Author: SSAdmin

Evaluating intrathecal delivery system

 

Here are reports on two research studies concerning baclofen – the first where it was found that long-term treatment with intrathecal compared with oral baclofen resulted in reduced spasm frequency and severity as well as greater dose stability, however no significant differences were found in pain, sleep, fatigue and quality of life measures between people taking either of the two.

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The second was a large study that effectively used CT scans to locate defects in intrathecal baclofen delivery systems. On this second study, Dr. Malin Dollinger, a medical practitioner who has HSP said “This is a very good article. I think everyone with a Baclofen pump should have a copy to show their pump doctor if there is ever any drug delivery problem. The average x-ray department may not have specific experience with CT scans of Baclofen pumps, and this article is a good guide.”

 

Abstract

BACKGROUND: Baclofen commonly is used to manage spasticity caused by central nervous system lesions or dysfunction. Although both intrathecal and oral delivery routes are possible, no study has directly compared clinical outcomes associated with these 2 routes of treatment.

OBJECTIVE: To compare spasticity levels, pain, sleep, fatigue, and quality of life between individuals receiving treatment with intrathecal versus oral baclofen.

DESIGN: Cross-sectional matched cohort survey study.

SETTING: Urban academic rehabilitation outpatient clinics.

PARTICIPANTS: Adult patients with spasticity, treated with intrathecal or oral baclofen for at least 1 year, matched 1:1 for age, gender, and diagnosis.

METHODS: Standardized surveys were administered during clinic appointments or by telephone.

MAIN OUTCOME MEASURES: Surveys included the Penn Spasm Frequency Scale, Brief Pain Inventory, Epworth Sleepiness Scale, Fatigue Severity Scale, Life Satisfaction Questionnaire, and Diener Satisfaction with Life Scale.

RESULTS: A total of 62 matched subjects were enrolled. The mean (standard deviation [SD]) age was 46 (11) years with a mean duration of intrathecal baclofen or oral baclofen treatment of 11 (6) and 13 (11) years, respectively. There were 40 (64%) male and 22 (36%) female subjects. Primary diagnoses included spinal cord injury (n = 38), cerebral palsy (n = 10), stroke (n = 10), and multiple sclerosis (n = 4). The mean (SD) dose of intrathecal and oral baclofen at the time of survey were 577 (1429) μg/day and 86 (50) mg/day, respectively. Patients receiving intrathecal compared with oral baclofen experienced significantly fewer (1.44 [0.92] versus 2.37 [1.12]) and less severe (1.44 [0.92] versus 2.16 [0.83]) spasms, respectively as measured by the Penn Spasm Frequency Scale (P < .01; P < .01).

There were no significant differences in pain, sleep, fatigue, and quality of life between groups. Subanalysis of patients with SCI mirrored results of the entire study sample, with significant decreases in spasm frequency and severity associated with intrathecal compared to oral baclofen (P < .01; P < .01), but no other between group differences. The mean (SD) percent change in dose of oral (21% [33%]) compared with intrathecal (3% [28%]) baclofen was significantly larger two years prior to the date of survey (P = .02).

CONCLUSIONS: Long-term treatment with intrathecal compared with oral baclofen is associated with reduced spasm frequency and severity as well as greater dose stability. These benefits must be weighed against the risks of internal pump and catheter placement in patients considering intrathecal baclofen therapy.

 

SOURCE: PM R. 2016 Jun;8(6):553-62. doi: 10.1016/j.pmrj.2015.10.005. Epub 2015 Oct 20.

 

Intrathecal Versus Oral Baclofen: A Matched Cohort Study of Spasticity, Pain, Sleep, Fatigue, and Quality of Life.

 

McCormick ZL1, Chu SK2, Binler D3, Neudorf D4, Mathur SN5, Lee J6, Marciniak C7.

 

1 Department of Physical Medicine & Rehabilitation (PM&R), Northwestern Feinberg School of Medicine/The Rehabilitation Institute of Chicago, McGaw Medical Center, Northwestern University Feinberg School of Medicine, 345 East Superior Street, Chicago, IL 60605(∗). Electronic address: zachary-mccormick@northwestern.edu.

2 Department of Physical Medicine & Rehabilitation (PM&R), Northwestern Feinberg School of Medicine/The Rehabilitation Institute of Chicago, Chicago, IL(†).

