40 results on '"Fehmi, Janev"'
Search Results
2. Overlapping central and peripheral nervous system syndromes in MOG antibody–associated disorders
- Author
-
Rinaldi, Simon, Davies, Alexander, Fehmi, Janev, Beadnall, Heidi N., Wang, Justine, Hardy, Todd A., Barnett, Michael H., Broadley, Simon A., Waters, Patrick, Reddel, Stephen W., Irani, Sarosh R., Brilot, Fabienne, Dale, Russell C., and Ramanathan, Sudarshini
- Published
- 2021
- Full Text
- View/download PDF
3. Autoimmune Nodopathies
- Author
-
Fehmi, Janev, primary and Rinaldi, Simon, additional
- Published
- 2019
- Full Text
- View/download PDF
4. Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study
- Author
-
Keddie, Stephen, Foldes, David, Caimari, Francisca, Baldeweg, Stephanie E., Bomsztyk, Joshua, Ziff, Oliver J., Fehmi, Janev, Cerner, Aviva, Jaunmuktane, Zane, Brandner, Sebastian, Yong, Kwee, Manji, Hadi, Carr, Aisling, Rinaldi, Simon, Reilly, Mary M., DʼSa, Shirley, and Lunn, Michael P.
- Published
- 2020
- Full Text
- View/download PDF
5. Contactin-1 links autoimmune neuropathy and membranous glomerulonephritis
- Author
-
Fehmi, Janev, primary, Davies, Alexander J., additional, Antonelou, Marilina, additional, Keddie, Stephen, additional, Pikkupeura, Sonja, additional, Querol, Luis, additional, Delmont, Emilien, additional, Cortese, Andrea, additional, Franciotta, Diego, additional, Persson, Staffan, additional, Barratt, Jonathan, additional, Pepper, Ruth, additional, Farinha, Filipa, additional, Rahman, Anisur, additional, Canetti, Diana, additional, Gilbertson, Janet A., additional, Rendell, Nigel B., additional, Radunovic, Aleksandar, additional, Minton, Thomas, additional, Fuller, Geraint, additional, Murphy, Sinead M., additional, Carr, Aisling S., additional, Reilly, Mary R., additional, Eftimov, Filip, additional, Wieske, Luuk, additional, Teunissen, Charlotte E., additional, Roberts, Ian S. D., additional, Ashman, Neil, additional, Salama, Alan D., additional, and Rinaldi, Simon, additional
- Published
- 2023
- Full Text
- View/download PDF
6. Antineurofascin IgG2‐associated paediatric autoimmune nodopathy.
- Author
-
Harris, Rachel E., Atherton, Mark, Naude, Johann Te Water, Bird‐Lieberman, Georgina A., Ramdas, Sithara, Fehmi, Janev, Rinaldi, Simon, and Ong, Min T.
- Subjects
DECISION making in children ,CHILD patients ,PEDIATRICS ,INTRAVENOUS immunoglobulins ,IMMUNOGLOBULINS - Abstract
In this case series of four paediatric patients, we present the first described cases of immunotherapy‐responsive autoimmune nodopathy with IgG2 antineurofascin antibodies. In three cases, the antineurofascin antibodies were predominantly of the IgG2 subclass, a novel finding in comparison to previously described adult cases where IgG4 and/or IgG1/3 have typically been described. One patient had low signal for IgG2 with predominant IgG1 and IgG4 antibodies, a pattern commonly seen in adult patients. Two patients had antibodies targeting all three neurofascin isoforms (155, 186, and 140), whereas antibodies in the sera from the third targeted only the nodal isoforms 186 and 140, and the fourth patient only neurofascin 155. The three patients with IgG2 predominant antibodies appear to be responsive to intravenous immunoglobulin (IVIG) to varying degrees thus far, whereas the patient with IgG1/4 antibodies had poor response to IVIG but good response to steroids. Although the full clinical significance of IgG2 predominant antineurofascin antibodies in the context of childhood polyneuropathy remains unclear, emerging evidence of serological‐phenotypic correlation may inform prognostication and therapeutic decision‐making, warranting further study into this area. What this paper adds: Paediatric immunotherapy‐responsive nodopathies were associated with antineurofascin antibodies predominantly of the IgG2 subclass in 3 out of 4 patients.Identification of antibodies and understanding their phenotypic relevance could predict response to treatment and guide therapeutic decision‐making in children. [ABSTRACT FROM AUTHOR]
- Published
- 2023
- Full Text
- View/download PDF
7. Treatment of CIDP
- Author
-
Fehmi, Janev, primary, Bellanti, Roberto, additional, Misbah, Siraj A, additional, Bhattacharjee, Anupam, additional, and Rinaldi, Simon, additional
