is anticipated that it will beused in European collaborative studies inMS.

The EDMUS system: basic conceptsThe aim of this database system is not toreplace the clinical notes, as is the case for theNorth American COSTAR system, but toprovide a minimal descriptive record forresearch purposes.

It is user-friendly and fast to facilitateregular and long term use, even in departmentswith limited facilities. In this respect, a mini-mal set of obligatory data has been defined.This does not exceed 15 items per patient andtakes only a few minutes to complete. Addi-tional complementary data may be entered fora more thorough description.The description of the clinical manifes-

tations ofMS often relies on retrospective dataprovided by the patient, his relatives or generalpractitioner. This is usually applicable to theonset of the disease but may also be relevant tothe earlier years of the disease. Consequently,in this system, priority has been given tosymptoms rather than signs, to include bothretrospective and prospective data.One of the main advantages of computerised

data processing is the ability to incorporatealgorithms in the programme which allowautomatic generation of data. Thus priority hasbeen given to analytical data from whichinterpretations such as classification of thecourse, severity and diagnostic confidence ofthe disease, are computer generated. Thisprocedure has many advantages. It is timesaving for the clinician and gives an overviewwhich may be used as a disease summary forany re-examination of the patient. It ensures astandardised way of classifying cases and pro-vides an up-to-date classification of the case.Lastly, it can be made compatible with anyfuture classification, as the data can be ana-lysed using new algorithms.

Flexibility is another essential characteristicof the EDMUS system and both basic andextended versions are available according tothe user's needs.The programme has been developed on the

Clinique deNeui'ologie, HopitalNeurologique, Lyon,FranceC ConfavreuxDepartment ofNeurology,Addenbrooke'sHospital, Cambridge,UKD A S CompstonDepartment ofNeurology, RadboudUniversity Hospital,Nijmegen, Netherlands0 R HommesInstitute of Neurology,The National Hospital,London, UKW I McDonaldA J ThompsonCorrespondence to:Professor Confavreux,Clinique de Neurologie,H6pital Neurologique,59 Boulevard Pinel,69003 Lyon, FranceReceived 19 March 1992and in revised form5 May 1992.Accepted 12 May 1992

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Confavreux, Compston, Hommes, McDonald, Thompson

standard database software OMNIS V (BlythSoftware). It runs on both Apple and IBM-PCpersonal microcomputers and does not requiresophisticated hardware. Apple computers of IILC, II Si, II Ci, PowerBook 140 and Power-Book 170 type are appropriate with a corememory size of 4 Megabytes, a hard disk of 40Megabytes and either a monochrome or a highdefinition colour screen. IBM-PC and compat-ible computers must be based on INTEL80386 or INTEL 80386 SX, with a > 20 Mhzclock speed, a similar core memory and harddisk as for Apple models. Apart from thestandard operating system (MS-DOS level3.xx), MS-WINDOWS 3 0x must be used.Data which are entered include the names of

the patients. This is necessary for any depart-ment using the system for its own patients aswell as for multicentre studies since severalrecords may have been registered from thesame patient. Consequently, several safetymeasures have been taken to preserveconfidentiality of the data, in accordance withthe recommendations of the French Commis-sion Nationale Informatique et Libertes(CNIL). Access to the EDMUS software andthe files requires specific passwords. These maybe updated at the convenience of the localdatabase manager, that is, the person who isresponsible for local use of the system. Thereare several levels for these passwords. Thedatabase manager level provides access to the

various possible operations on the files and tonamed data. By contrast, the lowest levelallows access to anonymous data only, anddoes not allow any modification to be done.An automatic procedure is available for sup-pressing the names of patients in the recordswhen files are to be duplicated. Any centremust store duplicated named files for safe-keeping. It is recommended that such copiesmight be limited in number to what is strictlynecessary for this purpose and stored in anappropriate protected place. Physical protec-tion is also required for the computer in whichthe named data are stored. Finally, any dataexchange between centres or researchers mustbe made through anonymous files.

