Ambulatory disabilities and the use of walking aids in patients with Hereditary Motor and Sensory Neuropathy type I (HMSN I)

Abstract

Purpose. 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.

Conclusion. 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.

Keywords:

• Hereditary motor and sensory neuropathies
• disability evaluation
• mobility limitation
• sickness impact profile
• quality of life
• motor activity