3 Rush University School of Medicine, Chicago, IL(‡).

4 Department of PM&R, University of California at Davis, Sacramento, CA(§).

5 Case Western Reserve University School of Medicine, Cleveland, OH(¶).

6 Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL(#).

7 Department of Physical Medicine & Rehabilitation (PM&R), Northwestern Feinberg School of Medicine/The Rehabilitation Institute of Chicago, Chicago, IL(‖).

 

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Abstract

AIM: To describe the computed tomography (CT) findings encountered when catheter patency is questionable. The role of CT in directing treatment is evaluated.

METHOD: Records of children with intrathecal baclofen pump management were reviewed. Only patients with CT evaluation who had revision pump/catheter surgery were included.

RESULTS: From 295 patients, 27 had CT contrast study; in three of them, baclofen could not be aspirated and the procedure was stopped, eight had normal scan and did not need surgery and 16 patients were reported. Four patients had normal CT (free contrast formed a perfect crescent shape), and had surgery because the pump battery was close to expiration. Five patients had inadequate fluid pooling (fluid was seen without a crescent shape). Five patients had fluid leak (fluid was seen around the pump or in the lumbar canal below catheter entrance level or outside the canal in the lumbar region). Two patients had catheter occlusion (fluid loculation around the catheter tip with no free flow).

INTERPRETATION: CT contrast study is safe and effective for locating defects in intrathecal baclofen delivery system. When catheter patency is questionable, CT plays an important role in directing the next step of management.

 

SOURCE: Dev Med Child Neurol. 2016 Apr;58(4):409-15. doi: 10.1111/dmcn.12893. Epub 2015 Sep 11.

 

Evaluation of intrathecal baclofen delivery system malfunction by computed tomography scan.

 

Abousamra O1, Rogers KJ1, McManus M1, Miller F1, Sees JP1.

1 Department of Orthopedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.

 

Coping with the stress of a chronic condition

 

Adam Lawrence, Chairman of the UK HSP Support Group, and known to the Australian HSP community through his annual global surveys, has reviewed a book ‘Living with the Enemy: Coping with the stress of chronic illness using CBT, mindfulness and acceptance’ by Dr. Ray Owen. Dr. Owen is a clinical psychologist who has worked in hospital, community and hospice settings for the past 20 years.

 

Here is Adam’s review from his regular blog …

 

At the HSP Support Group AGM in 2017 Robin Pajmans recommended the book “Living with the Enemy” by Ray Owen. I got this from my library to read. I found that there were a load of useful things in this. This post is a review of the book with a few points that I found useful from each chapter.

 

Overall the book covers several approaches for coping with the stress of a chronic condition or long term condition. It uses Cognitive Behavioural Therapy (CBT), Mindfulness and acceptance, and Acceptance Commitment Therapy (ACT).

 

The first chapter “knowing the enemy” is about understanding what people are dealing with in the case of a chronic condition. Whatever coping strategy is adopted, it cannot depend on finding a cure or a solution which sends all the symptoms away. With a chronic or long term condition these are not possible. It is better to avoid being in a fight with your condition.

 

The second chapter “getting stuck in the struggle” describes that having a long term condition (LTC) can have a huge impact on people, physically, practically and psychologically. When in a stressful situation our natural response is either to fight or flight – both of these can be difficult with an LTC.  If you are the type of person who urges to control, but your LTC is uncontrollable, it can set into unhelpful thinking patterns. The urge not to feel bad is strong, and many take the choice to miss out on things to do this – but the consequence of this is a more narrow life, missing out on things you enjoy. If you supress unwanted thoughts they will often reoccur. This chapter introduced me to the “hot cross bun model” which differentiates out what is a thought, a feeling, a behaviour and a physical symptom – and identifies which of these are in your control, and which are not.

 

Chapter 3, “troubling thoughts” identifies that having troubling thoughts can cause additional problems on top of an LTC. Some of those troubling thoughts are likely to be incorrect or at least distorted. Those who get caught up in such thoughts often stop noticing what is real. The chapter introduces setting up ‘rules of thumb’ to assess situations by and having a period of “worry time” each day to help resolve troubling thoughts.

 

Chapter 4, “unwanted feelings,” moves on to starting to accept the presence of unwanted thoughts. If you can accept some of these thoughts being in your mind, even if you dont like them, then you can avoid making them the centre of attention and free up your mind to do something more useful!

