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105. Meta-analyses of deflazacort versus prednisone/prednisolone in patients with nonsense mutation Duchenne muscular dystrophy

Author

Shieh, Perry B, primary, Elfring, Gary, additional, Trifillis, Panayiota, additional, Santos, Claudio, additional, Peltz, Stuart W, additional, Parsons, Julie A, additional, Apkon, Susan, additional, Darras, Basil T, additional, Campbell, Craig, additional, McDonald, Craig M, additional, Barohn, Richard J, additional, Bertini, Enrico, additional, Bushby, Kate, additional, Chabrol, Brigitte, additional, Ciafaloni, Emma, additional, Columer, Jaume, additional, Comi, Giacomi Pietro, additional, Connolly, Anne, additional, Finkel, Richard S, additional, Flanigan, Kevin M, additional, Goemans, Nathalie, additional, Guglieri, Michela, additional, Iannaccone, Susan T, additional, Jones, Kristi J, additional, Kaufmann, Petra, additional, Kirschner, Janbernd, additional, Mah, Jean K, additional, Mathews, Katherine, additional, Mercuri, Eugenio, additional, Muntoni, Francesco, additional, Nevo, Yoram, additional, Osorio, Andrés Nascimento, additional, Péréon, Yann, additional, Quinlivan, Rosaline, additional, Renfroe, J. Ben, additional, Russman, Barry, additional, Ryan, Monique, additional, Sampson, Jacinda, additional, Schara, Ulrike, additional, Selby, Kathryn, additional, Sejersen, Thomas, additional, Sproule, Douglas M, additional, Sweeney, H. Lee, additional, Tulinius, Már, additional, Vilchez, Juan J, additional, Vita, Giuseppe, additional, Voit, Thomas, additional, Burns-Wechsler, Stephanie, additional, Wong, Brenda, additional, Abresch, Ted, additional, Henricson, Erik K, additional, Coleman, Kim, additional, Eagle, Michelle, additional, Florence, Julaine, additional, Gappmaier, Ed, additional, McDonald, Craig, additional, Abdel-Hamid, Hoda Z, additional, Bloetzer, Clemens, additional, Butterfield, Russell J, additional, Chae, Jong-Hee, additional, Dastgir, Jahannaz, additional, Desguerre, Isabelle, additional, Escobar, Raul G, additional, Finanger, Erika, additional, Heydemann, Peter, additional, Hughes, Imelda, additional, Kaminska, Anna, additional, Karachunski, Peter, additional, Kudr, Martin, additional, Lotze, Timothy, additional, de Queiroz Campos Araujo, Alexandra Prufer, additional, Dutra de Resende, Maria Bernadete, additional, Tennekoon, Gihan, additional, Topaloglu, Haluk, additional, Torricelli, Ricardo Erazo, additional, Alfano, Lindsay N, additional, James, Meredith K, additional, Lowes, Linda, additional, Mayhew, Anna, additional, Mazzone, Elena S, additional, Nelson, Leslie, additional, and Rose, Kristy J, additional

Published
2021
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106. Health related quality of life in young, steroid-naïve boys with Duchenne muscular dystrophy

Author

Campbell, Craig, primary, McColl, Elaine, additional, McDermott, Michael P., additional, Martens, William B., additional, Guglieri, Michela, additional, Griggs, Robert C., additional, Straub, Volker, additional, Childs, Anne-Marie, additional, Ciafaloni, Emma, additional, Shieh, Perry B., additional, Spinty, Stefan, additional, Butterfield, Russell J., additional, Horrocks, Iain, additional, Roper, Helen, additional, Maggi, Lorenzo, additional, Baranello, Giovanni, additional, Flanigan, Kevin M., additional, Kuntz, Nancy L., additional, Manzur, Adnan Y., additional, Darras, Basil T., additional, Kang, Peter, additional, Mah, Jean K., additional, Mongini, Tiziana, additional, Ricci, Federica, additional, Morrison, Leslie, additional, Krzesniak-Swinarska, Monika, additional, von der Hagen, Maja, additional, Finkel, Richard S., additional, Kumar, Ashutosh, additional, Wicklund, Matthew, additional, McDonald, Craig M., additional, Henricson, Erik K., additional, Schara-Schmidt, Ulrike, additional, Wilichowski, Ekkehard, additional, Barohn, Richard J., additional, Statland, Jeffrey, additional, Kirschner, Janbernd, additional, Vita, Giuseppe, additional, Vita, Gian Luca, additional, Howard, James F., additional, Hughes, Imelda, additional, McMillan, Hugh J., additional, Pegoraro, Elena, additional, Bello, Luca, additional, Burnette, W. Bryan, additional, Thangarajh, Mathula, additional, and Chang, Taeun, additional

