UK Support Group 2015 AGM

In one weeks time it will be the UK HSP Support Group AGM in Leamington Spa. I'm looking forward to going and getting some first hand updates on whats going on.

There are two presentations this year:

Prof Henry Houlden - The National Hospital of Neurology and Neurosurgery: The differences and management of pure and complex HSP, Research and network update

Cahir O’Kane – Reader in Genetics – University of Cambridge

Obviously I'll put blog posts up on these after the event. Also a good opportunity to catch up with people. I've also nominated myself to go on the Groups' committee.....

100,000 genome project

I saw new reports the other month about the 100,000 genome project in England.

Genomics England are embarking on a project to decode and store the complete genomes of 100,000 people in England for the NHS (National Health Service). The project is set to leave a legacy for patients, the NHS and the UK economy. The project focuses on rare disease, cancer and infectious disease, and so it got my attention. You can read details here: http://www.genomicsengland.co.uk/the-100000-genomes-project/

I had a look at this in more detail, and you can find that HSP is already listed as one of the rare diseases which they are planning to cover in the project. The list is here: http://www.genomicsengland.co.uk/nominating-a-disease/

11 Genomic Medicine Centres (GMCs) have been set up to deal with the project. People who want to take part should be referred to GMCs by their clinicians.

Interestingly the list of GMCs for rare disease includes the East of England NHS GMC led by Cambridge University Hospitals NHS Foundation Trust. This is one of the key HSP research locations in the UK.

The site indicates that you are unlikely to be considered to take part unless you are already being cared for at one of the GMCs, which means that its people being seen in Cambridge who have the best chance of taking part. Also, the best chance for taking part is someone who has NOT yes had a genetic test undertaken.

So, it looks like I don't need to see about putting HSP forward for consideration in the list. I had thought about volunteering to take part, but seeings as I have already got a genetic test result and I'm not being cared for at Cambridge, its unlikely that I'd be considered for this.

Anyone reading who is being cared for at Cambridge and has a provisional diagnosis of HSP (or other diagnosis without genetic tests) then they are likely candidates and should talk to their clinician if interested in taking part.

Recent Research Papers

Having recently been alerted to the Ampyra/Dalfampridine story, I wondered if I was missing out on anything else. I used the http://www.ncbi.nlm.nih.gov/pubmed?term=((hereditary%20OR%20familial)%20AND%20%22spastic%20paraplegia%22)%20or%20%22strumpell%20lorrain%22 link to get my results and simply browsed interesting titles. The following is an overview of 10 interesting papers which I found, and why....

1) A treatable mimic of HSP

This paper from the Neurology Unit at the UK University of Sheffield reports a condition that is more rare than HSP - Cerebrotendinous xanthomatosis - which can cause spastic paraplegia. The condition is treatable with chenodeoxycholic acid if diagnosed in the early stages. The researchers hope that genetic sequencing may identify people earlier and allow treatment. It is not clear from the abstract if the person had been diagnosed with HSP, but I infer this from the title. I wonder if this might open up further potential for treatment for HSP?
http://www.ncbi.nlm.nih.gov/pubmed/25862734

2) Ampyra/Dalfampridine

No surprises to find this paper again.  This paper from the neurology unit at CHRU de Besancon in France showed that half of HSP patients in a trial responded favourably to treatment with Dalfampridine with improved walking. Another potential treatment for people.
http://www.ncbi.nlm.nih.gov/pubmed/25808501

3) Intrathecal Baclofen

Two papers report improvements in walking from HSP patients from the use of intrathecal baclofen. The first paper from the University Medical Center Groningen, in The Netherlands reports one patient who had improvements in walking from the use of both a test implant and a baclofen pump. The paper recommends the use of the pump for people who do not respond well to oral tablets. The second paper from the University of Athens Medical School, in Greece reports improvements in all 14 patients in the study which lasted on average for about 2 years. These patients had not responded well to oral tablets. The paper reports that intrathecal baclofen can improve walking/spasticity but that the improvement might be limited by either of the residual motor function or the patient continuing with their rehabilitation programme.
http://www.ncbi.nlm.nih.gov/pubmed/25626112
http://www.ncbi.nlm.nih.gov/pubmed/24973568

