985 results on '"Palace J"'
Search Results
2. Congenital Myasthenic Syndromes: A Retrospective Natural History Study of Respiratory Outcomes in A Single Centre
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Poulos, J, primary, Samuels, M, additional, Palace, J, additional, Beeson, D, additional, Robb, S, additional, Ramdas, S, additional, Chan, S, additional, and Munot, P, additional
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- 2023
- Full Text
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3. Treatment of MOG antibody associated disorders: results of an international survey
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Whittam, D. H., Karthikeayan, V., Gibbons, E., Kneen, R., Chandratre, S., Ciccarelli, O., Hacohen, Y., de Seze, J., Deiva, K., Hintzen, R. Q., Wildemann, B., Jarius, S., Kleiter, I., Rostasy, K., Huppke, P., Hemmer, B., Paul, F., Aktas, O., Pröbstel, A. K., Arrambide, G., Tintore, M., Amato, M. P., Nosadini, M., Mancardi, M. M., Capobianco, M., Illes, Z., Siva, A., Altintas, A., Akman-Demir, G., Pandit, L., Apiwattankul, M., Hor, J. Y., Viswanathan, S., Qiu, W., Kim, H. J., Nakashima, I., Fujihara, K., Ramanathan, S., Dale, R. C., Boggild, M., Broadley, S., Lana-Peixoto, M. A., Sato, D. K., Tenembaum, S., Cabre, P., Wingerchuk, D. M., Weinshenker, B. G., Greenberg, B., Matiello, M., Klawiter, E. C., Bennett, J. L., Wallach, A. I., Kister, I., Banwell, B. L., Traboulsee, A., Pohl, D., Palace, J., Leite, M. I., Levy, M., Marignier, R., Solomon, T., Lim, M., Huda, S., and Jacob, A.
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- 2020
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4. Demographic and clinical features of neuromyelitis optica: A review.
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Pandit, L, Asgari, N, Apiwattanakul, M, Palace, J, Paul, F, Leite, MI, Kleiter, I, Chitnis, T, and GJCF International Clinical Consortium & Biorepository for Neuromyelitis Optica
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GJCF International Clinical Consortium & Biorepository for Neuromyelitis Optica ,Humans ,Neuromyelitis Optica ,Adult ,Middle Aged ,Female ,Male ,NMO ,demographics ,epidemiology ,incidence ,prevalence ,Brain Disorders ,Eye Disease and Disorders of Vision ,Clinical Sciences ,Neurosciences ,Neurology & Neurosurgery - Abstract
The comparative clinical and demographic features of neuromyelitis optica (NMO) are not well known. In this review we analyzed peer-reviewed publications for incidence and prevalence, clinical phenotypes, and demographic features of NMO. Population-based studies from Europe, South East and Southern Asia, the Caribbean, and Cuba suggest that the incidence and prevalence of NMO ranges from 0.05-0.4 and 0.52-4.4 per 100,000, respectively. Mean age at onset (32.6-45.7) and median time to first relapse (8-12 months) was similar. Most studies reported an excess of disease in women and a relapsing course, particularly in anti-aquaporin 4 antibody (anti AQP4-IgG)-positive patients. Ethnicity may have a bearing on disease phenotype and clinical outcome. Despite limitations inherent to the review process, themes noted in clinical and demographic features of NMO among different populations promote a more global understanding of NMO and strategies to address it.
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- 2015
5. Neuromyelitis optica and multiple sclerosis: Seeing differences through optical coherence tomography
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Green, Ari, Bennett, JL, de, J, Lana-Peixoto, M, Palace, J, Waldman, A, Schippling, S, Tenembaum, S, Banwell, B, Greenberg, B, and Levy, M
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- 2015
6. MOG-Enzephalomyelitis: Internationale Empfehlungen zu Diagnose und Antikörpertestung
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Jarius, S., Paul, F., Aktas, O., Asgari, N., Dale, R. C., de Seze, J., Franciotta, D., Fujihara, K., Jacob, A., Kim, H. J., Kleiter, I., Kümpfel, T., Levy, M., Palace, J., Ruprecht, K., Saiz, A., Trebst, C., Weinshenker, B. G., and Wildemann, B.
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- 2018
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7. P-53 Efficacy and safety of ravulizumab in adults with anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder: Outcomes from the phase 3 CHAMPION-NMOSD trial
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Pittock, S., primary, Barnett, M., additional, Bennett, J.L., additional, Berthele, A., additional, de Sèze, J., additional, Levy, M., additional, Nakashima, I., additional, Oreja-Guevara, C., additional, Palace, J., additional, Paul, F., additional, Pozzilli, C., additional, Allen, K., additional, Mashhoon, Y., additional, Yountz, M., additional, and Kim, H.J., additional
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- 2023
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8. Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders
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Verschuuren, J.J., Palace, J., Murai, H., Tannemaat, M.R., Kaminski, H.J., and Bril, V.
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Lambert-Eaton Myasthenic Syndrome ,Myasthenia Gravis ,Humans ,Neurology (clinical) ,Neuromuscular Junction Diseases ,Autoimmune Diseases - Abstract
Myasthenia gravis and Lambert-Eaton myasthenic syndrome are antibody-mediated autoimmune diseases of the neuromuscular junction that usually present with weakness in ocular muscles and in proximal muscles of the limb and trunk. Prognosis regarding muscle strength, functional abilities, quality of life, and survival is generally good. However, some patients do not respond to treatment. Symptomatic drugs, corticosteroids, and steroid-sparing immunosuppressive drugs remain the cornerstone of treatment. In the past few years, new biological agents against complement, the FcRn receptor, or B-cell antigens have been tested in clinical trials. These new therapies extend the possibilities for targeted immunotherapies and promise exciting new options with a relatively rapid mode of action. Challenges in their use might occur, with barriers due to an increase in cost of care and additional considerations in the choice of drugs, and potential consequences of infection and vaccination due to the COVID-19 pandemic.
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- 2022
9. SA38 Mortality Estimates in Patients With Anti-Aquaporin-4 Autoantibody Positive Neuromyelitis Optica Spectrum Disorder
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Palace, J, primary, Eagle, N, additional, Leite, MI, additional, Kielhorn, A, additional, Powell, L, additional, and Johnston, K, additional
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- 2022
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10. VP.42 Clinical characteristics, molecular genetics and long-term clinical outcomes in 43 patients with congenital myasthenia syndrome due to RAPSYN mutation
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Ramdas, S., primary, Munot, P., additional, Cruz, P. Rodríguez, additional, Alabaf, S., additional, Robb, S., additional, Jayawant, S., additional, Beeson, D., additional, Jungbluth, H., additional, and Palace, J., additional
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- 2022
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11. MOG encephalomyelitis: international recommendations on diagnosis and antibody testing
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Jarius, S., Paul, F., Aktas, O., Asgari, N., Dale, R. C., de Seze, J., Franciotta, D., Fujihara, K., Jacob, A., Kim, H. J., Kleiter, I., Kümpfel, T., Levy, M., Palace, J., Ruprecht, K., Saiz, A., Trebst, C., Weinshenker, B. G., and Wildemann, B.
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- 2018
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12. Isolated new onset ‘atypical’ optic neuritis in the NMO clinic: serum antibodies, prognoses and diagnoses at follow-up
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Piccolo, L., Woodhall, M., Tackley, G., Juryńczyk, M., Kong, Y., Domingos, J., Gore, R., Vincent, A., Waters, P., Leite, M. I., and Palace, J.
