Overview

Overview

The Section of Amyotrophic Lateral Sclerosis and Related Motor Neuron Disorders in the Neuromuscular Center, headed by Dr. Erik P Pioro since 2000, provides diagnosis and management, as well as various research studies for hundreds of patients annually with ALS and other motor neuron diseases (MNDs) from Ohio and elsewhere. Every week, an average of 6 patients are newly diagnosed with ALS in the Neuromuscular Center and 8 previously diagnosed patients are managed in follow-up mostly in the ALS/MND multidisciplinary Team Clinic (an ALS Association Center of Excellence), co-directed by Drs. Rebecca M Kuenzler and Pioro.

Approximately 320 patients with ALS and related MNDs are provided ongoing care at any one time. Present research efforts in ALS and other MNDs are aimed at testing potential clinical therapies, characterizing the natural history of disease through big data analyses, identifying genetic and environmental factors predisposing to ALS/MND development, and examining the utility of novel neuroimaging techniques to noninvasively identify brain and spinal cord degeneration. A listing of ongoing and upcoming clinical trials in our section is available at clinicaltrials.gov. Successful completion of such multifaceted research efforts requires close collaboration with many talented individuals both internal and external to the Cleveland Clinic, as listed below. In addition to receiving clinical trial funding from various pharmaceutical companies, research support is or has been provided by NIH, CDC, the Packard Center for ALS Research, the ALS Association, and generous donors.

Funding

Northeast ALS (NEALS) Consortium
Neuraltus Pharmaceuticals, Inc.
Biogen/Idec, Inc.
Avanir Pharmaceuticals, Inc.
Centers for Disease Control, Federal Grant #NS42759
Sanofi Synthelabo, K08NS001846-01, Subaward No. IH-STTR-02
NIH, Rare Disease Clinical Research Network, Award No. TS15-001
Spastic Paraplegia Foundation

Publications

Publications

(in the past 3 years)

PLSFRS study group. Primary lateral sclerosis (PLS) functional rating scale: PLS-specific clinimetric scale. Mitsumoto H, Chiuzan C, Gilmore M, Zhang Y, Simmons Z, Paganoni S, Kisanuki YY, Zinman L, Jawdat O, Sorenson E, Floeter MK, Pioro EP, Fernandes Filho JAM, Heitzman D, Fournier CN, Oskarsson B, Heiman-Patterson T, Maragakis N, Joyce N, Hayat G, Nations S, Scelsa S, Walk D, Elman L, Hupf J, McHale B; Muscle Nerve. 2020 Feb;61(2):163-172. doi: 10.1002/mus.26765. Epub 2019 Dec 13.

Long-term edaravone efficacy in amyotrophic lateral sclerosis: Post-hoc analyses of Study 19 (MCI186-19). Shefner J, Heiman-Patterson T, Pioro EP, Wiedau-Pazos M, Liu S, Zhang J, Agnese W, Apple S., Muscle Nerve. 2020 Feb;61(2):218-221. doi: 10.1002/mus.26740. Epub 2019 Nov 11.

Palatal myoclonus, abnormal eye movements, and olivary hypertrophy in GAD65-related disorder. Macaron G, Willis MA, Ontaneda D, Fernandez H, Kim S, Jones SE, Pioro EP, Cohen JA., Neurology. 2019 Dec 31. pii:10.1212/WNL.0000000000008926. doi: 10.1212/WNL.0000000000008926. [Epub ahead of print] .

Pattern of lung function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation. Panchabhai TS, Mireles Cabodevila E, Pioro EP, Wang X, Han X, Aboussouan LS., ERJ Open Res. 2019 Sep 25;5(3). pii: 00044-2019. doi: 10.1183/23120541.00044-2019. eCollection 2019.

Pooled Resource Open-Access Als Clinical Trials Consortium. Stage-specific riluzole effect in amyotrophic lateral sclerosis: a retrospective study. Thakore NJ, Lapin BR, Pioro EP; Amyotroph Lateral Scler Frontotemporal Degener. 2019 Aug 27:1-4. doi: 10.1080/21678421.2019.1655060. [Epub ahead of print].