The EDMUS formGeneral structureThe form is made up of 2 sections. Section I isdevoted to: 1) the patient's identification; 2)the MS background, with genetic data and thepatient's medical history exclusive of MS; 3)the key-dates of the MS course in terms ofrelapses and onset of progression (fig 1); 4) thekey-dates of the non-reversible disability; 5)general comments, with mention of availablepatient's biological samples, if any, and key-words which may be specifically coined for thepatient and retrieved through the selectionprocedure of the programme (see below). Ithas a fixed length, that is, with newly available

Date of event onset Type UK

I IL °LE - UE - SS - SP - SX

EL U UJ

SymptomsFM - FS - OM - YE - BB

O: O: EO El EON PS- MT OT

E U UO

Figure 1 The "MS Course Key-Dates" window.This is devoted to the successive description of relapses and onset of progression, that is, the pivotal events which producemodifications in the symptoms (not signs only) of the disease. It is a part of Section I of the EDMUS form.Type:During the "no-sequelae period" of the relapsing-remitting phase, a distinction is made between: 1) the relapse at theonset of this period (RO); 2) subsequent relapses with a well identified date of onset and a complete remission (RR);3) relapses with complete remission but uncertain chronology (RU). The latter option may be necessary for retrospectivedata.

During the "sequelae period" of the relapsing-remitting phase, a distinction is made between: 1) the relapse at theonset of this period (SO); 2) subsequent relapses with a well identified date of onset and followed by a non-worsening(SN), or a worsening (SW), or a doubtful worsening (S?) ofpre-existing sequelae; 3) relapses with uncertainchronology (SU).

During the chronic progressive phase, a distinction is made between: 1) onset without any initial relapse (POP) andonset with an initial relapse (POR), which are mutually exclusive; 2) subsequent relapses with a well identifiedchronology (PR); 3) subsequent relapses with uncertain chronology (PU). The possible worsening effect of the relapseduring the chronic-progressive phase is not taken into account, as it is often too difficult to assess.

Symptoms:They are distributed in 14 categories, each of them with an appropriate abbreviation: lower extremity dysfunction (LE),upper extremity dysfunction (UE), sensory symptoms (SS), sphincter disturbance (SP), sexual disturbance (SX), facialmotor (FM), facial sensory (FS), oculomotor (OM), vestibular andlor cochlear (VE), bulbar (BB), optic neuritis(ON), psychiatric symptoms (PS), mental deterioration (MT) and other symptoms not covered by the precedingcategories (OT). Whenever symptoms related to the event to be described are unknown, the code "UK" is ticked.

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EDMUS, a European database for multiple sclerosis

information during follow up, the originaldocument is completed or, whenever neces-sary, modified but no extra sheets are used.The minimal set of Obligatory Data is identi-fied by heavy framing. Finally, a "Wait andSee" item has been prepared. It is usedwhenever the diagnosis of MS is doubtfulirrespective of assignment in the Poser classi-fication.2

Section II describes the Current Status (CS)of the patient, that is, the state of the patient atany given date in the disease, and includes: 1)the current disability scores; 2) current immu-nological treatments, if any; 3) current symp-toms and signs; 4) the results of currentinvestigations including electrophysiology,CSF analysis, MRI and other imaging tech-niques. One CS form should be completed forevery date under which the state of the patientis to be described. The CS can be documentedretrospectively, whenever it is considered ofinterest to record clinical or paraclinical datarelating to a previous date. It may also bedocumented prospectively, whenever thepatient is seen for follow up. Consequently,section II is made up of additive CS forms,corresponding to the number of assessmentscollected during the follow up of the patient. Inthis way it provides a longitudinal descriptionof the disease.