- Published
- 2022
- Full Text
- View/download PDF
8. Nodes, paranodes and neuropathies
- Author
-
Fehmi, Janev, Scherer, Steven S, Willison, Hugh J, and Rinaldi, Simon
- Published
- 2018
- Full Text
- View/download PDF
9. PO225 Innovation in neurology education in cambodia
- Author
-
Fehmi, Janev, Aron, Julien, and Sahai-Srivastava, Soma
- Published
- 2017
- Full Text
- View/download PDF
10. PO224 How effective is simulation-based teaching in cambodia?
- Author
-
Fehmi, Janev, Aron, Julien, Lim, Sengkhoun, Khoem, Namgech, Yi, Vannary, Ek, Vattanak, Ponndara, Ith, Bunsocheat, Mam, Aklinn, Nhem, and Sahai-Srivastava, Soma
- Published
- 2017
- Full Text
- View/download PDF
11. IgG1 pan-neurofascin antibodies define a severe and frequently fatal GBS-like neuropathy which responds to rituximab
- Author
-
Fehmi, Janev, primary, Jon Walters, R, additional, Lavin, Tim, additional, Keh, Ryan, additional, Rossor, Alexander M, additional, Munteanu, Tudor, additional, Delanty, Norman, additional, Roberts, Rhys, additional, Baumer, Dirk, additional, and Rinaldi, Simon, additional
- Published
- 2022
- Full Text
- View/download PDF
12. An International Perspective on Preceding Infections in Guillain-Barre Syndrome The IGOS-1000 Cohort
- Author
-
Leonhard, Sonja E., van der Eijk, Annemiek A., Andersen, Henning, Antonini, Giovanni, Arends, Samuel, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen J., Batstra, Manou R., Benedetti, Luana, van den Berg, Bianca, Van den Bergh, Peter, Burmann, Jan, Busby, Mark, Casasnovas, Carlos, Cornblath, David R., Davidson, Amy, Doets, Alex Y., van Doorn, Pieter A., de la Cour, Charlotte Dornonville, Feasby, Thomas E., Fehmi, Janev, Garcia-Sobrino, Tania, Goldstein, Jonathan M., Gorson, Kenneth C., Granit, Volkan, Hadden, Robert D. M., Harbo, Thomas, Hartung, Hans-Peter, Hasan, Imran, Holbech, Jakob, V, Holt, James K. L., Jahan, Israt, Islam, Zhahirul, Karafiath, Summer, Katzberg, Hans D., Kleyweg, Ruud P., Kolb, Noah, Kuitwaard, Krista, Kuwahara, Motoi, Kusunoki, Susumu, Luijten, Linda W. G., Kuwabara, Satoshi, Pan, Edward Lee, Lehmann, Helmar C., Maas, Marijke, Martin-Aguilar, Lorena, Al Miller, James, Mohammad, Quazi Deen, Monges, Soledad, Nedkova-Hristova, Velina, Nobile-Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Querol, Luis, Reisin, Ricardo, Van Rijs, Wouter, Rinaldi, Simon, Roberts, Rhys C., Roodbol, Joyce, Shahrizaila, Nortina, Sindrup, Soren Hein, Stein, Beth, Cheng-Yin, Tan, Tankisi, Hatice, Tio-Gillen, Anne P., Tous, Maria J. Sedano, Verboon, Christine, Vermeij, Frederique H., Visser, Leo H., Huizinga, Ruth, Willison, Hugh J., Jacobs, Bart C., Leonhard, Sonja E., van der Eijk, Annemiek A., Andersen, Henning, Antonini, Giovanni, Arends, Samuel, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen J., Batstra, Manou R., Benedetti, Luana, van den Berg, Bianca, Van den Bergh, Peter, Burmann, Jan, Busby, Mark, Casasnovas, Carlos, Cornblath, David R., Davidson, Amy, Doets, Alex Y., van Doorn, Pieter A., de la Cour, Charlotte Dornonville, Feasby, Thomas E., Fehmi, Janev, Garcia-Sobrino, Tania, Goldstein, Jonathan M., Gorson, Kenneth C., Granit, Volkan, Hadden, Robert D. M., Harbo, Thomas, Hartung, Hans-Peter, Hasan, Imran, Holbech, Jakob, V, Holt, James K. L., Jahan, Israt, Islam, Zhahirul, Karafiath, Summer, Katzberg, Hans D., Kleyweg, Ruud P., Kolb, Noah, Kuitwaard, Krista, Kuwahara, Motoi, Kusunoki, Susumu, Luijten, Linda W. G., Kuwabara, Satoshi, Pan, Edward Lee, Lehmann, Helmar C., Maas, Marijke, Martin-Aguilar, Lorena, Al Miller, James, Mohammad, Quazi Deen, Monges, Soledad, Nedkova-Hristova, Velina, Nobile-Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Querol, Luis, Reisin, Ricardo, Van Rijs, Wouter, Rinaldi, Simon, Roberts, Rhys C., Roodbol, Joyce, Shahrizaila, Nortina, Sindrup, Soren Hein, Stein, Beth, Cheng-Yin, Tan, Tankisi, Hatice, Tio-Gillen, Anne P., Tous, Maria J. Sedano, Verboon, Christine, Vermeij, Frederique H., Visser, Leo H., Huizinga, Ruth, Willison, Hugh J., and Jacobs, Bart C.
- Abstract
Background and Objectives Infections play a key role in the development of Guillain-Barre syndrome (GBS) and have been associated with specific clinical features and disease severity. The clinical variation of GBS across geographical regions has been suggested to be related to differences in the distribution of preceding infections, but this has not been studied on a large scale. Methods We analyzed the first 1,000 patients included in the International GBS Outcome Study with available biosamples (n = 768) for the presence of a recent infection with Campylobacter jejuni, hepatitis E virus, Mycoplasma pneumoniae, cytomegalovirus, and Epstein-Barr virus. Results Serologic evidence of a recent infection with C. jejuni was found in 228 (30%), M. pneumoniae in 77 (10%), hepatitis E virus in 23 (3%), cytomegalovirus in 30 (4%), and Epstein-Barr virus in 7 (1%) patients. Evidence of more than 1 recent infection was found in 49 (6%) of these patients. Symptoms of antecedent infections were reported in 556 patients (72%), and this proportion did not significantly differ between those testing positive or negative for a recent infection. The proportions of infections were similar across continents. The sensorimotor variant and the demyelinating electrophysiologic subtype were most frequent across all infection groups, although proportions were significantly higher in patients with a cytomegalovirus and significantly lower in those with a C. jejuni infection. C. jejuni-positive patients were more severely affected, indicated by a lower Medical Research Council sum score at nadir (p = 0.004) and a longer time to regain the ability to walk independently (p = 0.005). The pure motor variant and axonal electrophysiologic subtype were more frequent in Asian compared with American or European C. jejuni-positive patients (p < 0.001, resp. p = 0.001). Time to nadir was longer in the cytomegalovirus-positive patients (p = 0.004). Discussion Across geographical regions, the distribution o