Published
2021
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108. Editorial by concerned physicians: Unintended effect of the orphan drug act on the potential cost of 3,4-diaminopyridine

Author

Burns, Ted M., Smith, Gordon A., Allen, Jeffrey A., Amato, Anthony A., Arnold, David W., Barohn, Richard, Benatar, Michael, Bird, Shawn J., Bromberg, Mark, Chahin, Nizar, Ciafaloni, Emma, Cohen, Jeffrey A., Corse, Andrea, Crum, Brian A., David, William S., Dimberg, Elliot, Sousa, Eduardo A. De, Donofrio, Peter D., Dyck, James P. B., Engel, Andrew G., Ensrud, Erik R., Ferrante, Mark, Freimer, Miriam, Gable, Karissa L., Gibson, Summer, Gilchrist, James M., Goldstein, Jonathan M., Gooch, Clifton L., Goodman, Brent P., Gorelov, Dmitri, Gospe, Sidney M., Jr., Goyal, Namita A., Guidon, Amanda C., Guptill, Jeffrey T., Gutmann, Laurie, Gutmann, Ludwig, Gwathmey, Kelly, Harati, Yadollah, Harper, Michel C., Jr., Hehir, Michael K., Hobson-Webb, Lisa D., Howard, James F., Jr., Jackson, Carlayne E., Johnson, Nicholas, Jones, Sarah M., Juel, Vern C., Kaminski, Henry J., Karam, Chafic, Kennelly, Kathleen D., Khella, Sami, Khoury, Julie, Kincaid, John C., Kissel, John T., Kolb, Noah, Lacomis, David, Ladha, Shafeeq, Larriviere, Daniel, Lewis, Richard A., Li, Yuebing, Litchy, William J., Logigian, Eric, Lou, Jau-Shin, MacGowen, Daniel J.L., Maselli, Ricardo, Massey, Janice M., Mauermann, Michelle L., Mathews, Katherine D., Meriggioli, Matthew N., Miller, Robert G., Moon, Joon-Shik, Mozaffar, Tahseen, Nations, Sharon P., Nowak, Richard J., Ostrow, Lyle W., Pascuzzi, Robert M., Peltier, Amanda, Ruzhansky, Katherine, Richman, David P., Ross, Mark A., Rubin, DEVON I., Russell, James A., Sachs, George M., Salajegheh, Mohammad Kian, Saperstein, David S., Scelsa, Stephen, Selcen, Duygu, Shaibani, Aziz, Shieh, Perry B., Silvestri, Nicholas J., Singleton, Rob J., Smith, Benn E., So, Yuen T., Solorzano, Guillermo, Sorenson, Eric J., Srinivasen, Jayashri, Tavee, Jinny, Tawil, Rabi, Thaisetthawatkul, Pariwat, Thornton, Charles, Trivedi, Jaya, Vernino, Steven, Wang, Annabel K., Webb, Tyler A., Weiss, Michael D., Windebank, Anthony J., and Wolfe, Gil I.

Published
2016
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109. A secondary analysis of PAIN‐CONTRoLS: Pain's impact on sleep, fatigue, and activities of daily living.

Author

Bhai, Salman F., Brown, Alexandra, Gajewski, Byron, Kimminau, Kim S., Waitman, Lemuel R., Pasnoor, Mamatha, and Barohn, Richard J.

Abstract

Introduction/Aims: Peripheral neuropathies commonly affect quality of life of patients due to pain, sleep disturbances, and fatigue, although trials have not adequately explored these domains of care. The aim of this study was to assess the impact of nortriptyline, duloxetine, pregabalin, and mexiletine on pain, sleep, and fatigue in patients diagnosed with cryptogenic sensory polyneuropathy (CSPN). Methods: We implemented a Bayesian adaptive design to perform a 12‐wk multisite, randomized, prospective, open‐label comparative effectiveness study in 402 CSPN patients. Participants received either nortriptyline (n = 134), duloxetine (n = 126), pregabalin (n = 73), or mexiletine (n = 69). At prespecified analysis timepoints, secondary outcomes, Patient Reported Outcomes Measurement Information System (PROMIS) surveys including Short Form (SF)‐12, pain interference, fatigue, and sleep disturbance, were collected. Results: Mexiletine had the highest quit rate (58%) due to gastrointestinal side effects, while nortriptyline (38%) and duloxetine (38%) had the lowest quit rates. If tolerated for the full 12 wk of the study, mexiletine had the highest probability (>90%) of positive outcomes for improvements in pain interference and fatigue. There was no significant difference among the medications for sleep disturbance or SF‐12 scores. Adverse events and lack of efficacy were the two most common reasons for cessation of therapy. Discussion: Physicians caring for patients with CSPN should consider mexiletine to address pain and fatigue, although nortriptyline and duloxetine are better medications to trial first since they are better tolerated. Future research should compare other commonly used medications for CSPN to determine evidence‐based treatment strategies. See Editorial on pages 380‐381 in this issue [ABSTRACT FROM AUTHOR]

