Abstract Details

Title Energy Expenditure Index Correlates with Motor and Sensory Impairments and Disease Severity in Adrenomyeloneuropathy

Topic Child Neurology and Developmental Neurology

Presentation(s) P12 - Poster Session 12 (5:30 PM-6:30 PM)

Poster/Presentation
Number 4-008

Objective This study aimed to assess whether energy expenditure index (EEI) after the fast walking in individuals with adrenomyeloneuropathy (AMN) is associated with perceived exertion, impaired gait, postural sway, spasticity, and disease severity.

Background AMN is a progressive X-linked peroxisomal disorder presenting during adulthood. AMN is long tract disorder involving dorsal columns and corticospinal tracts, characterized by sensory ataxia, spastic paraparesis and bowel/bladder dysfunction. The ratio of heart rate (HR) per meter walk is a valid scale to represent energy consumption during walking and is known as the EEI.

Design/Methods

We conducted a cross-sectional analysis of baseline data collected from a clinical trial in adults with AMN. Perceived exertion was quantified in patients using the modified Borg scale (MBS). Gait parameters were measured by 3D motion capture at fast walking and postural sway was assessed in four static standing positions. The Ashworth scale and expanded disability status scale (EDSS) were used to evaluate spasticity and disease severity, respectively. The analysis was performed using multiple linear regressions adjusted for age and weight of participants.

Results Fifty-seven individuals (35 females and 22 males) with recorded resting HR and walking HR after the fast walking were included. EEI was significantly associated with MBS in both females (R²: 0.44, P-value: 0.0001) and males (R²: 0.68, P-value: 0.0001). Among females, EEI was correlated with cadence (R²: 0.35, P-value: 0.0001), stride length (R²: 0.32, P-value: 0.0003), double support time (R²: 0.29, P-value: 0.006), spasticity (R²: 0.22, P-value: 0.004), and pyramidal EDSS (R²: 0.18, P-value: 0.009). EEI was associated with pyramidal EDSS (R²: 0.39, P-value: 0.001), spasticity (R²: 0.39, P-value: 0.001), and cadence (R²: 0.27, P-value: 0.003) among males.

Conclusions

AMN symptoms correlate with increased effort with mobility, and EEI may be a mean to measure the impact of the severity of AMN symptoms on patients' quality of life.

Authors/Disclosures
Manouchehr Amanat Yazdi, MD PRESENTER	Mr. Amanat has nothing to disclose.
	No disclosure on file
	No disclosure on file
Gerald Raymond, MD (Johns Hopkins)	Dr. Raymond has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Bluebird bio. Dr. Raymond has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. Dr. Raymond has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Travere. Dr. Raymond has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant with Dept of HHS.
Bela Turk, MD (Kennedy Krieger Institute)	Dr. Turk has received intellectual property interests from a discovery or technology relating to health care. Dr. Turk has received publishing royalties from a publication relating to health care.
Seyed A. Fatemi, MD, MBA (Kennedy Krieger Institute, Johns Hopkins Medical Institute)	Dr. Fatemi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for RCO law. The institution of Dr. Fatemi has received research support from Minoryx. The institution of Dr. Fatemi has received research support from Viking Therapeutics. The institution of Dr. Fatemi has received research support from Swanbio Therapeutics . The institution of Dr. Fatemi has received research support from Autobahn Therapeutics. The institution of Dr. Fatemi has received research support from Nuvelution. The institution of Dr. Fatemi has received research support from NIH. The institution of Dr. Fatemi has received research support from A Cure for Ellie Foundation. The institution of Dr. Fatemi has received research support from Brian's Hope Foundation . Dr. Fatemi has received intellectual property interests from a discovery or technology relating to health care. Dr. Fatemi has a non-compensated relationship as a Director (Board member) with ALDConnect that is relevant to AAN interests or activities.
Amena S. Fine, MD, PhD (Kennedy Krieger Institute)	The institution of Dr. Fine has received research support from NIH.

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