- Published
- 2022
13. Treatment of CIDP.
- Author
-
Fehmi, Janev, Bellanti, Roberto, Misbah, Siraj A., Bhattacharjee, Anupam, and Rinaldi, Simon
- Subjects
- *
IMMUNOGLOBULINS , *ADRENOCORTICAL hormones , *PLASMA exchange (Therapeutics) , *NEURALGIA , *GUILLAIN-Barre syndrome - Abstract
Chronic inflammatory demyelinating polyneuropathy is a disabling but treatable disorder. However, misdiagnosis is common, and it can be difficult to optimise its treatment. Various agents are used both for first and second line. First-line options are intravenous immunoglobulin, corticosteroids and plasma exchange. Second-line therapies may be introduced as steroid-sparing agents or as more potent escalation therapy. It is also important to consider symptomatic treatment of neuropathic pain and non-pharmacological interventions. We discuss the evidence for the various treatments and explain the practicalities of the different approaches. We also outline strategies for monitoring response and assessing the ongoing need for therapy. [ABSTRACT FROM AUTHOR]
- Published
- 2023
- Full Text
- View/download PDF
14. Tumefactive demyelination presenting during bevacizumab treatment
- Author
-
Rice, Claire M, Rossiter, David, Fehmi, Janev, Stevens, James C, Renowden, Shelley A, Cohen, Nicki, Bailey, Clare, and Scolding, Neil J
- Published
- 2015
- Full Text
- View/download PDF
15. Leucine‐Rich Glioma‐Inactivated 1 versus Contactin‐Associated Protein‐like 2 Antibody Neuropathic Pain: Clinical and Biological Comparisons
- Author
-
Ramanathan, Sudarshini, primary, Tseng, Mandy, additional, Davies, Alexander J., additional, Uy, Christopher E., additional, Paneva, Sofija, additional, Mgbachi, Victor C., additional, Michael, Sophia, additional, Varley, James A., additional, Binks, Sophie, additional, Themistocleous, Andreas C., additional, Fehmi, Janev, additional, Anziska, Yaacov, additional, Soni, Anushka, additional, Hofer, Monika, additional, Waters, Patrick, additional, Brilot, Fabienne, additional, Dale, Russell C., additional, Dawes, John, additional, Rinaldi, Simon, additional, Bennett, David L., additional, and Irani, Sarosh R., additional
- Published
- 2021
- Full Text
- View/download PDF
16. IgG1 pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality
- Author
-
Fehmi, Janev, primary, Davies, Alexander J, additional, Walters, Jon, additional, Lavin, Timothy, additional, Keh, Ryan, additional, Rossor, Alexander M, additional, Munteanu, Tudor, additional, Delanty, Norman, additional, Roberts, Rhys, additional, Bäumer, Dirk, additional, Lennox, Graham, additional, and Rinaldi, Simon, additional
- Published
- 2021
- Full Text
- View/download PDF
17. Nodal and paranodal antibody-associated neuropathies
- Author
-
Fehmi, Janev, primary, Vale, Tom, additional, Keddie, Stephen, additional, and Rinaldi, Simon, additional
- Published
- 2021
- Full Text
- View/download PDF
18. FC 030CONTACTIN-1 IS A NOVEL ANTIGEN IN IDIOPATHIC MEMBRANOUS GLOMERULONEPHRITIS AND IN CIDP- ASSOCIATED GLOMERULONEPHRITIS
- Author
-
Salama, Alan, primary, Fehmi, Janev, additional, Antonelou, Marilina, additional, Barratt, Jonathan, additional, Ashman, Neil, additional, Carr, Aisling, additional, Reilly, Mary, additional, Keddie, Stephen, additional, Querol, Luis, additional, Pikkuoeura, Sonja, additional, Cortese, Andrea, additional, Delmont, Emilien, additional, Persson, Staffan, additional, Radunovic, Aleksandar, additional, Roberts, Ian Simon David, additional, Davies, Alexander, additional, and Rinaldi, Simon, additional
- Published
- 2021
- Full Text
- View/download PDF
19. Serum Contactin-1 in CIDP
- Author
-
Wieske, Luuk, Martín-Aguilar, Lorena, Fehmi, Janev, Lleixà, Cinta, Koel-Simmelink, Marleen J. A., Chatterjee, Madhurima, van Lierop, Zoë, Killestein, Joep, Verhamme, Camiel, Querol, Luis, Rinaldi, Simon, Teunissen, Charlotte E., Eftimov, Filip, and Universitat Autònoma de Barcelona
- Abstract
To investigate whether serum levels of contactin-1, a paranodal protein, correlate with paranodal injury as seen in patients with CIDP with antibodies targeting the paranodal region. Serum contactin-1 levels were measured in 187 patients with CIDP and 222 healthy controls. Paranodal antibodies were investigated in all patients. Serum contactin-1 levels were lower in patients (N = 41) with paranodal antibodies compared with patients (N = 146) without paranodal antibodies (p < 0.01) and showed good discrimination between these groups (area under the curve 0.84; 95% CI: 0.76-0.93). These findings suggest that serum contactin-1 levels have the potential to serve as a possible diagnostic biomarker of paranodal injury in CIDP. This study provides class II evidence that serum contactin-1 levels can discriminate between patients with CIDP with or without paranodal antibodies with a sensitivity of 71% (95% CI: 56%-85%) and a specificity of 97% (95% CI: 83%-100%).