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- 2016
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13. Artificial intelligence extension of the OSCAR-IB criteria
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Petzold, A., Albrecht, P., Balcer, L., Bekkers, E., Brandt, A. U., Calabresi, P. A., Deborah, O. G., Graves, J. S., Green, A., Keane, P. A., Nij Bijvank, J. A., Sander, J. W., Paul, F., Saidha, S., Villoslada, P., Wagner, S. K., Yeh, E. A., Aktas, O., Antel, J., Asgari, N., Audo, I., Avasarala, J., Avril, D., Bagnato, F. R., Banwell, B., Bar-Or, A., Behbehani, R., Manterola, A. B., Bennett, J., Benson, L., Bernard, J., Bremond-Gignac, D., Britze, J., Burton, J., Calkwood, J., Carroll, W., Chandratheva, A., Cohen, J., Comi, G., Cordano, C., Costa, S., Costello, F., Courtney, A., Cruz-Herranz, A., Cutter, G., Crabb, D., Delott, L., De Seze, J., Diem, R., Dollfuss, H., El Ayoubi, N. K., Fasser, C., Finke, C., Fischer, D., Fitzgerald, K., Fonseca, P., Frederiksen, J. L., Frohman, E., Frohman, T., Fujihara, K., Cuellar, I. G., Galetta, S., Garcia-Martin, E., Giovannoni, G., Glebauskiene, B., Suarez, I. G., Jensen, G. P., Hamann, S., Hartung, H. -P., Havla, J., Hemmer, B., Huang, S. -C., Imitola, J., Jasinskas, V., Jiang, H., Kafieh, R., Kappos, L., Kardon, R., Keegan, D., Kildebeck, E., Kim, U. S., Klistorner, S., Knier, B., Kolbe, S., Korn, T., Krupp, L., Lagreze, W., Leocani, L., Levin, N., Liskova, P., Preiningerova, J. L., Lorenz, B., May, E., Miller, D., Mikolajczak, J., Said, S. M., Montalban, X., Morrow, M., Mowry, E., Murta, J., Navas, C., Nolan, R., Nowomiejska, K., Oertel, F. C., Oh, J., Oreja-Guevara, C., Orssaud, C., Osborne, B., Outteryck, O., Paiva, C., Palace, J., Papadopoulou, A., Patsopoulos, N., Pontikos, N., Preising, M., Prince, J., Reich, D., Rejdak, R., Ringelstein, M., Rodriguez de Antonio, L., Sahel, J. -A., Sanchez-Dalmau, B., Sastre-Garriga, J., Schippling, S., Schuman, J., Shindler, K., Shin, R., Shuey, N., Soelberg, K., Specovius, S., Suppiej, A., Thompson, A., Toosy, A., Torres, R., Touitou, V., Trauzettel-Klosinski, S., van der Walt, A., Vermersch, P., Vidal-Jordana, A., Waldman, A. T., Waters, C., Wheeler, R., White, O., Wilhelm, H., Winges, K. M., Wiegerinck, N., Wiehe, L., Wisnewski, T., Wong, S., Wurfel, J., Yaghi, S., You, Y., Yu, Z., Yu-Wai-Man, P., Zemaitien≐, R., Zimmermann, H., Albrecht P., Petzold A., Balcer, L., Bekkers, E., Brandt, A. U., Calabresi, P. A., Deborah, O. G., Graves, J. S., Green, A., Keane, P. A., Nij Bijvank, J. A., Sander, J. W., Paul, F., Saidha, S., Villoslada, P., Wagner, S. K., Yeh, E. A., Aktas, O., Antel, J., Asgari, N., Audo, I., Avasarala, J., Avril, D., Bagnato, F. R., Banwell, B., Bar-Or, A., Behbehani, R., Manterola, A. B., Bennett, J., Benson, L., Bernard, J., Bremond-Gignac, D., Britze, J., Burton, J., Calkwood, J., Carroll, W., Chandratheva, A., Cohen, J., Comi, G., Cordano, C., Costa, S., Costello, F., Courtney, A., Cruz-Herranz, A., Cutter, G., Crabb, D., Delott, L., De Seze, J., Diem, R., Dollfuss, H., El Ayoubi, N. K., Fasser, C., Finke, C., Fischer, D., Fitzgerald, K., Fonseca, P., Frederiksen, J. L., Frohman, E., Frohman, T., Fujihara, K., Cuellar, I. G., Galetta, S., Garcia-Martin, E., Giovannoni, G., Glebauskiene, B., Suarez, I. G., P. , Jensen, G., Hamann, S., Hartung, H. -P., Havla, J., Hemmer, B., Huang, S. -C., Imitola, J., Jasinskas, V., Jiang, H., Kafieh, R., Kappos, L., Kardon, R., Keegan, D., Kildebeck, E., Kim, U. S., Klistorner, S., Knier, B., Kolbe, S., Korn, T., Krupp, L., Lagreze, W., Leocani, L., Levin, N., Liskova, P., Preiningerova, J. L., Lorenz, B., May, E., Miller, D., Mikolajczak, J., Said, S. M., Montalban, X., Morrow, M., Mowry, E., Murta, J., Navas, C., Nolan, R., Nowomiejska, K., Oertel, F. C., Oh, J., Oreja-Guevara, C., Orssaud, C., Osborne, B., Outteryck, O., Paiva, C., Palace, J., Papadopoulou, A., Patsopoulos, N., Pontikos, N., Preising, M., Prince, J., Reich, D., Rejdak, R., Ringelstein, M., Rodriguez de Antonio, L., Sahel, J. -A., Sanchez-Dalmau, B., Sastre-Garriga, J., Schippling, S., Schuman, J., Shindler, K., Shin, R., Shuey, N., Soelberg, K., Specovius, S., Suppiej, A., Thompson, A., Toosy, A., Torres, R., Touitou, V., Trauzettel-Klosinski, S., van der Walt, A., Vermersch, P., Vidal-Jordana, A., Waldman, A. T., Waters, C., Wheeler, R., White, O., Wilhelm, H., Winges, K. M., Wiegerinck, N., Wiehe, L., Wisnewski, T., Wong, S., Wurfel, J., Yaghi, S., You, Y., Yu, Z., Yu-Wai-Man, P., Zemaitien≐, R., and Zimmermann, H.
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0301 basic medicine ,Big Data ,medicine.medical_specialty ,Neurology ,media_common.quotation_subject ,Big data ,MEDLINE ,Reviews ,Socio-culturale ,Neurosciences. Biological psychiatry. Neuropsychiatry ,Review ,Public domain ,Retina ,Cohort Studies ,03 medical and health sciences ,Annotation ,0302 clinical medicine ,Artificial Intelligence ,medicine ,Humans ,Quality (business) ,RC346-429 ,Tomography ,media_common ,Image pattern recognition ,business.industry ,General Neuroscience ,Nervous System Diseases ,Tomography, Optical Coherence ,Algorithms ,030104 developmental biology ,Optical Coherence ,Imaging technology ,RC0321 ,Neurology. Diseases of the nervous system ,Neurology (clinical) ,Artificial intelligence ,sense organs ,business ,030217 neurology & neurosurgery ,RC321-571 - Abstract
Artificial intelligence (AI)‐based diagnostic algorithms have achieved ambitious aims through automated image pattern recognition. For neurological disorders, this includes neurodegeneration and inflammation. Scalable imaging technology for big data in neurology is optical coherence tomography (OCT). We highlight that OCT changes observed in the retina, as a window to the brain, are small, requiring rigorous quality control pipelines. There are existing tools for this purpose. Firstly, there are human‐led validated consensus quality control criteria (OSCAR‐IB) for OCT. Secondly, these criteria are embedded into OCT reporting guidelines (APOSTEL). The use of the described annotation of failed OCT scans advances machine learning. This is illustrated through the present review of the advantages and disadvantages of AI‐based applications to OCT data. The neurological conditions reviewed here for the use of big data include Alzheimer disease, stroke, multiple sclerosis (MS), Parkinson disease, and epilepsy. It is noted that while big data is relevant for AI, ownership is complex. For this reason, we also reached out to involve representatives from patient organizations and the public domain in addition to clinical and research centers. The evidence reviewed can be grouped in a five‐point expansion of the OSCAR‐IB criteria to embrace AI (OSCAR‐AI). The review concludes by specific recommendations on how this can be achieved practically and in compliance with existing guidelines.
