Turning strategies in patients with cerebellar ataxia

Mari, Silvia; Serrao, Mariano; Casali, Carlo; Conte, Carmela; Ranavolo, Alberto; Padua, Luca; Draicchio, Francesco; Iavicoli, Sergio; Monamì, Stefano; Sandrini, Giorgio; Pierelli, Francesco

doi:10.1007/s00221-012-3197-2

Cited by 38 publications

(36 citation statements)

References 30 publications

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“…Studies have demonstrated that, compared to healthy control subjects, ataxic patients exhibit shorter stride lengths [35–38] and larger stance widths [33, 37–40] during walking or turning, results similar to those in rats with olivocerebellar lesions. Further, the delayed heel-off time (i.e., approximately the beginning point of propulsion) and decreased duration from heel-off to toe-off observed in ataxic patients [35] are highly similar to the increased braking duration and decreased propulsion duration, respectively, exhibited by rats following the administration of 3-AP.…”

Section: Discussionmentioning

confidence: 96%

Gait analysis and the cumulative gait index (CGI): Translational tools to assess impairments exhibited by rats with olivocerebellar ataxia

Lambert¹,

Philpot²,

Engberg³

et al. 2014

Behavioural Brain Research

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Deviations from ‘normal’ locomotion exhibited by humans and laboratory animals may be determined using automated systems that capture both temporal and spatial gait parameters. Although many measures generated by these systems are unrelated and independent, some may be related and dependent, representing redundant assessments of function. To investigate this possibility, a treadmill-based system was used to capture gait parameters from normal and ataxic rats, and a multivariate analysis was conducted to determine deviations from normal. Rats were trained on the treadmill at two speeds, and gait parameters were generated prior to and following lesions of the olivocerebellar pathway. Control (non-lesioned) animals exhibited stable hindlimb gait parameters across assessments at each speed. Lesioned animals exhibited alterations in multiple hindlimb gait parameters, characterized by significant increases in stride frequency, braking duration, stance width, step angle, and paw angle and decreases in stride, stance, swing and propulsion durations, stride length and paw area. A principal component analysis of initial hindlimb measures indicated 3 uncorrelated factors mediating performance, termed rhythmicity, thrust and contact. Deviation in the performance of each animal from the group mean was determined for each factor and values summed to yield the Cumulative Gait Index (CGI), a single value reflecting variation within the group. The CGI for lesioned animals increased 2.3-fold relative to unlesioned animals. This study characterizes gait alterations in laboratory rats rendered ataxic by destruction of the climbing fiber pathway innervating Purkinje cells and demonstrates that a single index can be used to describe overall gait impairments.

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Section: Discussionmentioning

confidence: 96%

Gait analysis and the cumulative gait index (CGI): Translational tools to assess impairments exhibited by rats with olivocerebellar ataxia

Lambert¹,

Philpot²,

Engberg³

et al. 2014

Behavioural Brain Research

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“…Similarly, voluntary changes in step width and length have been shown to alter the stability of trunk motion [27]. In an examination of turning strategies of individuals with cerebellar ataxia, Mari et al [28] showed that during a demanding gait task, such as turning, ataxic patients displayed wider and shorter steps. The authors theorized that the observed gait difference between healthy and ataxic individuals was a compensatory strategy used to reduce instability.…”