Published
2022
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110. Contributors

Author

Abel, Naomi, primary, Agazzi, Siviero, additional, Ahmadian, Amir, additional, Akuthota, Venu, additional, Alaqeel, Ahmed, additional, Ali, Zarina S., additional, Alikhani, Puya, additional, Anastakis, Dimitri J., additional, Apaydin, Nihal, additional, Baccon, Jennifer, additional, Bahm, Jörg, additional, Banakhar, Mai A., additional, Barbe, Mary F., additional, Barohn, Richard J., additional, Belzberg, Allan, additional, Besson, Esther, additional, Birch, Rolfe M., additional, Blount, Jeffrey P., additional, Bozkurt, Murat, additional, Brandmeir, Nicholas, additional, Brown, Justin M., additional, Burchiel, Kim J., additional, Christensen, Kristian Friesgaard, additional, Câmara, Justin, additional, Cohen, Jeffrey A., additional, Corcos, Jacques, additional, Costanzo, Francesco, additional, Craig, Anita, additional, Dakwar, Elias, additional, Davis, Drew, additional, Davis, Karen D., additional, de Carvalho, Mamede, additional, De Groat, William C., additional, De Los Reyes, Kenneth, additional, Dimachkie, Mazen M., additional, Ditty, Benjamin J., additional, Doherty, Cahir, additional, Dorsi, Michael, additional, Dubal, Pariket M., additional, Eardley, W.G.P., additional, Egbuniwe, Obi, additional, Eloy, Jean Anderson, additional, Ezzati, Fatemeh, additional, Faris, C., additional, Fecho, Susan B., additional, Filler, Aaron G., additional, Fisher, Kurt W, additional, Fisher, Winfield S., additional, Flores, Leandro Pretto, additional, Gilbert, Alain, additional, Gomez-Amaya, Sandra M., additional, Gordon, Tessa, additional, Goswami, Ruma, additional, Goubier, Jean-Noël, additional, Griessenauer, Christoph J., additional, Guardia, Charles F., additional, Gupta, Amar, additional, Guyuron, Bahman, additional, Haroutounian, Simon, additional, Hassouna, Magdy, additional, Hassouna, Tarek, additional, Hattab, Eyas M, additional, Hayes, Seth B., additional, Heinen, Christian, additional, Hems, Tim, additional, Heuer, Gregory G., additional, Higgins, Diana M., additional, Hogan, Elizabeth, additional, Matthew Howe, B., additional, Illig, Karl A., additional, Jensen, Troels Staehelin, additional, Jones, Kathryn J., additional, Kerns, Robert D., additional, Khoury, E.F., additional, Knox, Aaron D.C., additional, Ko, Andrew L., additional, Kretschmer, Thomas, additional, Lalkhen, Abdul Ghaaliq, additional, Lee, Michelle, additional, Leonard, Garrett, additional, Levi, Allan D., additional, Liu, James K., additional, Mackinnon, Susan E., additional, Martin, Aaron, additional, Meadows, Rena M., additional, Mendoza, Paola, additional, Midha, Rajiv, additional, Miller, Joseph H., additional, Milner, Rachel, additional, Moore, Amy M., additional, Mowchun, Justin J., additional, Mulligan, Michael, additional, Neal, Sara, additional, Nguyen, Dianna Y., additional, Nikolajsen, Lone, additional, Oakes, W. Jerry, additional, Omar, Nidal B., additional, Ostrowska, Monika, additional, Patel, Daxa M., additional, Payne, Russell, additional, Phillips, Benjamin Z., additional, Pindrik, Jonathan, additional, Polasek, Katharine H., additional, Powell, Alexis, additional, Rasulic, Lukas, additional, Renton, Tara, additional, Richards, Alan T., additional, Rizk, Elias B., additional, Rocque, Brandon G., additional, Rosenbaum, Andrew J., additional, Rotshenker, Shlomo, additional, Rozzelle, Curtis J., additional, Ruggieri, Michael R., additional, Samardzic, Miroslav, additional, Sauvaget, E., additional, Schiefer, Matthew A., additional, Schmalz, Philip G.R., additional, Scioli-Salter, Erica, additional, Sengelaub, Dale R., additional, Sodde, Peter, additional, Spinner, Robert J., additional, Stewart, M.P.M., additional, Stommel, Elijah W., additional, Svider, Peter F., additional, Taira, Takaomi, additional, Teboul, Frédéric, additional, Tran, Richard T., additional, Tuite, Gerald F., additional, Tunstall, R.G., additional, Tyler, Dustin J., additional, Uhl, Richard L., additional, van Loveren, Harry, additional, Wellons, John C., additional, Yang, Aaron J., additional, Yang, Jian, additional, Young, Nathan P., additional, and Zager, Eric L., additional