 

Chapter 5, “living in the present”, notes that the mind tends to wander during the day, sometimes looking at the past, at the future or other places. Whilst the mind wanders of its own accord, if you get lost in unwanted thoughts this isn’t a happy place to be and by not paying attention to the present you miss out on what is happening in the here and now. The chapter introduces present moment awareness and mindfulness as techniques to help you focus on the present moment.

 

Chapter 6, “who am I now? a sense of self” separates out you as a person from the LTC that you have. It is the condition that is the burden, not you. The chapter sets out the concept of accepting the present and mourning the past. If you get stuck in a set story about yourself, the changing nature of an LTC means that this has potential to end up focusing on your limitations rather than your abilities.

 

Chapter 7, “living with purpose” sets out a method for working out your personal goals and purpose, but in the framework of LTCs being able to get in the way of some of your important goals. Knowing your values can help set your direction and give your life a better sense of purpose/fulfilment. It is important to note that goals are not the same as values. Once you know your values you can make sure that you not continually neglecting any of these.

 

Chapter 8, “taking action”, has the central premise that you need action in order to make a change. Base your actions on your values, choose goals that will matter to you personally, and make sure that they are smart (specific, measurable, achievable, relevant, time-bound). Note that you cannot change the behaviour of other people (but you can influence it).

 

Chapter 9 “putting it together” gives example action statements to make as a commitment: “I will do [action] in service of [value] and am willing to experience [unwanted thought/feeling] if that is what it takes”.

 

Regular readers of this blog will note that I’ve covered similar things like this before:

CBT: https://hspjourney.blogspot.co.uk/2014/04/stress-and-mood-management.html

Values: https://hspjourney.blogspot.co.uk/2016/01/depression-and-assessing-yourself.html

It also reminded me of Stephen Covey’s Seven Habits of Highly Effective People, in which there is much more on values, prioritising tasks and focusing on what is important.

 

The book is widely available in book stores and online in Australia for around $30.

 

SOURCE: http://hspjourney.blogspot.com/2018/03/living-with-enemy.html

 

 

Warning on imitations of current methodologies

 

Genetic diagnosis was confirmed in 62% of children in this study, mostly with complicated HSP – a significantly higher than normal percentage.

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New genotype – phenotype correlations were found in several of the genes identified, but caution was expressed regarding the choice of genetic testing technology, with the decision best based on clinical suspicion.

 

Abstract

Hereditary spastic paraplegias (HSP) are clinical and genetic heterogeneous diseases with more than 80 disease genes identified thus far. Studies on large cohorts of HSP patients showed that, by means of current technologies, the percentage of genetically solved cases is close to 50%. Notably, the percentage of molecularly confirmed diagnoses decreases significantly in sporadic patients.

 

To describe our diagnostic molecular genetic approach on patients with pediatric-onset pure and complex HSP, 47 subjects with HSP underwent molecular screening of 113 known and candidate disease genes by targeted capture and massively parallel sequencing. Negative cases were successively analyzed by multiplex ligation-dependent probe amplification (MLPA) analysis for the SPAST gene and high-resolution SNP array analysis for genome-wide CNV detection.

Diagnosis was molecularly confirmed in 29 out of 47 (62%) patients, most of whom had a clinical diagnosis of cHSP. Although SPG11 and SPG4 remain the most frequent cause of, respectively, complex and pure HSP, a large number of pathogenic variants were disclosed in POLR3A, FA2H, DDHD2, ATP2B4, ENTPD1, ERLIN2, CAPN1, ALS2, ADAR1, RNASEH2B, TUBB4A, ATL1, and KIF1A.

 

In a subset of these disease genes, phenotypic expansion and novel genotype-phenotype correlations were recognized. Notably, SNP array analysis did not provide any significant contribution in increasing the diagnostic yield.

 

Our findings document the high diagnostic yield of targeted sequencing for patients with pediatric-onset, complex, and pure HSP. MLPA for SPAST and SNP array should be limited to properly selected cases based on clinical suspicion.

 

SOURCE: Neurogenetics. 2018 May;19(2):111-121. doi: 10.1007/s10048-018-0545-9. Epub 2018 Apr 24. PMID: 29691679

 

The impact of next-generation sequencing on the diagnosis of pediatric-onset hereditary spastic paraplegias: new genotype-phenotype correlations for rare HSP-related genes.

 

Travaglini L1,2, Aiello C1,2, Stregapede F1,3, D’Amico A1,2, Alesi V4, Ciolfi A2, Bruselles A5, Catteruccia M1,2, Pizzi S2, Zanni G1,2, Loddo S4, Barresi S2, Vasco G6, Tartaglia M2, Bertini E7,8, Nicita F9,10.

