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2. Titin antibodies in “seronegative” myasthenia gravis — A new role for an old antigen

Author

Stergiou, C., Lazaridis, K., Zouvelou, V., Tzartos, J., Mantegazza, R., Antozzi, C., Andreetta, F., Evoli, A., Deymeer, F., Saruhan-Direskeneli, G., Durmus, H., Brenner, T., Vaknin, A., Berrih-Aknin, S., Behin, A., Sharshar, T., De Baets, M., Losen, M., Martinez-Martinez, P., Kleopa, K.A., Zamba-Papanicolaou, E., Kyriakides, T., Kostera-Pruszczyk, A., Szczudlik, P., Szyluk, B., Lavrnic, D., Basta, I., Peric, S., Tallaksen, C., Maniaol, A., Gilhus, N.E., Casasnovas Pons, C., Pitha, J., Jakubíkova, M., Hanisch, F., Bogomolovas, J., Labeit, D., Labeit, S., and Tzartos, S.J.

Published
2016
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6. Myasthenia gravis

Author

Gilhus, N.E., Tzartos, S., Evoli, A., Palaces, J., Burns, T.M., and Verschuuren, J.J.G.M.

Abstract

Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thynnectonny and innnnunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.

Published
2019

8. Long term outcome after poliomyelitis in different health and social conditions. (Research Report)

Author

Rekand, T., Korv, J., Farbu, E., Roose, M., Gilhus, N.E., Langeland, N., and Aarli, J.A.

Subjects
Social aspects, Care and treatment, Evaluation, Prognosis, Methods, Diseases -- Evaluation -- Social aspects -- Care and treatment -- Estonia -- Norway -- Prognosis, Rehabilitation -- Evaluation -- Methods -- Social aspects, Poliomyelitis -- Social aspects -- Prognosis -- Care and treatment
Abstract

Objective: To examine and compare the long term outcome after polio in an east European and a west European country with different access to rehabilitation and with different medical and [...]

Published
2003

9. Editorial Acknowledgment

Author

Aarvak, T., Abedi-Valugerdi, M., Abrahamsen, T., Akdis, C., Akuffo, H.O., Alarcon-Riquelme, M., Andersen, P., Arstila, P., Austgulen, R., Baecker-Allan, C., Baggiolini, M., Baker, B.S., Bakke, O., Bakken, V., Barclay, A.N., Bemark, M., Benestad, H.B., Berzins, K., Bjerkvik, R., Bjørge, L., Bland, P., Bolstad, A.I., Born, W., Bowman, S., Brinchmann, J., Britton, S., Brokstad, K.A., Brunet, L.R., Bruserud, Ø., Cardona, P.J., Ceyppens, J.L., Cirino, G., Cohn, M., Cox, R.J., Cunliffe, J., Dahlgren, U., Dellacasagrande, J., Ditzel, H., Dobryszycka, E., Dyrhol-Riise, A.M., Edwards, J.C., Egeland, T., Elsayed, S., Eray, M., Erb, K.J., Esch, T., Eugen-Olsen, J., Everse, L.A., Fagerhol, M., Farstad, I., Feldmann, M., Fernandez, C., Fleck, M., Franksson, R., Freitas, A., Garred, P., Gidlund, M., Gilhus, N.E., Goldbach-Mansky, R., Gordon, J., Gordon, T., Grandien, A., Gregerson, P., Grewal, H., Hakulinen, J., Halstensen, T., Haneberg, B., Hanej, S.H.E., Hansen, T., Harley, J.B., Haukenes, G., Hengartner, H., Henz, B.M., Holen, E., Hurme, M., Husby, G., Husby, S., Hörnqvist-Hultgren, E., Haaheim, L.R., Isenberg, D., Jahnson, F., Jakobsen, H., James, J.A., Jansson, J.O., Jarva, H., Jeansson, S., Johannessen, A.C., Johansen, F.E., Julkunen, I., Kalland, K.H., Kaye, J., Kazatchkine, M., Kihlström, E., Kohler, L., Konttinen, Y., Kotenko, S., Koutzov, S., Krammer, P., Kristoffersen, E., Kunzendorf, U., Lachmann, P., Langman, R., Langner, J., Lanzavecchia, L., Lappalainen, M., Laskay, T., Lea, T., Lefvert, A.K., Lehmann, A.C., Louka, A, Lúdviksson, B.R., Mariette, X., Marion, T., Marker-Herrmann, E., Mattsson, R., McAdam, S., Mellbye, O.J., Mellgren, G., Mellgren, S.I., Mestecky, J., Michaelsen, T., Michalek, S., Miettinen, A., Moestrup, S.K., Moretta, A., Moser, M., Mountz, J., Moutsopoulos, H., Munthe, L.A., Mustafa, T., Myrmel, H., Nannemark, G., Netea, M.G., Nilsen, R., Notarangelo, L.D., Ögmundsdóttir, H., Olsson, I., Olweus, J., Osland, A., Paludan, S.R., Parsons, K.R., Patarroyo, M., Paul, W.E., Perlman, P., Pollock, J.M., Puolakkainen, M., Quale, A., Rajewsky, K., Raman, C., Reichlin, M., Renkonen, R., Rese Shadidi, K., Ricciardi-Castagnoli, P., Rickinson, A.B., Ringdén, O., Risto, R., Rodinov, D., Rosenkrands, I., Rottenberg, M., Russel, M., Rygaard, J., Rødahl, E., Röllinghof, M., Sandberg, M., Schenk, K., Seppälä, I., Shevach, E., Shortman, K., Siegrist, C.A., Sigurdardóttir, S., Silvennoinen, O., Sinigaglia, F., Sistonen, L., Skarstein, K., Skogh, T., Smith, E., Spurkland, A., Stendahl, O., Sugden, B., Suri-Payer, E., Svennerholm, A.M., Szodoray, P., Theofilopoulos, A., Thorley-Lawson, D.A., Timonen, T., Trowsdale, J., Tuomo, T., Ulmer, A.J., Ulvestad, E., Ulvik, R., Vaerman, J.P., van, der Merwe J.P., van Eden, W., Vedeler, C., Vintermyr, O.K., Vyse, J., Wahlgren, M., Wahren-Herlenius, M., Walsh, L.J., Weetman, A.P., Westermark, P., Wiker, H., Yang, J., Yewdell, J.W., Youinou, P., and Zouali, M.

