Gait Difficulties and Postural Instability in Adrenoleukodystrophy.

Background: Gait and balance difficulties are among the most common clinical manifestations in adults with X-linked adrenoleukodystrophy, but little is known about the contributions of sensory loss, motor dysfunction, and postural control to gait dysfunction and fall risk. Objective: To quantify gait and balance deficits in both males and females with adrenoleukodystrophy and evaluate how environmental perturbations (moving surfaces and visual surrounds) affect balance and fall risk. Methods: We assessed sensory and motor contributions to gait and postural instability in 44 adult patients with adrenoleukodystrophy and 17 healthy controls using three different functional gait assessments (25 Foot Walk test, Timed Up and Go, and 6 Minute Walk test) and computerized dynamic posturography. Results: The median Expanded Disability Status Scale score for the patient cohort was 3.0 (range 0.0-6.5). Both males and females with adrenoleukodystrophy showed impairments on all three functional gait assessments relative to controls (P < 0.001). Performance on walking tests and Expanded Disability Status Scale scores correlated with incidence of falls on computerized dynamic posturography, with the 25 Foot Walk being a moderately reliable predictor of fall risk (area under the ROC curve = 0.7675, P = 0.0038). Conclusion: We demonstrate that gait difficulties and postural control deficits occur in patients with adrenoleukodystrophy, albeit at an older age in females. Postural deficits were aggravated by eyes closed and dynamic conditions that rely on vestibular input, revealing challenges to the interplay of motor, sensory and vestibular circuitry in adrenoleukodystrophy.

The effect of vibrotactile feedback on postural sway during locomotor activities.

BACKGROUND: Although significant progress has been achieved in developing sensory augmentation methods to improve standing balance, attempts to extend this research to locomotion have been quite limited in scope. The goal of this study was to characterize the effects of two real-time feedback displays on locomotor performance during four gait-based tasks ranging in difficulty. METHODS: Seven subjects with vestibular deficits used a trunk-based vibrotactile feedback system that provided real-time feedback regarding their medial-lateral (M/L) trunk tilt when they exceeded a subject-specific predefined tilt threshold during slow and self-paced walking, walking along a narrow walkway, and walking on a foam surface. Two feedback display configurations were evaluated: the continuous display provided real-time continuous feedback of trunk tilt, and the gated display provided feedback for 200 ms during the period immediately following heel strike. The root-mean-square (RMS) trunk tilt and percentage of time below the tilt thresholds were calculated for all locomotor tasks. RESULTS: Use of continuous feedback resulted in significant decreases in M/L trunk tilt and increases in percentage times below the tilt thresholds during narrow and foam trials. The gated display produced generally smaller changes. CONCLUSIONS: This preliminary study demonstrated that use of continuous vibrotactile feedback during challenging locomotor tasks allowed subjects with vestibular deficits to significantly decrease M/L RMS trunk tilt. Analysis of the results also showed that continuous feedback was superior.

Biofeedback improves postural control recovery from multi-axis discrete perturbations.