- Published
- 2021
20. Antibodies to the Caspr1/contactin-1 complex in chronic inflammatory demyelinating polyradiculoneuropathy
- Author
-
Pascual-Goñi, Elba, primary, Fehmi, Janev, additional, Lleixà, Cinta, additional, Martín-Aguilar, Lorena, additional, Devaux, Jérôme, additional, Höftberger, Romana, additional, Delmont, Emilien, additional, Doppler, Kathrin, additional, Sommer, Claudia, additional, Radunovic, Aleksandar, additional, Carvajal, Alejandra, additional, Smyth, Shane, additional, Williams, Laura, additional, Mazanec, Radim, additional, Potočková, Veronika, additional, Hinds, Nigel, additional, Cassereau, Julien, additional, Viala, Karine, additional, Lefilliatre, Mathilde, additional, Nicolas, Guillaume, additional, Foley, Peter, additional, Leypoldt, Frank, additional, Keddie, Stephen, additional, Lunn, Michael P, additional, Zimprich, Fritz, additional, Nunkoo, Vharoon Sharma, additional, Löscher, Wolfgang N, additional, Martínez-Martínez, Laura, additional, Díaz-Manera, Jordi, additional, Rojas-Garcia, Ricard, additional, Illa, Isabel, additional, Rinaldi, Simon, additional, and Querol, Luis, additional
- Published
- 2021
- Full Text
- View/download PDF
21. Epidemiological and cohort study finds no association between COVID-19 and Guillain-Barré syndrome
- Author
-
Keddie, Stephen, primary, Pakpoor, Julia, additional, Mousele, Christina, additional, Pipis, Menelaos, additional, Machado, Pedro M, additional, Foster, Mark, additional, Record, Christopher J, additional, Keh, Ryan Y S, additional, Fehmi, Janev, additional, Paterson, Ross W, additional, Bharambe, Viraj, additional, Clayton, Lisa M, additional, Allen, Claire, additional, Price, Olivia, additional, Wall, Jasmine, additional, Kiss-Csenki, Annamaria, additional, Rathnasabapathi, Devi Priya, additional, Geraldes, Ruth, additional, Yermakova, Tatyana, additional, King-Robson, Joshua, additional, Zosmer, Maya, additional, Rajakulendran, Sanjeev, additional, Sumaria, Sheetal, additional, Farmer, Simon F, additional, Nortley, Ross, additional, Marshall, Charles R, additional, Newman, Edward J, additional, Nirmalananthan, Niranjanan, additional, Kumar, Guru, additional, Pinto, Ashwin A, additional, Holt, James, additional, Lavin, Tim M, additional, Brennan, Kathryn M, additional, Zandi, Michael S, additional, Jayaseelan, Dipa L, additional, Pritchard, Jane, additional, Hadden, Robert D M, additional, Manji, Hadi, additional, Willison, Hugh J, additional, Rinaldi, Simon, additional, Carr, Aisling S, additional, and Lunn, Michael P, additional
- Published
- 2020
- Full Text
- View/download PDF
22. An International Perspective on Preceding Infections in Guillain-Barré Syndrome
- Author
-
Leonhard, Sonja E., van der Eijk, Annemiek A., Andersen, Henning, Antonini, Giovanni, Arends, Samuel, Attarian, Shahram, Barroso, Fabio A., Bateman, Kathleen J., Batstra, Manou R., Benedetti, Luana, van den Berg, Bianca, Van den Bergh, Peter, Bürmann, Jan, Busby, Mark, Casasnovas, Carlos, Cornblath, David R., Davidson, Amy, Doets, Alex Y., van Doorn, Pieter A., Dornonville de la Cour, Charlotte, Feasby, Thomas E., Fehmi, Janev, Garcia-Sobrino, Tania, Goldstein, Jonathan M., Gorson, Kenneth C., Granit, Volkan, Hadden, Robert D.M., Harbo, Thomas, Hartung, Hans-Peter, Hasan, Imran, Holbech, Jakob V., Holt, James K.L., Jahan, Israt, Islam, Zhahirul, Karafiath, Summer, Katzberg, Hans D., Kleyweg, Ruud P., Kolb, Noah, Kuitwaard, Krista, Kuwahara, Motoi, Kusunoki, Susumu, Luijten, Linda W.G., Kuwabara, Satoshi, Lee Pan, Edward, Lehmann, Helmar C., Maas, Marijke, Martín-Aguilar, Lorena, Miller, James A.L., Mohammad, Quazi Deen, Monges, Soledad, Nedkova-Hristova, Velina, Nobile-Orazio, Eduardo, Pardo, Julio, Pereon, Yann, Querol, Luis, Reisin, Ricardo, Van Rijs, Wouter, Rinaldi, Simon, Roberts, Rhys C., Roodbol, Joyce, Shahrizaila, Nortina, Sindrup, Søren Hein, Stein, Beth, Cheng-Yin, Tan, Tankisi, Hatice, Tio-Gillen, Anne P., Sedano Tous, María J., Verboon, Christine, Vermeij, Frederique H., Visser, Leo H., Huizinga, Ruth, Willison, Hugh J., and Jacobs, Bart C.