Published
2002

14. Neurofilament ELISA validation

Author

Petzold, A. Altintas, A. Andreoni, L. Bartos, A. Berthele, A. Blankenstein, M.A. Buee, L. Castellazzi, M. Cepok, S. Comabella, M. Constantinescu, C.S. Deisenhammer, F. Deniz, G. Erten, G. Espiño, M. Fainardi, E. Franciotta, D. Freedman, M.S. Giedraitis, V. Gilhus, N.E. Giovannoni, G. Glabinski, A. Grieb, P. Hartung, H.-P. Hemmer, B. Herukka, S.-K. Hintzen, R. Ingelsson, M. Jackson, S. Jacobsen, S. Jafari, N. Jalosinski, M. Jarius, S. Kapaki, E. Kieseier, B.C. Koel-Simmelink, M.J.A. Kornhuber, J. Kuhle, J. Kurzepa, J. Lalive, P.H. Lannfelt, L. Lehmensiek, V. Lewczuk, P. Livrea, P. Marnetto, F. Martino, D. Menge, T. Norgren, N. Papuć, E. Paraskevas, G.P. Pirttilä, T. Rajda, C. Rejdak, K. Ricny, J. Ripova, D. Rosengren, L. Ruggieri, M. Schraen, S. Shaw, G. Sindic, C. Siva, A. Stigbrand, T. Stonebridge, I. Topcular, B. Trojano, M. Tumani, H. Twaalfhoven, H.A.M. Vécsei, L. Van Pesch, V. Vanderstichele, H. Vedeler, C. Verbeek, M.M. Villar, L.M. Weissert, R. Wildemann, B. Yang, C. Yao, K. Teunissen, C.E.

Abstract

Background: Neurofilament proteins (Nf) are highly specific biomarkers for neuronal death and axonal degeneration. As these markers become more widely used, an inter-laboratory validation study is required to identify assay criteria for high quality performance. Methods: The UmanDiagnostics NF-light ®enzyme-linked immunoabsorbent assays (ELISA) for the neurofilament light chain (NfL, 68 kDa) was used to test the intra-assay and inter-laboratory coefficient of variation (CV) between 35 laboratories worldwide on 15 cerebrospinal fluid (CSF) samples. Critical factors, such as sample transport and storage, analytical delays, reaction temperature and time, the laboratories' accuracy and preparation of standards were documented and used for the statistical analyses. Results: The intra-laboratory CV averaged 3.3% and the inter-laboratory CV 59%. The results from the test laboratories correlated with those from the reference laboratory (R = 0.60, p < 0.0001). Correcting for critical factors improved the strength of the correlation. Differences in the accuracy of standard preparation were identified as the most critical factor. Correcting for the error introduced by variation in the protein standards improved the correlation to R = 0.98, p < 0.0001 with an averaged inter-laboratory CV of 14%. The corrected overall inter-rater agreement was subtantial (0.6) according to Fleiss' multi-rater kappa and Gwet's AC1 statistics. Conclusion: This multi-center validation study identified the lack of preparation of accurate and consistent protein standards as the main reason for a poor inter-laboratory CV. This issue is also relevant to other protein biomarkers based on this type of assay and will need to be solved in order to achieve an acceptable level of analytical accuracy. The raw data of this study is available online. © 2009 Elsevier B.V.