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- 2021
14. Myasthenia Gravis: Basic Designs, Sample Sizes and Pitfalls
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Palace, J. and Guiloff, Roberto J., editor
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- 2001
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15. A multicentric investigation of the diagnostic accuracy of cortical lesions and central vein sign in multiple sclerosis
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Cagol, A., Cortese, R., Barakovic, M., Schaedelin, S., Ruberte, E., Absinta, M., Barkhof, F., Calabrese, M., Marco Castellaro, Ciccarelli, O., Cocozza, S., Stefano, N., Enzinger, C., Filippi, M., Jurynczyk, M., Maggi, P., Mahmoudi, N., Montalban, X., Palace, J., Pontillo, G., Rocca, M. A., Ropele, S., Rovira, A., Schoonheim, M. M., Sowa, P., Strijbis, E., Wattjes, M. P., Wuerfel, J., Kappos, L., and Granziera, C.
- Published
- 2022
16. The influence of HLA‐DRB1*15 on the relationship between microglia and neurons in multiple sclerosis normal appearing cortical grey matter
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Yates, RL, Pansieri, J, Li, Q, Bell, JS, Yee, SA, Palace, J, Esiri, MM, and DeLuca, GC
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Inflammation ,Neurons ,Multiple Sclerosis ,General Neuroscience ,Humans ,Microglia ,Neurology (clinical) ,Gray Matter ,HLA-DRB1 Chains ,Pathology and Forensic Medicine - Abstract
Cortical tissue injury is common in multiple sclerosis (MS) and associates with disability progression. We have previously shown that HLA-DRB1*15 genotype status associates with the extent of cortical inflammatory pathology. In the current study, we sought to examine the influence of HLA-DRB1*15 on relationships between inflammation and neurodegeneration in MS. Human post-mortem MS cases (n = 47) and controls (n = 10) were used. Adjacent sections of motor cortex were stained for microglia (Iba1+, CD68+, TMEM119+), lymphocytes (CD3+, CD8+), GFAP+ astrocytes, and neurons (NeuN+). A subset of MS cases (n = 20) and controls (n = 7) were double-labeled for neurofilament and glutamic acid decarboxylase 65/67 (GAD+) to assess the extent of the inhibitory synaptic loss. In MS cases, microglial protein expression positively correlated with neuron density (Iba1+: r = 0.548, p < 0.001, CD68+: r = 0.498, p = 0.001, TMEM119+ r = 0.437, p = 0.003). This finding was restricted to MS cases not carrying HLA-DRB1*15. Evidence of a 14% reduction in inhibitory synapses in MS was detected (MS: 0.299 ± 0.006 synapses/μm2 neuronal membrane versus control: 0.348 ± 0.009 synapses/μm2 neuronal membrane, p = 0.005). Neurons expressing inhibitory synapses were 24% smaller in MS cases compared to the control (MS: 403 ± 15 μm2 versus control: 531 ± 29 μm2, p = 0.001), a finding driven by HLA-DRB1*15+ cases (15+: 376 ± 21 μm2 vs. 15−: 432 ± 22 μm2, p = 0.018). Taken together, our results demonstrate that HLA-DRB1*15 modulates the relationship between microglial inflammation, inhibitory synapses, and neuronal density in the MS cortex.
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- 2021
17. Absence of astrocytic outer retinal layer thinning in AQP4-IgG seropositive neuromyelitis optica spectrum disorders
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Lu, A., Zimmermann, H. G., Specovius, S., Motamedi, S., Chien, C., Lana-Peixoto, M. Aurelio, Fontenelle, M. Andrade, Ashtari, F., Kafieh, R., Pandit, L., Dcunha, A., Kim, H., Hyun, J. -W., Leocani, L., Pisa, M., Radaelli, M., Siritho, S., May, E. F., Tongco, C., de Seze, J., Senger, T., Palace, J., Roca-Fernandez, A., Stiebel-Kalish, H., Asgari, N., Soelberg, K. K., Martinez-Lapiscina, E. H., Havla, J., Mao-Draayer, Y., Rimler, Z., Reid, A., Marignier, R., Calvo, A., Altintas, A., Tanriverdi, U., Ringelstein, M., Albrecht, P., Tavares, I. M., Bichuetti, D., Jacob, A., Huda, S., de Castillo, I. S., Petzold, A., Green, A. J., Yeaman, M. R., Smith, T. J., Cook, L., Paul, F., Brandt, A. U., Oertel, F. C., and Consortiu, GJCF Int Clinical
- Published
- 2021
18. P.048 International MAGNIMS-CMSC-NAIMS consensus recommendations on the use of standardized MRI in MS
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Traboulsee, A, primary, Wattjes, M, additional, Ciccarelli, O, additional, Reich, D, additional, Banwell, B, additional, de Stefano, N, additional, Enzinger, C, additional, Fazekas, F, additional, Filippi, M, additional, Frederiksen, J, additional, Gasperini, C, additional, Hacohen, Y, additional, Kappos, L, additional, Li, DK, additional, Mankad, K, additional, Montalban, X, additional, Newsome, S, additional, Oh, J, additional, Palace, J, additional, Rocca, M, additional, Sastre-Garriga, J, additional, Tintore, M, additional, Vrenken, H, additional, Yours, T, additional, Barkhof, F, additional, and Rovira, A, additional
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- 2021
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19. The use of OCT in good visual acuity MOGAD and AQP4-NMOSD patients; with and without optic neuritis
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Roca-Fernández, A, primary, Camera, V, additional, Loncarevic-Whitaker, G, additional, Messina, S, additional, Mariano, R, additional, Vincent, A, additional, Sharma, S, additional, Leite, MI, additional, and Palace, J, additional
- Published
- 2021
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20. Systemic Vascular Disease Burden in Multiple Sclerosis: a post mortem case - control study: O23
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Geraldes, R., Esiri, M., Palace, J., and DeLuca, G.
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- 2016
21. Stratification and monitoring of natalizumab-associated progressive multifocal leukoencephalopathy risk: recommendations from an expert group
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McGuigan, C, Craner, M, Guadagno, J, Kapoor, R, Mazibrada, G, Molyneux, P, Nicholas, R, Palace, J, Pearson, O R, Rog, D, and Young, C A
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- 2016
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22. The role of pontine lesion location in differentiating multiple sclerosis from vascular risk factor-related small vessel disease
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Geraldes, R., Jurynczyk, M., dos Passos, G. R., Pichler, A., Chung, K., Hagens, M., Ruggieri, S., Auger, C., Sastre-Garriga, J., Enzinger, C., Chard, D., Barkhof, F., Gasperini, C., Rovira, A., DeLuca, G., Palace, J., MAGNIMS Study, Group, Filippi, M., Rocca, M. A., Institut Català de la Salut, [Geraldes R, Juryńczyk M, Rodrigues dos Passos G] Nuffield Department of Clinical Neurosciences, Oxford, UK. [Pichler A] Department of Neurology, Medical University of Graz, Graz, Austria/Division of Neuroradiology, Vascular & Interventional Radiology, Medical University of Graz, Graz, Austria. [Chung K] NMR Research Unit, Queen Square Multiple Sclerosis Centre, University College London Institute of Neurology, London, UK. [Hagens M] MS Center Amsterdam, Department of Neurology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands. [Auger C, Rovira A] Secció de Neuroradiologia, Servei de Radiologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Sastre-Garriga J] Servei de Neurologia/ Neuroimmunologia, Centre d’Esclerosi Múltiple de Catalunya (CEMCAT), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain, Vall d'Hebron Barcelona Hospital Campus, Neurology, Amsterdam Neuroscience - Neuroinfection & -inflammation, Amsterdam Neuroscience - Brain Imaging, Radiology and nuclear medicine, Geraldes, R., Jurynczyk, M., dos Passos, G. R., Pichler, A., Chung, K., Hagens, M., Ruggieri, S., Auger, C., Sastre-Garriga, J., Enzinger, C., Chard, D., Barkhof, F., Gasperini, C., Rovira, A., Deluca, G., Palace, J., MAGNIMS Study, Group, Filippi, M., and Rocca, M. A.
