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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

Differential diagnosis of NMO/NMO-SD associated LETM.a, a 90 year-old woman with left-sided chest pain, a three-day progressive paraparesis and Herpes simplex lesions gluteal. Inlay, the lesion involves the central regions of the spinal cord. CSF, 160 cells/μl (lymphocytes, (activated) lymphocytes, monocytes, neutrophils, eosinophils), no intrathecal Ig synthesis and no oligoclonal bands. Sera AQP4-ab negative. Diagnosis, HZV myelitis sine herpete (PCR from the vesicle contents, positive for HSV; PCR from CSF, negative for herpes simplex virus, but positive for varicella zoster virus.) .b, a 34 year old woman with peracute tetraparesis after infection of the upper respiratory tract and pneumonia. CSF, 65 cells/μl, elevated total protein and IgG concentration, positive OCB. Serum AQP4-ab were negative. Diagnosis, postinfectious myelitis (complement binding reaction and ELISA for mycoplasma pneumoniae were both positive). In a follow-up after 6 months the AQP4-ab were again negative.c, a 27 year-old woman with dysaesthesia and right-sided hemiparesis. MRI, several confluent lesions which are located at the lateral and posterior regions of the spinal cord. CSF, 9 cells/µl, intrathecal IgG synthesis and oligoclonal bands type 2b. Serum AQP4-ab were negative. Diagnosis, relapsing-remitting multiple sclerosis (Barkhof criteria fulfilled). D, NMO associated LETM, a 19 year-old women; CSF (3times), elevated cell counts, 9/µl, 181/µl and 207/µl, elevated total protein, but no IgG synthesis and no OCB. AQP4-ab were repeatedly positive (patient 71, Figure 2).
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pone-0079649-g003: Differential diagnosis of NMO/NMO-SD associated LETM.a, a 90 year-old woman with left-sided chest pain, a three-day progressive paraparesis and Herpes simplex lesions gluteal. Inlay, the lesion involves the central regions of the spinal cord. CSF, 160 cells/μl (lymphocytes, (activated) lymphocytes, monocytes, neutrophils, eosinophils), no intrathecal Ig synthesis and no oligoclonal bands. Sera AQP4-ab negative. Diagnosis, HZV myelitis sine herpete (PCR from the vesicle contents, positive for HSV; PCR from CSF, negative for herpes simplex virus, but positive for varicella zoster virus.) .b, a 34 year old woman with peracute tetraparesis after infection of the upper respiratory tract and pneumonia. CSF, 65 cells/μl, elevated total protein and IgG concentration, positive OCB. Serum AQP4-ab were negative. Diagnosis, postinfectious myelitis (complement binding reaction and ELISA for mycoplasma pneumoniae were both positive). In a follow-up after 6 months the AQP4-ab were again negative.c, a 27 year-old woman with dysaesthesia and right-sided hemiparesis. MRI, several confluent lesions which are located at the lateral and posterior regions of the spinal cord. CSF, 9 cells/µl, intrathecal IgG synthesis and oligoclonal bands type 2b. Serum AQP4-ab were negative. Diagnosis, relapsing-remitting multiple sclerosis (Barkhof criteria fulfilled). D, NMO associated LETM, a 19 year-old women; CSF (3times), elevated cell counts, 9/µl, 181/µl and 207/µl, elevated total protein, but no IgG synthesis and no OCB. AQP4-ab were repeatedly positive (patient 71, Figure 2).

Mentions: OCB were negative in 57 out of 67 NMO/NMO-SD patients (85%) (Figure 3). For 4 patients there were no data available on OCB (NMO, n=2; NMO-SD, n=2) (Figure 1, patient numbers 12, 41, 53, 56). OCB (type 2 or 3) were positive in 10 NMO/NMO-SD (12.7%; NMO, n=8 and NMO-SD, n=2 (Figure 1, patient numbers 2, 19, 27, 29, 31, 32, 57, 60, 67, 70). In 4 (out of 67 patients) who had more than one CSF examination a switch from OCB positive to OCB negative or vice versa could be observed (Figure 1, patient numbers 15, 22, 23, 37).


