Telemedicine Chapter 17: Telemedicine and Progressive Supranuclear Palsy

This chapter is part of Literature reviews carried out for the Heath Service Executive National Telehealth Steering Group April – July 2020

Systematic Reviews

Theodoros, Deborah et al (2019) [Systematic Review] Technology‐ enabled management of communication and swallowing disorders in Parkinson’s disease: a systematic scoping review[1]

Background: Communication and swallowing disorders are highly prevalent in people with Parkinson’s disease (PD). Maintenance of functional communication and swallowing over time is challenging for the person with PD and their families and may lead to social isolation and reduced quality of life if not addressed. Speech and language therapists (SLTs)face the conundrum of providing sustainable and flexible services to meet the changing needs of people with PD. Motor, cognitive and psychological issues associated with PD, medication regimens and dependency on others often impede attendance at a centre-based service. The access difficulties experienced by people with PD require a disruptive service approach to meet their needs. Technology-enabled management using information and telecommunications technologies to provide services at a distance has the potential to improve access, and enhance the quality of SLT services to people with PD. Aims: To report the status and scope of the evidence for the use of technology in the management of the communication and swallowing disorders associated with PD. Methods and Procedures: Studies were retrieved from four major databases: PubMed, CINAHL, EMBASE and Medline via Web of Science. Data relating to the types of studies, level of evidence, context, nature of the management undertaken, participant perspectives and the types of technologies involved were extracted for the review. Main Contribution: A total of 17 studies were included in the review, 15 of which related to the management of communication and swallowing disorders in PD with two studies devoted to participant perspectives. The majority of the studies reported on the treatment of the speech disorder in PD using Lee Silverman Voice Treatment (LSVT LOUD®). Synchronous and asynchronous technologies were used in the studies with a predominance of the former. There was a paucity of research in the management of cognitive-communication and swallowing disorders. Conclusions and Implications: Research evidence supporting technology-enabled management of the communication and swallowing disorders in PD is limited and predominantly low in quality. The treatment of the speech disorder online is the most developed aspect of the technology-enabled management pathway. Future research needs to address technology-enabled management of cognitive-communication and swallowing disorders and the use of a more diverse range of technologies and management approaches to optimize SLT service delivery to people with PD.

Miscellaneous

De Vos, Maarten etal(2020) Discriminating progressive supranuclear palsy from Parkinson’s disease using wearable technology and machine learning[2]

Background: Progressive supranuclear palsy (PSP), a neurodegenerative conditions may be difficult to discriminate clinically from idiopathic Parkinson’s disease (PD). It is critical that we are able to do this accurately and as early as possible in order that future disease modifying therapies for PSP may be deployed at a stage when they are likely to have maximal benefit. Analysis of gait and related tasks is one possible means of discrimination. Research Question: Here we investigate a wearable sensor array coupled with machine learning approaches as a means of disease classification. Methods: 21 participants with PSP, 20 with PD, and 39 healthy control (HC) subjects performed a two-minute walk, static sway test, and timed up-and-go task, while wearing an array of six inertial measurement units. The data were analysed to determine what features discriminated PSP from PD and PSP from HC. Two machine learning algorithms were applied, Logistic Regression (LR) and Random Forest (RF). Results: 17 features were identified in the combined dataset that contained independent information. The RF classifier outperformed the LR classifier, and allowed discrimination of PSP from PD with 86 % sensitivity and 90 % specificity, and PSP from HC with 90% sensitivity and 97 % specificity. Using data from the single lumbar sensor only resulted in only a modest reduction in classification accuracy, which could be restored using 3 sensors: lumbar, right arm and foot. However,for maximum specificity the full six sensor array was needed. Significance: A wearable sensor array coupled with machine learning methods can accurately discriminate PSP from PD. Choice of array complexity depends on context; for diagnostic purposes a high specificity is needed suggesting the more complete array is advantageous, while for subsequent disease tracking a simpler system may suffice.