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medicine.medical_specialty ,cerebral small vessel disease ,differential diagnosis ,imaging ,Multiple sclerosis ,Esclerosi múltiple ,Disease ,Vascular risk ,Cervell - Vasos sanguinis - Malalties ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Short Reports ,Risk Factors ,Internal medicine ,Pons ,Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Imaging::Tomography::Magnetic Resonance Imaging [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT] ,enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::trastornos cerebrovasculares::enfermedades de los pequeños vasos cerebrales [ENFERMEDADES] ,Medicine ,Humans ,diagnóstico::técnicas y procedimientos diagnósticos::diagnóstico por imagen::tomografía::imagen por resonancia magnética [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS] ,030304 developmental biology ,0303 health sciences ,Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Cerebrovascular Disorders::Cerebral Small Vessel Diseases [DISEASES] ,business.industry ,Brain ,Nervous System Diseases::Autoimmune Diseases of the Nervous System::Demyelinating Autoimmune Diseases, CNS::Multiple Sclerosis [DISEASES] ,medicine.disease ,Magnetic Resonance Imaging ,Neurology ,enfermedades del sistema nervioso::enfermedades autoinmunitarias del sistema nervioso::enfermedades autoinmunes desmielinizantes del SNC::esclerosis múltiple [ENFERMEDADES] ,Cardiology ,Imatgeria per ressonància magnètica ,Neurology (clinical) ,Small vessel ,Differential diagnosis ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
Esclerosi múltiple; Diagnòstic diferencial; Imatges Esclerosis múltiple; Diagnóstico diferencial; Imagen Multiple sclerosis; Differential diagnosis; Imaging Background: Differentiating multiple sclerosis (MS) from vascular risk factor (VRF)-small vessel disease (SVD) can be challenging. Objective and Methods: In order to determine whether or not pontine lesion location is a useful discriminator of MS and VRF-SVD, we classified pontine lesions on brain magnetic resonance imaging (MRI) as central or peripheral in 93 MS cases without VRF, 108 MS patients with VRF and 43 non-MS cases with VRF. Results: MS without VRF were more likely to have peripheral pons lesions (31.2%, 29/93) than non-MS with VRF (0%, 0/43) (Exp(B) = 29.8; 95% confidence interval (CI) = (1.98, 448.3); p = 0.014) but there were no significant differences regarding central pons lesions between MS without VRF (5.4%, 5/93) and non-MS with VRF patients (16.3%, 7/43) (Exp(B) = 0.89; 95% CI = (0.2, 3.94); p = 0.87). The presence of peripheral pons lesions discriminated between MS and VRF-SVD with 100% (95% CI = (91.8, 100)) specificity. The proportion of peripheral pons lesions in MS with VRF (30.5%, 33/108) was similar to that seen in MS without VRF (31.2%, 29/93, p = 0.99). Central lesions occurred in similar frequency in MS with VRF (8.3%, 9/108) and non-MS with VRF (16.3%, 7/43, p = 0.15). Conclusion: Peripheral pons lesion location is a good discriminator of MS from vascular lesions. The author(s) received no financial support for the research, authorship and/or publication of this article.
- Published
- 2020
23. Long-term safety and efficacy of Eculizumab in Aquaporin-4 IgG-positive NMOSD
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Wingerchuk, D.M., Fujihara, K., Palace, J., Berthele, A., Levy, M., Kim, H.J., Nakashima, I., Oreja-Guevara, C., Wang, K.-C., Miller, L., Shang, S., Sabatella, G., Yountz, M., Pittock, S.J., Villa, A., Garcea, O., Manin, A., Melamud, L., Aguirre, F., Fernandez, V., Munoz, D.J., Amor, J., Bocchiardo, C., Obregon, C.D.D., Laffue, A., Paez, M.F., Perez, R.M., Rocchi, V., Teijeiro, L., De Virgiliis, M., Cordoba, M., Ingolotti, M., Lupinacci, A., Ballario, C., Chiesa, A., Gomez, H., Mainella, C., Lattini, H., Barnett, M., Barton, J., Beadnall, H., Garber, J., Hardy, T., Pollard, J., Trewin, B., Shuey, N., Bryson, A., French, A., Laing, J., Law, L.Y., Plummer, C., Sanders, L., Sedal, L., Winkel, A., Neal, A., Habek, M., Adamec, I., Barun, B., Crnosija, L., Gabelic, T., Pitha, J., Nytrova, P., Novakova, I., Tyblova, M., Krasulova, E., Pavlickova, J., Petersen, T., Rasmussen, P., Stilund, M., Svendsen, K., Diem, R., Platten, M., Berberich, A., Jaschonek, H., Wildemann, B., Biberacher, V.M., Brinkhoff, K., Golkowski, D., Kowarik, M., Lehmann-Horn, K., Pongratz, V., Zettl, U., Klinke, J., Loebermann, M., Meister, S., Rimmele, F., Winkelmann, A., Lau, A.Y.L., Au, L.W.C., Fan, S.Y.F., Ip, V.H.L., Ma, S.H., Ma, K.Y.K., Mok, C.T.V., Patti, F., Messina, S., Proietto, M., Filla, A., Morra, V.B., Costabile, T., Nozzolillo, A., Sacc, Sacca, Tackley, George, and MATUR, ZELİHA
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0301 basic medicine ,medicine.medical_specialty ,Neuromyelitis optica ,business.industry ,Maintenance dose ,Eculizumab ,Placebo ,Interim analysis ,medicine.disease ,Confidence interval ,ddc ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,Aquaporin 4 ,Neurology ,Internal medicine ,Medicine ,Neurology (clinical) ,Adverse effect ,business ,Research Articles ,030217 neurology & neurosurgery ,Research Article ,medicine.drug - Abstract
Objective\udDuring PREVENT (NCT01892345), eculizumab significantly reduced relapse risk versus placebo in patients with aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorder (AQP4-IgG+ NMOSD). We report an interim analysis of PREVENT's ongoing open-label extension (OLE; NCT02003144) evaluating eculizumab's long-term safety and efficacy.\ud\udMethods\udPatients who completed PREVENT could enroll in the OLE to receive eculizumab (maintenance dose = 1,200 mg/2 weeks, after a blinded induction phase). Safety and efficacy data from PREVENT and its OLE (interim data cut, July 31, 2019) were combined for this analysis.\ud\udResults\udAcross PREVENT and the OLE, 137 patients received eculizumab and were monitored for a median (range) of 133.3 weeks (5.1–276.9 weeks), for a combined total of 362.3 patient-years (PY). Treatment-related adverse event (AE) and serious adverse event (SAE) rates were 183.5 in 100 PY and 8.6 in 100 PY, respectively. Serious infection rates were 10.2 in 100 PY in eculizumab-treated patients versus 15.1 in 100 PY in the PREVENT placebo group. No patient developed a meningococcal infection. At 192 weeks (3.7 years), 94.4% (95% confidence interval [CI], 88.6–97.3) of patients remained adjudicated relapse-free. The adjudicated annualized relapse rate was 0.025 (95% CI = 0.013–0.048) in all eculizumab-treated patients versus 0.350 (95% CI = 0.199–0.616) in the PREVENT placebo group. During the OLE, 37% of patients (44 of 119 patients) stopped or decreased background immunosuppressive therapy use.\ud\udInterpretation\udThis analysis demonstrates that eculizumab's long-term safety profile in NMOSD is consistent with its established profile across other indications. This analysis also demonstrated the sustained ability of long-term eculizumab treatment to reduce relapse risk in patients with AQP4-IgG+ NMOSD. ANN NEUROL 2021;89:1088–1098
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- 2021
24. Myelin oligodendrocyte glycoprotein antibodies predict a non-MS demyelination course
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HACOHEN, Y, ABSOUD, M, DEIVA, K, HEMINGWAY, C, PALACE, J, WASSMER, E, TARDIEU, M, VINCENT, A, WATERS, P, and LIM, M
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- 2015
25. Short-term adaptation to a simple motor task: A physiological process preserved in multiple sclerosis
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Mancini, L., Ciccarelli, O., Manfredonia, F., Thornton, J.S., Agosta, F., Barkhof, F., Beckmann, C., De Stefano, N., Enzinger, C., Fazekas, F., Filippi, M., Gass, A., Hirsch, J.G., Johansen-Berg, H., Kappos, L., Korteweg, T., Manson, S.C., Marino, S., Matthews, P.M., Montalban, X., Palace, J., Polman, C., Rocca, M., Ropele, S., Rovira, A., Wegner, C., Friston, K., Thompson, A., and Yousry, T.