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)

Differential diagnosis of NMO/NMO-SD associated LETM.a, a 90 year-old woman with left-sided chest pain, a three-day progressive paraparesis and Herpes simplex lesions gluteal. Inlay, the lesion involves the central regions of the spinal cord. CSF, 160 cells/μl (lymphocytes, (activated) lymphocytes, monocytes, neutrophils, eosinophils), no intrathecal Ig synthesis and no oligoclonal bands. Sera AQP4-ab negative. Diagnosis, HZV myelitis sine herpete (PCR from the vesicle contents, positive for HSV; PCR from CSF, negative for herpes simplex virus, but positive for varicella zoster virus.) .b, a 34 year old woman with peracute tetraparesis after infection of the upper respiratory tract and pneumonia. CSF, 65 cells/μl, elevated total protein and IgG concentration, positive OCB. Serum AQP4-ab were negative. Diagnosis, postinfectious myelitis (complement binding reaction and ELISA for mycoplasma pneumoniae were both positive). In a follow-up after 6 months the AQP4-ab were again negative.c, a 27 year-old woman with dysaesthesia and right-sided hemiparesis. MRI, several confluent lesions which are located at the lateral and posterior regions of the spinal cord. CSF, 9 cells/µl, intrathecal IgG synthesis and oligoclonal bands type 2b. Serum AQP4-ab were negative. Diagnosis, relapsing-remitting multiple sclerosis (Barkhof criteria fulfilled). D, NMO associated LETM, a 19 year-old women; CSF (3times), elevated cell counts, 9/µl, 181/µl and 207/µl, elevated total protein, but no IgG synthesis and no OCB. AQP4-ab were repeatedly positive (patient 71, Figure 2).
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Related In: Results  -  Collection

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getmorefigures.php?uid=PMC3818238&req=5

pone-0079649-g003: Differential diagnosis of NMO/NMO-SD associated LETM.a, a 90 year-old woman with left-sided chest pain, a three-day progressive paraparesis and Herpes simplex lesions gluteal. Inlay, the lesion involves the central regions of the spinal cord. CSF, 160 cells/μl (lymphocytes, (activated) lymphocytes, monocytes, neutrophils, eosinophils), no intrathecal Ig synthesis and no oligoclonal bands. Sera AQP4-ab negative. Diagnosis, HZV myelitis sine herpete (PCR from the vesicle contents, positive for HSV; PCR from CSF, negative for herpes simplex virus, but positive for varicella zoster virus.) .b, a 34 year old woman with peracute tetraparesis after infection of the upper respiratory tract and pneumonia. CSF, 65 cells/μl, elevated total protein and IgG concentration, positive OCB. Serum AQP4-ab were negative. Diagnosis, postinfectious myelitis (complement binding reaction and ELISA for mycoplasma pneumoniae were both positive). In a follow-up after 6 months the AQP4-ab were again negative.c, a 27 year-old woman with dysaesthesia and right-sided hemiparesis. MRI, several confluent lesions which are located at the lateral and posterior regions of the spinal cord. CSF, 9 cells/µl, intrathecal IgG synthesis and oligoclonal bands type 2b. Serum AQP4-ab were negative. Diagnosis, relapsing-remitting multiple sclerosis (Barkhof criteria fulfilled). D, NMO associated LETM, a 19 year-old women; CSF (3times), elevated cell counts, 9/µl, 181/µl and 207/µl, elevated total protein, but no IgG synthesis and no OCB. AQP4-ab were repeatedly positive (patient 71, Figure 2).
Mentions: OCB were negative in 57 out of 67 NMO/NMO-SD patients (85%) (Figure 3). For 4 patients there were no data available on OCB (NMO, n=2; NMO-SD, n=2) (Figure 1, patient numbers 12, 41, 53, 56). OCB (type 2 or 3) were positive in 10 NMO/NMO-SD (12.7%; NMO, n=8 and NMO-SD, n=2 (Figure 1, patient numbers 2, 19, 27, 29, 31, 32, 57, 60, 67, 70). In 4 (out of 67 patients) who had more than one CSF examination a switch from OCB positive to OCB negative or vice versa could be observed (Figure 1, patient numbers 15, 22, 23, 37).

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