 

1 Unit of Neuromuscular and Neurodegenerative Disorders, Ospedale Pediatrico Bambino Gesù, Polo di Ricerca S. Paolo, V.le S. Paolo, 15, 00146, Rome, Italy.

2 Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Rome, Italy.

3 Department of Sciences, Roma Tre University, Rome, Italy.

4 Clinical Genetics, Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Rome, Italy.

5 Department of Oncology and Molecular Medicine, Istituto Superiore di Sanità, Rome, Italy.

6 Movement Analysis and Robotics Laboratory (MARLab), Neurorehabilitation Unit, Ospedale Pediatrico Bambino Gesù, Rome, Italy.

7 Unit of Neuromuscular and Neurodegenerative Disorders, Ospedale Pediatrico Bambino Gesù, Polo di Ricerca S. Paolo, V.le S. Paolo, 15, 00146, Rome, Italy. enricosilvio.bertini@opbg.net.

8 Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Rome, Italy. enricosilvio.bertini@opbg.net.

9 Unit of Neuromuscular and Neurodegenerative Disorders, Ospedale Pediatrico Bambino Gesù, Polo di Ricerca S. Paolo, V.le S. Paolo, 15, 00146, Rome, Italy. nicita.f@gmail.com.

10 Genetics and Rare Diseases Research Division, Ospedale Pediatrico Bambino Gesù, Rome, Italy. nicita.f@gmail.com.

 

Effective treatment available

 

Botox injections to the larynx together with voice therapy have been effective in a first-ever identified case of a voice problem known as spasmodic dysphonia, associated with SPG7 HSP.

 

Spasmodic dysphonia is a disorder in which the muscles that generate a person’s voice go into periods of spasm. This results in breaks or interruptions in the voice, often every few sentences, which can make a person difficult to understand.

 

The issue has previously been identified in some cases of spastic paraplegia complicated by other coexisting conditions, but never in any of the 79 HSP types thus far defined.

 

Abstract

Objective: To describe a case of spasmodic dysphonia associated with hereditary spastic paraplegia (HSP) type 7.

Background: HSP is a heterogeneous group of inherited disorders characterized by lower limb predominant spasticity with or without weakness. HSP can be classified as uncomplicated or complicated based on the presence of other neurological or systemic features.

HSP7 is an autosomal recessive disorder caused by a mutation in the SPG7 gene on chromosome 16q. HSP7 is usually complicated, with cerebellar ataxia as the most frequent additional feature. Dystonia is rarely seen in HSP7; to date only cervical dystonia has been reported.

Methods: We describe a case of a 26-year-old man who presented with a progressive gait disorder and abnormal speech. At age 17, he began to toe-walk. Over time, he developed a limping, unsteady gait with leg stiffness and falls. At age 25, his voice became progressively hoarse and strangulated without dysphagia. His parents are first cousins and a paternal uncle has global developmental delay.

Examination revealed normal strength, diffuse hyperreflexia in the arms and legs, bilateral positive Hoffmann’s reflexes, increased spastic tone in the legs with bilateral ankle clonus and upgoing toes. He had pronounced foot plantar flexion when walking. His gait did not change when running or walking backwards. His voice had a strained quality with occasional breaks on voice phonation. His voice quality normalized when laughing and the strain and breaks resolved with whispering.

Results: A brain MRI was normal. A cervical spine MRI revealed mild flattening of the cord. Nerve conduction studies and electromyography were normal. Laryngovideostroboscopy revealed normal laryngeal function with occasional spasm with connected speech.

Trio whole exome sequencing detected a homozygous, previously reported pathogenic nonsense variant, p.L78X (c.233T>A), in the SPG7 gene. Clinical Sanger sequencing confirmed the presence of the homozygous variant in the patient.

Conclusions: He is being treated with botulinum toxin injections to the larynx for spasmodic dysphonia and to the bilateral gastrocnemius for gait dysfunction, thought to be a combination of spasticity and dystonia. The injections and voice therapy successfully improved his voice.

We believe this is the first description of spasmodic dysphonia, a focal dystonia of the laryngeal muscles, in association with a SPG7 mutation.

 

SOURCE: Mov Disord. 2017; 32 (suppl 2). http://www.mdsabstracts.org/abstract/spasmodic-dysphonia-in-hereditary-spastic-paraplegia-type-7/. Accessed May 26, 2018.