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- 2009
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26. Mind the gap: from neurons to networks to outcomes in multiple sclerosis
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Chard, D. T., Alahmadi, A. A. S., Audoin, B., Charalambous, T., Enzinger, C., Hulst, H. E., Rocca, M. A., Rovira, A., Sastre-Garriga, J., Schoonheim, M. M., Tijms, B., Tur, C., Gandini Wheeler-Kingshott, C. A. M., Wink, A. M., Ciccarelli, O., Barkhof, F., De Stefano, N., Filippi, M., Frederiksen, J. L., Gasperini, C., Kappos, L., Palace, J., Yousry, T., Vrenken, H., University College of London [London] (UCL), Department of Diagnostic Radiology, Faculty of Applied Medical Science, King Abdulaziz University (KAU), Jeddah, Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - APHM] (CEMEREM), Hôpital de la Timone [CHU - APHM] (TIMONE)-Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), AP-HM, CHU Timone, Pole de Neurosciences Cliniques, Department of Neurology, Marseille, France., Department of Neurology, Research Unit for Neuronal Repair and Plasticity, Medical University of Graz, Graz, Department of Radiology, Division of Neuroradiology, Vascular and Interventional Radiology, Medical University of Graz, Graz, Department of Anatomy and Neurosciences, MS Center Amsterdam, Amsterdam Neuroscience, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Neuroimaging Research Unit and Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Universitat Autònoma de Barcelona (UAB), Alzheimer Center Amsterdam, Department of Neurology, Amsterdam Neuroscience, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Department of Neurology, Luton and Dunstable University Hospital, Luton, Università degli Studi di Pavia = University of Pavia (UNIPV), Brain MRI 3T Research Center, IRCCS Mondino Foundation, Pavia, Department of Radiology & Nuclear Medicine, Amsterdam Neuroscience, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, NMR Research Unit, Queen Square MS Centre, Department of Neuroinflammation, UCL Institute of Neurology, Faculty of Brain Sciences, University College London, London, National Institute for Health Research (NIHR) University College London Hospitals (UCLH) Biomedical Research Centre, London, Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - AP-HM] (CEMEREM), Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)-Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)- Hôpital de la Timone [CHU - APHM] (TIMONE), Section of Neuroradiology, Department of Radiology Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Servei de Neurologia/Neuroimmunologia, Multiple Sclerosis Centre of Catalonia (Cemcat), Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Department of Brain and Behavioural Sciences, University of Pavia, Pavia, Institutes of Neurology and Healthcare Engineering, University College London, London, Chard, Declan T, Alahmadi, Adnan A S, Audoin, Bertrand, Charalambous, Thali, Enzinger, Christian, Hulst, Hanneke E, Rocca, Maria A, Rovira, Àlex, Sastre-Garriga, Jaume, Schoonheim, Menno M, Tijms, Betty, Tur, Carmen, Gandini Wheeler-Kingshott, Claudia A M, Wink, Alle Meije, Ciccarelli, Olga, Barkhof, Frederik, MAGNIMS Study, Group, and Filippi, Massimo
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Multiple Sclerosis ,Grey matter ,Network topology ,030218 nuclear medicine & medical imaging ,White matter ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,0302 clinical medicine ,Alzheimer Disease ,medicine ,Humans ,Default mode network ,ComputingMilieux_MISCELLANEOUS ,Inflammation ,Neurons ,Artificial neural network ,business.industry ,Multiple sclerosis ,[SCCO.NEUR]Cognitive science/Neuroscience ,Brain ,medicine.disease ,medicine.anatomical_structure ,Neurology (clinical) ,Disconnection ,Alzheimer's disease ,Nerve Net ,business ,Neuroscience ,030217 neurology & neurosurgery - Abstract
MRI studies have provided valuable insights into the structure and function of neural networks, particularly in health and in classical neurodegenerative conditions such as Alzheimer disease. However, such work is also highly relevant in other diseases of the CNS, including multiple sclerosis (MS). In this Review, we consider the effects of MS pathology on brain networks, as assessed using MRI, and how these changes to brain networks translate into clinical impairments. We also discuss how this knowledge can inform the targeting of MS treatments and the potential future directions for research in this area. Studying MS is challenging as its pathology involves neurodegenerative and focal inflammatory elements, both of which could disrupt neural networks. The disruption of white matter tracts in MS is reflected in changes in network efficiency, an increasingly random grey matter network topology, relative cortical disconnection, and both increases and decreases in connectivity centred around hubs such as the thalamus and the default mode network. The results of initial longitudinal studies suggest that these changes evolve rather than simply increase over time and are linked with clinical features. Studies have also identified a potential role for treatments that functionally modify neural networks as opposed to altering their structure.
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- 2021
27. International Consensus Guidance for Management of Myasthenia Gravis 2020 Update
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Narayanaswami, P, Sanders, DB, Wolfe, G, Benatar, M, Cea, G, Evoli, A, Gilhus, NE, Illa, I, Kuntz, NL, Massey, J, Melms, A, Murai, H, Nicolle, M, Palace, J, Richman, D, and Verschuuren, J
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education - Abstract
Objective To update the 2016 formal consensus-based guidance for the management of myasthenia gravis (MG) based on the latest evidence in the literature. Methods In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness method was used to develop consensus recommendations pertaining to 7 treatment topics. In February 2019, the international panel was reconvened with the addition of one member to represent South America. All previous recommendations were reviewed for currency, and new consensus recommendations were developed on topics that required inclusion or updates based on the recent literature. Up to 3 rounds of anonymous e-mail votes were used to reach consensus, with modifications to recommendations between rounds based on the panel input. A simple majority vote (80% of panel members voting "yes") was used to approve minor changes in grammar and syntax to improve clarity. Results The previous recommendations for thymectomy were updated. New recommendations were developed for the use of rituximab, eculizumab, and methotrexate as well as for the following topics: early immunosuppression in ocular MG and MG associated with immune checkpoint inhibitor treatment. Conclusion This updated formal consensus guidance of international MG experts, based on new evidence, provides recommendations to clinicians caring for patients with MG worldwide.
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- 2021
28. Myelin-oligodendrocyte glycoprotein antibody-associated disease
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Marignier, R. Hacohen, Y. Cobo-Calvo, A. Pröbstel, A.-K. Aktas, O. Alexopoulos, H. Amato, M.-P. Asgari, N. Banwell, B. Bennett, J. Brilot, F. Capobianco, M. Chitnis, T. Ciccarelli, O. Deiva, K. De Sèze, J. Fujihara, K. Jacob, A. Kim, H.J. Kleiter, I. Lassmann, H. Leite, M.-I. Linington, C. Meinl, E. Palace, J. Paul, F. Petzold, A. Pittock, S. Reindl, M. Sato, D.K. Selmaj, K. Siva, A. Stankoff, B. Tintore, M. Traboulsee, A. Waters, P. Waubant, E. Weinshenker, B. Derfuss, T. Vukusic, S. Hemmer, B.