Published
2015
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112. Rare variants in SQSTM1 and VCP genes and risk of sporadic inclusion body myositis

Author

Hanna, Michael G., Houlden, Henry, Machado, Pedro M., Gang, Qiang, Bettencourt, Conceicao, Healy, Estelle, Parton, Matthew, Holton, Janice L., Brady, Stefen, Hilton-Jones, David, Shieh, Perry B., Zanoteli, Edmar, de Camargo, Leonardo Valente, De Paepe, Boel, De Bleecker, Jan, Shaibani, Aziz, Ripolone, Michela, Violano, Raffaella, Moggio, Maurizio, Barohn, Richard J., Dimachkie, Mazen M., McVey, April L., Pasnoor, Mamatha, Glenn, Melanie, Jawdat, Omar, Statland, Jeffrey, Rico, Gabrielle, Mora, Marina, Mantegazza, Renato, Zanotti, Simona, Needham, Merrilee, Mastaglia, Frank, Liang, Christina, Dalakas, Marinos C., Biba, Angie, Chinoy, Hector, Lilleker, James B., Lamb, Janine, Platt, Hazel, Cooper, Robert G., Miller, James A.L., Roberts, Mark, Househam, Elizabeth, Hilton, David, Shivane, Aditya, Bartlett, Amy, Kissel, John T., Runk, Heidi, Wicklund, Matthew, Saperstein, David S., McKinney, Lynette R., Bettencourt, Conceição, Pittman, Alan M., Hughes, Deborah, and Singleton, Andrew B.

Published
2016
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117. Phase II Study of Arimoclomol in IBM FDA-OOPD

Author

Dimachkie, Mazen M., primary, Hanna, Michael, additional, Machado, Pedro, additional, Herbelin, Laura, additional, Pasnoor, Mamatha, additional, Jawdat, Omar, additional, McVey, April, additional, Statland, Jeffery, additional, Glenn, Melanie D., additional, Greensmith, Linda, additional, Martens, Bill, additional, Tawil, Rabi, additional, McDermott, Michael, additional, and Barohn, Richard J., additional

Published
2021
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121. Safety, efficacy, and tolerability of efgartigimod in patients with generalised myasthenia gravis (ADAPT): a multicentre, randomised, placebo-controlled, phase 3 trial