4) Robotic Gait Training

Two papers present improvements in gait obtained by robotic training. The first paper, from the University Hospital of Pisa in Italy reports that 13 patients with uncomplicated HSP got improvements in gait/walking and balance after a 6 week robotic gait training programme. The benefits were maintained at a re-test 2 months later. The recommend that robotic gait training is considered in exercise routines. The second paper, from Seoul National University Hospital in Korea reports that one patient improved walking speed and balance after a 6 week programme of robotic gait training and physiotherapy. They note that whilst speed and balance improved the gait itself did not (kinematics and kinetics).
http://www.ncbi.nlm.nih.gov/pubmed/25547770
http://www.ncbi.nlm.nih.gov/pubmed/25255290

5) Pelvic Floor Training

This paper, from the University of São Paulo in Brazil reports one woman with HSP who had bladder and bowel complaints, pelvic pain and pain during intercourse. A course of perineal and pelvic floor stretching was developed which resulted in less pain and improvements in bowel and bladder function.
http://www.ncbi.nlm.nih.gov/pubmed/25478261

6) Botox

This paper, from the Radboud University Medical Centre in The Netherlands reports that 15 patients with HSP were given a course of Botulinum toxin type-A in their calf muscles together with daily calf muscle stretching over 18 weeks improved walking speed and muscle tone. It is not clear from the abstract which was deemed to be more important - the Botox or the stretching.
http://www.ncbi.nlm.nih.gov/pubmed/25325386

7) Gait Variation

This paper, from the Geneva University Hospitals in Switzerland reports a gait analysis of 6 patients with HSP from the same family, with a follow up between 4 and 15 years after. Their analysis shows that there is a large variation in walking ability within one family (the inference is that they all have the same type of HSP). Over the period of the study three of the patients had improved their gait (from childhood) and three had worsened (within adulthood). There was no statistical difference between the sets.
http://www.ncbi.nlm.nih.gov/pubmed/25218933

8) Stem Cell Research

This paper, from Germany & USA, describes that stem cells derived from normal adult cells have been shown to have some relevance to HSP - potentially allowing a model of the neurons.
http://www.ncbi.nlm.nih.gov/pubmed/24821704

9) Temperature Effects

This paper, from the USA, describes that drosophila (fruit flies) with HSP have improved mobility and survive longer when reared in colder temperatures. They suggest that mild hypothermia might hold promise as a therapeutic approach for HSP. I note, however that many people complain that HSP appears worse in the cold.
http://www.ncbi.nlm.nih.gov/pubmed/24906373

10) Japanese Research Group

I note this paper, from Japan, simply because it was produced by the Japan Spastic Paraplegia Research Consortium (JASPAC). I wasn't aware of JASPAC previously, and this simply puts me on a mission to try and find out more about what they are doing. There are also other papers above from areas of the world which I've not seen before in HSP reporting, again opening up further investigation.
http://www.ncbi.nlm.nih.gov/pubmed/25296875

Exercise Routines

I've been doing my stretches now for just over 3 months. The main thing to report is that my hamstrings are getting longer. I cant touch the floor with my fingertips yet, but I'm much closer now than I have ever been able to (well, since childhood, I dont remember if I was able to do this as a small child.....)

This is quite a pleasing result in itself, and shows that the advice from my physio is good - as this was one of the aims. I'm looking at this with the following perspective - improvement in movement now helps slow HSP's trajectory down.

Back in the past (before we had children) I used to go to the gym regularly, and I did regular stretches there too. I didnt notice any lengthening of my hamstrings there (went a couple of times a week for a few years). The main difference is that I've had precise advice about this, although stretches that I used to do are similar to those now, and that I'm now doing this twice a day. Perhaps its the twice-a-day part, perhaps I'm holding them for longer now. I'm not sure.

On this topic there was a link on one of the Facebook groups to the Australian HSP site with a letter from Dr Fink, which was posted in February this year.

