Cognitive-behavioral screening reveals prevalent impairment in a large multicenter ALS cohort

Jennifer Murphy, Pam Factor-Litvak, Raymond Goetz, Catherine Lomen-Hoerth, Peter L. Nagy, Jonathan Hupf, Jessica Singleton, Susan Woolley, Howard Andrews, Daragh Heitzman, Richard S. Bedlack, Jonathan S. Katz, Richard J. Barohn, Eric J. Sorenson, Björn Oskarsson, J. Americo M.Fernandes Filho, Edward J. Kasarskis, Tahseen Mozaffar, Yvonne D. Rollins, Sharon P. NationsAndrea J. Swenson, Boguslawa A. Koczon-Jaremko, Hiroshi Mitsumoto

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63 Scopus citations


Objectives: To characterize the prevalence of cognitive and behavioral symptoms using a cognitive/behavioral screening battery in a large prospective multicenter study of amyotrophic lateral sclerosis (ALS). Methods: Two hundred seventy-four patients with ALS completed 2 validated cognitive screening tests and 2 validated behavioral interviews with accompanying caregivers. We examined the associations between cognitive and behavioral performance, demographic and clinical data, and C9orf72 mutation data. Results: Based on the ALS Cognitive Behavioral Screen cognitive score, 6.5% of the sample scored below the cutoff score for frontotemporal lobar dementia, 54.2% scored in a range consistent with ALS with mild cognitive impairment, and 39.2% scored in the normal range. The ALS Cognitive Behavioral Screen behavioral subscale identified 16.5% of the sample scoring below the dementia cutoff score, with an additional 14.1% scoring in the ALS behavioral impairment range, and 69.4% scoring in the normal range. Conclusions: This investigation revealed high levels of cognitive and behavioral impairment in patients with ALS within 18 months of symptom onset, comparable to prior investigations. This investigation illustrates the successful use and scientific value of adding a cognitive-behavioral screening tool in studies of motor neuron diseases, to provide neurologists with an efficient method to measure these common deficits and to understand how they relate to key clinical variables, when extensive neuropsychological examinations are unavailable. These tools, developed specifically for patients with motor impairment, may be particularly useful in patient populations with multiple sclerosis and Parkinson disease, who are known to have comorbid cognitive decline.

Original languageEnglish
Pages (from-to)813-820
Number of pages8
Issue number9
StatePublished - Mar 1 2016

Bibliographical note

Funding Information:
The authors are deeply grateful to the patients and their families who enthusiastically participated in this labor-intensive study. The authors express their thanks to Annette Kirshner at NIEHS for her kind advice and strong support. This study was sponsored by NIEHS (R01ES016348), MDA Wings Over Wall Street, and The Anthony Senerchia Jr. ALS Charitable Foundation. MDA Wings, Adams Foundation, and Ride for Life supported part of the study as well. J. Murphy is employed by the CRO INC research. P. Factor-Litvak, R. Goetz, C. Lomen-Hoerth, P. Nagy, J. Hupf, J. Singleton, S. Woolley, and H. Andrews report no disclosures relevant to the manuscript. D. Heitzman has received financial support from the Muscular Dystrophy Association for the ALS MDA Center at Texas Neurology. He has received research support from National Institutes of Health (via Columbia University Subcontract), Biogen Idec, Questcor Pharmaceuticals, Merz Pharmaceuticals, and Cytokinetics for research trials in which he was the site investigator. R. Bedlack has research grants from the ALS Association, Motor Neurone Disease Association, and Cytokinetics, and is a paid consultant for the ALS Association and Neuraltus Pharmaceuticals. J. Katz, R. Barohn, and E. Sorenson report no disclosures relevant to the manuscript. B. Oskarsson is a speaker for Grifols, his research is supported by Novartis, Cytokinetics, the ALS Association, NIH UL1 TR 000002, and linked award KL2 TR 000134, NINDS U10 NS077422-01, and NINDS 1 U01 NS049640-02, serves on the Board of the Greater Sacramento ALS Association, and he practices clinical neuromuscular medicine and bills for this. J. Fernandes Filho and E. Kasarskis report no disclosures relevant to the manuscript. T. Mozaffar has received personal compensation from consulting activities to Baxter, Biogen Idec, BioMarin, California Stem Cell Inc., Crescent (a Walgreens company), CSL Behring, Genzyme, Grifols, Idera, NuFactor, and Ultragenyx. Dr. Mozaffar received funding from NIH (NS049203) and received clinical research support from ALSTDI, Alexion, Alnylam, Amicus, Biogen Idec, BioMarin, CSL, Cytokinetics, FDA, Grifols, Genzyme, GSK, ISIS, Neuraltus, Novartis, and Ultragenyx. Dr. Mozaffar is currently serving as the chair of the medical advisory board for the Myositis Association. Y. Rollins, S. Nations, A. Swenson, and B. Koczon-Jaremko report no disclosures relevant to the manuscript. H. Mitsumoto received research support from SPF, MDA Wings Over Wall Street, NIEHS (R01ES016348), and private donations from Mr. and Mrs. David Marren. Go to for full disclosures.

Publisher Copyright:
© 2016 American Academy of Neurology.

ASJC Scopus subject areas

  • Clinical Neurology


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