Author

Howard, James F, primary, Bril, Vera, additional, Vu, Tuan, additional, Karam, Chafic, additional, Peric, Stojan, additional, Margania, Temur, additional, Murai, Hiroyuki, additional, Bilinska, Malgorzata, additional, Shakarishvili, Roman, additional, Smilowski, Marek, additional, Guglietta, Antonio, additional, Ulrichts, Peter, additional, Vangeneugden, Tony, additional, Utsugisawa, Kimiaki, additional, Verschuuren, Jan, additional, Mantegazza, Renato, additional, De Bleecker, Jan L., additional, De Koning, Kathy, additional, De Mey, Katrien, additional, De Pue, Annelien, additional, Mercelis, Rudolf, additional, Wyckmans, Maren, additional, Vinck, Caroline, additional, Wagemaekers, Linda, additional, Baets, Jonathan, additional, Ng, Eduardo, additional, Shabanpour, Jafar, additional, Daniyal, Lubna, additional, Mannan, Shabber, additional, Katzberg, Hans D., additional, Genge, Angela, additional, Siddiqi, Zaeem, additional, Junkerová, Jana, additional, Horakova, Jana, additional, Reguliova, Katerina, additional, Tyblova, Michaela, additional, Jurajdova, Ivana, additional, Novakova, Iveta, additional, Jakubikova, Michala, additional, Pitha, Jiri, additional, Vohanka, Stanislav, additional, Havelkova, Katerina, additional, Horak, Tomas, additional, Bednarik, Josef, additional, Horakova, Mageda, additional, Meisel, Andreas, additional, Remstedt, Dike, additional, Heibutzki, Claudia, additional, Kohler, Siegfried, additional, Gerischer, Lea, additional, Hoffman, Sarah, additional, Stascheit, Frauke, additional, Vissing, John, additional, Zafirakos, Lizzie, additional, Khatri, Kuldeep Kumar, additional, Autzen, Anne, additional, Godtfeldt Stemmerik, Mads Peter, additional, Andersen, Henning, additional, Attarian, Shahram, additional, Salort-Campana, Emmanuelle, additional, Delmont, Emilien, additional, Grapperon, Aude-Marie, additional, Kouton, Ludivine, additional, Tsiskaridze, Alexander, additional, Rózsa, Csilla, additional, Jakab, Gedeonne Margo, additional, Toth, Szilvia, additional, Szabo, Gyorgyi, additional, Bors, David, additional, Szabo, Eniko, additional, Campanella, Angela, additional, Vanoli, Fiammetta, additional, Frangiamore, Rita, additional, Antozzi, Carlo, additional, Bonanno, Silvia, additional, Maggi, Lorenzo, additional, Giossi, Riccardo, additional, Saccà, Francesco, additional, Marsili, Angela, additional, Pane, Chiara, additional, Puorro, Giorgia, additional, Reia, Antonio, additional, Antonini, Giovanni, additional, Alfieri, Girolamo, additional, Morino, Stefania, additional, Garibaldi, Matteo, additional, Fionda, Laura, additional, Leonardi, Luca, additional, Konno, Shingo, additional, Uzawa, Akiyuki, additional, Sakuma, Kaoru, additional, Watanabe, Chiho, additional, Ozawa, Yukiko, additional, Yasuda, Manato, additional, Onishi, Yosuke, additional, Samukawa, Makoto, additional, Tsuda, Tomoko, additional, Suzuki, Yasushi, additional, Ishida, Sayaka, additional, Watanabe, Genya, additional, Takahashi, Masanori, additional, Nakamura, Hiroko, additional, Sugano, Erina, additional, Kubota, Tomoya, additional, Imai, Tomihiro, additional, Suzuki., Mari, additional, Mori, Ayako, additional, Yamamoto, Daisuke, additional, Ikeda, Kazuna, additional, Hisahara, Shin, additional, Masuda, Masayuki, additional, Takaki, Miki, additional, Minemoto, Kanako, additional, Ido, Nobuhiro, additional, Naito, Makiko, additional, Okubo, Yoshihiko, additional, Sugimoto, Takamichi, additional, Takematsu, Yuka, additional, Kamei, Ayumi, additional, Shimizu, Mihiro, additional, Naito, Hiroyuki, additional, Nomura, Eiichi, additional, Van Heur, Marjolein, additional, Peters, Anne-Marie, additional, Tannemaat, Martijn, additional, Ruiter, Annabel, additional, Keene, Kevin, additional, Halas, Marek, additional, Szczudlik, Andrzej, additional, Pinkosz, Marta, additional, Frasinska, Monika, additional, Zwolinska, Grazyna, additional, Kostera-Pruszczyk, Anna, additional, Golenia, Aleksandra, additional, Szczudlik, Piotr, additional, Szczechowski, Lech, additional, Pasko, Aneta, additional, Poverennova, Irina, additional, Urtaeva, Lubov, additional, Kuznetsova, Nadezhda, additional, Romanova, Tatiana, additional, Nadezhda, Malkova, additional, Lapochka, Elena, additional, Korobko, Denis, additional, Vergunova, Ilona, additional, Melnikova, Anna, additional, Bulatova, Ekaterina, additional, Antipenko, Elena, additional, Basta, Ivana, additional, Bozovic, Ivo, additional, Lavrnic, Dragana, additional, Stojanovic, Vidosava Rakocevic, additional, Beydoun, Said, additional, Akhter, Salma, additional, Malekniazi, Ali, additional, Darki, Leila, additional, Pimentel, Norianne, additional, Cannon, Victoria, additional, Chopra, Manisha, additional, Traub, Rebecca, additional, Mozaffar, Tahseen, additional, Hernandez, Isela, additional, Turner, Ivonne, additional, Habib, Ali, additional, Goyal, Namita, additional, Kak, Manisha, additional, Velasquez, Erik, additional, Lam, Lucy, additional, Suresh, Niraja, additional, Farias, Jerrica, additional, Jones, Sarah, additional, Wagoner, Mary, additional, Eggleston, Debbie, additional, Bertorini, Tulio, additional, Benzel, Cindy, additional, Henegar, Robert, additional, Pillai, Rekha, additional, Bharavaju-Sanka, Ratna, additional, Paiz, Carolyn, additional, Jackson, Carlayne, additional, Ruzhansky, Katherine, additional, Dimitrova, Diana, additional, Visser, Amy, additional, Chahin, Nizar, additional, Levine, Todd, additional, Lisak, Robert, additional, Jia, Kelly, additional, Mada, Flicia, additional, Bernitsas, Evanthia, additional, Pasnoor, Mamatha, additional, Roath, Katherine, additional, Colgan, Samantha, additional, Currence, Melissa, additional, Heim, Andrew, additional, Barohn, Richard, additional, Dimachkie, Mazen, additional, Statland, Jeffrey, additional, Jawdat, Omar, additional, Jabari, Duaa, additional, Farmakidis, Constantine, additional, Gilchrist, James, additional, Li, Yuebing, additional, Caristo, Irys, additional, Hastings, Debbie, additional, Morren, John Anthony, additional, Weiss, Michael, additional, Muppidi, Srikanth, additional, Nguyen, Tia, additional, Welsh, Lesly, additional, So, Yuen, additional, Goyal, Neelam, additional, Pulley, Michael, additional, Bailey, Cathy, additional, Quraishi, Zubair, additional, Berger, Alan, additional, Sahagian, Gregory, additional, Camberos, Yasmin, additional, and Frishberg, Benjamin, additional