I quite like the general concept of this. I'll simplify the whole thing into a couple of points:

• Find out what makes walking difficult. Get advice on how to improve this.
• Frequency of exercise is as important as what you are doing
• Stretching, balance, core exercises, and aerobic conditioning are all important.
• You should expect some improvement

Reviewing what I do against this, its all there - I get balance/core from Pilates and aerobic from cycling to work.

The full letter is copied below, and you can read it in the context of the site here: http://www.hspersunite.org.au/exercise-in-hsp/:

Hello everyone,

As requested, this is a brief overview of my recommendations for exercise in HSP and PLS (Primary Lateral Sclerosis). One caveat: my recommendations are not based on scientific research of exercise methods in HSP and PLS. These recommendations are based on talking with many individuals with gait disturbance and finding what seems to be helpful.

Identify the factors that make walking difficult. HSP and PLS affect walking differently in each person. For some individuals, spasticity (affecting hamstrings, quadriceps, adductors, “heel cords” in variable proportion) is the major problem. In other individuals weakness (hip flexion, foot dorsiflexion, hamstrings for example) or endurance is the major problem. Often weakness (in certain muscles more than others) and spasticity (in certain muscles more than others) occur together (in variable proportions) with balance difficulty and slowness in muscle activation.
Consultation with a neurologist, physiatrist, physical therapist, personal trainer are often helpful in identifying which factors are particularly problematic. This is the basis for developing a function-specific exercise program.
The basic concepts are to

a) find the problems,
b) address the problems specifically both as isolated exercises and importantly, through complex task-based exercises;
c) keep score of your progress,
d) when tasks become easier, change the routine to make things more challenging;
e) expect improvement (recognizing it will be slow);
f) core muscle exercise and aerobic conditioning are key.

Here are a few notes:

Develop an exercise program that:

a) “starts low and goes slow” (begin with something you’re capable of and increase the frequency and intensity by approximately 10% each week)
b) is graded (increasing intensity and frequency)
c) is monitored (by you, keeping track of performance, and by your therapist or trainer)
d) addresses the function-specific goals
e) is varied (monotonous routines are difficult to maintain)
f) has days off each week where other exercises are performed
g) ideally is done with exercise partners (activities that are performed completely alone are difficult to maintain).

Both complex/contextual exercises (e.g. climbing gym, water aerobics, kicking a weighted ball) and isolated exercises (leg lifts, abdominal exercise “crunch” machine at the gym) are useful.

In my view, the value of stretching, balance, core exercises, and aerobic conditioning can not be overstated and should have a central place in the exercise routine. In my opinion, “exercise frequency” (4 to 10 times a week) is at least as important if not more important than the intensity of a given exercise period.

I hope this is helpful.

Sincerely,

John

John K. Fink, M.D.

Another Physiotherapist Visit

Recently I had my third trip to the physiotherapist. This is my last appointment for a while. I've got an "open appointment" (which means that I can get in quickly if there is a dramatic change), and another "normal" appointment booked in for three months time.

Since my previous appointment I've been trying hard to get that elusive second set of stretches in, which I now do by getting up a few minutes earlier each day and doing them first thing. I'm currently limiting these to my hamstrings as these are the muscles which get the least stretching in my day-to-day life, but I also realise I'm being a bit stubborn and I'll probably get the calves and roll-downs added in soon too.

Effectively, its a question of trying to introduce stretches into everyday life, so my stretching/exercising changes are:

• Stretches in the morning (as described above)
• Raising up onto my toes when cleaning teeth (to exercise calves)
• Changing the way I pedal my bike to exercise calves
• Checking my posture to make sure I'm sitting more upright
• Checking my leg position when sitting so they are not tucked under (hamstrings)
• Standing with my feet apart sometime to stretch my hip adductor's 
• Improving my posture when making cups of tea at work (hamstring stretch)
• Lowering ironing board height when ironing to get hamstrings to stretch.
• Stretches in the evening
• Laying with my legs apart in bed some of the time to stretch my hip adductor's

 Has this made any difference? This is a difficult question to answer. I think that I have managed to get my hamstrings to lengthen a bit, but I dont really know for sure about anything else. But, this is the "new" life I lead, and I wont know how much these exercises will slow HSPs trajectory down.