Section: Discussionmentioning

confidence: 99%

Early presentation of gait impairment in Wolfram Syndrome

Pickett

Duncan

Hoekel

et al. 2012

Orphanet J Rare Dis

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BackgroundClassically characterized by early onset insulin-dependent diabetes mellitus, optic atrophy, deafness, diabetes insipidus, and neurological abnormalities, Wolfram syndrome (WFS) is also associated with atypical brainstem and cerebellar findings in the first decade of life. As such, we hypothesized that gait differences between individuals with WFS and typically developing (TD) individuals may be detectable across the course of the disease.MethodsGait was assessed for 13 individuals with WFS (min 6.4 yrs, max 25.8 yrs) and 29 age-matched, typically developing individuals (min 5.6 yrs, max 28.5 yrs) using a GAITRite ® walkway system. Velocity, cadence, step length, base of support and double support time were compared between groups.ResultsAcross all tasks, individuals with WFS walked slower (p = 0.03), took shorter (p ≤ 0.001) and wider (p ≤ 0.001) steps and spent a greater proportion of the gait cycle in double support (p = 0.03) compared to TD individuals. Cadence did not differ between groups (p = 0.62). Across all tasks, age was significantly correlated with cadence and double support time in the TD group but only double support time was correlated with age in the WFS group and only during preferred pace forward (rs= 0.564, p = 0.045) and dual task forward walking (rs= 0.720, p = 0.006) tasks. Individuals with WFS also had a greater number of missteps during tandem walking (p ≤ 0.001). Within the WFS group, spatiotemporal measures of gait did not correlate with measures of visual acuity. Balance measures negatively correlated with normalized gait velocity during fast forward walking (rs = −0.59, p = 0.03) and percent of gait cycle in double support during backward walking (rs = −0.64, p = 0.03).ConclusionsQuantifiable gait impairments can be detected in individuals with WFS earlier than previous clinical observations suggested. These impairments are not fully accounted for by the visual or balance deficits associated with WFS, and may be a reflection of early cerebellar and/or brainstem abnormalities. Effective patient-centered treatment paradigms could benefit from a more complete understanding of the progression of motor and other neurological symptom presentation in individuals with WFS.

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“…These findings have revealed biomechanical abnormalities in spatiotemporal parameters, center of mass (CoM) and center of pressure (CoP) trajectories, joint kinematics and kinetics, muscles activation pattern and upper body control [118–123]. Patients have difficulties in steady state linear walking, but also in gait initiation, termination, and turning [124–129]. All of these locomotor abnormalities reflect poor limb coordination and impaired balance, which greatly restrict patients in their activities of daily life and predispose them to falls [130].…”

Section: Gait/posture In Cerebellar Disorders (Carlo Casali and Mariamentioning

confidence: 99%

Consensus Paper: Revisiting the Symptoms and Signs of Cerebellar Syndrome

Bodranghien¹,

et al. 2015

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The cerebellum is involved in sensorimotor operations, cognitive tasks and affective processes. Here, we revisit the concept of the cerebellar syndrome in the light of recent advances in our understanding of cerebellar operations. The key symptoms and signs of cerebellar dysfunction, often grouped under the generic term of ataxia, are discussed. Vertigo, dizziness, and imbalance are associated with lesions of the vestibulo-cerebellar, vestibulo-spinal, or cerebellar ocular motor systems. The cerebellum plays a major role in the online to long-term control of eye movements (control of calibration, reduction of eye instability, maintenance of ocular alignment). Ocular instability, nystagmus, saccadic intrusions, impaired smooth pursuit, impaired vestibulo-ocular reflex (VOR), and ocular misalignment are at the core of oculomotor cerebellar deficits. As a motor speech disorder, ataxic dysarthria is highly suggestive of cerebellar pathology. Regarding motor control of limbs, hypotonia, a- or dysdiadochokinesia, dysmetria, grasping deficits and various tremor phenomenologies are observed in cerebellar disorders to varying degrees. There is clear evidence that the cerebellum participates in force perception and proprioceptive sense during active movements. Gait is staggering with a wide base, and tandem gait is very often impaired in cerebellar disorders. In terms of cognitive and affective operations, impairments are found in executive functions, visual-spatial processing, linguistic function, and affective regulation (Schmahmann’s syndrome). Nonmotor linguistic deficits including disruption of articulatory and graphomotor planning, language dynamics, verbal fluency, phonological, and semantic word retrieval, expressive and receptive syntax, and various aspects of reading and writing may be impaired after cerebellar damage. The cerebellum is organized into (a) a primary sensorimotor region in the anterior lobe and adjacent part of lobule VI, (b) a second sensorimotor region in lobule VIII, and (c) cognitive and limbic regions located in the posterior lobe (lobule VI, lobule VIIA which includes crus I and crus II, and lobule VIIB). The limbic cerebellum is mainly represented in the posterior vermis. The cortico-ponto-cerebellar and cerebello-thalamocortical loops establish close functional connections between the cerebellum and the supratentorial motor, paralimbic and association cortices, and cerebellar symptoms are associated with a disruption of these loops.

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Turning strategies in patients with cerebellar ataxia

Cited by 38 publications

References 30 publications

Gait analysis and the cumulative gait index (CGI): Translational tools to assess impairments exhibited by rats with olivocerebellar ataxia

Gait analysis and the cumulative gait index (CGI): Translational tools to assess impairments exhibited by rats with olivocerebellar ataxia

Early presentation of gait impairment in Wolfram Syndrome

Consensus Paper: Revisiting the Symptoms and Signs of Cerebellar Syndrome

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