The motor function measure to study limitation of activity in children and adults with Charcot-Marie-Tooth disease.

OBJECTIVE: To study the applicability and responsiveness of the motor function measure (total score and sub-scores D1, D2 and D3) in patients with Charcot-Marie-Tooth disease. PATIENTS AND METHODS: Two hundred and thirty-three patients aged 4-86 years were included in the descriptive study. Scores and sub-scores were analyzed by age and by disease subtypes. Sensitivity to change (responsiveness) was estimated in patients having had at least two evaluations with at least six months between the first and the second. RESULTS: Motor function measure scores decrease with age, especially sub-scores D1 and D3. There were no significant differences between the scores according to type of Charcot-Marie-Tooth disease. The scores were significantly higher for ambulatory than for non-ambulatory patients. Significant responsiveness was demonstrated only in type 2 Charcot-Marie-Tooth disease. DISCUSSION/CONCLUSIONS: Our results suggest that, especially for D1 and D3 sub-scores, the motor function measure is a reliable and valid outcome measure that can be usefully applied in longitudinal follow-up. Studies of longer duration could demonstrate its responsiveness in other Charcot-Marie-Tooth disease subtypes.

Acquired visual field defects rehabilitation: critical review and perspectives.

Visual field deficit (VFD) is one of the most commonly observed symptoms following brain injury. Persistent VFD and defective exploratory oculomotor scanning patterns often cause severe impairment in daily activities, particularly as regards visual exploration and reading. Homonymous hemianopia is consequently a powerful negative predictor of patient outcome. In spite of these quantitative and qualitative factors, there currently exists no consensus on rehabilitative therapy and treatment. Different approaches have nevertheless been developed, all of them having one therapeutic principle in common; repeated practice of a specific visual task, with the hope/expectation that improved performance will extend to a wide range of ecologically useful visual functions. The four main available methods aim at replacing part of the intact visual field with part of the damaged visual field (optical therapy using prisms), at partially restoring the lost visual field region (restorative therapies), at stimulating detection capacities in the blind field (stimulation or blindsight) or at substituting for the lost region by reorganizing the control of visual information processing and eye movements (compensatory therapies). This review explores the key data relative to these different approaches in terms of behavioral or imagery results. It also aims at critically analyzing the advantages and limits of each one. The importance of strict assessment in terms of deficiencies or disabilities is underlined. Finally, upon consideration of these data taken as a whole, it is suggested that efficient treatment would probably have to associate general components and more specific elements, according to what may be done with regard to other aspects of cognitive rehabilitation.