The MS course key-dates formThis form is devoted to a successive descrip-tion of the relapses and the onset of progres-sion, that is, the pivotal events which producemodifications in the symptoms (not signs only)of the disease. As shown in the diagram (fig 1),two distinct phases are considered. During therelapsing-remitting phase, the relapses are sepa-rated by periods of clinical inactivity. A relapseis defined3 as the occurrence of new symp-toms, the re-appearance of former ones, or theworsening of current symptoms of at least 24hours duration. It has a subacute onset; theinitial phase is followed by a plateau and,usually, by improvement which may be com-plete. Transient worsening of symptoms asso-ciated with a rise in temperature is notconsidered as a relapse. Relapses must beseparated by at least one month, and symp-toms occurring within a month are consideredas part of the same episode. Within therelapsing-remitting phase, a no sequelae periodand a sequelae period may be considered.During the latter, the sequelae must produce atleast a minimum ambulation-related disabilityor a significant not ambulation-related dis-ability, such as, grade 3 on the EIS-DSSadapted scale (see below). The chronic-progressive phase is defined3 as continuousworsening of symptoms and signs of grade 3 ormore on the EIS-DSS adapted scale (seebelow) for a minimum of six months, whethersupervened by relapses or not. According tothis scheme, there is a logical sequence in thedescription of the course. With time, the nosequelae period may be followed in the samepatient by sequelae within the relapsing-remit-ting phase and then by chronic-progressivedisease.

Specific definitions and abbreviations havebeen adopted for the characterisation of therelapses and the onset of progression. They aredisplayed in fig 1. For each event, the date ofonset and concurrent symptoms should bespecified. Fourteen categories of symptomshave been considered. They are displayed withthe corresponding abbreviations in fig 1. In thecase of retrospective data, symptoms observedduring the relapse or the onset of progressionmay be unknown. It is then possible to tick theappropriate code. In this part of the EDMUSform, the symptoms are entered in a conciseway. If a more thorough description is desiredat the clinical (or paraclinical) level, a CurrentStatus form may also be completed relating tothe same assessment.

The disability scalesThe Kurtzke DSS scale4 has served as thestarting model since it is widely used in clinicalpractice and research. However, it has beenadapted to be unambiguous, notably by notmixing functional systems and by being quickto assess, even retrospectively. It is mainlybased on symptoms and walking abilities. ThisEDMUS impairment scale (EIS-DSS adapted;Appendix 1) provides good inter-rater agree-ment (unpublished observations). The otherdisability scales which have been selected areKurtzke's EDSS and functional systemsscales5 6 and the ambulation index.7

The automated functions of the EDMUSsoftwareCross-checking of the dataThe windows which are displayed on thescreen are directly derived from the EDMUSchart and, as the data is being entered, the useris automatically guided by the programme. Forinstance, the unmarried name and the preg-nancy boxes will appear on the screen only if afemale patient is being registered.

Consistency is maintained within any givenwindow. For example, in the Course Key-Dates window, whenever a relapse or the onsetof progression is described, its type and date ofonset are compared with any preceding events.In the case of inconsistency, a warning messageappears and an appropriate correction isrequested. A similar control is ensured for theDisability Key-Dates window where dates forthe various disability levels must be entered ina logical chronological order.

Overall consistency is also checked automat-ically. With the exception of the birth date, nodate can be entered if it precedes the date ofthe first relapse in cases with a remittent onsetor the date of the onset of progression in caseswith a progressive onset which has beenregistered in the Course Key-Dates window.Similarly, in the Current Status window, it isimpossible to enter an EIS-DSS adapted dis-ability level which would be lower than thenon-reversible disability level already recordedin the Disability Key-Dates window.

