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Postural instability in Charcot-Marie-Tooth type 1A patients is strongly associated with reduced somatosensation

In order to determine the influence of somatosensory impairments, due to the loss
of large myelinated fibres, on the postural stability of Charcot-Marie-Tooth 1A
(CMT) patients, a cross-sectional balance assessment was done. Nine CMT patients
were compared with eight patients with a distal type of Spinal Muscular Atrophy
(SMA), and 11 healthy control subjects. The balance assessment consisted of four
tasks: quiet barefoot standing on a stable versus compliant surface, with eyes
opened or closed. Force plate signals were used to calculate the velocity of the
centre of pressure of the ground reaction forces. The patients' distal muscle
force (MRC scale), vibration detection threshold (Rydel-Seiffer tuning fork) and
superficial tactile sensation (Semmes-Weinstein monofilaments) were clinically
assessed. Compared to the healthy subjects, postural stability of both patient
groups was seriously impaired, however, increased visual dependency was only
found in the CMT patients. The postural instability of the CMT patients
correlated significantly with decreased vibration sense only. The strength of the
correlation increased with task complexity. It is concluded that somatosensory
deficits substantially contribute to impaired postural stability and increased
visual dependency in CMT patients.
CI - Copyright 2010 Elsevier B.V. All rights reserved.

Langue : ANGLAIS

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