Published
2010

15. Cognitive Rehabilitation

Author

Cappa, S.F., Benke, T., Clarke, S., Rossi, B., Stemmer, B., van Heugten, C.M., Hughes, R., Brainin, M., Gilhus, N.E., Neuropsychology & Psychopharmacology, and RS: FPN NPPP I

Published
2008
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16. Report of the task force on pre‐graduate education in Europe of the Education Committee of the European Federation of Neurological Societies

Author

LOPES LIMA, J.M., MESEC, A., WILKINSON, I.M.J., WILES, C.M., GILHUS, N.E., ZIMPRICH, F., and ALEJSEENKO, Y.V.

Abstract

Submitted by lara martins (laramartins.defi@chporto.min-saude.pt) on 2011-07-06T10:16:14Z No. of bitstreams: 1 Report of the Task Force on pre-graduate education... [Eur J Neurol. 2008] -.pdf: 12935 bytes, checksum: b2b4a27be988f471ae9b0055cfe94f75 (MD5) Made available in DSpace on 2011-07-06T10:16:14Z (GMT). No. of bitstreams: 1 Report of the Task Force on pre-graduate education... [Eur J Neurol. 2008] -.pdf: 12935 bytes, checksum: b2b4a27be988f471ae9b0055cfe94f75 (MD5) Previous issue date: 2006-12

Published
2006

17. Neurofilament ELISA validation.

Author

Petzold, A., Altintas, A., Andreoni, L., Bartos, A., Berthele, A., Blankenstein, M.A., Buee, L., Castellazzi, M., Cepok, S., Comabella, M., Constantinescu, C.S., Deisenhammer, F., Deniz, G., Erten, G., Espino, M., Fainardi, E., Franciotta, D., Freedman, M.S., Giedraitis, V., Gilhus, N.E., Giovannoni, G., Glabinski, A., Grieb, P., Hartung, H.P., Hemmer, B., Herukka, S.K., Hintzen, R., Ingelsson, M., Jackson, S., Jacobsen, S., Jafari, N., Jalosinski, M., Jarius, S., Kapaki, E., Kieseier, B.C., Koel-Simmelink, M.J., Kornhuber, J., Kuhle, J., Kurzepa, J., Lalive, P.H., Lannfelt, L., Lehmensiek, V., Lewczuk, P., Livrea, P., Marnetto, F., Martino, D., Menge, T., Norgren, N., Papuc, E., Paraskevas, G.P., Pirttilä, T., Rajda, C., Rejdak, K., Ricny, J., Ripova, D., Rosengren, L., Ruggieri, M., Schraen, S., Shaw, G., Sindic, C., Siva, A., Stigbrand, T., Stonebridge, I., Topcular, B., Trojano, M., Tumani, H., Twaalfhoven, H.A., Vecsei, L., Pesch, V. Van, Vanderstichele, H., Vedeler, C., Verbeek, M.M., Villar, L.M., Weissert, R., Wildemann, B., Yang, C., Yao, K., Teunissen, C.E., Petzold, A., Altintas, A., Andreoni, L., Bartos, A., Berthele, A., Blankenstein, M.A., Buee, L., Castellazzi, M., Cepok, S., Comabella, M., Constantinescu, C.S., Deisenhammer, F., Deniz, G., Erten, G., Espino, M., Fainardi, E., Franciotta, D., Freedman, M.S., Giedraitis, V., Gilhus, N.E., Giovannoni, G., Glabinski, A., Grieb, P., Hartung, H.P., Hemmer, B., Herukka, S.K., Hintzen, R., Ingelsson, M., Jackson, S., Jacobsen, S., Jafari, N., Jalosinski, M., Jarius, S., Kapaki, E., Kieseier, B.C., Koel-Simmelink, M.J., Kornhuber, J., Kuhle, J., Kurzepa, J., Lalive, P.H., Lannfelt, L., Lehmensiek, V., Lewczuk, P., Livrea, P., Marnetto, F., Martino, D., Menge, T., Norgren, N., Papuc, E., Paraskevas, G.P., Pirttilä, T., Rajda, C., Rejdak, K., Ricny, J., Ripova, D., Rosengren, L., Ruggieri, M., Schraen, S., Shaw, G., Sindic, C., Siva, A., Stigbrand, T., Stonebridge, I., Topcular, B., Trojano, M., Tumani, H., Twaalfhoven, H.A., Vecsei, L., Pesch, V. Van, Vanderstichele, H., Vedeler, C., Verbeek, M.M., Villar, L.M., Weissert, R., Wildemann, B., Yang, C., Yao, K., and Teunissen, C.E.