Variation in noninvasive ventilation use in amyotrophic lateral sclerosis. Thakore NJ, Lapin BR, Pioro EP, Aboussouan LS., Neurology. 2019 Jul 16;93(3):e306-e316. doi: 0.1212/WNL.0000000000007776. Epub 2019 Jun 10.

Unbiased MRI Analyses Identify Micropathologic Differences Between Upper Motor Neuron-Predominant ALS Phenotypes. Rajagopalan V, Pioro EP., Front Neurosci. 2019 Jul 12;13:704. doi: 10.3389/fnins.2019.00704. eCollection 2019.

Contributing Members of the NEALS Bulbar Subcommittee. Provisional best practices guidelines for the evaluation of bulbar dysfunction in amyotrophic lateral sclerosis.Pattee GL, Plowman EK, Focht Garand KL, Costello J, Brooks BR, Berry JD, Smith RA, Atassi N, Chapin JL, Yunusova Y, McIlduff CE, Young E, Macklin EA, Locatelli ER, Silani V, Heitzman D, Wymer J, Goutman SA, Gelinas DF, Perry B, Nalipinski P, Stipancic K, O'Brien M, Sullivan SL, Pioro EP, Gargiulo G, Green JR; Muscle Nerve. 2019 May;59(5):531-536. doi: 10.1002/mus.26408. Epub 2019 Jan 22.

Airlie House ALS Clinical Trials Guidelines Group. Revised Airlie House consensus guidelines for design and implementation of ALS clinical trials. van den Berg LH, Sorenson E, Gronseth G, Macklin EA, Andrews J, Baloh RH, Benatar M, Berry JD, Chio A, Corcia P, Genge A, Gubitz AK, Lomen-Hoerth C, McDermott CJ, Pioro EP, Rosenfeld J, Silani V, Turner MR, Weber M, Brooks BR, Miller RG, Mitsumoto H; Neurology. 2019 Apr 2;92(14):e1610-e1623. doi:10.1212/WNL.0000000000007242. Epub 2019 Mar 8.

Longitudinal 18F-FDG PET and MRI Reveal Evolving Imaging Pathology That Corresponds to Disease Progression in a Patient With ALS-FTD. Rajagopalan V, Pioro EP. Front Neurol. 2019b Mar 19;10:234. doi: 10.3389/fneur.2019.00234. eCollection 2019.

Deconstructing progression of amyotrophic lateral sclerosis in stages: a Markov modeling approach. Thakore N J, Lapin BR, Kinzy T G, Pioro E P. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 2018 Nov;19(7-8), 483-494. doi:10.1080/21678421.2018.1484925.

Additional evidence for a therapeutic effect of dextromethorphan/quinidine on bulbar motor function in persons with ALS: A quantitative speech analysis.Br J Clin Pharmacol. 2018 Aug 28. doi: 10.1111/bcp.13745. [Epub ahead of print] Green JR, Allison KM, Cordella C, Richburg BD, Pattee GL, Berry JD, Macklin EA, Pioro EP, Smith RA.

Deconstructing progression of amyotrophic lateral sclerosis in stages: a Markov modeling approach. Amyotroph Lateral Scler Frontotemporal Degener. 2018 Jul 12:1-11. doi: 10.1080/21678421.2018.1484925. [Epub ahead of print] Thakore NJ, Lapin BR, Kinzy TG, Pioro EP. 

Distal Predominance of Electrodiagnostic Abnormalities in Early-Stage Amyotrophic Lateral Sclerosis. Muscle Nerve. 2018 May 9. doi: 10.1002/mus.26158. [Epub ahead of print]. Shayya L, Babu S, Pioro EP, Li J, Li Y.

Assessment of bulbar function in amyotrophic lateral sclerosis: validation of a self-report scale (Center for Neurologic Study Bulbar  Function Scale). Eur J Neurol. 2018 Jul;25(7):907-e66. doi: 10.1111/ene.13638. Epub 2018 Apr 16. Smith RA, Macklin EA, Myers KJ, Pattee GL, Goslin KL, Meekins GD, Green JR, Shefner JM, Pioro EP.

