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Gait Difficulties and Postural Instability in Adrenoleukodystrophy.

Neha P Godbole1, Reza Sadjadi1,2, Madeline A DeBono1

  • 1Department of Neurology, Massachusetts General Hospital, Boston, MA, United States.

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|July 5, 2021
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Gait and balance problems are common in X-linked adrenoleukodystrophy (X-ALD). This study quantifies deficits and identifies the 25 Foot Walk test as a predictor of fall risk in X-ALD patients.

Keywords:
adrenoleukodystrophybalancecomputerized dynamic posturographygaitmyelopathyspinal cord disease

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Area of Science:

  • Neurology
  • Movement Science
  • Genetics

Background:

  • Gait and balance impairments are common in X-linked adrenoleukodystrophy (X-ALD).
  • Contributions of sensory loss, motor dysfunction, and postural control to gait dysfunction and fall risk in X-ALD are not well understood.
  • Deficits may manifest differently in males and females.

Purpose of the Study:

  • Quantify gait and balance deficits in adult males and females with X-ALD.
  • Evaluate the impact of environmental perturbations (moving surfaces, visual surrounds) on balance and fall risk.
  • Identify predictors of fall risk in X-ALD patients.

Main Methods:

  • Assessed 44 adult X-ALD patients and 17 healthy controls.
  • Utilized functional gait assessments (25 Foot Walk, Timed Up and Go, 6 Minute Walk tests).
  • Employed computerized dynamic posturography to evaluate sensory and motor contributions to postural instability.

Main Results:

  • X-ALD patients exhibited significant impairments in all functional gait assessments compared to controls (P < 0.001).
  • Performance on walking tests and Expanded Disability Status Scale scores correlated with fall incidence.
  • The 25 Foot Walk test demonstrated moderate reliability in predicting fall risk (AUC = 0.7675, P = 0.0038).

Conclusions:

  • Gait difficulties and postural control deficits are present in X-ALD patients, appearing later in females.
  • Postural deficits worsen under conditions challenging vestibular input (eyes closed, dynamic surfaces).
  • Findings highlight complex interplay issues within motor, sensory, and vestibular circuitry in X-ALD.