Abstract

Contains fulltext : 89601.pdf (publisher's version ) (Closed access), BACKGROUND: Neurofilament proteins (Nf) are highly specific biomarkers for neuronal death and axonal degeneration. As these markers become more widely used, an inter-laboratory validation study is required to identify assay criteria for high quality performance. METHODS: The UmanDiagnostics NF-light (R)enzyme-linked immunoabsorbent assays (ELISA) for the neurofilament light chain (NfL, 68kDa) was used to test the intra-assay and inter-laboratory coefficient of variation (CV) between 35 laboratories worldwide on 15 cerebrospinal fluid (CSF) samples. Critical factors, such as sample transport and storage, analytical delays, reaction temperature and time, the laboratories' accuracy and preparation of standards were documented and used for the statistical analyses. RESULTS: The intra-laboratory CV averaged 3.3% and the inter-laboratory CV 59%. The results from the test laboratories correlated with those from the reference laboratory (R=0.60, p<0.0001). Correcting for critical factors improved the strength of the correlation. Differences in the accuracy of standard preparation were identified as the most critical factor. Correcting for the error introduced by variation in the protein standards improved the correlation to R=0.98, p<0.0001 with an averaged inter-laboratory CV of 14%. The corrected overall inter-rater agreement was subtantial (0.6) according to Fleiss' multi-rater kappa and Gwet's AC1 statistics. CONCLUSION: This multi-center validation study identified the lack of preparation of accurate and consistent protein standards as the main reason for a poor inter-laboratory CV. This issue is also relevant to other protein biomarkers based on this type of assay and will need to be solved in order to achieve an acceptable level of analytical accuracy. The raw data of this study is available online.

Published
2010

18. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases

Author

Elovaara, I., Apostolski, S., Doorn, P. van, Gilhus, N.E., Hietaharju, A., Honkaniemi, J., van, Schaik,I, Scolding, N., Udd, B., Sørensen, Per Soelberg, Elovaara, I., Apostolski, S., Doorn, P. van, Gilhus, N.E., Hietaharju, A., Honkaniemi, J., van, Schaik,I, Scolding, N., Udd, B., and Sørensen, Per Soelberg

Abstract

Despite high-dose intravenous immunoglobulin (IVIG) is widely used in treatment of a number of immune-mediated neurological diseases, the consensus on its optimal use is insufficient. To define the evidence-based optimal use of IVIG in neurology, the recent papers of high relevance were reviewed and consensus recommendations are given according to EFNS guidance regulations. The efficacy of IVIG has been proven in Guillain-Barre syndrome (level A), chronic inflammatory demyelinating polyradiculoneuropathy (level A), multifocal mononeuropathy (level A), acute exacerbations of myasthenia gravis (MG) and short-term treatment of severe MG (level A recommendation), and some paraneoplastic neuropathies (level B). IVIG is recommended as a second-line treatment in combination with prednisone in dermatomyositis (level B) and treatment option in polymyositis (level C). IVIG should be considered as a second or third-line therapy in relapsing-remitting multiple sclerosis, if conventional immunomodulatory therapies are not tolerated (level B), and in relapses during pregnancy or post-partum period (good clinical practice point). IVIG seems to have a favourable effect also in paraneoplastic neurological diseases (level A), stiff-person syndrome (level A), some acute-demyelinating diseases and childhood refractory epilepsy (good practice point) Udgivelsesdato: 2008/9

Published
2008

35. Disease severity and outcome in thymoma myasthenia gravis: a long-term observation study.

Author

Romi, F., Gilhus, N.E., Varhaug, J.E., Myking, A., and Aarli, J.A.

