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Neuromyelitis optica in Austria in 2011: to bridge the gap between neuroepidemiological research and practice in a study population of 8.4 million people.

Aboul-Enein F, Seifert-Held T, Mader S, Kuenz B, Lutterotti A, Rauschka H, Rommer P, Leutmezer F, Vass K, Flamm-Horak A, Stepansky R, Lang W, Fertl E, Schlager T, Heller T, Eggers C, Safoschnik G, Fuchs S, Kraus J, Assar H, Guggenberger S, Reisz M, Schnabl P, Komposch M, Simschitz P, Skrobal A, Moser A, Jeschow M, Stadlbauer D, Freimüller M, Guger M, Schmidegg S, Franta C, Weiser V, Koppi S, Niederkorn-Duft M, Raber B, Schmeissner I, Jecel J, Tinchon A, Storch MK, Reindl M, Berger T, Kristoferitsch W - PLoS ONE (2013)

Bottom Line: Sex ratio (m:f = 1:7) and the proportion of patients without oligoclonal bands in cerebrospinal fluid (86.6%) were in line with previously published results.All identified patients were Caucasians.This database is presented in detail and provides the basis for further studies and international cooperation in order to investigate this rare disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Sozialmedizinisches Zentrum Ost Donauspital, Vienna, Austria ; Department of Neurology, Karl Landsteiner Institute for Neuroimmunological and Neurodegenerative Disorders, Vienna, Austria.

ABSTRACT

Background: In 2008 the Austrian Task Force for Neuromyelitis Optica (NMO) started a nation-wide network for information exchange and multi-centre collaboration. Their aim was to detect all patients with NMO or NMO spectrum disorders (NMO-SD) in Austria and to analyse their disease courses and response to treatment.

Methods: (1) As of March 2008, 1957 serum samples (of 1557 patients) have been tested with an established cell based immunofluorescence aquaporin-4 antibody (AQP4-ab) assay with a high sensitivity and specificity (both >95%). All tests were performed in a single reference laboratory (Clinical Dept. of Neurology of the Innsbruck Medical University). (2) A nation-wide survey with several calls for participation (via email newsletters, articles in the official journal of the Austrian Society of Neurology, and workshops) was initiated in 2008. All collected data will be presented in a way that allows that every individual patient can be traced back in order to ensure transparency and to avoid any data distortion in future meta-analyses. The careful and detailed presentation allows the visualization and comparison of the different disease courses in real time span. Failure and response to treatment are made visible at one glance. Database closure was 31 December 2011. All co-operators were offered co-authorship.

Results: All 71 NMO- or NMO-SD patients with AQP4-ab positivity (age range 12.3 to 79.6 years) were analysed in detail. Sex ratio (m:f = 1:7) and the proportion of patients without oligoclonal bands in cerebrospinal fluid (86.6%) were in line with previously published results. All identified patients were Caucasians.

Conclusions: A nationwide collaboration amongst Austrian neurologists with good network communications made it possible to establish a database of 71 AQP4-ab positive patients with NMO/NMO-SD. This database is presented in detail and provides the basis for further studies and international cooperation in order to investigate this rare disease.

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Related in: MedlinePlus

Demographic and clinical data, traceable for each individual patient.A, patients aged 16 to 53 years; B, patients aged 54 to 83 years. Note, the data of the two ‘AQP4-ab negative’ patients were also presented here in Figure 1 to ensure full transparency. However, both ‘AQP4-ab negative’ patients have no ID numbers. The ID numbers were reserved for the included ‘AQP4-ab positive’ patients. Both ‘AQP4-ab negative’ patients were clearly marked as ‘AQP4-ab negative’ and were also not included in further analyses (e.g. Figure 2).Dashed line, lifespan; bold line, disease duration; continuous bold vertical line, publication date of Lennon et al., Lancet Neurol 2004; continuous dashed vertical line, start of AQP4-Ab testing at the Innsbruck Medical University. White circle, unilateral optic neuritis (ON); black circle, bilateral ON; square, longitudinally transverse myelitis (LETM); triangle, myelitis less than 3 vertebral segments; white rectangle (brain lesion); black rectangle, tumor-like lesion; all ON and LETM (or myelitis) were treated with intravenous methylprednisolone (ivMP), unless otherwise indicated. A dot within a circle or square indicates that in addition to ivMP PLEX was performed; a solitary black dot indicates PLEX apart from a relapse straight cross in green, rituximab (RTX); straight cross in orange, natalizumab; straight cross in blue, intravenous cyclophosphamide (CTX) or mitoxantrone (MXT); oblique cross in red, peroral immunsuppressive therapy with azathioprine (AZA) and/or prednsiolone (PRED) and/or mycophenolate (MMF) and/or methotrexate (MTX) and/or cyclosporine A (CSA) and/or CTX; oblique cross in purple, intravenous immunoglobuline (IVIG); oblique cross in grey, interferon beta; oblique cross in yellow, glatiramer-acetate.
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pone-0079649-g001: Demographic and clinical data, traceable for each individual patient.A, patients aged 16 to 53 years; B, patients aged 54 to 83 years. Note, the data of the two ‘AQP4-ab negative’ patients were also presented here in Figure 1 to ensure full transparency. However, both ‘AQP4-ab negative’ patients have no ID numbers. The ID numbers were reserved for the included ‘AQP4-ab positive’ patients. Both ‘AQP4-ab negative’ patients were clearly marked as ‘AQP4-ab negative’ and were also not included in further analyses (e.g. Figure 2).Dashed line, lifespan; bold line, disease duration; continuous bold vertical line, publication date of Lennon et al., Lancet Neurol 2004; continuous dashed vertical line, start of AQP4-Ab testing at the Innsbruck Medical University. White circle, unilateral optic neuritis (ON); black circle, bilateral ON; square, longitudinally transverse myelitis (LETM); triangle, myelitis less than 3 vertebral segments; white rectangle (brain lesion); black rectangle, tumor-like lesion; all ON and LETM (or myelitis) were treated with intravenous methylprednisolone (ivMP), unless otherwise indicated. A dot within a circle or square indicates that in addition to ivMP PLEX was performed; a solitary black dot indicates PLEX apart from a relapse straight cross in green, rituximab (RTX); straight cross in orange, natalizumab; straight cross in blue, intravenous cyclophosphamide (CTX) or mitoxantrone (MXT); oblique cross in red, peroral immunsuppressive therapy with azathioprine (AZA) and/or prednsiolone (PRED) and/or mycophenolate (MMF) and/or methotrexate (MTX) and/or cyclosporine A (CSA) and/or CTX; oblique cross in purple, intravenous immunoglobuline (IVIG); oblique cross in grey, interferon beta; oblique cross in yellow, glatiramer-acetate.

