Stumbling, struggling, and shame due to spasticity: a qualitative study of adult persons with hereditary spastic paraplegia

Kerstens, Hans C. J. W.; Satink, Ton; Nijkrake, Maarten J.; Swart, B.J.M. de; Lith, B.J.H. van; Geurts, Alexander C. H.; Sanden, Maria W. G. Nijhuis-van der

doi:10.1080/09638288.2019.1610084

Cited by 17 publications

(23 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Our results further showed that pain, fatigue, and autonomic problems are major (non-motor) symptoms in patients with pure HSP. Only few previous studies have mentioned pain as an important problem in this population [15,16,18,37], even though pain in the legs and/or back was reported by 72% of our participants. This number was similar to the reported frequency in previous studies [15,18].…”

Section: Muscular and Non-motor Symptomscontrasting

confidence: 55%

“…The structure and content of the web-based survey were designed by a team of expert physicians, researchers, physical therapists, and persons with HSP. Part of the questionnaire was based on a previous international survey of patients living with spasticity [9], while other questions were based on a qualitative study in patients with pure HSP who were interviewed about the daily life consequences of spastic paraparesis and their related healthcare needs (note: data on healthcare needs are reported elsewhere) [16]. The structure and formulation of the questions and predefined answering options were improved during an iterative process, until all authors agreed on the final questionnaire.…”

Section: Web-based Questionnairementioning

confidence: 99%

“…In our clinical experience, low back pain often has a continuous character, possibly related to postural deviation (i.e., anterior pelvic tilt with lumbar hyperlordosis). On average, the severity of pain yielded a median score of 4 on a numeric rating scale (0-10), but from a recent qualitative study, using semi-structured interviews in 14 patients with pure HSP, we learned that, in individual patients, pain may be severe enough to seriously affect their quality of life [16]. Besides pain, many participants experienced fatigue, both physically and mentally, with median severity scores of 6 and 5, respectively.…”

Section: Muscular and Non-motor Symptomsmentioning

confidence: 99%

“…Besides pain, many participants experienced fatigue, both physically and mentally, with median severity scores of 6 and 5, respectively. From our recent qualitative study it became clear that spasticity and muscle stiffness impact on physical fitness, while the high levels of attention needed to cope with balance and gait problems seem to cause mental fatigue [16]. Generally, fatigue and pain are serious problems in many types of chronic neurological disorders such as spinal cord injury, stroke, Parkinson's disease, multiple sclerosis, and neuromuscular disease, which require specific clinical attention and treatment [30,[38][39][40][41][42].…”

Section: Muscular and Non-motor Symptomsmentioning

confidence: 99%

“…In addition, some studies mentioned the occurrence of pain, fatigue, urinary symptoms, sleeping problems, unpredictable day-to-day fluctuations, activity limitations and participation restrictions. However, these studies included either small patients samples [15,16] or lumped patients with pure and complex HSP [17][18][19].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands

Lith

Kerstens

Bemd

et al. 2020

Orphanet J Rare Dis

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Section: Muscular and Non-motor Symptomscontrasting

confidence: 55%

Section: Web-based Questionnairementioning

confidence: 99%

Section: Muscular and Non-motor Symptomsmentioning

confidence: 99%

Section: Muscular and Non-motor Symptomsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands

Lith

Kerstens

Bemd

et al. 2020

Orphanet J Rare Dis

Self Cite

View full text Add to dashboard Cite

show abstract

Mobile digital gait analysis objectively measures progression in hereditary spastic paraplegia

Loris

Ollenschläger

Greinwalder

et al. 2023

Ann Clin Transl Neurol

View full text Add to dashboard Cite

Progressive spasticity and gait impairment is the functional hallmark of hereditary spastic paraplegia (HSP), but due to inter‐individual variability, longitudinal studies on its progression are scarce. We investigated the progression of gait deficits via mobile digital measurements in conjunction with clinical and patient‐reported outcome parameters. Our cohort included adult HSP patients (n = 55) with up to 77 months of follow‐up. Gait speed showed a significant association with SPRS progression. Changes in stride time and gait variability correlated to fear of falling and quality of life, providing evidence that gait parameters are meaningful measures of HSP progression.

show abstract

Specific Gait Changes in Prodromal Hereditary Spastic Paraplegia Type 4: preSPG4 Study

et al. 2022

View full text Add to dashboard Cite

Background In hereditary spastic paraplegia type 4 (SPG4), subclinical gait changes might occur years before patients realize gait disturbances. The prodromal phase of neurodegenerative disease is of particular interest to halt disease progression by future interventions before impairment has manifested. Objective The objective of this study was to identify specific movement abnormalities before the manifestation of gait impairment and quantify disease progression in the prodromal phase. Methods Seventy subjects participated in gait assessment, including 30 prodromal SPAST pathogenic variant carriers, 17 patients with mild‐to‐moderate manifest SPG4, and 23 healthy control subjects. An infrared‐camera‐based motion capture system assessed gait to analyze features such as range of motion and continuous angle trajectories. Those features were correlated with disease severity as assessed by the Spastic Paraplegia Rating Scale, neurofilament light chain as a fluid biomarker indicating neurodegeneration, and motor‐evoked potentials. Results Compared with healthy control subjects, we found an altered gait pattern in prodromal pathogenic variant carriers during the swing phase in the segmental angle of the foot (Dunn's post hoc test, q = 3.1) and heel ground clearance (q = 2.8). Furthermore, range of motion of segmental angle was reduced for the foot (q = 3.3). These changes occurred in prodromal pathogenic variant carriers without quantified leg spasticity in clinical examination. Gait features correlated with neurofilament light chain levels, central motor conduction times of motor‐evoked potentials, and Spastic Paraplegia Rating Scale score. Conclusions Gait analysis can quantify changes in prodromal and mild‐to‐moderate manifest SPG4 patients. Thus, gait features constitute promising motor biomarkers characterizing the subclinical progression of spastic gait and might help to evaluate interventions in early disease stages. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society

show abstract

Stumbling, struggling, and shame due to spasticity: a qualitative study of adult persons with hereditary spastic paraplegia

Cited by 17 publications

References 25 publications

Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands

Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands

Mobile digital gait analysis objectively measures progression in hereditary spastic paraplegia

Specific Gait Changes in Prodromal Hereditary Spastic Paraplegia Type 4: preSPG4 Study

Contact Info

Product

Resources

About