BACKGROUND: Multi-axis vibrotactile feedback has been shown to significantly reduce the root-mean-square (RMS) sway, elliptical fits to sway trajectory area, and the time spent outside of the no feedback zone in individuals with vestibular deficits during continuous multidirectional support surface perturbations. The purpose of this study was to examine the effect of multidirectional vibrotactile biofeedback on postural stability during discrete multidirectional support surface perturbations. METHODS: The vibrotactile biofeedback device mapped tilt estimates onto the torso using a 3-row by 16-column tactor array. The number of columns displayed was varied to determine the effect of spatial resolution upon subject response. Torso kinematics and center of pressure data were measured in six subjects with vestibular deficits. Transient and steady state postural responses with and without feedback were characterized in response to eight perturbation directions. Four feedback conditions in addition to the tactors off (no feedback) configuration were evaluated. Postural response data captured by both a force plate and an inertial measurement unit worn on the torso were partitioned into three distinct phases: ballistic, recovery, and steady state. RESULTS: The results suggest that feedback has minimal effects during the ballistic phase (body's outbound trajectory in response to the perturbation), and the greatest effects during the recovery (return toward baseline) and steady state (post-recovery) phases. Specifically, feedback significantly decreases the time required for the body tilt to return to baseline values and significantly increases the velocity of the body's return to baseline values. Furthermore, feedback significantly decreases root mean square roll and pitch sway and significantly increases the amount of time spent in the no feedback zone. All four feedback conditions produced comparable performance improvements. Incidences of delayed and uncontrolled responses were significantly reduced with feedback while erroneous (sham) feedback resulted in poorer performance when compared with the no feedback condition. CONCLUSIONS: The results show that among the displays evaluated in this study, no one tactor column configuration was optimal for standing tasks involving discrete surface perturbations. Feedback produced larger effects on body tilt versus center of pressure parameters. Furthermore, the subjects' performance worsened when erroneous feedback was provided, suggesting that vibrotactile stimulation applied to the torso is actively processed and acted upon rather than being responsible for simply triggering a stiffening response.

Assessment of vibrotactile feedback on postural stability during pseudorandom multidirectional platform motion.

This study uses frequency-domain techniques and stabilogram diffusion analysis (SDA) to investigate the effect of vibrotactile feedback during continuous multidirectional perturbations of a support platform. Eight subjects with vestibular deficits were subjected to two-axis pseudorandom surface platform motion while donning a multiaxis vibrotactile feedback device that mapped body tilt estimates onto their torsos via a 3-row by 16-column array of tactile vibrators (tactors). Four tactor display configurations with spatial resolutions ranging between 22.5 degrees and 90 degrees, in addition to the tactors off configuration, were evaluated. Power spectral density functions of body sway in the anterior-posterior (A/P) and medial-lateral (M/L) directions, and transfer functions between platform motion and body sway, were computed at frequencies ranging from 0.0178 to 3.56 Hz. Cross-spectral analysis revealed that the A/P responses were not significantly driven by M/L inputs, and vice versa, thus supporting the notion of independent A/P and M/L postural control. Vibrotactile feedback significantly decreased A/P and M/L spectral power, decreased transfer function gains up to a frequency of 1.8 and 0.6 Hz in the A/P and M/L directions, respectively, and increased phase leads above 0.3 Hz. SDA showed significantly decreased transition time for both A/P and M/L tilts, and decreased transition displacement and short-term diffusion coefficients for A/P tilt. However, the spatial resolution of the tactor displays did not affect subjects' performance, thereby supporting the use of a lower spatial resolution display in future device designs.

Optokinetic nystagmus as a measure of visual function in severely visually impaired patients.

PURPOSE: To evaluate the efficacy of using optokinetic nystagmus (OKN) as an objective measurement of vision in severely visually impaired patients, in whom it is difficult to measure visual function reliably. Objective visual acuity (VA) measurements would be useful in the pre-and postoperative assessment of severely visually impaired patients who are potential candidates for visual rehabilitation strategies, such as retinal prostheses, neural and stem cell transplantation, and molecular approaches. METHODS: Full-field visual stimuli were used to evoke horizontal OKN responses in 17 subjects. Eye movements were recorded and analyzed to determine the maximum stimulus velocity (V(max)) at which subjects could maintain an OKN response. This endpoint was compared to logMAR VA and Goldmann visual field (VF) test RESULTS: results. V(max) was dependent on VA, VF, and the spatial frequency (SF) of the stimulus, yielding the equation V(max) = 14.2 . log(VA) - 6.20 . log(SF) + 0.22 . VF + 25.0. The findings suggest that V(max) in the presence of full-field OKN stimuli may provide an objective measure of VA and peripheral vision. CONCLUSIONS: OKN testing may be useful as an additional, more objective means of assessing visual function in a select group of severely visually impaired patients who are being considered as candidates for new visual rehabilitative strategies.