Generation of dataAt the same time as analytical data are regis-tered, further information is automatically

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generated and updated as additional informa-tion is entered. For instance, the date of thelast examination is deduced from the latestdate among the Course Key-Dates, the Disa-bility Key-Dates and the Current Status win-dows. This allows an appropriate calculation ofthe duration of disease and length of follow upfor any case. Similarly, there are algorithms forthe classification of the disease course, severityand confidence of diagnosis.The classification of the disease course is

made through the Course Key-Dates windowaccording to established criteria.8The remittentforms show events (relapses) in the exacerbat-ing-remitting phase and none (onset of pro-gression and subsequent relapses, if any) in thechronic-progressive phase, while the remittent-progressive forms show events in both phases,and the progressive forms displays the chronic-progressive phase only. This latter category issubdivided into with and without superimposedrelapses.The classification of the severity of disease is

generated from the Disability Key-Dates win-dow according to a previous grade proposal.8When severe non-reversible disability hasdeveloped (grade . 7 on the EIS-DSS adaptedscale), the interval to severe disability is calcu-lated from the date of onset of the disease, thatis, the date of the first event in the Course Key-Dates window. The following definitions areapplied:Hyperacute form: interval to severe non-revers-ible disability < 5 years.Acute form: interval to severe non-reversibledisability = 5-10 years.Subacute form: interval to severe non-reversibledisability = 10-15 years.Intermediateform: interval to severe non-revers-ible disability 2 15 years.Benign form: residual disability less than grade3 with a disease duration greater than 20years.

El Pathological verification

rCl Time Dissemination

Not assessable form: case that fails to fulfil thepreceding categories.The generation of the diagnosis classifica-

tion is more complex. It is displayed on theDiagnosis Synopsis window (fig 2) which hasbeen designed to display items relevant to thegeneration of the diagnosis using the Posercriteria.2 These items are picked up from thewhole case record. In this way, it is very easy toknow if a given examination has already beendone, what has been its diagnostic value, andthe rationale for doing or repeating it. Theseitems are grouped according to the 3 criteria ofthe Poser classification: 1) dissemination oflesions in time; 2) dissemination of lesions inspace, evidence of which may be clinical orparaclinical (evoked potentials, CT scan orMRI examinations); 3) quantitative and/orqualitative abnormalities of immunoglobulinsin the cerebrospinal fluid.The criteria for dissemination in time and the

date of completion are generated directly fromthe second event, if any, which has beenentered in the Course Key-Dates window. Fordissemination in space, several steps are fol-lowed. First, relevant data are extracted fromsections I and II of the record. The involve-ment of the neuraxis is demonstrated clinicallywhenever neuraxis symptoms and/or signs havebeen entered in the Course Key-Dates, Disa-bility Key-Dates or Current Status windows. Itis also demonstrated electrophysiologicallywhen brainstem auditory, somatosensory ormotor evoked potentials provide positiveresults. The earliest date of positivity amongthese items serves as the date of completion forthe neuraxis item. A similar procedure isfollowed for the optic nerve. The imaging itemis generated as positive as soon as the MRIexamination (or the CT scan for earlierpatients) has been entered in a Current Statuswindow as strongly suggestive ofMS accordingto Paty's criteria.9 In a second step, the criteria

Decease date | i

Onset date

Rapce dissemination Onset date

.Neuraxis . Onset datej~ S....Optic Nerve.......onsetdste jjjj.........O

Clinical 0 - 0 +

Clinical: 0 - 0 YEP: OND 0- 0SEP:OND 0- 0~~~~~~~~YE: M 0-

SEP 0 ND 0 '

BAEP: 0 ND 0 o + maging . Onset date Ii.

Mt1EP: 0 ND 0 0-O+CT scan: 0 ND 0 - 0 +

MRI: O ND 0 - 0 +

FCerebro-Spinal Fluid -0 ND-0 ----0 +

IgG index: OND 0- 0+ Oligoclonalbands: OND 0- 0+I I~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

[:E -Wait and See-Figure 2 The "Diagnosis Synopsis" window. This is a window of the EDMUS programme but not a part of theEDMUS form. It displays data which are automatically extracted from the whole case record, such as, Sections I and IIof the EDMUS form. From these data, the diagnosis is automatically classified in accordance with the Poser's criteria(see text). SEP = somato-sensory evoked potentials; BAEP = brainstem auditory evoked potentials; MEP = motorevoked potentials; VEP = visual evoked potentials; MRI = magnetic resonance imaging; ND = not done.