- Abstract
Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently identified autoimmune disorder that presents in both adults and children as CNS demyelination. Although there are clinical phenotypic overlaps between MOGAD, multiple sclerosis, and aquaporin-4 antibody-associated neuromyelitis optica spectrum disorder (NMOSD) cumulative biological, clinical, and pathological evidence discriminates between these conditions. Patients should not be diagnosed with multiple sclerosis or NMOSD if they have anti-MOG antibodies in their serum. However, many questions related to the clinical characterisation of MOGAD and pathogenetic role of MOG antibodies are still unanswered. Furthermore, therapy is mainly based on standard protocols for aquaporin-4 antibody-associated NMOSD and multiple sclerosis, and more evidence is needed regarding how and when to treat patients with MOGAD. © 2021 Elsevier Ltd
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- 2021
29. Optical coherence tomography in aquaporin-4-IgG positive neuromyelitis optica spectrum disorders: a collaborative multi-center study
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Oertel, F. C., Specovius, S., Zimmermann, H., Chien, C., Motamedi, S., Cook, L., Lana-Peixoto, M. A., Fontenelle, M., Kim, H. J., Hyun, J. -W, Palace, J., Roca-Fernandez, A., Ashtari, F., Kafieh, R., Pandit, L., D Cunha, A., Aktas, O., Ringelstein, M., May, E., Tongco, C., Leocani, L., Pisa, M., Radaelli, M., Martinez-Lapiscina, E., Stiebel-Kalish, H., Siritho, S., Seze, J., Senger, T., Havla, J., Marignier, R., Nerrant, E., Calvo, A. Cobo, Bichuetti, D., Ivan Tavares, Asgari, N., Soelberg, K. K., Altintas, A., Tanriverdi, U., Jacob, A., Huda, S., Rimler, Z., Mao-Draayer, Y., Castillo, I. Soto, Green, A., Yeaman, M., Smith, T., Brandt, A., and Paul, F.
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- 2020
30. Reproducibility of fMRI in the clinical setting: Implications for trial designs
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Bosnell, R., Wegner, C., Kincses, Z.T., Korteweg, T., Agosta, F., Ciccarelli, O., De Stefano, N., Gass, A., Hirsch, J., Johansen-Berg, H., Kappos, L., Barkhof, F., Mancini, L., Manfredonia, F., Marino, S., Miller, D.H., Montalban, X., Palace, J., Rocca, M., Enzinger, C., Ropele, S., Rovira, A., Smith, S., Thompson, A., Thornton, J., Yousry, T., Whitcher, B., Filippi, M., and Matthews, P.M.
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- 2008
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31. Congenital Myasthenic Syndromes in childhood: Diagnostic and management challenges
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Kinali, M., Beeson, D., Pitt, M.C., Jungbluth, H., Simonds, A.K., Aloysius, A., Cockerill, H., Davis, T., Palace, J., Manzur, A.Y., Jimenez-Mallebrera, C., Sewry, C., Muntoni, F., and Robb, S.A.
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- 2008
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32. Neuroprotection and repair
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Palace, J.
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- 2008
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33. The impact of neuromyelitis optica spectrum disorder on pregnancy outcome: T203
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Nour, M. M., Coutinho, E., Nakashima, I., Sousa, F., Revis, J., Santos, E., George, J., Kitley, J., Misu, T., Woodhall, M., Silva, A. M., Vincent, A., Waters, P., Palace, J., Fujihara, K., and Leite, M. I.
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- 2014
34. Congenital myasthenic syndromes: Epidemiology, clinical phenotyping and aids to diagnosis in the neuromuscular clinic: P19
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Healy, E. G., Carr, A. S., Tirupathi, S., Pang, K., Herron, B. M., Cardwell, C., Palace, J., Beeson, D., and McConville, J.
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- 2014
35. Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica
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Viegas, S., Weir, A., Esiri, M., Kuker, W., Waters, P., Leite, M.I., Vincent, A., and Palace, J.
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Hypothalamus -- Research ,Hypothalamus -- Physiological aspects ,Hypothalamus -- Diseases ,Neuromyelitis optica -- Diagnosis ,Neuromyelitis optica -- Case studies ,Neuromyelitis optica -- Care and treatment ,Biopsy -- Usage ,Health ,Psychology and mental health - Published
- 2009
36. Impairment of movement-associated brain deactivation in multiple sclerosis: further evidence for a functional pathology of interhemispheric neuronal inhibition
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Manson, S. C., Wegner, C., Filippi, M., Barkhof, F., Beckmann, C., Ciccarelli, O., De Stefano, N., Enzinger, Christian, Fazekas, F., Agosta, F., Gass, A., Hirsch, J., Johansen-Berg, H., Kappos, L., Korteweg, T., Polman, C., Mancini, L., Manfredonia, F., Marino, S., Miller, D. H., Montalban, X., Palace, J., Rocca, M., Ropele, S., Rovira, A., Smith, S., Thompson, A., Thornton, J., Yousry, T., Frank, J. A., and Matthews, P. M.
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- 2008
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37. MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain: A review of reported findings
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Seewann, A., Enzinger, C., Filippi, M., Barkhof, F., Rovira, A., Gass, A., Miller, D., Montalban, X., Thompson, A., Yousry, T., Tintore, M., de Stefano, N., Palace, J., Rovaris, M., Polman, C., Fazekas, F., and for the MAGNIMS network
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- 2008
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38. Epidemiology of neuromyelitis optica spectrum disorder and its prevalence and incidence worldwide
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Hor, J.Y., Asgari, N., Nakashima, I., Broadley, S.A., Leite, M.I., Kissani, N., Jacob, A., Marignier, R., Weinshenker, B.G., Paul, F., Pittock, S.J., Palace, J., Wingerchuk, D.M., Behne, J.M., Yeaman, M.R., and Fujihara, K.
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Function and Dysfunction of the Nervous System - Abstract
Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon inflammatory disease of the central nervous system, manifesting clinically as optic neuritis, myelitis, and certain brain and brainstem syndromes. Cases clinically diagnosed as NMOSD may include aquaporin 4 (AQP4)-antibody-seropositive autoimmune astrocytopathic disease, myelin oligodendrocyte glycoprotein (MOG)-antibody-seropositive inflammatory demyelinating disease, and double-seronegative disease. AQP4-antibody disease has a high female-to-male ratio (up to 9:1), and its mean age at onset of ~40 years is later than that seen in multiple sclerosis. For MOG-antibody disease, its gender ratio is closer to 1:1, and it is more common in children than in adults. Its clinical phenotypes differ but overlap with those of AQP4-antibody disease and include acute disseminated encephalomyelitis, brainstem and cerebral cortical encephalitis, as well as optic neuritis and myelitis. Double-seronegative disease requires further research and clarification. Population-based studies over the past two decades report the prevalence and incidence of NMOSD in different populations worldwide. One relevant finding is the varying prevalence observed in different racial groups. Consistently, the prevalence of NMOSD among Whites is ~1/100,000 population, with an annual incidence of
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- 2020
39. Distinguishing neurosarcoidosis from multiple sclerosis based on cerebrospinal fluid analysis: A retrospective cohort study
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Arun, T, Pattison, L, and Palace, J
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- 2020
40. Myasthenic syndromes due to defects in COL13A1 and in the N‐linked glycosylation pathway
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Beeson, D, Cossins, J, Rodriguez Cruz, PM, Maxwell, S, Liu, W-W, and Palace, J
- Abstract
The congenital myasthenic syndromes (CMS) are hereditary disorders of neuromuscular transmission. The number of cases recognized, at around 1:100,000 in the United Kingdom, is increasing with improved diagnosis. The advent of next‐generation sequencing has facilitated the discovery of many genes that harbor CMS‐associated mutations. An emerging group of CMS, characterized by a limb‐girdle pattern of muscle weakness, is caused by mutations in genes that encode proteins involved in the initial steps of the N‐linked glycosylation pathway, which is surprising, since this pathway is found in all mammalian cells. However, mutations in these genes may also give rise to multisystem disorders (congenital disorders of glycosylation) or muscle disorders where the myasthenic symptoms constitute only one component within a wider phenotypic spectrum. We also report a CMS due to mutations in COL13A1, which encodes an extracellular matrix protein that is concentrated at the neuromuscular junction and highlights a role for these extracellular matrix proteins in maintaining synaptic stability that is independent of the AGRN/MuSK clustering pathway. Knowledge about the neuromuscular synapse and the different proteins involved in maintaining its structure as well as function enables us to tailor treatments to the underlying pathogenic mechanisms.
