Neurostatus Systems AG

Neurostatus
About

PLEASE NOTE: Since the beginning of August 2016 all Neurostatus products and services are operated and maintained by the Department of Neurology at the University Hospital Basel UHBS. Contact the UHBS also for a Licence to capture and validate electronic Neurostatus/EDSS examination data.

 

The Neurostatus/EDSS scale is the widely accepted primary endpoint of most clinical trials in Multiple Sclerosis. The products and services are developed in collaboration with the scientific experts of the MS team at the University Hospital Basel (Prof. L. Kappos) and cover the whole process from training and certification and Neurostatus/EDSS raters to electronic data capture, data management and scientific review within clinical trials.

HISTORICAL BACKGROUND

The (E)DSS was initially introduced in 1955 as the Disability Status Scale, a 10-step overall neurological disability rating scale. It was subsequently extended in 1983 by the introduction of half steps, leading to a total of 20 steps ranging from 0 to 10, separated by half-point increments (Expanded Disability Status Scale).

Since the 1982 Prof. L. Kappos was involved in the planning and conducting of numerous clinical MS trial and subsequently improved and standardized the EDSS scale (references), which became the Neurostatus, consisting of the Neurostatus Definitions and Scoring Sheet.

In parallel Neurostatus Systems and it's predecessor companies developed various training and certification programs in collaboration with the Prof. L. Kappos' MS team at the University Hospital Basel. All started with training videos in 1994, continued with a training CD/DVD Rom (1998/2005) and lead to the online certification platform Neurostatus e-Test in 2003.

In 2007 the development of the Neurostatus Algorithm, a digitized version of the complete Neurostatus Definitions, began and was finally validated and implemented in 2011. The complete Neurostatus e-Scoring system, including mobile electronic data capture and data management was introduced in 2013.

Neurostatus/EDSS has become a key endpoint of most phase III clinical trials in Multiple Sclerosis and is accepted by health authorities as a basis for label claims on disability.

STRUCTURE OF NEUROSTATUS/EDSS

The EDSS is based on a standardised neurological examination, but focuses on the symptoms that occur frequently in MS.

Assessment of the seven Functional Systems (FS: visual, brainstem, pyramidal, cerebellar, sensory, bowel & bladder and cerebral) is based on a standardised neurological examination.

Based on the FS scores and on the assessment of the ambulation, the EDSS step is determined. The range of the EDSS includes 20 half steps from 0 to 10, with EDSS step 0 corresponding to a completely normal examination and EDSS step 10 to death due to MS. For EDSS ratings between 0 and 4, the scale relies mainly on the scores of the individual FS. For ratings over 4, the EDSS is primarily determined by the ability and range of walking.

WORLDWIDE DISTRIBUTION

Neurostatus products are used worldwide:

  • Approx. 70 ongoing MS and NMO studies use Neurostatus e-Test
  • 2 studies use Neurostatus e-Scoring since 2013
  • 3 Studies e-Scoring Algorithm since 2011
  • More than 130 studies use Neurostatus e-Test Qualification since 2003
  • More than 10’000 Neurologists have active accounts on Neurostatus.net
  • More than 25’000 active e-Test Qualifications
  • Approx. 80 new registrations monthly on Neurostatus.net
  • Approx. 10'000 Training DVD ROMs delivered
Neurostatus Systems AG
Close

References

Amato MP, Fratiglioni L, Groppi C, Siracusa G, Amaducci L. Interrater reliability in assessing functional systems and disability on the Kurtzke scale in multiple sclerosis. Arch Neurol. 1988;45(7):746‐8.

Andersson PB, Goodkin DE. Outcome measures in multiple sclerosis clinical trials. Baillieres Clin Neurol. 1997;6(3):409‐28.

Butzkueven H, Chapman J, Cristiano E, Grand'Maison F, Hoffmann M, Izquierdo G, Jolley D, Kappos L, Leist T, Pöhlau D, Rivera V, Trojano M, Verheul F, Malkowski JP. MSBase: an international, online registry and platform for collaborative outcomes research in multiple sclerosis. Mult Scler. 2006;12(6):76974.