But, I do know that my cycling is becoming very important. We recently had the school easter holidays, where I spent time with my family instead of cycling to work. I didnt realise how much stiffer I had become over this short time until after I did my first set of evening stretches after my first cycle to/from work. The stretches were so much easier after this. It makes me think that keeping active is the key to this, and I must try to make an effort to get some exercise in on days when I dont cycle to work. My physiotherapist thought that cycling was good because it is repetitive.

One other development is that I am now the proud owner of one UNS (universal night split). I have one of these: http://www.completecareshop.co.uk/orthopaedic-aids/night-splints/universal-night-splint-large

The job of the UNS is to hold the foot so that a long term gentle stretch can be given to the foot muscles. I am to to trial this between now and my next physio appointment. In the ideal world I would wear this for 6 hours a day on each foot - but I dont have time for that! Whilst the website indicates that this can be worn at night my physio advises that this should be worn in the day. The agreed trial is to wear this for an hour a day on each foot, and to adjust so that I can feel the stretch. I have noticed that when in bed and laying on my back my feet tend to point away rather than up, so this is a good stretch to do, being against what my feet are wanting to do.

The main problem with the trial is finding 2 hours a day when I am sitting down at home able to do this. I work from home one day a week, and that is easy, and I can grab the odd half an hour here or there when watching the TV or writing this blog (have just swapped from right to left foot!). This means I'm perhaps getting a few hours each week rather than an hour a foot per day. Effectively, without making "drastic" changes at home, I'll only be able to get this length of time using the UNS at work, although that might have a few health and safety consequences!

Outcomes from the trial may be continued use, or to stop using for a while, or to only use when needed. Update in 3 months!

The final observation for the day is that my UNS is indeed an Ankle-Foot-Orthotic or AFO, so in the autumn I'll have to answer my mobility question differently when I get my survey up and running!

New study of Dalfampridine/Ampyra for use with HSP

There has just been a new study published which shows that Dalfampridine/Ampyra/Fampridine has use as a treatment for HSP.

The abstract is:
Dalfampridine in hereditary spastic paraplegia: a prospective, open study.

Béreau M, Anheim M, Chanson JB, Tio G, Echaniz-Laguna A, Depienne C, Collongues N, de Sèze J of the Département de Neurologie, CHRU de Besançon, Besançon, France.

Our aim was to support the use of dalfampridine as a treatment for patients affected with hereditary spastic paraplegia (HSP). We performed a prospective, uncontrolled, proof of concept, open trial. We included 12 HSP patients defining the total group (TG) who received dalfampridine 10 mg twice daily for 2 weeks. Efficacy assessment was based on walking ability improvement. The Timed-25-Foot Walk Test, the Spastic Paraplegia Rating Scale (SPRS), and the 12-item Multiple Sclerosis Walking Scale (MSWS-12) were performed before and after treatment. Safety assessment was based on adverse events occurrence. A significant improvement in SPRS (p = 0.0195) and MSWS-12 (p = 0.0429) was noted after treatment in the TG. No serious adverse events were noted. This interventional study provides encouraging results supporting the use of dalfampridine in HSP.

This is an electronic publication ahead of the March edition of the Journal of Neurology.

http://www.ncbi.nlm.nih.gov/pubmed/25808501
http://link.springer.com/article/10.1007/s00415-015-7707-6

This paper is reported here:
http://www.medpagetoday.com/MeetingCoverage/AAN/45552
which reports:

12 patients with HSP were given the drug -- at 10 milligrams twice a day -- for 15 days.

50% of patients (6) improved on 3 measures of walking ability

The improvements were clinically meaningful as well as statistically significant

The drug was well tolerated and would likely be without significant adverse effects for an even longer treatment period.

There is almost no carry-over effect: "If you stop the drug, one day later there is no effect."

The drug is currently approved for use in multiple sclerosis

Within my HSP survey I had one respondent who was taking this drug for HSP, who indicated that this was being taken to improve gait, and rated this medicine as 5 out of 5 for benefits and would recommend to others.