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EDMUS, a European database for multiple sclerosis

for dissemination in space and date at whichthis was established are completed wheneverany of the following combinations from theneuraxis, optic nerve and imaging items isobtained: neuraxis + optic nerve + imaging;neuraxis + optic nerve; neuraxis + imaging;optic nerve + imaging. The CSF criteria are

generated as positive as soon as the presence ofoligoclonal banding has been entered and/orthe IgG index'0 is higher than 070 in a

Current Status window. The earliest date ofCSF positivity serves as the date of completionof the CSF item.

Finally, the time, space and CSF criteria are

automatically combined by the programme togenerate and classify the diagnosis in accord-ance with Poser's guidelines.2Clinically definite case: "time + space" what-ever the "CSF" result.Laboratory-supported definite case: "time +CSF" or "space + CSF".Clinically probable case: "time" only or "space"only.Laboratory-supported case: "CSF" only.Suspected case: case that fails to fulfil thepreceding categories.

Case selection and export facilitiesA selection of the records from the whole fileaccording to any criterion, isolated or incombination, is possible through the Selectionwindow (fig 3). The list of the selected recordsmay be printed. Data from these records may

be transferred to standard softwares, forinstance EXCEL (Microsoft Corporation) or

SPSS (SPSS Incorporation) for statisticalanalysis.

Present and future developmentsDiffusion of the EDMUS systemThe first version of the EDMUS system is nowavailable and may be ordered through theClinique de Neurologie, H6pital Neurolo-gique, Lyon, France (cost = 600 ECUs).Colleagues are advised to contact the EDMUSSteering Committee member in their own

country for further information. The LyonClinique de Neurologie will act as EDMUSmanaging office and will provide assistance inseveral areas: knowledge of the system, localimplementation of the system, conversion (ifpossible) of previously collected data to theEDMUS system, and long call assistance viaFax. It will also act as an EDMUS coordinat-ing centre. Such a structure is necessary forseveral reasons: 1) to distribute, maintain andcontinue to develop the system; 2) to promoteand coordinate collaborative studies; 3) toestablish a connexion with tissue bankingfacilities. In this respect, it may be noted that,for any record on the EDMUS system, it ispossible to register the availability of biologicalmaterial, either DNA, cells, CSF, serum or

brain tissue. Any local EDMUS user will havethe possibility offorwarding anonymous files to

2;ender _ Delay of first examination (yrs)i Botlh from:[ (incl.) to: F clUiIOM Disease duration (yrs)O F from:Ir iIO(incl.)to: |I(excl.)

FBackground L

Twins: N.C.Caucasoids : N.C.

Familial MS : ®) N.C.Particular Onset : N.C.

-nset SymptomsLong Tracts: N.C. 0 Incl udeBrainstem: N.C. 0 Include

Optic Ne u ritis ®:i N.C. 0 Incl ude

0 Yes0 Yes0 Yes0 Yes

0 Exclude0 Exclude0 Exclude

O NoO NoO NoO No

OL /$/t/edEl IwsoledLI/Woleed

.L

I

Disability delay ( yrs)Moderate from: ] (i ncl.) to li i (exc .)

Severe from: 111 (i ncl.) to: | (excl.)

-Keywords contCnt (or) I-1- ~~ ~ ~ ~~11

IIL-Dagnoss-

Time Dissemi nation: i N.C. 0 I ncl ude 0 ExcludeSpace Dissemi nation: ® N.C. 0 I ncl ude 0 Excl ude

Ti me and/or Space Diss.:® N.C. 0 I ncl ude 0 Excl ude........................................................................................................................ .................................................

CSF: ®) N.C. 0 + 0 -........................................................................................................................ .............

Classification: Z N.C.Cli nically Definite : I ncl ude 0 Exclude

Lab. Supported Definite : ® Include 0 ExcludeClinically Probable : I ncl ude 0 Excl ude

Lab. Supported Probable () I ncl ude 0 ExcludeSuspected : ( I ncl ude 0 Exclude

........................................................................................................................ ............................

