International validation of meaningfulness of postural sway and gait to assess myeloneuropathy in adults with adrenoleukodystrophy.

Autor: Yska HAF; Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands., Turk BR; Kennedy Krieger Institute, Baltimore, Maryland, USA.; Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland, USA., Fatemi A; Kennedy Krieger Institute, Baltimore, Maryland, USA.; Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland, USA., Goodman J; Kennedy Krieger Institute, Baltimore, Maryland, USA., Voermans M; Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands., Amos D; Kennedy Krieger Institute, Baltimore, Maryland, USA., Amanat M; Kennedy Krieger Institute, Baltimore, Maryland, USA., van de Stadt S; Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands., Engelen M; Emma Children's Hospital, Department of Neurology and Pediatric Neurology, Amsterdam Leukodystrophy Center, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands., Smith-Fine A; Kennedy Krieger Institute, Baltimore, Maryland, USA.; Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland, USA., Keller J; Kennedy Krieger Institute, Baltimore, Maryland, USA.; Department of Physical Medicine & Rehabilitation, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
Jazyk: angličtina
Zdroj: Journal of inherited metabolic disease [J Inherit Metab Dis] 2024 Nov; Vol. 47 (6), pp. 1336-1347. Date of Electronic Publication: 2024 May 25.
DOI: 10.1002/jimd.12753
Abstrakt: Background: The most common manifestation of X-linked adrenoleukodystrophy (ALD) is a slowly progressive myeloneuropathy, which leads to imbalance and gait disturbances. The variable progression of the disease complicates evaluation of its progression rate. Wearable sensors allow for easy and frequent balance and gait collection. This study reports baseline data from a longitudinal study on the quantitative assessment of balance and gait with wearable sensors and their clinical relevance.
Methods: Data were collected from adult patients in two institutions. Postural body sway and gait parameters were measured using accelerometers. Disease severity was measured by the Expanded Disability Severity Scale (EDSS). Falling frequency and quality of life (QOL) were collected in men. The relationship between sway and gait variables and EDSS score, participants' use of a walking aid, and falling frequency was evaluated.
Results: One hundred twenty individuals with ALD were included. Sway variables significantly differentiate participants' assistive device use. Sway and gait variables were correlated to the EDSS in both sexes. Both gait speed and sway were correlated with falling frequency in men from one institution. Select QOL subscores were correlated with the EDSS in males from one institution. Accelerometry generated comparable results across sites.
Discussion: This study confirms the clinical correlation between spinal cord disease and imbalance and gait in ALD. For the first time, this study shows clinically meaningful relationships for sway and gait with use of an assistive device, falling frequency and QOL. Wearable accelerometers are a valid means to measure sway and gait in ALD. These measures are promising outcomes for clinical trial designs to assess myeloneuropathy in ALD and to monitor disease progression in individuals.
(© 2024 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.)
Databáze: MEDLINE
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