 

Spasmodic Dysphonia in Hereditary Spastic Paraplegia Type 7

 

Hall, N. Stong, N. Lippa, M. Pitman, S. Pullman, O. Levy (New York, NY, USA)

Connecting with research, clinical trials management

 

SAN FRANCISCO, March 22, 2018 /PRNewswire/

Invitae Corporation (NYSE: NVTA) one of the fastest growing genetic information companies, today announced an expansion of its network of rare and ultra-rare patient registries in its Patient Insights Network^TM (PIN) program to include five additional collaborations with leading advocacy groups across a variety of genetic conditions.

 

The SP Foundation in the USA, with whom this Foundation partners and collaborates, is one of the five organisations to join the PIN program.

 

The programs are designed to empower patients to be active participants in their networks and further expand Invitae’s work to connect patients with rare genetic disorders to research, clinical trials and information on managing their condition.

 

The first PIN to launch is led by No Stomach For Cancer, a leading research and advocacy organization focused on stomach cancer. Known as the Global Gastric Cancer Registry, the program enables patients, advocates, clinicians and researchers to share and access deidentified data on a number of different stomach cancers, including Diffuse Gastric Cancer, Proximal Stomach Cancer and Noncardia (Distal) Stomach Cancer.

 

“Having access to information on clinical experience and outcomes continues to help improve care for patients and accelerates access to clinical trials for both patients and researchers. These networks are making a powerful contribution to advancing our understanding of diseases, particularly in rare disorders.” said Robert Nussbaum, M.D., chief medical officer of Invitae.

 

Read the full article …

 

“There is nothing that can be done”

 

Hands up how many HSPers have heard these words from a neurologist?

 

Unfortunately, all too many! What would be much more helpful as well as more accurate is if the neurologist said “there is nothing that I can do to help you treat the disease itself, but there are things that others can do and that you can do for yourself that can make a big difference in your functionality and quality of life”.

 

 

It is a fact that a fit, flexible HSPer who undertakes a regular program of appropriately prescribed exercise and movement, and maintains good bodyweight experiences significantly higher functionality and quality of life than does an HSPer who doesn’t exercise and is overweight.

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The difference in quality of life is not restricted to physical capabilities and mobility, it extends to improvements in aspects of mental health such as mood, outlook and attitude, and the avoidance of depression.

 

Neurophysiotherapy

One of the best investments that HSPers can make is to see a specialist neurophysiotherapist or at least a physiotherapist with neurological training. The ultimate aim is to have a program of movement and exercise that can be largely self-managed. It is specifically designed with their symptom profile, functionality and capability in mind.

 

The first step is assessment of functionality and capability, especially in regard to mobility but also in a broader sense. This includes detailed assessment of the nature, type and extent of symptoms – which muscles are spastic, which muscles are weak, the relative balance of muscles, skeletal or structural issues with the back, legs and feet, as well as the joints. A thorough assessment of what is going on with the parts as well as in the ‘whole’.

 

Based on that, there may be a necessity for particular types of treatment, which could be for shorter or longer term. At the same time, the neurophysiotherapist is developing a plan and program of movement and exercise aimed at maximising functionality and mobility. In the early stages, this program may be implemented under the guidance of the neurophysiotherapist, who will be assessing and monitoring performance and adjusting the program to avoid adverse effects and maximise positive impact. Feedback from the HSPer is important in this process of monitoring and improvement.

 

Such programs are designed for regular implementation, perhaps with a daily or twice-daily component, and other parts of the program to be done three times a week. This is largely self-monitored and managed, with return visits to the neurophysiotherapist at intervals for monitoring, assessing progress and increasing the level of challenge as appropriate.

 

Even from the start, such programs are ‘owned’ by the HSPer. The neurophysiotherapist assists and facilitates, but it should not be thought of as “going for treatment”. The neurophysiotherapist’s role is best seen as expert support for a self-managed program of self-improvement.

 

Other allied health professionals

It may be that the neurophysiotherapist recommends that you see a podiatrist, occupational therapist or even an orthopaedic specialist to deal with particular issues that you have.

 

Take charge!

HSPers need to be their own best friends in the quest to maximise functionality and mobility. Take the lead, take charge, make it happen! There is a listing of community recommended medical practitioners on the website. The Australian Physiotherapy Association also has a search facility on their website where you can enter location even sort by neurological specialty. If you have no luck identifying a suitable physiotherapist, email us and we will do our best to help you find one.