Wait and See :* N.C. 0 Incl ude 0 Exclude

Figure 3 The "Selection" window is a window of the EDMUS programme but not a part of the EDMUSform. It displays the pre-established criteriawhich may be used, in isolation or in combination, for the selection of records from the whole file.M = male; F = female; yrs = years; incl = inclusive; excl = exclusive; NC = not concerned. Delays and durations are calculated by comparing

the date of onset of the disease and the date of onset of the relevant item. For instance, in "delay offirst examination" calculations are made from thedate of onset of the disease to the date offirst examination of the patient in the department, data which are present in Section I of the EDMUS form. Itis then possible to select records for example, with a minimum (inclusive) of 6 months and a maximum of 10 years (exclusive) for the "delay offirstexamination" by entering 0O5 and 10 in the appropriate boxes. "Key-words": by entering a given key-word in the appropriate box, it is possible toselect records for which such a key-word has been entered in the "Comments" part of Section I of the EDMUS form.

-Non Reversible DisabilityCurrent Level

i N.C. 0 None 0 Moderate

Age (yrs)at onset from: | (ncl.) to I (excl.)

at last info. from: (ncl.) to: | (excl.)

MS Courc - N.C.Remittent: ® Include 0 Exclude

Remittent Progressive: (®) Include 0 ExcludePProgressive: ( I ncl ude O Excl ude

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the coordinating centre via a disquette or

modem. The coordinating centre will providethe users with the general characteristics of thefiles which have been collected and help in theretrieval of records when requested. Access to

and disposal of any given record will beallowed only with the formal agreement of theEDMUS user in charge of this record.Confidentiality of the data will also, be pre-

served at this level. In these various tasks, thecoordinating centre will continue to be assistedand permanently controlled by the EDMUSSteering Committee members.

Development of future versions of the EDMUSsystemThe EDMUS system will evolve in agreementwith the users and the steering committeemembers recommendations. For example, newalgorithms could be incorporated into thesystem, if considered relevant. Moreover,besides the present Basic Version of theEDMUS system, it is necessary to prepareseveral extended versions aimed at specificstudies. Thus an MRI Extended Version isanticipated for MRI monitoring treatment ofMS, in collaboration with the MRI Committeeof the European Concerted Action for Multi-ple Sclerosis"; a Register Version, displayingonly the minimal set of obligatory data of theBasic Version, is planned for epidemiologicalsurveys, and a Genetic Version for GAMES(Genetic Analysis of Multiple Sclerosis in theEuropean Society).

Collaborative activitiesWhile the EDMUS system may be used withinindividual departments, the aim of this projectis to share data from different centres andpromote collaboration between them. Themembers of the EDMUS Steering Committeehave already agreed to register systematically inthe EDMUS system all newly diagnosed cases

of MS in their departments, to test thereliability of the system. A collaborative pro-

spective study of the relationship between MSand pregnancy is in preparation. There are

many other areas to which the system may beadapted, including genetic analysis of twinsand multiplex families, MS in immigrants toEurope, longitudinal MRI studies and thera-peutic trials. Such collaborative Europeanstudies within the European Concerted Actionfor MS will be greatly enhanced through theuse of this commonly agreed language. It ishoped that the National MS Societies, theEuropean MS Platform and the EuropeanEconomic Community will contribute to set-ting up and maintaining a central facility whichcould be pivotal in all future research in MSthroughout Europe.

The continuous contribution ofMr Albert Biron, DepartementInformatique des Hospices Civils de Lyon, in the design and theimplementation of the EDMUS system and of Mr Jean MarcAzerad, Option Performance, in the development of theprogramme have been appreciated. The Committee gratefullyacknowledge the support of the European Charcot Foundation,Hospices Civils de Lyon, Association pour la Recherche sur laSclerose En Plaques (ARSEP), Ligue Francaise contre laSclerose En Plaques (LFSEP).