Cohen JA, Reingold SC, Polman CH, Wolinsky JS; International Advisory Committee on Clinical Trials in Multiple Sclerosis. Disability outcome measures in multiple sclerosis clinical trials: current status and future prospects. Lancet Neurol. 2012;11(5):46776.

Francis DA, Bain P, Swan AV, Hughes RA. An assessment of disability rating scales used in multiple sclerosis. Arch Neurol. 1991;48(3):299301.

Goodkin DE, Cookfair D, Wende K, Bourdette D, Pullicino P, Scherokman B, Whitham R. Interand intrarater scoring agreement using grades 1.0 to 3.5 of the Kurtzke Expanded Disability Status Scale (EDSS). Multiple Sclerosis Collaborative

Research Group. Neurology. 1992;42(4):85963.

Hobart J, Freeman J, Thompson A. Kurtzke scales revisited: the application of psychometric methods to clinical intuition. Brain. 2000;123 ( Pt 5):102740.

Hobart J, Kalkers N, Barkhof F, Uitdehaag B, Polman C, Thompson A. Outcome measures for multiple sclerosis clinical trials: relative measurement precision of the Expanded Disability Status Scale and Multiple Sclerosis Functional Composite. Mult Scler. 2004;10(1):416.

Hyland M, Rudick RA. Challenges to clinical trials in multiple sclerosis: outcome measures in the era of diseasemodifying drugs. Curr Opin Neurol. 2011;24(3):25561. Review.

Kappos L. European Study Group on Interferon β‐1b in Secondary Progressive MS. Placebocontrolled multicentre randomised trial of interferon β‐1b in treatment of secondary progressive multiple sclerosis. Lancet. 1988; 352:14911497.

Kappos L. Immunsuppressive Therapie der multiplen Sklerose mit Cyclosporin A und Azathioprin. Langzeiteffekte, Risiken, kernspintomographische und immunologische Befunde. Springer, 1990.

Kappos L, LechnerScott J, Lienert C. Neurostatus Neurostatus training and documentation DVD for a standardized neurological examination and assessment of Kurtzke’s functional systems and Expanded Disability Status Scale for MS patients. 2002, 2007, Basel, Switzerland. Available at: http://neurostatus.net. Accessed March 30, 2015.

Kappos L, Patzold U, Dommasch D, Poser S, Haas J, Krauseneck P, Malin JP, Fierz W, von Graffenried B, Gugerli US. Cyclosporine vs azathioprine in the longterm treatment of MS results of the German multicenter study. Ann Neurol 1988;23:5663.

Kragt JJ, Thompson AJ, Montalban X, Tintoré M, Río J, Polman CH, Uitdehaag BM. Responsiveness and predictive value of EDSS and MSFC in primary progressive MS. Neurology. 2008;70(13 Pt 2):108491.

Kurtzke JF. A new scale for evaluating disability in multiple sclerosis. Neurology 1955;5:580–3.

Kurtzke JF. On the evaluation of disability in multiple sclerosis. Neurology 1961;11:686–94. 

Kurtzke JF. Further notes on disability evaluation in multiple sclerosis, with scale modifications. Neurology1965;15:654–61.

Kurtzke JF. Clinical manifestations of multiple sclerosis. Multiple sclerosis and other demyelinating diseases. In: VinkenPJ,Bruyn GW, editors. Handbook of clinical neurology,vol. 9. Amsterdam: NorthHolland;1970:161–216.

Kurtzke JF. Rating neurologic impairment in multiple sclerosis. An expanded disability status scale(EDSS). Neurology1983;33:1444–52.

Kurtzke JF. The Disability Status Scale for multiple sclerosis: Apologia pro DSS sua. Neurology 1989a;39:291–302.

Kurtzke JF. Origin of DSS: to present the plan. Mult Scler. 2007;13(1):1203.

Kurtzke JF. Historical and clinical perspectives of the Expanded Disability Status Scale. Neuroepidemiology 2008;31:1–9.