 

Help with travel planning and getting around

 

Long-term member of the HSP community, Jason Jones from Wollongong, has been involved in the development of a mobile phone app using location-based technology in Google Maps.

The app helps wheelchair users find the best travel routes based on the person’s ability in using a wheelchair.

 

Here is the story from the SYDNEY.NEWS:

 

“Four out of five wheelchair users had not been anywhere new in the last year, and the main reason was the unreliability of accessibility information”

 

A mobile phone app that helps wheelchair users find the best routes to travel based on their ability is being developed by researchers at Wollongong.

In an Australian first, researchers have developed the application that shows wheelchair users the best routes to travel around the University of Wollongong campus.

They say the Navability app could revolutionise the way wheelchair users get around.

Jason Jones, who has struggled with hereditary spastic paraplegia for the past two decades, has helped to develop the program

Since 2014 the 45-year-old’s condition has progressively worsened, forcing him to use a wheelchair.

Mr Jones said the app would make a huge difference to the everyday lives of people with a disability, making it easier for them to get around and socialise.

“This month I went to a Sydney McDonald’s and this one had stairs going into it. How does a person in a wheelchair get there?” he said.

“Now I can log places that aren’t accessible and people will know not to go there.”

A more accessible world for wheelchair users

The brainchild of Briometrix, the app aims to make moving around easier and safer for people with a disability by translating wheelchair user generated data into navigation routes.

The app uses location-based technology in Google Maps and monitors exercise, to create routes based on a person’s ability to propel and manoeuvre a wheelchair.

“Measuring the actual effort that somebody takes to push a wheelchair, it starts to measure combining all of those parameters,” University of Wollongong associate professor Robert Gorkin said.

Read the full article …

 

SOURCE: https://thesydney.news/2017/12/11/google-maps-inspired-app-could-revolutionise-the-way-wheelchair-users-get-around/

Google Maps-Inspired App could Revolutionise the way Wheelchair Users Get Around

11 December 2017

Monash Children’s Hospital, Victoria

 

Monash Children’s Hospital in Victoria runs a highly successful Bicycle Skills Program, the brainchild of physiotherapist Rachel Toovey. “We had a growing number of children and their families requesting to learn how to ride a bike” Ms Toovey said. “It was something they wanted to do with their family or friends, or a bike education program at school was coming up and they didn’t want to be the only kid with training wheels.” Rachel said most children learned to ride on their own after three days.

 

While the main focus of the program has been kids with cerebral palsy, kids with HSP and similar conditions are also welcome to participate. Young HSP community member Ryan is a graduate of the program and is featured in the video.

 

It is a school holiday program run over 3 consecutive days at Fregon Reserve, Browns Rd, Clayton (at the back of Monash Children’s Hospital). It has been running for more than 5 years with well over 100 children having participated. The goal is to develop the skill to be able to ride a two-wheel bike. Children aged 6 – 15 years with neuro-motor difficulties (such as cerebral palsy, acquired brain injury, HSP) who are able to walk independently without aids are eligible to participate.

 

For more information on the referral process to get into the program, contact Rachel or Melanie.

There is also a program run by the Rehabilitation Department at the Royal Children’s Hospital for VPRS clients. See https://www.vprs.org.au/services/ for more information.

If you think this is a great idea, but you don’t live in Melbourne, what about taking the lead in your area to get a bike program like this going? Contact the Foundation with the best number to call you on and we can share some ideas to help get started.

 

But does warming the legs help?

 

Using heat packs on the legs for 30 minutes improved walking speed by 12% and coordination by 22%, with the gains maintained for at least one hour. The effectiveness of using hot packs to warm cold legs in people with HSP was assessed by walking speed and coordination tests.

So if you get stiff legs in cold weather, and can feel hot and cold on your legs, try using heat packs, electric blankets or a hot bath for 30 minutes before moving about or walking to improve mobility functioning and performance.

 

Abstract

OBJECTIVES: Cooling of the lower limb in people with Hereditary and Spontaneous Spastic Paraparesis (pwHSSP) has been shown to affect walking speed and neuromuscular impairments. The investigation of practical strategies, which may help to alleviate these problems is important. The potential of superficial heat to improve walking speed has not been explored in pwHSSP. Primary objective was to explore whether the application of superficial heat (hot packs) to lower limbs in pwHSSP improves walking speed. Secondary objective was to explore whether wearing insulation after heating would prolong any benefits.