- Published
- 2022
- Full Text
- View/download PDF
23. Immunoadsorption and Plasma Exchange in Seropositive and Seronegative Immune-Mediated Neuropathies
- Author
-
Davies, Alexander J., primary, Fehmi, Janev, additional, Senel, Makbule, additional, Tumani, Hayrettin, additional, Dorst, Johannes, additional, and Rinaldi, Simon, additional
- Published
- 2020
- Full Text
- View/download PDF
24. Motor neuropathy with conduction block due to pan‐neurofascin antibodies in a patient with chronic lymphocytic leukemia
- Author
-
Li, Vivien, primary, Schon, Frederick, additional, Fehmi, Janev, additional, Modarres, Hamid, additional, Rinaldi, Simon, additional, and Rossor, Alexander M., additional
- Published
- 2020
- Full Text
- View/download PDF
25. Contactin-1 Antibodies Link Autoimmune Neuropathies to Nephrotic Syndrome
- Author
-
Fehmi, Janev, primary, Davies, A J, additional, Antonelou, Marilina, additional, Teunissen, Charlotte E., additional, Keddie, Stephen, additional, Pikkupeura, Sonja, additional, Cortese, Andrea, additional, Querol, Luis, additional, Delmont, Emilien, additional, Franciotta, Diego, additional, Persson, Staffan, additional, Barratt, Jonathan, additional, Radunovic, Aleksandar, additional, Minton, Thomas, additional, Fuller, Geraint, additional, Murphy, Sinead M., additional, Carr, Aisling S., additional, Reilly, M M, additional, Wieske, Luuk, additional, Eftimov, Filip, additional, Roberts, Ian SD, additional, Ashman, Neil, additional, Salama, Alan D., additional, and Rinaldi, Simon, additional
- Published
- 2020
- Full Text
- View/download PDF
26. IgG1 pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality.
- Author
-
Fehmi, Janev, Davies, Alexander J., Walters, Jon, Lavin, Timothy, Keh, Ryan, Rossor, Alexander M., Munteanu, Tudor, Delanty, Norman, Roberts, Rhys, Bäumer, Dirk, Lennox, Graham, and Rinaldi, Simon
- Subjects
RITUXIMAB ,CELL adhesion molecules ,INTRAVENOUS immunoglobulins ,IMMUNOGLOBULINS ,NEUROPATHY ,SENSORY neurons ,CRANIAL nerves ,DYSAUTONOMIA ,THERAPEUTIC use of immunoglobulins ,AUTOANTIBODIES ,RESEARCH ,PERIPHERAL neuropathy ,ADRENOCORTICAL hormones ,RESEARCH methodology ,MEDICAL cooperation ,EVALUATION research ,COMPARATIVE studies ,RESEARCH funding - Abstract
Objectives: We aimed to define the clinical and serological characteristics of pan-neurofascin antibody-positive patients.Methods: We tested serum from patients with suspected immune-mediated neuropathies for antibodies directed against nodal/paranodal protein antigens using a live cell-based assay and solid-phase platform. The clinical and serological characteristics of antibody-positive and seronegative patients were then compared. Sera positive for pan-neurofascin were also tested against live myelinated human stem cell-derived sensory neurons for antibody binding.Results: Eight patients with IgG1-subclass antibodies directed against both isoforms of the nodal/paranodal cell adhesion molecule neurofascin were identified. All developed rapidly progressive tetraplegia. Cranial nerve deficits (100% vs 26%), autonomic dysfunction (75% vs 13%) and respiratory involvement (88% vs 14%) were more common than in seronegative patients. Four patients died despite treatment with one or more modalities of standard immunotherapy (intravenous immunoglobulin, steroids and/or plasmapheresis), whereas the four patients who later went on to receive the B cell-depleting therapy rituximab then began to show progressive functional improvements within weeks, became seronegative and ultimately became functionally independent.Conclusions: IgG1 pan-neurofascin antibodies define a very severe autoimmune neuropathy. We urgently recommend trials of targeted immunotherapy for this serologically classified patient group. [ABSTRACT FROM AUTHOR]- Published
- 2021
- Full Text
- View/download PDF
27. Untangling a case of painful neuropathy
- Author
-
Keddie, Stephen, primary, Fehmi, Janev, additional, Jaunmuktane, Zane, additional, D'Sa, Shirley, additional, Stevens, James C, additional, and Lunn, Michael PT, additional
- Published
- 2019
- Full Text
- View/download PDF
28. A rare case of Miller Fisher variant of Guillain-Barré Syndrome (GBS) induced by a checkpoint inhibitor
- Author
-
McNeill, Caitlin Jane, primary, Fehmi, Janev, additional, Gladwin, James, additional, and Price, Christopher, additional
