Dataset Information

Wearable sensors detect impaired gait and coordination in LBSL during remote assessments.

ABSTRACT:

Background

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a rare leukodystrophy with motor impairment due to biallelic mutations in DARS2, which encodes mitochondrial aspartyl tRNA synthetase. Progressive ataxia is the primary feature.

Objective

The study objective is to determine the feasibility of remotely collecting quantitative gait and balance measures in LBSL.

Methods

The study design uses wearable accelerometers and the scale for the assessment and rating of ataxia (SARA) scale to assess gait and postural sway in LBSL and control participants' homes through video conferencing.

Results

Lateral step variability (LSV), which indicates stride variability, and elevation of the step at mid-swing are increased for LBSL patients during brief walking tests. During stance with the eyes closed, LBSL participants show rapid accelerations and decelerations of body movement covering a large sway area and path. Both the LSV and sway area during stance with the feet together and eyes closed correlate strongly with the SARA.

Conclusions

Wearable accelerometers are valid and sensitive for detecting ataxia in LBSL patients during remote assessments. The finding of large increases in the sway area during stance with the eyes closed is intriguing since dorsal column dysfunction is universally seen in LBSL. This approach can be applied to related rare diseases that feature ataxia.

SUBMITTER: Smith Fine A

PROVIDER: S-EPMC8994975 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature

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Publications

Wearable sensors detect impaired gait and coordination in LBSL during remote assessments.

Smith Fine Amena A Kaufman Miriam M Goodman Jordan J Turk Bela B Bastian Amy A Lin Doris D Fatemi Ali A Keller Jennifer J

Annals of clinical and translational neurology 20220308 4

<h4>Background</h4>Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a rare leukodystrophy with motor impairment due to biallelic mutations in DARS2, which encodes mitochondrial aspartyl tRNA synthetase. Progressive ataxia is the primary feature.<h4>Objective</h4>The study objective is to determine the feasibility of remotely collecting quantitative gait and balance measures in LBSL.<h4>Methods</h4>The study design uses wearable accelerometers and the ...[more]

PMID: 35257509

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Dataset Information

Wearable sensors detect impaired gait and coordination in LBSL during remote assessments.

Background

Objective

Methods

Results

Conclusions

Publications

Wearable sensors detect impaired gait and coordination in LBSL during remote assessments.

Similar Datasets

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