Mentions: Thirdly, the same highly sensitive and specific cell-based immunofluorescence assay was used to detect AQP4-ab in the sera of all patients and, essential for quality insurance, the testing was performed centrally, i.e. by the same laboratory at the Clinical Department of Neurology, Innsbruck Medical University (Figure 1) [10]. The following is the result of a nationwide epidemiological study of patients with NMO/NMO-SD in Austria. With a supposed prevalence of NMO of between 0.3 and 3 people per 100,000 people in Caucasian populations [3,4,11,12], at least 25 and up to 250 NMO patients may be expected in Austria. By the end of 2011 we had identified 36 patients with NMO and 35 patients with NMO-SD; all 71 patients were seropositive for AQP4-ab. Hereby follows the individual clinical key data for each patient who met the stringent inclusion criteria. The data are presented in detail so they can be used for meta-analyses and larger epidemiological studies outside Austria.


Neuromyelitis optica in Austria in 2011: to bridge the gap between neuroepidemiological research and practice in a study population of 8.4 million people.

Aboul-Enein F, Seifert-Held T, Mader S, Kuenz B, Lutterotti A, Rauschka H, Rommer P, Leutmezer F, Vass K, Flamm-Horak A, Stepansky R, Lang W, Fertl E, Schlager T, Heller T, Eggers C, Safoschnik G, Fuchs S, Kraus J, Assar H, Guggenberger S, Reisz M, Schnabl P, Komposch M, Simschitz P, Skrobal A, Moser A, Jeschow M, Stadlbauer D, Freimüller M, Guger M, Schmidegg S, Franta C, Weiser V, Koppi S, Niederkorn-Duft M, Raber B, Schmeissner I, Jecel J, Tinchon A, Storch MK, Reindl M, Berger T, Kristoferitsch W - PLoS ONE (2013)

Demographic and clinical data, traceable for each individual patient.A, patients aged 16 to 53 years; B, patients aged 54 to 83 years. Note, the data of the two ‘AQP4-ab negative’ patients were also presented here in Figure 1 to ensure full transparency. However, both ‘AQP4-ab negative’ patients have no ID numbers. The ID numbers were reserved for the included ‘AQP4-ab positive’ patients. Both ‘AQP4-ab negative’ patients were clearly marked as ‘AQP4-ab negative’ and were also not included in further analyses (e.g. Figure 2).Dashed line, lifespan; bold line, disease duration; continuous bold vertical line, publication date of Lennon et al., Lancet Neurol 2004; continuous dashed vertical line, start of AQP4-Ab testing at the Innsbruck Medical University. White circle, unilateral optic neuritis (ON); black circle, bilateral ON; square, longitudinally transverse myelitis (LETM); triangle, myelitis less than 3 vertebral segments; white rectangle (brain lesion); black rectangle, tumor-like lesion; all ON and LETM (or myelitis) were treated with intravenous methylprednisolone (ivMP), unless otherwise indicated. A dot within a circle or square indicates that in addition to ivMP PLEX was performed; a solitary black dot indicates PLEX apart from a relapse straight cross in green, rituximab (RTX); straight cross in orange, natalizumab; straight cross in blue, intravenous cyclophosphamide (CTX) or mitoxantrone (MXT); oblique cross in red, peroral immunsuppressive therapy with azathioprine (AZA) and/or prednsiolone (PRED) and/or mycophenolate (MMF) and/or methotrexate (MTX) and/or cyclosporine A (CSA) and/or CTX; oblique cross in purple, intravenous immunoglobuline (IVIG); oblique cross in grey, interferon beta; oblique cross in yellow, glatiramer-acetate.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3818238&req=5

