Evidence established in large study
Mobile digital gait measurements in this large study of 55 people with HSP over more than six years has established that changes in gait are meaningful measures of HSP progression.
The changes in gait were correlated with clinical measures such as score on the Spastic Paraplegia Rating Scale (SPRS) and participant reported factors such as fear of falling and quality of life.
The potential exists for development of these mobile digital gait measurements into a biomarker of disease status and progression suitable for use in clinical trials, which is something badly needed and currently lacking.
Abstract
Progressive spasticity and gait impairment are the functional hallmarks 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.
SOURCE: Ann Clin Transl Neurol. 2023 Jan 9. doi: 10.1002/acn3.51725. Online ahead of print. PMID: 36622133 © 2023 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.
Mobile digital gait analysis objectively measures progression in hereditary spastic paraplegia
Evelyn Loris 1 , Malte Ollenschläger 1 2 , Teresa Greinwalder 1 , Björn Eskofier 2 , Jürgen Winkler 1 3 , Heiko Gaßner 1 4 , Martin Regensburger 1 3
1. Department of Molecular Neurology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany.
2. Machine Learning and Data Analytics Lab, Department Artificial Intelligence in Biomedical Engineering, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany.
3. Center for Rare Diseases Erlangen (ZSEER), Universitätsklinikum Erlangen, Erlangen, Germany.
4. Fraunhofer IIS, Fraunhofer Institute for Integrated Circuits IIS, 91058, Erlangen, Germany.
My HSP came on only as I reached 70 yo. But it is getting much worse and now at 80 have lost my mobility (I use an electric wheelchair or scooter). Have full use of my arms and very little dementia but some disphagia. Will I get ALS symptoms as I get older?
Editor’s Note: Firstly – HSP does not become ALS/motor neurone disease. If you have HSP, you have HSP. Secondly – there can be some symptom overlap between HSP and ALS, and indeed, some symptom overlap between several neurodegenerative disorders. Thirdly, there is no way to predict HSP progression in any particular case, either in type of symptoms or their severity. While general statements about type of symptoms, severity of symptoms and rate of disease progression can be made about different forms and types of the HSPs, the evidence base for predictability at the individual level is currently lacking.