- Published
- 2019
- Full Text
- View/download PDF
29. Epidemiological and cohort study finds no association between COVID-19 and Guillain-Barré syndrome.
- Author
-
Keddie, Stephen, Pakpoor, Julia, Mousele, Christina, Pipis, Menelaos, Machado, Pedro M, Foster, Mark, Record, Christopher J, Keh, Ryan Y S, Fehmi, Janev, Paterson, Ross W, Bharambe, Viraj, Clayton, Lisa M, Allen, Claire, Price, Olivia, Wall, Jasmine, Kiss-Csenki, Annamaria, Rathnasabapathi, Devi Priya, Geraldes, Ruth, Yermakova, Tatyana, and King-Robson, Joshua
- Subjects
COVID-19 ,COVID-19 pandemic ,GUILLAIN-Barre syndrome ,COHORT analysis ,SARS-CoV-2 - Abstract
Reports of Guillain-Barré syndrome (GBS) have emerged during the Coronavirus disease 2019 (COVID-19) pandemic. This epidemiological and cohort study sought to investigate any causative association between COVID-19 infection and GBS. The epidemiology of GBS cases reported to the UK National Immunoglobulin Database was studied from 2016 to 2019 and compared to cases reported during the COVID-19 pandemic. Data were stratified by hospital trust and region, with numbers of reported cases per month. UK population data for COVID-19 infection were collated from UK public health bodies. In parallel, but separately, members of the British Peripheral Nerve Society prospectively reported incident cases of GBS during the pandemic at their hospitals to a central register. The clinical features, investigation findings and outcomes of COVID-19 (definite or probable) and non-COVID-19 associated GBS cases in this cohort were compared. The incidence of GBS treated in UK hospitals from 2016 to 2019 was 1.65-1.88 per 100 000 individuals per year. GBS incidence fell between March and May 2020 compared to the same months of 2016-19. GBS and COVID-19 incidences during the pandemic also varied between regions and did not correlate with one another (r = 0.06, 95% confidence interval: -0.56 to 0.63, P = 0.86). In the independent cohort study, 47 GBS cases were reported (COVID-19 status: 13 definite, 12 probable, 22 non-COVID-19). There were no significant differences in the pattern of weakness, time to nadir, neurophysiology, CSF findings or outcome between these groups. Intubation was more frequent in the COVID-19 affected cohort (7/13, 54% versus 5/22, 23% in COVID-19-negative) attributed to COVID-19 pulmonary involvement. Although it is not possible to entirely rule out the possibility of a link, this study finds no epidemiological or phenotypic clues of SARS-CoV-2 being causative of GBS. GBS incidence has fallen during the pandemic, which may be the influence of lockdown measures reducing transmission of GBS inducing pathogens such as Campylobacter jejuni and respiratory viruses. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
30. Untangling a case of painful neuropathy.
- Author
-
Keddie, Stephen, Fehmi, Janev, Jaunmuktane, Zane, D'Sa, Shirley, Stevens, James C., and Lunn, Michael P. T.
- Subjects
- *
BLOOD testing , *DIFFERENTIAL diagnosis , *ELECTRON microscopy , *HEMOSTASIS , *MONOCLONAL gammopathies , *POLYNEUROPATHIES , *VASCULAR endothelial growth factors , *POEMS syndrome ,VASCULAR disease diagnosis ,PERIPHERAL neuropathy diagnosis - Published
- 2020
- Full Text
- View/download PDF
31. IgG1pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality
- Author
-
Fehmi, Janev, Davies, Alexander J, Walters, Jon, Lavin, Timothy, Keh, Ryan, Rossor, Alexander M, Munteanu, Tudor, Delanty, Norman, Roberts, Rhys, Ba¨umer, Dirk, Lennox, Graham, and Rinaldi, Simon
- Abstract
ObjectivesWe aimed to define the clinical and serological characteristics of pan-neurofascin antibody-positive patients.MethodsWe tested serum from patients with suspected immune-mediated neuropathies for antibodies directed against nodal/paranodal protein antigens using a live cell-based assay and solid-phase platform. The clinical and serological characteristics of antibody-positive and seronegative patients were then compared. Sera positive for pan-neurofascin were also tested against live myelinated human stem cell-derived sensory neurons for antibody binding.ResultsEight patients with IgG1-subclass antibodies directed against both isoforms of the nodal/paranodal cell adhesion molecule neurofascin were identified. All developed rapidly progressive tetraplegia. Cranial nerve deficits (100% vs 26%), autonomic dysfunction (75% vs 13%) and respiratory involvement (88% vs 14%) were more common than in seronegative patients. Four patients died despite treatment with one or more modalities of standard immunotherapy (intravenous immunoglobulin, steroids and/or plasmapheresis), whereas the four patients who later went on to receive the B cell-depleting therapy rituximab then began to show progressive functional improvements within weeks, became seronegative and ultimately became functionally independent.ConclusionsIgG1pan-neurofascin antibodies define a very severe autoimmune neuropathy. We urgently recommend trials of targeted immunotherapy for this serologically classified patient group.
- Published
- 2021
- Full Text
- View/download PDF
32. Nodes, paranodes and neuropathies
- Author
-
Fehmi, Janev, primary, Scherer, Steven S, additional, Willison, Hugh J, additional, and Rinaldi, Simon, additional
- Published
- 2017
- Full Text
- View/download PDF
33. FEMORAL NEUROPATHY FROM ILIACUS HAEMATOMA AS FIRST PRESENTATION OF HAEMOPHILIA
- Author
-
Fehmi, Janev, primary and Morgan, Catherine, additional
- Published
- 2016
- Full Text
- View/download PDF
34. Out of hours intravenous fluid therapy: a prompt to guide prescribing
- Author
-
Fehmi, Janev, primary, Carpenter, Alexander, additional, Townsend, Matthew, additional, Sheppeard, Rhian, additional, Gannon, Elizabeth, additional, Hewitson, Lynsey, additional, Auerbach, Natalie, additional, and Thomas, Katherine, additional
- Published
- 2015
- Full Text
- View/download PDF
35. Long-term survival of neonatal mitochondrial complex III deficiency associated with a novel BCS1L gene mutation
- Author
-
Tuppen, Helen A.L., primary, Fehmi, Janev, additional, Czermin, Birgit, additional, Goffrini, Paola, additional, Meloni, Francesca, additional, Ferrero, Iliana, additional, He, Langping, additional, Blakely, Emma L., additional, McFarland, Robert, additional, Horvath, Rita, additional, Turnbull, Douglass M., additional, and Taylor, Robert W., additional
- Published
- 2010
- Full Text
- View/download PDF
36. 09.24 Phone a friend?