Nigro, Salvatore et al (2020) [Cohort Study] Automated MRI Classification in Progressive Supranuclear Palsy: a Large International Cohort Study[3]

Background: The Magnetic Resonance Parkinsonism Index is listed as one of the most reliable imaging morphometric markers for diagnosis of progressive supranuclear palsy (PSP). However, the use of this index in diagnostic workup has been limited until now by the low generalizability of published results because of small monocentric patient cohorts, the lack of data validation in independent patient series, and manual measurements used for index calculation. The objectives of this study were to investigate the generalizability of Magnetic Resonance Parkinsonism Index performance validating previously established cutoff values in a large international cohort of PSP patients subclassified into PSP-Richardson’s syndrome and PSP-parkinsonism and to standardize the use of the automated Magnetic Resonance Parkinsonism Index by providing a web-based platform to obtain homogenous measures around the world. Methods: In a retrospective international multicenter study, a total of 173 PSP patients and 483 non-PSP participants were enrolled. A web-based platform was used to calculate automated Magnetic Resonance Parkinsonism Index values. Results: Magnetic Resonance Parkinsonism Index values showed optimal performance in differentiating PSP-Richardson’s syndrome and PSP-parkinsonism patients from non-PSP participants (93.6% and 86.5% of accuracy, respectively). The Magnetic Resonance Parkinsonism Index was also able to differentiate PSP-Richardson’s syndrome and PSP-parkinsonism patients in an early stage of the disease from non-PSP participants (90.1% and 85.9%, respectively). The web-based platform provided the automated Magnetic Resonance Parkinsonism Index calculation in 94% of cases. Conclusions: Our study provides the first evidence on the generalizability of automated Magnetic Resonance Parkinsonism Index measures in a large international cohort of PSP-Richardson’s syndrome and PSP-parkinsonism patients. The web-based platform enables widespread applicability of the automated Magnetic Resonance Parkinsonism Index to different clinical and research settings.

Nordio, S et al(2018)[Review]Effect of lee silverman voice treatment (LSVT LOUD)on dysphagia in patients with parkinsonism (progressive supranuclear palsy and multiple system atrophy) [4]

Telerehabilitation is the use of telecommunications technology for rehabilitation. Recently, some studies have shown positive effects of telerehabilitation of swallowing disorders, yet there are no systematic reviews verifying the evidence. The aim of this review is to assess the effects of telerehabilitation in the field of dysphagia as an alternative to face-toface patient care, considering swallowing recovery and/or quality of life in different patient populations. We searched the Cochrane Library, MEDLINE, EMBASE, Google Scholar, Google Search and the grey literature from inception until December 2016 for publications written in English (keywords: telerehabilitation, telemedicine, dysphagia, swallowing disorders), which resulted in 330 records. Abstract screening and data extraction was carried out independently by two reviewers. Four papers were selected to read in full, and the methodological quality of the studies included was evaluated using Cochrane Collaboration’s tool for assessing risk of bias. One study met our inclusion criteria which showed that telerehabilitation improves adherence to treatment compared to patient-directed intervention. Although adherence is an important factor that influences the treatment outcome, clinical outcomes have to be examined in randomised controlled trials in order to reach evidence in this field. Lastly, this systematic review did not demonstrate the efficacy of telerehabilitation compared with face-to face therapy.

Clerici, Ilaria et al (2017) [Clinical Trial] Rehabilitation in Progressive Supranuclear Palsy: Effectiveness of Two Multidisciplinary Treatments[5]

Background: to date, there are no medical or surgical treatments for progressive supranuclear palsy (PSP). It is possible to speculate that patients with PSP could benefit from rehabilitative treatments designed for Parkinson’s disease, including the use of robot-assisted walking training. Objective: to evaluate whether the use of the robotic device Lokomat® is superior in PSP patients to the use of treadmill with visual cues and auditory feedbacks [treadmill-plus]in the context of an aerobic, multidisciplinary, intensive, motor-cognitive and goal-based rehabilitation treatment (MIRT) conceived for Parkinsonian patients. Methods: we enrolled twenty-four PSP patients. Twelve subjects underwent a 4-week MIRT exploiting the use of the treadmill-plus (MIRT group). Twelve subjects underwent the same treatment, but replacing the treadmill-plus with Lokomat® (MIRT-Lokomat group). Subjects were evaluated with clinical and functional scales at admission and discharge. The primary outcomes were the total PSP Rating Scale (PSPRS) score and its limb and gait sub-scores. Secondary outcomes were Berg Balance Scale (BBS), Six Minutes Walking test (6MWT) and the number of falls. Results: total PSPRS, PSPRS-gait sub-score, BBS, 6MWT and number of falls improved significantly in both groups (p ≤ 0.003 all, except 6MWT, p = 0.032 and p = 0.018 in MIRT-Lokomat and MIRT group respectively). The PSPRS-limb sub-score improved significantly only in the MIRT group (p = 0.002). A significant difference between groups was observed only for total PSPRS, indicating a slightly better improvement for patients in the MIRT group (p = 0.047). No differences between groups were revealed for the other outcomes, indicating that the effect of rehabilitation was similar in both groups. Conclusions: Lokomat® training, in comparison with treadmill-plus training, does not provide further benefits in PSP patients undergoing MIRT. Our findings suggest the usefulness of an aerobic, multidisciplinary, intensive, motor-cognitive and goal-based approach for the rehabilitation of patients suffering from such a complex disease as PSP. Trial registration: This trial was registered on ClinicalTrials.gov, NCT02109393.