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- 2020
41. Serological and experimental studies in different forms of myasthenia gravis
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Vincent, A, Huda, S, Cao, M, Cetin, H, Koneczny, I, Rodriguez Cruz, PM, Jacobson, L, Viegas, S, Jacob, S, Woodhall, M, Nagaishi, A, Maniaol, A, Damato, V, Leite, MI, Cossins, J, Webster, R, Palace, J, and Beeson, D
- Abstract
Antibodies to the acetylcholine receptor (AChR) have been recognized for over 40 years and have been important in the diagnosis of myasthenia gravis (MG), and its recognition in patients of different ages and thymic pathologies. The 10–20% of patients who do not have AChR antibodies are now known to comprise different subgroups, the most commonly reported of which is patients with antibodies to muscle‐specific kinase (MuSK). The use of cell‐based assays has extended the repertoire of antibody tests to clustered AChRs, low‐density lipoprotein receptor–related protein 4, and agrin. Autoantibodies against intracellular targets, namely cortactin, titin, and ryanodine receptor (the latter two being associated with the presence of thymoma), may also be helpful as biomarkers in some patients. IgG4 MuSK antibodies are clearly pathogenic, but the coexisting IgG1, IgG2, and IgG3 antibodies, collectively, have effects that question the dominance of IgG4 as the sole pathologic factor in MuSK MG. After a brief historical review, we define the different subgroups and summarize the antibody characteristics. Experiments to demonstrate the in vitro and in vivo pathogenic roles of MuSK antibodies are discussed.
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- 2020
42. The neuromuscular junction and wide heterogeneity of congenital myasthenic syndromes
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Rodríguez Cruz, P, Palace, J, and Beeson, D
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Myasthenic Syndromes, Congenital ,neuromuscular transmission ,COL13A1 ,neuromuscular junction ,β2-adrenergic agonists ,GMPPB ,Review ,Synaptic Transmission ,Acetylcholine ,lcsh:Chemistry ,SNARE complex ,Genetic Heterogeneity ,Phenotype ,lcsh:Biology (General) ,lcsh:QD1-999 ,health services administration ,Mutation ,presynaptic CMS ,congenital myasthenic syndromes ,N-glycosylation pathway ,Humans ,Receptors, Cholinergic ,lcsh:QH301-705.5 ,health care economics and organizations - Abstract
Congenital myasthenic syndromes (CMS) are genetic disorders characterised by impaired neuromuscular transmission. This review provides an overview on CMS and highlights recent advances in the field, including novel CMS causative genes and improved therapeutic strategies. CMS due to mutations in SLC5A7 and SLC18A3, impairing the synthesis and recycling of acetylcholine, have recently been described. In addition, a novel group of CMS due to mutations in SNAP25B, SYT2, VAMP1, and UNC13A1 encoding molecules implicated in synaptic vesicles exocytosis has been characterised. The increasing number of presynaptic CMS exhibiting CNS manifestations along with neuromuscular weakness demonstrate that the myasthenia can be only a small part of a much more extensive disease phenotype. Moreover, the spectrum of glycosylation abnormalities has been increased with the report that GMPPB mutations can cause CMS, thus bridging myasthenic disorders with dystroglycanopathies. Finally, the discovery of COL13A1 mutations and laminin α5 deficiency has helped to draw attention to the role of extracellular matrix proteins for the formation and maintenance of muscle endplates. The benefit of β2-adrenergic agonists alone or combined with pyridostigmine or 3,4-Dyaminopiridine is increasingly being reported for different subtypes of CMS including AChR-deficiency and glycosylation abnormalities, thus expanding the therapeutic repertoire available.
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- 2020
43. Impact of Eculizumab on Hospitalization Rates and Relapse Treatment in Patients With AQP4+NMOSD: the Phase 3 PREVENT Study
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Kim, H., Pittock, S., Berthele, A., Fujihara, K., Levy, M., Palace, J., Wingerchuk, D., and Ondokuz Mayıs Üniversitesi
- Abstract
Americas-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ACTRIMS) Forum -- FEB 27-29, 2020 -- West Palm Beach, FL WOS: 000532412600226 … Americas Comm Treatment & Res Multiple Sclerosis
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- 2020
44. BMJ Open
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Specovius, S. (Svenja), Zimmermann, H. (Hanna) G. (G), Oertel, F. (Frederike) C. (Cosima), Chien, C. (Claudia), Bereuter, C. (Charlotte), Cook, L. (Lawrence) J. (J), Lana Peixoto, M. (Marco) A. (Aurélio), Fontenelle, M. (Mariana) A. (Andrade), Kim, H. (Ho) J. (Jin), Hyun, J. (Jae-Won), Jung, S. (Su-Kyung), Palace, J. (Jacqueline), Roca-Fernandez, A. (Adriana), Diaz, A. (Alejandro) R. (Rubio), Leite, M. (Maria) I. (Isabel), Sharma, S. (Srilakshmi) M. (M), Ashtari, F. (Fereshte), Kafieh, R. (Rahele), Dehghani, A. (Alireza), Pourazizi, M. (Mohsen), Pandit, L. (Lekha), Dcunha, A. (Anitha), Aktas, O. (Orhan), Ringelstein, M. (Marius), Albrecht, P. (Philipp), May, E. (Eugene), Tongco, C. (Caryl), Leocani, L. (Letizia), Pisa, M. (Marco), Radaelli, M. (Marta), Martinez-Lapiscina, E. (Elena) H. (H), Stiebel-Kalish, H. (Hadas), Hellmann, M. (Mark), Lotan, I. (Itay), Siritho, S. (Sasitorn), De Sèze, J. (Jérôme), Senger, T. (Thomas), Havla, J. (Joachim), Marignier, R. (Romain), Tilikete, C. (Caroline), Cobo Calvo, A. (Alvaro), Bichuetti, D. (Denis) B. (Bernardi), Tavares, I. (Ivan) M. (Maynart), Asgari, N. (Nasrin), Soelberg, K. (Kerstin), Altintas, A. (Ayse), Yildirim, R. (Rengin), Tanriverdi, U. (Uygur), Jacob, A. (Anu), Huda, S. (Saif), Rimler, Z. (Zoe), Reid, A. (Allyson), Mao-Draayer, Y. (Yang), de Castillo, I. (Ibis) S. (Soto), Yeaman, M. (Michael) R. (R), Smith, T. (Terry) J. (J), Brandt, A. (Alexander) U. (U), and Paul, F. (Friedemann)
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Sciences du Vivant [q-bio]/Neurosciences [q-bio.NC] - Abstract
PURPOSE: Optical coherence tomography (OCT) captures retinal damage in neuromyelitis optica spectrum disorders (NMOSD). Previous studies investigating OCT in NMOSD have been limited by the rareness and heterogeneity of the disease. The goal of this study was to establish an image repository platform, which will facilitate neuroimaging studies in NMOSD. Here we summarise the profile of the Collaborative OCT in NMOSD repository as the initial effort in establishing this platform. This repository should prove invaluable for studies using OCT to investigate NMOSD. PARTICIPANTS: The current cohort includes data from 539 patients with NMOSD and 114 healthy controls. These were collected at 22 participating centres from North and South America, Asia and Europe. The dataset consists of demographic details, diagnosis, antibody status, clinical disability, visual function, history of optic neuritis and other NMOSD defining attacks, and OCT source data from three different OCT devices. FINDINGS TO DATE: The cohort informs similar demographic and clinical characteristics as those of previously published NMOSD cohorts. The image repository platform and centre network continue to be available for future prospective neuroimaging studies in NMOSD. For the conduct of the study, we have refined OCT image quality criteria and developed a cross-device intraretinal segmentation pipeline. FUTURE PLANS: We are pursuing several scientific projects based on the repository, such as analysing retinal layer thickness measurements, in this cohort in an attempt to identify differences between distinct disease phenotypes, demographics and ethnicities. The dataset will be available for further projects to interested, qualified parties, such as those using specialised image analysis or artificial intelligence applications.
