Ambulatory disabilities and the use of walking aids in patients with hereditary motor and sensory neuropathy type I (HMSN I).
OBJECTIVE
To determine the level of ambulatory disability and the use of walking aids in well-ambulant Hereditary Motor and Sensory Neuropathy type I (HMSN I) patients, and to identify the related demographic, physical and psychological variables.


METHODS
Seventy-five well-ambulant HMSN I patients, aged 20-58 years, were measured in a cross-sectional assessment, addressing disability of ambulation and mobility (Sickness Impact Profile), demographics, muscle strength (Medical Research Council), use of walking aids, physical activity (actometer), fatigue (Checklist Individual Strength), and quality of life (EuroQoL).


RESULTS
Seventy-two percent of the patients perceived a significant amount of ambulatory disability. These patients were less active, and more fatigued compared to patients without ambulatory disability, and healthy reference groups. The total patient sample showed marked distal paresis (mean MRC = 3.3), a high level of pain-discomfort (76%), but normal levels of employment (62.7%) and anxiety-depression (20%). Walking aids were used by 49% of the patients. These patients were older, less active, more fatigued, had less muscle strength, and perceived more disabilities of ambulation and mobility than non-users. Of the patients without walking aids, 41% perceived a significant amount of ambulatory disabilities.


CONCLUSIONS
Ambulatory disability frequently occurred in well-ambulant HMSN I patients. The use of walking aids was not completely in accordance with the perceived ambulatory disability. Therefore prescription requires specific attention as well as complaints about pain and fatigue.
van der Linden
Marleen H
MH
Department of Rehabilitation Medicine, Radboud University Nijmegen Medical Centre, The Netherlands.
Kalkman
Joke S
JS
Hendricks
Henk T
HT
Schillings
Maartje L
ML
Zwarts
Machiel J
MJ
Bleijenberg
Gijs
G
van Engelen
Baziel G M
BG
D000328:Adult; D003430:Cross-Sectional Studies; D004185:Disability Evaluation; D006233:Persons with Disabilities / Q000534:rehabilitation*; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000534:rehabilitation*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009983:Orthopedic Equipment / Q000706:statistics & numerical data*; D012959:Socioeconomic Factors