Citations 3
Prospective Validation of the New PLS Diagnostic Criteria From PLS Natural History Study: EMG and Neuro
Sorenson, Eric J. , Heitzman, Daragh, Lee, Ikjae , Jang, Grace E. , Elman, Lauren , Habib, Ali A. , Wymer, James , Hayat, Ghazala , Fernandes, J Americo M, Floeter, Mary Kay, Ajroud‐Driss, Senda , Gwathmey, Kelly , Ej, Kasarskis, Kisanuki, Yasushi, Lomen-Hoerth, Catherine, Walk, David , Johnston, Wendy S, Diaz, Frank, Maragakis, Nicholas J, Paganoni, Sabrina, Shah, Jaimin, Zinman, Lorne, Heiman-Patterson, Terry, Jawdat, Omar, Fournier, Christina N, Pulley, Michael , Scelsa, Stephen N. , Shoesmith, Christen , Sherman, Alex, Hoover, Benjamin N, Yun, Rebecca, Cheung, Ken, Mitsumoto, Hiroshi , Group, for the PLS NHS Study, Maragakis, Nicholas J. , Sorenson, Eric , Gwathmey, Kelly , Walk, David , Paganoni, Sabrina , Shah, Jaimin , Pulley, Michael T , Simmons, Zachary , Maragakis, Nicholas , Wymer, James, Goutman, Stephen A. , Kasarskis, Edward J. , Lomen‐Hoerth, Catherine , Johnston, Wendy , Diaz, Frank , Oskarsson, Björn , Zinman, Lorne , Heiman‐Patterson, Terry , Fournier, Christina , Simmons, Zachary , Hoover, Benjamin N.
Muscle & Nerve | March 8, 2026 | Volume: 73 | Issue: 6 | Pages: 976-983
ABSTRACT Background Primary lateral sclerosis (PLS) is an ultrarare upper motor neuron syndrome with a prognosis unique from classical ALS. The study of PLS is complicated by its rarity and the difficulty distinguishing PLS from ALS. We present data from a 1‐year prospective follow‐up study on PLS and efforts to distinguish it from ALS. Methods Seventy‐six PLS participants enrolled in this prospective natural history study. EMG studies, blood neurofilament light chain levels (NfLs), and demographic characteristics were obtained at baseline. At 1‐year follow‐up, repeat EMG studies were conducted to determine which participants fulfilled criteria for ALS. Baseline characteristics were then compared to determine features that predict reclassification. Results Seventy participants completed 1‐year follow‐up. Five of the 70 were reclassified to ALS (7.1%). Those reclassified had higher trends in baseline blood NfL levels (91.4 vs. 34.0 pg/mL, p = 0.13) and shorter symptom duration (39 vs. 69 months in the PLS group, p = 0.15). Reclassification was noted in both probable and definite PLS participants. All cases with a symptomatic duration of less than 2 years retained the PLS phenotype (5 of 5). NfL levels over 90 pg/mL predicted reclassification with 94% specificity and 60% sensitivity. No other features predicted reclassification to ALS. Conclusions In our population, reclassification of PLS to ALS occurred at a low frequency at 1 year follow‐up (7.1%). Baseline NfL was the strongest predictor in differentiating UMN dominant ALS from PLS at 1‐year follow‐up. Based on our data, we propose EMG and NfL criteria for enrollment in future PLS trials.
Preface: promoting research in PLS: current knowledge and future challenges
Mitsumoto, Hiroshi , Conference, all Delegates of the PLS, Ajroud‐Driss, Senda , Andres, Patricia L., Andrews, Jinsy , Babu, Suma , Barohn, Richard J., Benatar, Michael, Chew, Sheena , Conwit, Robin , Davis, Frank, Drory, Vivian E., Elman, Lauren , Fernandes, J. Americo, Ferrey, Dominic, Finegan, Eoin, Fink, John K. , Floeter, Mary Kay, Fournier, Christina , Genge, Angela , Govindarajan, Raghav , Haase, Georg, Hardiman, Orla , Harms, Matthew B. , Hayat, Ghazala , Heiman‐Patterson, Terry , Hill, Bryan Alan, Hübers, Annemarie , Huey, Edward D. , Jawdat, Omar, Kano, Osamu , Kau, Kristen, Kiernan, Matthew C. , Kisanuki, Yasushi, Kurent, Jerome E., Kwan, Justin , Lange, Dale J. , Ludolph, Albert C. , Mackenzie, Ian R. , Manfredi, Giovanni , Marren, David, Family, and, Morita, Mitsuya , Murphy, Jennifer , Nations, Sharon , Oskarsson, Björn , Pellerin, David , Ravits, John , Rezania, Kourosh , Scelsa, Stephen N. , Siddique, Nailah, Simmons, Zachary , Statland, Jeffrey , Blitterswijk, Marka van , Berg, Leonard van den , Walk, David , Warden, Deborah L., Wymer, James , van Blitterswijk, Marka , Simmons, Zachary , Oskarsson, Björn , Hardiman, Orla , Kiernan, Matthew C , Walk, David , Turner, Martin R. , Conference, all Delegates of the PLS, Área-Gómez, Estela , Solano, Juan Marcos , Bede, Peter , Corcia, Philippe , Cudkowicz, Merit , Carvalho, Mamede A. de , Factor‐Litvak, Pam , Granit, Volkan , Family, and, Paganoni, Sabrina , Rouleau, Guy Armand , Siddique, Teepu , Silani, Vincenzo , Traynor, Bryan J.
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration | February 20, 2021 | Volume: 21 | Issue: sup1 | Pages: 1-2
Primary lateral sclerosis (PLS) functional rating scale: PLS‐specific clinimetric scale
Mitsumoto, Hiroshi , Chiuzan, Codruța , Gilmore, Madison , Zhang, Yuan , Simmons, Zachary , Kisanuki, Yasushi, Zinman, Lorne , Jawdat, Omar, Sorenson, Eric J. , Floeter, Mary Kay, Filho, J. Americo M. Fernandes, Heitzman, Daragh, Fournier, Christina , Oskarsson, Björn , Heiman‐Patterson, Terry , Maragakis, Nicholas J. , Joyce, Nanette C. , Hayat, Ghazala , Nations, Sharon , Scelsa, Stephen N. , Walk, David , Elman, Lauren , Hupf, Jonathan , McHale, Brittany , group, the PLSFRS study, Sorenson, Eric, Simmons, Zachary , Oskarsson, Björn , Maragakis, Nicholas , Walk, David , Paganoni, Sabrina , Pioro, Erik P. , group, the PLSFRS study
Muscle & Nerve | November 23, 2019 | Volume: 61 | Issue: 2 | Pages: 163-172
AbstractIntroductionOur research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS).MethodsA prototype of the PLS Functional Rating Scale (PLSFRS) was generated. Seventy‐seven participants with PLS were enrolled and evaluated at 21 sites that comprised the PLSFRS study group. Participants were assessed using the PLSFRS, Neuro‐Quality of Life (QoL), Schwab‐England Activities of Daily Living (ADL), and the Clinical Global Impression of Change scales. Participants completed telephone assessments at 12, 24, and 48 weeks after enrollment.ResultsThe PLSFRS demonstrated internal consistency as well as intrarater, interrater, telephone test‐retest reliability, and construct validity. Significant changes in disease progression were detected at 6 and 12 months; changes measured by the PLSFRS vs the ALSFRS‐R were significantly higher.DiscussionThe PLSFRS is a valid tool to assess the natural history of PLS in a shorter study period.