pone-0079649-g001: Demographic and clinical data, traceable for each individual patient.A, patients aged 16 to 53 years; B, patients aged 54 to 83 years. Note, the data of the two ‘AQP4-ab negative’ patients were also presented here in Figure 1 to ensure full transparency. However, both ‘AQP4-ab negative’ patients have no ID numbers. The ID numbers were reserved for the included ‘AQP4-ab positive’ patients. Both ‘AQP4-ab negative’ patients were clearly marked as ‘AQP4-ab negative’ and were also not included in further analyses (e.g. Figure 2).Dashed line, lifespan; bold line, disease duration; continuous bold vertical line, publication date of Lennon et al., Lancet Neurol 2004; continuous dashed vertical line, start of AQP4-Ab testing at the Innsbruck Medical University. White circle, unilateral optic neuritis (ON); black circle, bilateral ON; square, longitudinally transverse myelitis (LETM); triangle, myelitis less than 3 vertebral segments; white rectangle (brain lesion); black rectangle, tumor-like lesion; all ON and LETM (or myelitis) were treated with intravenous methylprednisolone (ivMP), unless otherwise indicated. A dot within a circle or square indicates that in addition to ivMP PLEX was performed; a solitary black dot indicates PLEX apart from a relapse straight cross in green, rituximab (RTX); straight cross in orange, natalizumab; straight cross in blue, intravenous cyclophosphamide (CTX) or mitoxantrone (MXT); oblique cross in red, peroral immunsuppressive therapy with azathioprine (AZA) and/or prednsiolone (PRED) and/or mycophenolate (MMF) and/or methotrexate (MTX) and/or cyclosporine A (CSA) and/or CTX; oblique cross in purple, intravenous immunoglobuline (IVIG); oblique cross in grey, interferon beta; oblique cross in yellow, glatiramer-acetate.
Mentions: Thirdly, the same highly sensitive and specific cell-based immunofluorescence assay was used to detect AQP4-ab in the sera of all patients and, essential for quality insurance, the testing was performed centrally, i.e. by the same laboratory at the Clinical Department of Neurology, Innsbruck Medical University (Figure 1) [10]. The following is the result of a nationwide epidemiological study of patients with NMO/NMO-SD in Austria. With a supposed prevalence of NMO of between 0.3 and 3 people per 100,000 people in Caucasian populations [3,4,11,12], at least 25 and up to 250 NMO patients may be expected in Austria. By the end of 2011 we had identified 36 patients with NMO and 35 patients with NMO-SD; all 71 patients were seropositive for AQP4-ab. Hereby follows the individual clinical key data for each patient who met the stringent inclusion criteria. The data are presented in detail so they can be used for meta-analyses and larger epidemiological studies outside Austria.

Bottom Line: Sex ratio (m:f = 1:7) and the proportion of patients without oligoclonal bands in cerebrospinal fluid (86.6%) were in line with previously published results.All identified patients were Caucasians.This database is presented in detail and provides the basis for further studies and international cooperation in order to investigate this rare disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Sozialmedizinisches Zentrum Ost Donauspital, Vienna, Austria ; Department of Neurology, Karl Landsteiner Institute for Neuroimmunological and Neurodegenerative Disorders, Vienna, Austria.

ABSTRACT

Background: In 2008 the Austrian Task Force for Neuromyelitis Optica (NMO) started a nation-wide network for information exchange and multi-centre collaboration. Their aim was to detect all patients with NMO or NMO spectrum disorders (NMO-SD) in Austria and to analyse their disease courses and response to treatment.

Methods: (1) As of March 2008, 1957 serum samples (of 1557 patients) have been tested with an established cell based immunofluorescence aquaporin-4 antibody (AQP4-ab) assay with a high sensitivity and specificity (both >95%). All tests were performed in a single reference laboratory (Clinical Dept. of Neurology of the Innsbruck Medical University). (2) A nation-wide survey with several calls for participation (via email newsletters, articles in the official journal of the Austrian Society of Neurology, and workshops) was initiated in 2008. All collected data will be presented in a way that allows that every individual patient can be traced back in order to ensure transparency and to avoid any data distortion in future meta-analyses. The careful and detailed presentation allows the visualization and comparison of the different disease courses in real time span. Failure and response to treatment are made visible at one glance. Database closure was 31 December 2011. All co-operators were offered co-authorship.

Results: All 71 NMO- or NMO-SD patients with AQP4-ab positivity (age range 12.3 to 79.6 years) were analysed in detail. Sex ratio (m:f = 1:7) and the proportion of patients without oligoclonal bands in cerebrospinal fluid (86.6%) were in line with previously published results. All identified patients were Caucasians.

Conclusions: A nationwide collaboration amongst Austrian neurologists with good network communications made it possible to establish a database of 71 AQP4-ab positive patients with NMO/NMO-SD. This database is presented in detail and provides the basis for further studies and international cooperation in order to investigate this rare disease.

Show MeSH
Related in: MedlinePlus