## Abstract Very late‐onset Friedreich's ataxia (VLOFA) is characterized by symptomatic onset after 40 years of age and, usually, a benign phenotype. We describe a sporadic case with onset at 53 years of age and a novel VLOFA phenotype mimicking multiple system atrophy (MSA) of cerebellar type asso
Perturbation of precision grip in Friedreich's ataxia and late-onset cerebellar ataxia
✍ Scribed by Dr. Joachim Hermsdöourfer; Karl Wessel; Norbert Mai; Christian Marquardt
- Publisher
- John Wiley and Sons
- Year
- 1994
- Tongue
- English
- Weight
- 463 KB
- Volume
- 9
- Category
- Article
- ISSN
- 0885-3185
No coin nor oath required. For personal study only.
✦ Synopsis
Abstract
Perturbations of precision grip were tested in 7 patients with Friedreich's ataxia (FA) and 11 patients with late‐onset cerebellar ataxia (CA). Subjects were instructed to hold a small compressible manipulandum between thumb and index finger and to resist any perturbation of maintained finger position. A sudden increase of load induced a displacement of fingers until this was stopped by subjects' active intervention. The amount of initial displacement emerged as a highly sensitive parameter to differentiate the clinical subgroups: Responses in FA patients were missing or massively delayed, whereas displacements in CA patients were normal or only moderately abnormal. This discrimination of impaired hand function in FA and CA patients has not been possible by using only tasks of isometric grip force control. We concluded that our task relies more on intact sensory afferents, which are known to be impaired in FA, than on cerebellar function. In a second task the stiffness of the maintained grip was determined. On the average, preresponse stiffness was lower in FA patients as compared with CA patients and normal controls. However, stiffness appeared to be an independent parameter that did not influence the amount of displacement in the perturbation task.
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