 

METHODS: A randomised crossover study design with 21 pwHSSP. On two separate occasions two hot packs and an insulating wrap (Neo-G™) were applied for 30minutes to the lower limbs of pwHSSP. On one occasion the insulating wrap was maintained for a further 30minutes and on the other occasion it was removed. Measures of temperature (skin, room and core), walking speed (10 metre timed walk) and co-ordination (foot tap time) were taken at baseline (T1), after 30 mins (T2) and at one hour (T3).

 

RESULTS: All 21 pwHSSP reported increased lower limb stiffness and decreased walking ability when their legs were cold. After thirty minutes of heating, improvements were seen in walking speed (12.2%, P<0.0001, effect size 0.18) and foot tap time (21.5%, P<0.0001, effect size 0.59). Continuing to wear insulation for a further 30minutes gave no additional benefit; with significant improvements in walking speed maintained at one hour (9.9%, P>0.001) in both conditions.

 

CONCLUSIONS: Application of 30minutes superficial heating moderately improved walking speed in pwHSSP with effects maintained at 1hour. The use of hot packs applied to lower limbs should be the focus of further research for the clinical management of pwHSSP who report increased stiffness of limbs in cold weather and do not have sensory deficits.

 

SOURCE: Ann Phys Rehabil Med. 2017 Dec 11. pii: S1877-0657(17)30453-0. doi: 10.1016/j.rehab.2017.12.001. [Epub ahead of print] PMID: 29241713 Copyright © 2017 Elsevier Masson SAS. All rights reserved.

 

Effects of superficial heating and insulation on walking speed in people with hereditary and spontaneous spastic paraparesis: A randomised crossover study.

 

Denton AL1, Hough AD2, Freeman JA2, Marsden JF2.

1 School of Health Professions, Faculty of Health and Human Sciences, Plymouth University, Peninsula Allied Health Centre, Derriford Road, Plymouth, PL6 8BH, United Kingdom. Electronic address: amanda.denton@plymouth.ac.uk.

2 School of Health Professions, Faculty of Health and Human Sciences, Plymouth University, Peninsula Allied Health Centre, Derriford Road, Plymouth, PL6 8BH, United Kingdom.

Real-world tasks or Virtual Reality games?

 

30 people with traumatic spinal cord injury were put into two groups to do training designed to improve balance. One group used virtual reality game-based training and the other used real-world task-specific training.

 

It turns out that virtual reality game-based training is significantly better in improving balance and functional performance.

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While this study was with spinal cord injury participants, the findings may well be relevant to people with HSP.

 

Abstract

Objective: To determine whether there is any difference between virtual reality game-based balance training and real-world task-specific balance training in improving sitting balance and functional performance in individuals with paraplegia.

 

Methods: The study was a pre-test – post-test experimental design. There were 30 participants (28 males, 2 females) with traumatic spinal cord injury randomly assigned to 2 groups (group A and B). The levels of spinal injury of the participants were between T6 and T12. The virtual reality game-based balance training and real-world task-specific balance training were used as interventions in groups A and B, respectively. The total duration of the intervention was 4 weeks, with a frequency of 5 times a week; each training session lasted 45 minutes. The outcome measures were modified Functional Reach Test (mFRT), t-shirt test, and the self-care component of the Spinal Cord Independence Measure-III (SCIM-III).

 

Results: There was a significant difference for time (p = .001) and Time × Group effect (p = .001) in mFRT scores, group effect (p = .05) in t-shirt test scores, and time effect (p = .001) in the self-care component of SCIM-III.

 

Conclusions: Virtual reality game-based training is better in improving balance and functional performance in individuals with paraplegia than real-world task-specific balance training.

 

SOURCE: Top Spinal Cord Inj Rehabil. 2017 Summer;23(3):263-270. doi: 10.1310/sci16-00003. Epub 2017 May 4. PMID: 29339902 PMCID: PMC5562034 [Available on 2018-06-01]

 

Study on the Effectiveness of Virtual Reality Game-Based Training on Balance and Functional Performance in Individuals with Paraplegia.

 

Khurana M1, Walia S1, Noohu MM2.

1 ISIC Institute of Rehabilitation Sciences, New Delhi, India.

2 Centre for Physiotherapy and Rehabilitation Sciences, Jamia Millia Islamia, New Delhi, India.