 

Reflections on 2014 Survey Results

I was asked my thoughts on my recently published survey results.

The thing which I have found most surprising is the lack of evidence of the benefits of the more common medications for relieving HSP symptoms, particularly Diazepam and Gabapentin (1 paper only each) and Tizanidine, Amitripyline and Pregabalin (no papers). 

There is some indication that medication for spasticity from MS and Parkinsons can help with HSP, but again there is no evidence. 

I’m also surprised at the supplements that people take, there were plenty of people taking one or more dietary supplements without being sure that there was any benefit from doing so. 

Overall, there is little evidence to help people make choices about medication and supplements. 

I’ll put the caveat in that my search for papers was 'only' from the PubMed website (http://www.ncbi.nlm.nih.gov/pubmed) using keyword searches. There may be other papers out there covering these issues which I don’t know about, in which case I’d be pleased to pointed in other directions......

A trip to the physiotherapist

I have now been to see a Neurophysiotherapist twice, with another appointment in a month's time.

I noted down what I was told in my notebook, which was just after my notes from my appointment at the HSP clinic last year. Here I found out that physio is a good thing to do. Muscles need working on daily in order to slow down HSP's trajectory, and that physio has the same objective as baclofen.

My first appointment showed that I have similar tightness in both legs, that my range of movement is good in that I can hold at the limits of the range and move through the range. I have a touch more clonus in my left leg than my right leg. My sensation is OK. We didn't discuss pins and needles as these are transient. I didnt realise I had any clonus, so I need to look into that!

There were 8 things for me to concentrate on:

1. When sitting, make sure I don't tuck my legs in. My foot should be flat on the floor.
2. Calf stretches twice a day, 30s at a time
3. Hamstring stretches twice a day, 30s at a time
4. Flex my toes up to strengthen my shins
5. Raise myself up onto my toes to strengthen my calfs
6. Stand with my toes on a step and lower my heels to stretch my calves
7. Lift the outer edges of my feet to help increase range of motion
8. Concentrate on my posture, keeping up straight.

The advice was to try and integrate as many of these into my normal routine as possible, which makes doing them as easy and straightforward as possible.

The first I found easy, and I hadn't realised how much I was tucking my feet under. I started doing one set of stretches just before bed, but found it difficult to find the time to get a second set in. The other points didn't get much of a look in.

In terms of stretches, my calf stretch was a lunge, concentrating on keeping my heel on the ground, my leg straight and my bum down. I prefer to do this leaning against a wall (like this: http://ryanchiropracticpllc.com/resources/stretches/calf-stretch/). My hamstring stretch was to raise my foot up on to a chair (actually chose bed rather than chair) and to concentrate on keeping my leg straight. (like this: http://www.topendsports.com/medicine/stretches/hamstring-standing.htm)

As I have been doing these things I have noticed that my muscles feel like they have been doing some exercise most of the time, which shows that my legs are being worked more than they were before.

Given that I go to Pilates once a week and am regularly cycling to work we didn't look at core strength or general levels of activity. For readers, the advice on hamstrings and calfs applies to everyone!

My second visit was about a month later. We talked about how I had been doing, and I explained that it was difficult to find the time to fit all of these things into my routine. I sought information on how often and for how long I needed to be doing these things.

The detailed points become:

1. Dont tuck legs in: no change.
2. Calf stretches: Variation, alternate between doing legs individually and both legs together.
3. Hamstring stretches: no change
4. Flex toes up: no new info.
5. Raise onto my toes: do whilst cleaning teeth (couple of mins, twice a day)
6. Stand on step and lower heels: didnt discuss
7. Lift outer edges of feet: didnt discuss 
8. Concentrate on my posture: no change

In addition, we talked about:

• An alternative hamstring stretch where I sit on the floor with my legs out straight and apart, sitting up straight. Initially I would keep one leg out straight at a time, like this http://www.fitness.com/exercises/192/seated_single_leg_hamstring_stretch.php. 
• Another hamstring alternative is where I sit on a chair with a neutral back and raise my legs up, like this http://www.ortho.microport.com/kneesite/Patients/preopex.asp. 
• These two can be undertaken instead of the hamstring stretch above, and be bought into a second set of stretches each day. As you may suspect from all this discussion on hamstrings, it is my hamstrings which are short.
• Stretches of my adductors, where I spread my legs apart, either sitting or standing.
• I should also finish each set of stretches with a couple of roll-downs, like this http://www.theepochtimes.com/n2/health/move-of-the-week-the-roll-down-166973.html. I should try to reduce the flex in my knee to make the hamstring stretch a bit bigger.

