VALUES: A National Multicenter Study Demonstrating Gender Differences in Amyotrophic Lateral Sclerosis with Behavioral Impairment

Abstract

SUMMARY Aim: This study aims to characterize gender-specific behavioral profiles in nondemented amyotrophic lateral sclerosis subjects with behavioral changes (ALSbi). Methods: Caregivers of 106 subjects (53 male) completed an amyotrophic lateral sclerosis standardized clinical interview framed around the Frontal Behavioral Inventory (FBI) structured questionnaire to rate patients on behaviors associated with declines in frontal and temporal self-regulatory capacities. Subjects evaluated evidenced two or more nonoverlapping behavioral symptoms. Inventory items were grouped into disinhibited, apathetic and stereotypic subclasses for between-gender evaluation by independent t-tests and within-gender evaluation by Mann–Whitney U and Spearman rho. Results: Males showed greater disinhibition (p = 0.000), and loss of insight (p = 0.011), jocularity (p = 0.009) and impulsivity (p = 0.005). Females showed significant relationships between personal neglect and indifference (p = 0.000), impulsivity (p = 0.0.013) and jocularity (p = 0.013). Conclusion: Gender differences are evident in ALSbi, characterized by gender-specific loss of social sophistication, and their characterization may allow for better detection of female ALSbi patients, as well as contribute to treatment planning and quality of life for ALSbi patients and their caregivers.

Acknowledgements

The following investigators collaborated in this study: Claire Flaherty (principal investigator), and Allyson Brothers, Darren Hoffer, Marissa Harrison, Chengwu Yang, Richard S Legro and Zachery Simmons (coinvestigators). The authors are deeply grateful to the patients and caregivers for their willingness to participate in this study.

Financial & competing interests disclosure

This work was supported in part by the Paul and Harriett Campbell fund for ALS Research and the Zimmerman Family Love Fund. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Ethical conduct of research

The authors state that they have obtained appropriate institutional review board approval or have followed the principles outlined in the Declaration of Helsinki for all human or animal experimental investigations. In addition, for investigations involving human subjects, informed consent has been obtained from the participants involved.

Additional information

Notes on contributors

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For a full list of contributors please see end of this article