Kurtzke JF. On the origin of EDSS. Multiple Sclerosis and Related Disorders 2015; 4:95103.

Kuzma JW, Namerow NS, Tourtellotte WW, Sibley WA, Kurtzke JF, Rose AS, Dixon WJ. An assessment of the reliability of three methods used in evaluating the status of multiple sclerosis patients. J Chronic Dis. 1969;21(11):80314.

LechnerScott J, Kappos L, Hofman M, Polman CH, Ronner H, Montalban X, Tintore M, Frontoni M, Buttinelli C, Amato MP, Bartolozzi ML, Versavel M, Dahlke F, Kapp JF, Gibberd R. Can the Expanded Disability Status Scale be assessed by telephone? Mult Scler. 2003;9(2):1549.

MeyerMoock S, Feng YS, Maeurer M, Dippel FW, Kohlmann T. Systematic literature review and validity evaluation of the Expanded Disability Status Scale (EDSS) and the Multiple Sclerosis Functional Composite (MSFC) in patients with multiple sclerosis. BMC Neurol. 2014;14:58.

Montalban X, Tintoré M, Río J, Galán I, Codina A. Interobserver variability in the evaluation of functional systems and Kurzke expanded disability status scale in a multiple sclerosis patient. Rev Neurol. 1996;24(130):6302.

Noseworthy JH. Clinical scoring methods for multiple sclerosis. Ann Neurol. 1994;36 Suppl:S805.

Noseworthy JH, Vandervoort MK, Hopkins M, Ebers GC. A referendum on clinical trial research in multiple sclerosis: the opinion of the participants at the Jekyll Island workshop. Neurology. 1989;39(7):97781.

Noseworthy JH, Vandervoort MK, Wong CJ, Ebers GC. Interrater variability with the Expanded Disability Status Scale (EDSS) and Functional Systems (FS) in a multiple sclerosis clinical trial. The Canadian Cooperation MS Study Group. Neurology. 1990;40(6):9715. 

Patzold U, Pocklington P. Course of multiple sclerosis. First results of a prospective study carried out of 102 MS patients from 19761980. Acta Neurol Scand. 1982; 65: 248266.

Patzold U, Weinrich W. Vorschlag einer neurologischen Befunddokumentation mittels Markierungsbeleg. Nervenarzt. 1975;46:550556.

Rudick R, Antel J, Confavreux C, Cutter G, Ellison G, Fischer J, Lublin F, Miller A, Petkau J, Rao S, Reingold S, Syndulko K, Thompson A, Wallenberg J, Weinshenker B, Willoughby E. Clinical outcomes assessment in multiple sclerosis. Ann Neurol. 1996;40(3):46979. Review. 

Rudick RA, Cutter G, Reingold S. The multiple sclerosis functional composite: a new clinical outcome measure for multiple sderosis trials. Mult Scler. 2002;8(5):35965.

Rudick RA, Kappos L. Measuring disability in relapsingremitting MS. Neurology 2010; 75(4): 296297.

Sharrack B, Hughes RA. Clinical scales for multiple sclerosis. J Neurol Sci. 1996;135(1):19.

Sharrack B, Hughes RA, Soudain S, Dunn G. The psychometric properties of clinical rating scales used in multiple sclerosis. Brain. 1999;122 ( Pt1):14159.

VerdierTaillefer MH, Zuber M, LyonCaen O, Clanet M, Gout O, Louis C, Alpérovitch A. Observer disagreement in rating neurologic impairment in multiple sclerosis: facts and consequences. Eur Neurol. 1991;31(2):1179.

Whitaker JN, McFarland HF, Rudge P, Reingold SC. Outcomes assessment in multiple sclerosis clinical trials: a critical analysis. Mult Scler. 1995;1(1):3747.

Willoughby EW, Paty DW. Scales for rating impairment in multiple sclerosis: a critique. Neurology. 1988;38(11):17938.

Wingerchuk DM, Noseworthy JH, Weinshenker BG. Clinical outcome measures and rating scales in multiple sclerosis trials. Mayo Clin Proc. 1997;72(11):10709.