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ABSTRACT: Background
Hereditary sensory and autonomic neuropathy type III features marked ataxic gait that progressively worsens over time. We assessed whether proprioceptive disturbances can explain the ataxia.Methods
Proprioception at the knee joint was assessed using passive joint angle matching in 18 patients and 14 age-matched controls; 5 patients with cerebellar ataxia were also studied. Ataxia was quantified using the Brief Ataxia Rating Score, which ranged from 7 to 26 of 30.Results
Neuropathy patients performed poorly in judging joint position: mean absolute error was 8.7° ± 1.0°, and the range was very wide (2.8°-18.1°); conversely, absolute error was only 2.7° ± 0.3° (1.6°-5.5°) in the controls and 3.0° ± 0.2° (2.1°-3.4°) in the cerebellar patients. This error was positively correlated to the degree of ataxia in the neuropathy patients but not the cerebellar patients.Conclusions
These results suggest that poor proprioceptive acuity at the knee joint is a major contributor to the ataxic gait associated with hereditary sensory and autonomic neuropathy type III.
SUBMITTER: Macefield VG
PROVIDER: S-EPMC3694996 | biostudies-literature | 2013 Jun
REPOSITORIES: biostudies-literature
Macefield Vaughan G VG Norcliffe-Kaufmann Lucy J LJ Axelrod Felicia B FB Kaufmann Horacio H
Movement disorders : official journal of the Movement Disorder Society 20130516 6
<h4>Background</h4>Hereditary sensory and autonomic neuropathy type III features marked ataxic gait that progressively worsens over time. We assessed whether proprioceptive disturbances can explain the ataxia.<h4>Methods</h4>Proprioception at the knee joint was assessed using passive joint angle matching in 18 patients and 14 age-matched controls; 5 patients with cerebellar ataxia were also studied. Ataxia was quantified using the Brief Ataxia Rating Score, which ranged from 7 to 26 of 30.<h4>Re ...[more]