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- 2020
45. Impact of Eculizumab on Quality of Life in AQP4+NMOSD: Findings From the PREVENT Study
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Berthele, A., Pittock, S., Fujihara, K., Kim, H., Levy, M., Palace, J., Wingerchuk, D., and Ondokuz Mayıs Üniversitesi
- Abstract
Americas-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ACTRIMS) Forum -- FEB 27-29, 2020 -- West Palm Beach, FL WOS: 000532412600227 … Americas Comm Treatment & Res Multiple Sclerosis
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- 2020
46. Measurement of Whole-Brain and Gray Matter Atrophy in Multiple Sclerosis: Assessment with MR Imaging
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Storelli, L, Rocca, M, Pagani, E, Van Hecke, W, Horsfield, M, De Stefano, N, Rovira, A, Sastre-Garriga, J, Palace, J, Sima, D, Smeets, D, Filippi, M, Group, Magnims Study, Storelli, Loredana, Rocca, Maria A., Pagani, Elisabetta, Van Hecke, Wim, Horsfield, Mark A., De Stefano, Nicola, Rovira, Alex, Sastre-Garriga, Jaume, Palace, Jacqueline, Sima, Diana, Smeets, Dirk, and Filippi, Massimo
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Adult ,Male ,Radiology, Nuclear Medicine and Imaging ,medicine.medical_specialty ,Multiple Sclerosis ,Reproducibility of Result ,Gray (unit) ,030218 nuclear medicine & medical imaging ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Software ,Atrophy ,Multiple Sclerosi ,Image Processing, Computer-Assisted ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Gray Matter ,Cross-Sectional Studie ,business.industry ,Multiple sclerosis ,Reproducibility of Results ,Brain ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Mr imaging ,Cross-Sectional Studies ,Female ,Radiology ,business ,030217 neurology & neurosurgery ,Human - Abstract
Purpose To compare available methods for whole-brain and gray matter (GM) atrophy estimation in multiple sclerosis (MS) in terms of repeatability (same magnetic resonance [MR] imaging unit) and reproducibility (different system/field strength) for their potential clinical applications. Materials and Methods The softwares ANTs-v1.9, CIVET-v2.1, FSL-SIENAX/SIENA-5.0.1, Icometrix-MSmetrix-1.7, and SPM-v12 were compared. This retrospective study, performed between March 2015 and March 2017, collected data from (a) eight simulated MR images and longitudinal data (2 weeks) from 10 healthy control subjects to assess the cross-sectional and longitudinal accuracy of atrophy measures, (b) test-retest MR images in 29 patients with MS acquired within the same day at different imaging unit field strengths/manufacturers to evaluate precision, and (c) longitudinal data (1 year) in 24 patients with MS for the agreement between methods. Tissue segmentation, image registration, and white matter (WM) lesion filling were also evaluated. Multiple paired t tests were used for comparisons. Results High values of accuracy (0.87-0.97) for whole-brain and GM volumes were found, with the lowest values for MSmetrix. ANTs showed the lowest mean error (0.02%) for whole-brain atrophy in healthy control subjects, with a coefficient of variation of 0.5%. SPM showed the smallest mean error (0.07%) and coefficient of variation (0.08%) for GM atrophy. Globally, good repeatability (P > .05) but poor reproducibility (P < .05) were found for all methods. WM lesion filling technique mainly affected ANTs, MSmetrix, and SPM results (P < .05). Conclusion From this comparison, it would be possible to select a software for atrophy measurement, depending on the requirements of the application (research center, clinical trial) and its goal (accuracy and repeatability or reproducibility). An improved reproducibility is required for clinical application. © RSNA, 2018 Online supplemental material is available for this article.
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- 2018
47. Inflammation versus neurodegeneration: Consequences for treatment
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Palace, J.
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- 2007
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48. Urgent challenges in quantification and interpretation of brain grey matter atrophy in individual MS patients using MRI
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Amiri, Houshang, de Sitter, Alexandra, Bendfeldt, Kerstin, Battaglini, Marco, Gandini Wheeler-Kingshott, Claudia A M, Calabrese, Massimiliano, Geurts, Jeroen J G, Rocca, Maria A, Sastre-Garriga, Jaume, Enzinger, Christian, de Stefano, Nicola, Filippi, Massimo, Rovira, Álex, Barkhof, Frederik, Vrenken, Hugo, Barkhof, F, Vrenken, H, Ciccarelli, O, Fredriksen, J L, Palace, J, Rovira, A, Sastre-Garriga, J, Amiri, Houshang, de Sitter, Alexandra, Bendfeldt, Kerstin, Battaglini, Marco, Gandini Wheeler-Kingshott, Claudia A. M., Calabrese, Massimiliano, Geurts, Jeroen J. G., Rocca, Maria A., Sastre-Garriga, Jaume, Enzinger, Christian, de Stefano, Nicola, Filippi, Massimo, Rovira, Álex, Barkhof, Frederik, and Vrenken, Hugo
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Radiology, Nuclear Medicine and Imaging ,Brain atrophy ,TR ,Neurology ,White Matter/pathology ,CTh ,brain extraction tool ,lcsh:RC346-429 ,Magnetic Resonance Imaging/methods ,030218 nuclear medicine & medical imaging ,deep grey matter ,0302 clinical medicine ,Nuclear Medicine and Imaging ,TI, inversion time ,FA, fractional anisotropy ,Gray Matter ,repetition time ,VBM ,TE, echo time ,Brain Mapping ,medicine.diagnostic_test ,Grey matter atrophy ,DGM ,Regular Article ,GM ,CTh, cortical thickness ,Multiple Sclerosis/pathology ,diffusion tensor imaging ,Magnetic Resonance Imaging ,White Matter ,TR, repetition time ,medicine.anatomical_structure ,DTI ,lcsh:R858-859.7 ,CNS ,TI ,Radiology ,fractional anisotropy ,MRI ,TE ,FA ,inversion time ,medicine.medical_specialty ,Grey matter ,Multiple Sclerosis ,Cognitive Neuroscience ,Cognitive neuroscience ,Gray Matter/pathology ,lcsh:Computer applications to medicine. Medical informatics ,CNS, central nervous system ,echo time ,MS, multiple sclerosis ,Multiple sclerosis ,VBM, voxel-based morphometry ,03 medical and health sciences ,Magnetic resonance imaging ,Atrophy ,BET, brain extraction tool ,DGM, deep grey matter ,DTI, diffusion tensor imaging ,GM, grey matter ,MRI, magnetic resonance imaging ,WM, white matter ,medicine ,voxel-based morphometry ,Humans ,Multiple sclerosi ,Radiology, Nuclear Medicine and imaging ,WM ,Intensive care medicine ,lcsh:Neurology. Diseases of the nervous system ,business.industry ,MS ,cortical thickness ,BET ,central nervous system ,medicine.disease ,Atrophy/pathology ,Neurology (clinical) ,Clinical trial ,business ,030217 neurology & neurosurgery - Abstract
Atrophy of the brain grey matter (GM) is an accepted and important feature of multiple sclerosis (MS). However, its accurate measurement is hampered by various technical, pathological and physiological factors. As a consequence, it is challenging to investigate the role of GM atrophy in the disease process as well as the effect of treatments that aim to reduce neurodegeneration. In this paper we discuss the most important challenges currently hampering the measurement and interpretation of GM atrophy in MS. The focus is on measurements that are obtained in individual patients rather than on group analysis methods, because of their importance in clinical trials and ultimately in clinical care. We discuss the sources and possible solutions of the current challenges, and provide recommendations to achieve reliable measurement and interpretation of brain GM atrophy in MS., Highlights • Accurate measurement of brain GM atrophy in MS is hampered by various physiological, pathological and technical factors. • Challenges to achieve accuracy in quantification and interpretation of atrophy in MS are discussed. • Recommendations on how to achieve reliable measurement and interpretation of GM atrophy in MS are suggested.
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- 2018
49. Beta-Interferon treatment does not always slow the progression of axonal injury in multiple sclerosis
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Parry, A., Corkill, R., Blamire, A. M., Palace, J., Narayanan, S., Arnold, D., Styles, P., and Matthews, P. M.
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- 2003
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50. PATHOGENIC MECHANISMS OF RAPSN MUTATIONS IN CONGENITAL MYASTHENIC SYNDROMES: 34
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Cheung, J., Cossins, J., Liu, W., Belaya, K., Palace, J., and Beeson, D.
- Published
- 2013
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