Published
2021
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122. A Phonetic Complexity-Based Approach For Intelligibility and Articulatory Precision Testing: A Preliminary Study on Talkers With Amyotrophic Lateral Sclerosis

Author

Kuruvilla-Dugdale, Mili, Custer, Claire, Heidrick, Lindsey, Barohn, Richard, and Govindarajan, and Raghav

Subjects
Speech intelligibility -- Analysis, Amyotrophic lateral sclerosis -- Analysis -- Psychological aspects, Phonetics -- Psychological aspects, Health
Abstract

Purpose: This study describes a phonetic complexity-based approach for speech intelligibility and articulatory precision testing using preliminary data from talkers with amyotrophic lateral sclerosis. Method: Eight talkers with amyotrophic lateral sclerosis and 8 healthy controls produced a list of 16 low and high complexity words. Sixty-four listeners judged the samples for intelligibility, and 2 trained listeners completed phoneme-level analysis to determine articulatory precision. To estimate percent intelligibility, listeners orthographically transcribed each word, and the transcriptions were scored as being either accurate or inaccurate. Percent articulatory precision was calculated based on the experienced listeners' judgments of phoneme distortions, deletions, additions, and/or substitutions for each word. Articulation errors were weighted based on the perceived impact on intelligibility to determine word-level precision. Results: Between-groups differences in word intelligibility and articulatory precision were significant at lower levels of phonetic complexity as dysarthria severity increased. Specifically, more severely impaired talkers showed significant reductions in word intelligibility and precision at both complexity levels, whereas those with milder speech impairments displayed intelligibility reductions only for more complex words. Articulatory precision was less sensitive to mild dysarthria compared to speech intelligibility for the proposed complexity-based approach. Conclusions: Considering phonetic complexity for dysarthria tests could result in more sensitive assessments for detecting and monitoring dysarthria progression., The current clinical gold standard to detect and track dysarthria progression in talkers with amyotrophic lateral sclerosis (ALS) involves a brief perceptual evaluation based on sentence or word reading to [...]

Published
2018
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127. Tocilizumab is safe and tolerable and reduces C‐reactive protein concentrations in the plasma and cerebrospinal fluid of ALS patients

Author

Milligan, Carol, primary, Atassi, Nazem, additional, Babu, Suma, additional, Barohn, Richard J., additional, Caress, James B., additional, Cudkowicz, Merit E., additional, Evora, Armineuza, additional, Hawkins, Gregory A., additional, Wosiski‐Kuhn, Marlena, additional, Macklin, Eric A., additional, Shefner, Jeremy M., additional, Simmons, Zachary, additional, Bowser, Robert P., additional, and Ladha, Shafeeq S., additional

Published
2021
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134. Abstracts from the ASENT 2008 Annual Meeting March 6–8, 2008

Author

VanDenburgh, Amanda, Shah, Manasee V., Hua, S., Abu-Shakra, S., Wagg, Jon, Khariton, Tatiana, Beddingfield, F. C., Brin, M. F., Herr, Barbara E., Hart, Kimberly A., McDermott, Michael, Griggs, Robert C., Herbelin, Laura, Barohn, Richard, Loane, David J., Byrnes, Kimberly R., Stoica, Bogdan A., Pajoohesh-Ganji, Ahdeah, Faden, Alan I., Nahab, Fatta, Wittevrongel, Loretta, Hallett, Mark, Li, Ingrid, Brady, Linda, Driscoll, Jamie, Guyer, Mark, Yao, Yong, Almeida, Luis, Maia, Joana, Soares-da-Silva, Patricio, Rocha, Jose Francisco, Lopes, Carlos, Nunes, Teresa, Machado, Rita, Costa, Raquel, Torrão, Leonel, Loureiro, Ana-Isabel, Vaz-da-Silva, Manuel, Wright, Lyndon, Falcão, Amilcar, Coelho, Rui, Albino-Teixeira, Antonio, Cusack, Noel, Hurtt, Mark, Katner, Simon, Torchia, John, Solomon, Bruce, Guinn, Robyn, Rohde, Candace, Thorne, Christine, Cusack, Noel J., Aungst, Bruce, Venkatarangan, Lata, Behrens, Davette, Brown, Foster, Meltzer, Peter, Bakshi, Rohit, Neema, Mohit, Healy, Brian C., Liptak, Zsuzsanna, Betensky, Rebecca A., Buckle, Guy J., Gauthier, Susan A., Stankiewicz, James, Meier, Dominik, Egorova, Svetlana, Arora, Ashish, Guss, Zachary D., Glanz, Bonnie I., Khoury, Samia J., Guttmann, Charles R. G., Weiner, Howard L., Guss, Jason G., and CINCH Study Group