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene. Neuron. 2018 Mar 21;97(6):1268-1283.e6. doi: 10.1016/j.neuron.2018.02.027. Nicolas A, Kenna KP, Renton AE, … Pioro EP, … Shaw CE, Traynor BJ, Landers JE. 

MR Imaging-based Estimation of Upper Motor Neuron Density in Patients with Amyotrophic Lateral Sclerosis: A Feasibility Study. Radiology. 2018 Jun;287(3):955-964. doi: 10.1148/radiol.2018162967. Epub 2018 Jan 23. Chen J, Kostenko V, Pioro EP, Trapp BD.

Trajectories of impairment in amyotrophic lateral sclerosis: Insights from the Pooled Resource Open-Access ALS Clinical Trials cohort. Muscle  Nerve. 2018 Jun;57(6):937-945. doi: 10.1002/mus.26042. Epub 2018 Jan 11. Thakore NJ, Lapin BR, Pioro EP; Pooled Resource Open-Access ALS Clinical Trials Consortium.

Laughter, crying and sadness in ALS. J Neurol Neurosurg  Psychiatry. 2017 Oct;88(10):825-831. doi: 10.1136/jnnp-2017-315622. Epub 2017 Jun 1. Thakore NJ, Pioro EP.

Positron emission tomography imaging in a case of E200K mutation-related spongiform encephalopathy with non-diagnostic magnetic resonance imaging and cerebrospinal fluid testing. SAGE Open Med Case Rep. 2017 Mar 29;5:2050313X17700347. doi: 10.1177/2050313X17700347. eCollection 2017. George P, Newey CR, Mente KP, Pioro EP. 

Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: A novel motor neuron disorder? Muscle Nerve. 2017  Dec;56(6):1164-1168. doi: 10.1002/mus.25669. Epub 2017 May 15. Delva A, Thakore N, Pioro EP, Poesen K, Saunders-Pullman R, Meijer IA, Rucker JC, Kissel JT, Van Damme P.

Differential involvement of corticospinal tract (CST) fibers in UMN-predominant ALS patients with or without CST hyperintensity:  A diffusion tensor tractography study. Neuroimage Clin. 2017 Feb 22;14:574-579. doi: 10.1016/j.nicl.2017.02.017. eCollection 2017. Rajagopalan V, Pioro EP. 

Erratum to: Enhanced Bulbar Function in Amyotrophic Lateral Sclerosis: The Nuedexta Treatment Trial. Neurotherapeutics. 2017 Jul;14(3):830. doi: 10.1007/s13311-017-0517-z. Smith R, Pioro E, Myers K, Sirdofsky M, Goslin K, Meekins G, Yu H, Wymer J, Cudkowicz M, Macklin EA, Schoenfeld D, Pattee G.

Enhanced Bulbar Function in Amyotrophic Lateral Sclerosis: The Nuedexta Treatment Trial. Neurotherapeutics. 2017 Jul;14(3):762-772. doi: 10.1007/s13311-016-0508-5. Erratum in: Neurotherapeutics. 2017 Mar 10. Smith R, Pioro E, Myers K, Sirdofsky M, Goslin K, Meekins G, Yu H, Wymer J, Cudkowicz M, Macklin EA, Schoenfeld D, Pattee G.

Optimizing muscle selection for electromyography in amyotrophic lateral sclerosis. Muscle Nerve 2017 Jul;56(1):36-44. Epub 2017 Feb 15. Babu S, Pioro EP, Li J, Li Y.




Members & Collaborations

Members & Collaborations

Cleveland Clinic

External Relationships and Collaborations

  • Michael Benatar, MD (University of Miami, Miami, FL)
  • Walter Bradley, MD (University of Miami, Miami, FL)
  • Mark Griswold, PhD (Case Western Reserve University, Cleveland, OH)
  • Julian Grosskreutz, PD, Dr. med. (Friedrich Schiller University, Jena, Germany)
  • Dan Ma, PhD (Case Western Reserve University, Cleveland, OH)
  • Hiroshi Mitsumoto, MD (Columbia Presbyterian Medical Center)
  • Elijah Stommel, MD (Dartmouth College, Hanover, NH