1 Confavreux C, Moreau T, Wolfson C, et al. Natural historyand prognosis in multiple sclerosis: the Lyon MS data-base. In: Battaglia MA, ed. Multiple sclerosis research.Amsterdam: Elsevier 1989:199-203.

2 Poser CM, Paty DW, Scheinberg L, et al. New diagnosticcriteria for multiple sclerosis: guidelines for researchprotocols. Ann Neurol 1983;13:227-31.

3 Schumacher GA, Beebe G, Kibler RF, et al. Problems ofexperimental trials of therapy in MS: report by the panelon the evaluation of experimental trials of therapy in MS.Ann NYAcad Sci 1965;122:552-68.

4 Kurtzke JF. On the evaluation of disability in multiplesclerosis. Neurology 1961;11:686-94.

5 Kurtzke JF. Further notes on disability evaluation inmultiple sclerosis, with scale modifications. Neurology1965;15:654-61.

6 Kurtzke JF. Rating neurological impairment in multiplesclerosis: an expanded disability status scale (EDSS).Neurology 1983;33:1444-52.

7 Hauser SL, Dawson DM, Lehrich JR, et al. Intensiveimmunosuppression in progressive multiple sclerosis: arandomized, three-arm study of high-dose intravenouscyclophosphamide, plasma exchange, and ACTH. N EngI7Med 1983;308:173-80.

8 Confavreux C, Aimard G, Devic M. Course and prognosisof Multiple Sclerosis assessed by the computerized dataprocessing of 349 patients. Brain 1980;103:281-300.

9 Lee KH, Hashimoto SA, Hooge JP, et al. Magneticresonance imaging of the head in the diagnosis of multiplesclerosis: a prospective 2 year follow up with comparisonof clinical evaluation, evoked potentials, oligoclonal band-ing, and CT. Neurology 1991;41:657-60.

10 Delpech B, Lichtblau F. Etude quantitative des immuno-globulines G et de l'albumine du liquide cephalo-rachidien. Clin Chim Acta 1972;37:15-23.

11 Miller DH, Barkoff F, Berry I, Kappos L, Scotti G,Thompson AJ. Magnetic resonance imaging in monitor-ing the treatment of multiple sclerosis: Concerted ActionGuidelines. J Neurol Neurosurg Psychiat 199 1;54:683-8.

APPENDIX

Appendix 1: The EDMUS Impairment Scale (DSSadapted):

EIS (DSS adapted)Score0 Normal findings on neurological examination.1 No disability. Minimal signs on neurological

examination.2 Minimal and not ambulation-related disability. Able

to run.3 Unlimited walking distance (WD) without rest, but

unable to run; or a significant not ambulation-related disability.

4 Walks without aid. Limited WD, but > 500 meterswithout rest.

5 Walks without aid. WD < 500 meters without rest.6 Walks with uni or bilateral support. WD < 100

meters without rest.7 Home restricted. A few steps with wall or furniture

assistance. WD < 10 meters without rest.8 Chair restricted. Unable to take a step. Some

effective use of arms.9 Bedridden and totally helpless.10 Death due to MS.

Appendix 2: List of the EDMUS Steering Committeemembers

Belgium and Luxemburg: Dr Richard Gonsette,Melsbroek.

Denmark: Dr Niels Koch-Henriksen, Odense.Germany: Professor Hans-Peter Hartung, Wurzburg.France: Professor Christian Confavreux (President),

Lyon; Dr Annick Alperovitch, Villejuif; ProfessorGilles Edan, Rennes.

Ireland: Dr Michael Hutchinson, Dublin.Italy: Professor Giancarlo Comi, Milan.The Netherlands: Professor Otto Hommes (Project-

leader of the European Concerted Action for MS),Nijmegen.

Portugal: Professor Antonio Magalhaes, Lisboa.Spain: Dr Oscar Fernandez, Malaga.Switzerland: Dr Ludwig Kappos, Basel.United Kingdom: Professor Alastair Compston,

Cambridge; Dr Alan Thompson, London.

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EDMUS, Europeandatabase for multiple sclerosis