Published
2008
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135. Books to Read Before You Graduate High School

Author

Barohn, Richard J., primary, Freeman, Joshua, additional, Kissel, John, additional, Wallis, Marc, additional, Katz, Jonathan, additional, Levine, Todd, additional, Saperstein, Dave, additional, Jawdat, Omar, additional, Barohn, Joel, additional, Ensrud, Erik R., additional, Gorson, Kenneth, additional, Herbelin, Laura, additional, Swerdlow, Russel, additional, Burns, Jeff, additional, Jackson, Carlayne, additional, and Dubinsky, Richard, additional

Published
2021
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139. Whole-genome analysis of sporadic amyotrophic lateral sclerosis

Author

Dunckley, Travis, Huentelman, Matthew J., Craig, David W., Pearson, John V., Szelinger, Szabolcs, Joshipura, Keta, Halperin, Rebecca F., Stamper, Chelsea, Jensen, Kendall R., Letizia, David, Hesterlee, Sharon E., Pestronk, Alan, Levine, Todd, Bertorini, Tulio, Graves, Michael C., Mozaffar, Tahseen, Jackson, Calayne E., Bosch, Peter, McVey, April, Dick, Arthur, Barohn, Richard, Lomen-Hoerth, Catherine, Rosenfeld, Jeffrey, OEConnor, Daniel T., Kuixing Zhang, Crook, Richard, Ryberg, Henrik, Hutton, Michael, Katz, Jonathan, Simpson, Ericka P., Hiroshi MitsumotoBowser, Robert, Miller, Robert G., Appel, Stanley H., and Stephan, Dietrich A.

Subjects
Amyotrophic lateral sclerosis -- Genetic aspects, Genetic susceptibility -- Research
Abstract

A genomewide case control association study is carried out to identify the specific genetic variants associated with sporadic amyotrophic lateral sclerosis (ALS). Results concluded that variants of the uncharacterized gene, FLJ10986 may confer susceptibility to sporadic ALS.

Published
2007

140. Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial

Author

Gordon, Paul H, Moore, Dan H, Miller, Robert G, Florence, Julaine M, Verheijde, Joseph L, Doorish, Carolyn, Hilton, Joan F, Spitalny, G Mark, MacArthur, Robert B, Mitsumoto, Hiroshi, Neville, Hans E, Boylan, Kevin, Mozaffar, Tahseen, Belsh, Jerry M, Ravits, John, Bedlack, Richard S, Graves, Michael C, McCluskey, Leo F, Barohn, Richard J, and Tandan, Rup

Published
2007
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145. The clinical spectrum of primary lateral sclerosis

Author

Barohn, Richard J., primary, Fink, John K., additional, Heiman-Patterson, Terry, additional, Huey, Edward D., additional, Murphy, Jennifer, additional, Statland, Jeffrey M., additional, Turner, Martin R., additional, and Elman, Lauren, additional

Published
2020
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148. Contributors

