2020, Article / Letter to editor (Orphanet Journal of Rare Diseases, vol. 15, iss. 1, (2020))Background Hereditary spastic paraplegia (HSP) is a group of inherited disorders characterized by progressive spastic paresis of the lower limbs. Treatment is often focused on reducing spasticity and its physical consequences. To better address individual patients' needs, we investigated a broad range of experienced complaints, activity limitations, and loss of motor capacities in pure HSP. In addition, we aimed to identify patient characteristics that are associated with increased fall risk and/or reduced walking capacity. Methods We developed and distributed an HSP-specific online questionnaire in the Netherlands. A total of 109 out of 166 questionnaires returned by participants with pure HSP were analyzed. Results Participants experienced the greatest burden from muscle stiffness and limited standing and walking activities, while 72% reported leg and/or back pain. Thirty-five and 46% reported to use walking aids (e.g. crutches) indoors and outdoors, respectively; 57% reported a fall incidence of at least twice a year ('fallers'); in 51% a fall had led to an injury at least once; and 73% reported fear of falling. Duration of spasticity and incapacity to rise from the floor were positively associated with being a 'faller', whereas non-neurological comorbidity and wheelchair use were negatively associated. Higher age, experienced gait problems, not being able to stand for 10 min, and incapacity to open a heavy door showed a negative association with being a 'walker without aids' (> 500 m). Conclusions Our results emphasize the large impact of spastic paraparesis on the lives of people with pure HSP and contribute to a better understanding of possible targets for rehabilitation.
2012, Article / Letter to editor (Gait & Posture, vol. 36, iss. 1, (2012), pp. 127-132)The objective of the present study was to examine the attentional demands of gait adaptations required to walk over irregular terrain in community-dwelling people with chronic stroke. Eight community ambulators (>6 months post-stroke, aged 57 ± 15 years) and eight age-matched healthy controls participated in the study. As the primary motor task, participants walked on a treadmill while they quickly reacted to a sudden obstacle in front of the affected (in the stroke group) or left (in healthy controls) leg. The secondary, cognitive task was an auditory Stroop task. Outcomes were avoidance success rate and muscle reaction times of the biceps and rectus femoris (motor task), and a composite score of accuracy and verbal reaction time (cognitive task). Success rates did not differ between single- and dual-task conditions in either group, while muscle reaction times deteriorated equally during the dual task in both groups. However, compared with the Stroop scores just before and after obstacle crossing, the scores while crossing the obstacle deteriorated more in the stroke group than in the controls (p=0.012). The higher dual-task costs on the Stroop task reflect greater attentional demands during walking and crossing obstacles. The absence of dual-task effects on obstacle avoidance performance suggests that the people with stroke used a "posture-first strategy". The results imply that common daily life tasks such as obstacle crossing while walking require disproportionate attention even in well-recovered people with stroke.