Subjects
*MYASTHENIA gravis, *AUTOANTIBODIES, *MUSCLES, *RYANODINE
Abstract

Thymomas occurring in myasthenia gravis (MG) are usually of the cortical subtype and are usually treated by thymectomy. However, the factors that influence MG outcome in thymoma MG patients are not known. In a long-term study, MG severity and treatment was observed in 24 thymoma and 24 non-thymoma MG patients for up to 30 years, and the occurrence of muscle autoantibodies was assayed. The rate of complete stable remission was low and did not differ between the two groups. There was no significant difference in MG severity between thymoma and non-thymoma MG patients at any time during the study. Titin and ryanodine receptor (RyR) antibody occurrence was significantly higher in thymoma MG patients. Four thymoma (all titin and RyR antibody positive) and two non-thymoma (both titin and one RyR antibody positive) MG patients died from MG-related respiratory insufficiency. Seventy percent of thymoma and 75% of non-thymoma MG patients were treated with immunosuppressive drugs. The number of patients who received plasmapheresis did not differ in those who were treated because of acute MG deterioration, irrespective of planned surgery .This study shows equal MG severity and outcome in thymoma and non-thymoma MG, but the presence of RyR antibodies in thymoma MG and titin/RyR in non-thymoma MG indicates a less favorable prognosis. [ABSTRACT FROM AUTHOR]

Published
2003
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36. MR analysis of the transverse ligament in the late stage of whiplash injury.

Author

Krakenes, J., Kaale, B.R., Nordli, H., Moen, G., Rorvik, J., and Gilhus, N.E.

Subjects
WHIPLASH injuries, LIGAMENT injuries, CERVICAL vertebrae, MAGNETIC resonance imaging, RADIOSCOPIC diagnosis, MEDICAL radiography
Abstract

Purpose: To analyse and classify structural changes in the transverse ligament in the late stage of whiplash injury by use of high-resolution MRI, and to evaluate the reliability of our classification.Material and Methods: Ninety-two whiplash-injured (2-9 years previously, mean 6 years) and 30 non-injured individuals underwent proton-weighted MR imaging of the craniovertebral junction in three orthogonal planes. Structural changes in the transverse ligaments were graded twice (grades 1-3) based on increased signal, independently by 3 radiologists with a 4-month interval. Inter- and intraobserver statistics were calculated by ordinary and weighted kappa (K).Results: Image quality was excellent in 109 cases and slightly reduced in 13. Twenty-two out of 30 ligaments in the control group were classified as normal (73%) compared with only 32 out of 92 in the injured group (36%). Two or all 3 observers agreed in their grading in 101 out of 122 ligaments (83%). Intraobserver agreement (weighted K) was fair to good (0.33-0.73). Pair-wise interobserver agreement was fair (0.24-0.39). Reasons for divergent grading were insufficient knowledge of normal variations, low signal intensity in the peridental soft tissue obscuring the ligament and interpretation flaw.Conclusion: Whiplash trauma can damage the transverse ligament. By use of high-resolution proton-weighted MR images such lesions can be detected and classified. The reliability of this classification still needs improvement. [ABSTRACT FROM AUTHOR]

Published
2003
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37. EFNS Task Force Report: a questionnaire-based survey on the service provision and quality assurance for determination of diagnostic autoantibody tests in European neuroimmunology centres.

Author

Willison, H.J., Ang, W., Gilhus, N.E., Graus, F., Liblau, R., Vedeler, C., and Vincent, A.

Subjects
AUTOANTIBODIES, POTASSIUM channels
Abstract

Examines the impact of autoantibodies on patients with neurological diseases. Correlation between antineuronal antibodies and paraneoplastic syndromes; Role of antibodies in voltage gated calcium channels and potassium channels; Measurement of the antiglycolipid antibodies.

Published
2000
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44. Increased bacterial antibody titres and IgA deficiency in epileptic patients.

Author

Iivanainen, M., Syrjanen, J., Waltimo, O., Valtonen, V.V., and Gilhus, N.E.

Subjects
IMMUNOGLOBULIN A, GENETICS of epilepsy
Abstract

Examines the prevalence of increased bacterial antibody titres and Immunoglobulin A (IgA) deficiency in epileptic patients. Effect of Phenytoin on IgA; Activation of bacterial seizures with infections; Association of epileptic seizures with bacterial antibody.

Published
1987
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