Author

Abou-Hamden, Amal, primary, Amato, Anthony A., additional, Ashwal, Stephen, additional, Axelrod, Felicia B., additional, Bale, James F., additional, Banwell, Brenda, additional, Baranano, Kristin W., additional, Barkovich, A. James, additional, Barohn, Richard J., additional, Batshaw, Mark L., additional, Ben-Sira, Liat, additional, Birnbaum, Angela K., additional, Boustany, Rose-Mary N., additional, Brooks-Kayal, Amy, additional, Brown, Lawrence W., additional, Camfield, Carol S., additional, Camfield, Peter R., additional, Casselbrant, Margaretha L., additional, Chiriboga, Claudia A., additional, Christian, Susan L., additional, Cilio, Maria Roberta, additional, Connolly, Anne M., additional, Conway, Jeannine M., additional, Cornes, Susannah, additional, Coulter, David L., additional, Cowan, Tina M., additional, Das, Soma, additional, De Vivo, Darryl C., additional, de Vries, Linda S., additional, Desai, Jay, additional, Descartes, Maria, additional, deVeber, Gabrielle, additional, DiMauro, Salvatore, additional, Dobyns, William B., additional, Dong, Qing, additional, Drake, James M., additional, Duhaime, Ann-Christine, additional, du Plessis, Adre J., additional, El-Bitar, Mohamad K., additional, Enns, Gregory M., additional, Escolar, Diana M., additional, Evans, Owen B., additional, Fatemi, S. Ali, additional, Ferriero, Donna M., additional, Filipek, Pauline A., additional, Frank, Yitzchak, additional, Fredrick, Douglas R., additional, Freeze, Hudson H., additional, Friedman, Neil R., additional, Furman, Joseph M., additional, Garg, Bhuwan P., additional, Ghosh, Debabrata, additional, Gilles, Elizabeth E., additional, Giza, Christopher C., additional, Glaser, Carol A., additional, Gleeson, Joseph G., additional, Graham, John M., additional, Gressens, Pierre, additional, Guerrini, Renzo, additional, Gupta, Nalin, additional, Hahn, Jin S., additional, Harini, Chellamani, additional, Heatwole, Chad, additional, Hirtz, Deborah G., additional, Holmes, Gregory L., additional, Holshouser, Barbara A., additional, Ichord, Rebecca N., additional, Jafar-Nejad, Paymaan, additional, Jensen, Frances E., additional, Johnston, Michael V., additional, Jordan, Lori, additional, Khakoo, Yasmin, additional, Khasraw, Mustafa, additional, Kirton, Adam, additional, Kissel, John T., additional, Klein, Ophir, additional, Knupp, Kelly, additional, Korf, Bruce R., additional, Kotagal, Suresh, additional, Leber, Steven, additional, Leppik, Ilo E., additional, Lerman-Sagie, Tally, additional, Lerner, Jason T., additional, Leshner, Robert T., additional, Leventer, Richard J., additional, Lewis, Donald W., additional, Lewis, Paul F., additional, Lichter-Konecki, Uta, additional, Limperopoulos, Catherine, additional, Louie, Janice K., additional, Luc, Quyen N., additional, MacDonald, Tobey J., additional, Makhani, Naila, additional, Malinger, Gustavo, additional, Mandelbaum, David E., additional, Marcuccilli, Charles J., additional, Maricich, Stephen M., additional, Martin, Lee J., additional, Mennella, Julie A., additional, Ment, Laura R., additional, Michelson, David J., additional, Mikhail, Fady M., additional, Millen, Kathleen J., additional, Miller, Steven P., additional, Mink, Jonathan W., additional, Mirzaa, Ghayda, additional, Mitchell, Wendy G., additional, Moodley, Manikum, additional, Morton, Lawrence D., additional, Moxley, Richard T., additional, Muppidi, Srikanth, additional, Nash, Kendall, additional, Nass, Ruth, additional, Noetzel, Michael J., additional, Nordli, Douglas R., additional, Northington, Frances J., additional, Ouvrier, Robert, additional, Packer, Roger J., additional, Packman, Seymour, additional, Parsons, Julie A., additional, Partridge, John C., additional, Pastores, Gregory M., additional, Patterson, Marc C., additional, Pellock, John M., additional, Perkin, Ronald M., additional, Rapin, Isabelle, additional, Raymond, Gerald V., additional, Rendleman, Rebecca, additional, Rho, Jong M., additional, Roddy, Sarah M., additional, Rosenthal, Stephen M., additional, Rosman, N. Paul, additional, Ross, M. Elizabeth, additional, Rust, Robert S., additional, Sanchez-Lara, Pedro A., additional, Sanger, Terence D., additional, Sanmaneechai, Oranee, additional, Schaad, Urs B., additional, Scher, Mark S., additional, Schor, Nina F., additional, Segal, Michael M., additional, Shaywitz, Bennett A., additional, Shaywitz, Sally E., additional, Sherr, Elliott H., additional, Shevell, Michael I., additional, Shinnar, Shlomo, additional, Shu, Stanford K., additional, Silverstein, Faye S., additional, Singer, Harvey S., additional, Sladky, John T., additional, Smith, Stephen A., additional, Soul, Janet S., additional, Stafstrom, Carl E., additional, Strober, Jonathan B., additional, Sullivan, Joseph, additional, Swaiman, Kenneth F., additional, Sweney, Matthew T., additional, Swoboda, Kathryn J., additional, Täuber, Martin G., additional, Taylor, Donald A., additional, Tein, Ingrid, additional, Thiele, Elizabeth A., additional, Trauner, Doris A., additional, Tuchman, Roberto, additional, Vanderver, Adeline, additional, Hirtum-Das, Michéle Van, additional, Vedanarayanan, V. Venkataraman, additional, Vexler, Zinaida S., additional, Vezina, Gilbert, additional, Scheven, Emily von, additional, Wagner, Ann, additional, Wainwright, Mark S., additional, Walkup, John T., additional, Walsh, Laurence E., additional, Wang, Ching H., additional, Wheless, James W., additional, Wolf, Nicole I., additional, Wolfe, Gil I., additional, Wu, Yvonne W., additional, Wycliffe, Nathaniel D., additional, Yager, Jerome Y., additional, Zimmer, Jennifer A., additional, Zoghbi, Huda Y., additional, and Zupanc, Mary L., additional

Published
2012
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