- Author
-
Minton, Thomas, Fuller, Geraint, Rinaldi, Simon, Fehmi, Janev, Bosnell, Rose, and Barton, Eleanor
- Abstract
A 72-year-old man with type 2 diabetes mellitus was admitted under nephrology with malaise. He was diagnosed with acute kidney injury secondary to urinary retention and hydronephrosis. Over 10 days during this admission he developed progressive symmetrical limb paraesthesia and weakness with areflexia.Neurophysiological studies demonstrated a severe demyelinating polyneuropathy with widespread motor slowing and prolonged distal motor latencies. CSF was acellular with a protein of 5.9 g/L. He was treated with IV immunoglobulins and stabilised for 2 weeks. He then deteriorated over the next four weeks despite IV methylprednisolone, oral corticosteroids and 5 cycles of plasma exchange. He had profound limb weakness (0/5) and required ventilation. He had hypoalbuminaemia, with 1.5 g proteinuria consistent with a nephrotic syndrome.We phoned a friend.IgG4 anti–contactin-1 (CNTN1) antibodies were identified in serum with a CNTN1 at 1:1600.He received 4 cycles of Rituximab. After 6 weeks he was off ventilation with grade 4 power in both arms.Recently autoantibodies against node of Ranvier proteins have emerged in a subset of patients with CIDP. These patients have a homogenous clinical phenotype with an aggressive polyneuropathy, proteinuria and poor response to conventional therapy, but seem to respond to other agents, including Rituximab and Cyclophosphamide.
- Published
- 2019
- Full Text
- View/download PDF
37. IgG 1 pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality.
- Author
-
Fehmi J, Davies AJ, Walters J, Lavin T, Keh R, Rossor AM, Munteanu T, Delanty N, Roberts R, Bäumer D, Lennox G, and Rinaldi S
- Subjects
- Adrenal Cortex Hormones therapeutic use, Adult, Aged, Female, Humans, Immunoglobulins, Intravenous therapeutic use, Male, Middle Aged, Peripheral Nervous System Diseases drug therapy, Peripheral Nervous System Diseases immunology, Autoantibodies, Immunoglobulin G immunology, Peripheral Nervous System Diseases mortality
- Abstract
Objectives: We aimed to define the clinical and serological characteristics of pan-neurofascin antibody-positive patients., Methods: We tested serum from patients with suspected immune-mediated neuropathies for antibodies directed against nodal/paranodal protein antigens using a live cell-based assay and solid-phase platform. The clinical and serological characteristics of antibody-positive and seronegative patients were then compared. Sera positive for pan-neurofascin were also tested against live myelinated human stem cell-derived sensory neurons for antibody binding., Results: Eight patients with IgG
1 -subclass antibodies directed against both isoforms of the nodal/paranodal cell adhesion molecule neurofascin were identified. All developed rapidly progressive tetraplegia. Cranial nerve deficits (100% vs 26%), autonomic dysfunction (75% vs 13%) and respiratory involvement (88% vs 14%) were more common than in seronegative patients. Four patients died despite treatment with one or more modalities of standard immunotherapy (intravenous immunoglobulin, steroids and/or plasmapheresis), whereas the four patients who later went on to receive the B cell-depleting therapy rituximab then began to show progressive functional improvements within weeks, became seronegative and ultimately became functionally independent., Conclusions: IgG1 pan-neurofascin antibodies define a very severe autoimmune neuropathy. We urgently recommend trials of targeted immunotherapy for this serologically classified patient group., Competing Interests: Competing interests: JF has received research grants from the Guarantors of Brain and GBS|CIDP Foundation International. TL has received speaker’s honoraria from CSL Behring and Akcea. AJD, JW, RK, AMR, TM, ND, RR, DB and GL report no disclosures or conflicts of interest. SR has received speaker’s honoraria from Fresenius, Alnylam and Excemed, and payments to provide expert medicolegal advice on inflammatory neuropathies and their treatment. He has received complimentary registration and prize money from the Peripheral Nerve Society, and a travel bursary from the European School of Neuroimmunology. He is a member of the GBS and Associated Inflammatory Neuropathies (GAIN) patient charity medical advisory board. He runs a not-for-profit nodal/paranodal antibody testing service at the Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK, in partnership with Clinical Laboratory Immunology of Oxford University Hospitals., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.)- Published
- 2021
- Full Text
- View/download PDF
38. Epidemiological and cohort study finds no association between COVID-19 and Guillain-Barré syndrome.
- Author
-
Keddie S, Pakpoor J, Mousele C, Pipis M, Machado PM, Foster M, Record CJ, Keh RYS, Fehmi J, Paterson RW, Bharambe V, Clayton LM, Allen C, Price O, Wall J, Kiss-Csenki A, Rathnasabapathi DP, Geraldes R, Yermakova T, King-Robson J, Zosmer M, Rajakulendran S, Sumaria S, Farmer SF, Nortley R, Marshall CR, Newman EJ, Nirmalananthan N, Kumar G, Pinto AA, Holt J, Lavin TM, Brennan KM, Zandi MS, Jayaseelan DL, Pritchard J, Hadden RDM, Manji H, Willison HJ, Rinaldi S, Carr AS, and Lunn MP
- Subjects
- Adult, Aged, Aged, 80 and over, Cohort Studies, Female, Humans, Male, Middle Aged, Prospective Studies, Retrospective Studies, SARS-CoV-2, United Kingdom epidemiology, Young Adult, COVID-19 epidemiology, Guillain-Barre Syndrome epidemiology
- Abstract
Reports of Guillain-Barré syndrome (GBS) have emerged during the Coronavirus disease 2019 (COVID-19) pandemic. This epidemiological and cohort study sought to investigate any causative association between COVID-19 infection and GBS. The epidemiology of GBS cases reported to the UK National Immunoglobulin Database was studied from 2016 to 2019 and compared to cases reported during the COVID-19 pandemic. Data were stratified by hospital trust and region, with numbers of reported cases per month. UK population data for COVID-19 infection were collated from UK public health bodies. In parallel, but separately, members of the British Peripheral Nerve Society prospectively reported incident cases of GBS during the pandemic at their hospitals to a central register. The clinical features, investigation findings and outcomes of COVID-19 (definite or probable) and non-COVID-19 associated GBS cases in this cohort were compared. The incidence of GBS treated in UK hospitals from 2016 to 2019 was 1.65-1.88 per 100 000 individuals per year. GBS incidence fell between March and May 2020 compared to the same months of 2016-19. GBS and COVID-19 incidences during the pandemic also varied between regions and did not correlate with one another (r = 0.06, 95% confidence interval: -0.56 to 0.63, P = 0.86). In the independent cohort study, 47 GBS cases were reported (COVID-19 status: 13 definite, 12 probable, 22 non-COVID-19). There were no significant differences in the pattern of weakness, time to nadir, neurophysiology, CSF findings or outcome between these groups. Intubation was more frequent in the COVID-19 affected cohort (7/13, 54% versus 5/22, 23% in COVID-19-negative) attributed to COVID-19 pulmonary involvement. Although it is not possible to entirely rule out the possibility of a link, this study finds no epidemiological or phenotypic clues of SARS-CoV-2 being causative of GBS. GBS incidence has fallen during the pandemic, which may be the influence of lockdown measures reducing transmission of GBS inducing pathogens such as Campylobacter jejuni and respiratory viruses., (© Crown copyright 2020.)