Dunlop, Susan Rebecca et al (2016) The Cure PSP Care Guide: A Telephonic Nursing Intervention for Individuals and Families Living With Progressive Supranuclear Palsy[6]

Progressive supranuclear palsy (PSP)is a rare, progressive, and terminal neurodegenerative disease characterized by problems with ambulation, balance, mobility, vision, speech, swallowing, and behavior during the 7- to 10-year course of the illness. Substantial evidence in the nursing literature supports the benefits of patient education, self-management, chronic disease management, telehealth, and nurse navigation programs, which enhance patient and caregiver knowledge, improve day-to-day management by developing an awareness of resources, decrease dependence on services, and address caregiver needs. The Cure PSP Care Guide is a targeted telehealth nursing intervention aimed at providing knowledge, guidance, and resources to the vulnerable individuals and families living with PSP; identifying local resources; and building community. During the course of two telephone calls, individuals and their caregivers are assessed to develop a Cure PSP Care Guide designed to provide guidance along the trajectory. A knowledge assessment, self-efficacy scale, and Caregiver Strain Index are administered before and after the intervention to determine the program intervention effect. Caregiver knowledge assessments improved after the intervention, whereas strain scores were static. Qualitative data show the ability of the intervention to address caregiver needs for knowledge and support, daily management tips, and resource identification. The preliminary quantitative and qualitative data collected on this pilot project justify further exploration of the use of telehealth to remotely deliver nurse case management to the vulnerable individuals and families living with PSP.


[1] Theodoros D, Aldridge D, Hill AJ, Russell T. Technology-enabled management of communication and swallowing disorders in
Parkinson’s disease: a systematic scoping review. Int J Lang Commun Disord.2019;542.:170‐188. doi:10.1111/1460-6984.12400

[2] De Vos M, Prince J, Buchanan T, FitzGerald JJ, Antoniades CA. Discriminating progressive supranuclear palsy from Parkinson’s
disease using wearable technology and machine learning. Gait Posture. 2020;77:257‐263. doi:10.1016/j.gaitpost.2020.02.007

[3] Nigro S, Antonini A, Vaillancourt DE et al Automated MRI Classification in Progressive Supranuclear Palsy: a Large
International Cohort Study [published online ahead of print, 2020 Feb 24]. Mov Disord. 2020;10.1002/mds.28007. doi:10.1002/mds.28007

[4] Nordio S, Innocenti T, Agostini M, Meneghello F, Battel I. The efficacy of telerehabilitation in dysphagic patients: a systematic
review. Acta Otorhinolaryngol Ital. 2018;382.:79‐85. doi:10.14639/0392-100X-1816

[5] Clerici I, Ferrazzoli D, Maestri R et al Rehabilitation in progressive supranuclear palsy: Effectiveness of two multidisciplinary
treatments. PLoS One. 2017;122.:e0170927. Published 2017 Feb 3. doi:10.1371/journal.pone.0170927

[6] Dunlop SR, Kent VP, Lashley M, Caruana T. The Cure PSP Care Guide: A Telephonic Nursing Intervention for Individuals and
Families Living With Progressive Supranuclear Palsy. J Neurosci Nurs. 2016;482.:105‐106. doi:10.1097/JNN.0000000000000194