- Published
- 2021
- Full Text
- View/download PDF
39. Overlapping central and peripheral nervous system syndromes in MOG antibody-associated disorders.
- Author
-
Rinaldi S, Davies A, Fehmi J, Beadnall HN, Wang J, Hardy TA, Barnett MH, Broadley SA, Waters P, Reddel SW, Irani SR, Brilot F, Dale RC, and Ramanathan S
- Subjects
- Adult, Aged, Autoantibodies immunology, Autoantigens immunology, Cohort Studies, Demyelinating Autoimmune Diseases, CNS immunology, Female, Humans, Male, Middle Aged, Myelitis, Transverse complications, Myelitis, Transverse immunology, Optic Neuritis complications, Optic Neuritis immunology, Demyelinating Autoimmune Diseases, CNS complications, Myelin-Oligodendrocyte Glycoprotein immunology, Peripheral Nervous System Diseases epidemiology
- Abstract
Objective: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with CNS demyelination inclusive of optic neuritis (ON) and transverse myelitis (TM). To examine whether peripheral nervous system (PNS) involvement is associated with MOG antibody-associated disorders (MOGAD), we performed detailed characterization of an Australasian MOGAD cohort., Methods: Using a live cell-based assay, we diagnosed 271 adults with MOGAD (2013-2018) and performed detailed clinical and immunologic characterization on those with likely PNS involvement., Results: We identified 19 adults with MOGAD and PNS involvement without prior TM. All patients had CNS involvement including ON (bilateral [n = 3], unilateral [n = 3], and recurrent [n = 7]), a cortical lesion (n = 1), meningoencephalitis (n = 1), and subsequent TM (n = 4). Clinical phenotyping and neurophysiology were consistent with acute inflammatory demyelinating polyneuropathy (n = 1), myeloradiculitis (n = 3), multifocal motor neuropathy (n = 1), brachial neuritis (n = 2), migrant sensory neuritis (n = 3), and paresthesia and/or radicular limb pain (n = 10). Onset MRI spine was consistent with myeloradiculitis with nerve root enhancement in 3/19 and normal in 16/19. Immunotherapy resulted in partial/complete PNS symptom resolution in 12/15 (80%) (steroids and/or IV immunoglobulin n = 9, rituximab n = 2, and plasmapheresis n = 1). We identified serum antibodies targeting neurofascin 155, contactin-associated protein 2, or GM1 in 4/16 patients with MOGAD PNS compared with 0/30 controls ( p = 0.01). There was no binding to novel cell surface antigens using an in vitro myelinating sensory neuronal coculture model., Conclusions: Myeloradiculitis, combined central and peripheral demyelination syndromes, and inflammatory neuropathies may be associated with MOGAD and may be immunotherapy responsive. We identified a subgroup who may have pathology mediated by coexistent autoantibodies., (Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)
- Published
- 2020
- Full Text
- View/download PDF
40. A rare case of Miller Fisher variant of Guillain-Barr é Syndrome (GBS) induced by a checkpoint inhibitor.
- Author
-
McNeill CJ, Fehmi J, Gladwin J, and Price C
- Subjects
- Aged, Diagnosis, Differential, Guillain-Barre Syndrome drug therapy, Humans, Male, Miller Fisher Syndrome chemically induced, Antineoplastic Agents, Immunological adverse effects, Miller Fisher Syndrome diagnosis, Nivolumab adverse effects
- Abstract
With the recent development of novel, more potent cancer treatment, in particular, immune 'checkpoint inhibitors', cases of neurological immune-related adverse events are on the rise. Although rare, this includes Guillain-Barré Syndrome (GBS). We present the case of a 68-year-old male who was admitted with sudden onset of worsening neurological symptoms following immunotherapy treatment. These symptoms progressed quickly to respiratory failure requiring intubation and admission to the intensive care unit. He was thoroughly investigated and is believed to have an axonal neuropathy in the form of Miller Fisher Syndrome (MFS) variant of GBS, secondary to immunotherapy treatment. He was initially treated with intravenous immunoglobulin, and later, perhaps more effectively, with high dose steroids which significantly improved his symptoms. This case of checkpoint inhibitor-induced MFS is one of few in the literature and is an important reminder of the potential for new immunotherapeutic agents to cause significant neurotoxic effects. These should be promptly and thoroughly investigated, in particular, as the management of these patients can differ from standard treatments used in these conditions., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)
- Published
- 2019
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.