We tried out a Theraband/Pilates band and that can be another way to stretch/flex muscles. I have one to try, for example http://www.easyvigour.net.nz/fitness/h_Hamstring_Stretch.htm. We also discussed a universal night splint (or UNS) as a way of getting my ankle to stay at 90 degrees, and although the discussion started as something for the future, I'll be getting one to try at my next appointment.

Since this second appointment I've been trying much harder to get a second set of stretches in each day, which is a bit of a challenge. So, I've been trying to do a set when I get up, and I manage this most days. It is clear that my muscles are much tighter in the mornings. I've been raising myself up onto my toes when I'm cleaning my teeth and raising the outer edges of my feet wherever possible. Also, whenever I'm sitting on the floor I'm trying not to sit on my knees because that is my hamstrings at their shortest and I'm trying to get my legs out straight, so there is some re-jigging there as well. Actually this happens quite a lot (reading stories to children at bed time, playing Lego/cars/trains/etc, sorting laundry etc.) I'm prefering to do my hamstring stretches standing rather than sitting on the floor, and I need to try and bring some variation into the mix.

I also told my Pilates instructor about my hamstrings being short so that she can give me advice about if I need to do any of the exercises differently. For example, my Physio suggested that I should perhaps use a stool/step instead of kneeling for some exercises, which I had been doing as it is very difficult for me to get an upright neutral back whilst sitting with my legs out straight in front of me.

2014 Survey Results

So, it is rare disease day again. I am pleased to publish the results of my second HSP survey which I launched in September. Many thanks are due to the 97 people who gave their time and completed the survey - this wouldn't have been possible without you.

The full analysis can be found here: 

https://drive.google.com/file/d/0BzEoTkR5HCWhbEJMcVZncFNGV00/view?usp=sharing&resourcekey=0-5WakXsT4x1aiVBwu1sG2jw

Here is a short version of the 2014 suvey results....

This reports my analysis of the results of an on-line survey for people with HSP launched in September 2014. There were 97 respondents who completed the survey, predominantly from the USA and the UK.

Mobility Analysis

All 97 respondents gave answers to this question. Around half of respondents use walking sticks/poles/crutches/canes and around two fifths of respondents use a wheelchair or mobility scooter. FES is the mobility aid used by the least number of people, with a take-up of around 5%.

The results also allow the distribution of respondents within a scale of mobility which I devised last year. This is an “HSP mobility score” which allows me to cross-reference mobility against the other questions in the questionnaire. The definition of the HSP mobility score is;

0. No mobility effects
1. Can walk without aids but some effects
2. Orthotics/AFO/FES and/or Sticks/Poles/Crutches/Canes some of the time
3. Sticks/Poles/Crutches/Canes and Frame/Chair some of the time
4. Sticks/Poles/Crutches/Canes most of the time
5. Sticks/Poles/Crutches/Canes all of the time
6. Rollator/Walking frame most of the time
7. Rollator/Walking frame all of the time
8. Wheelchair/Mobility scooter most of the time
9. Wheelchair/Mobility scooter all of the time

Table 7 – Overview of mobility aids used

Mobility Aids Used - Overview:	Respondents	Percentage	Mobility Score
Those without aids	19	20%	0-1
Those who use mobility aids some of the time	24	25%	2-3
Those who use sticks most/all of the time	24	25%	4-5
Those who use frames most/all of the time	13	13%	6-7
Those who use chairs most/all of the time	17	18%	8-9

Medication

I wanted to explore which reasons people take which medicines for and how effective they perceive those to be. In total 91 respondents answered this question. There were 68 people who indicated that they took at least one medication and 23 respondents who indicated that they did not take any medication at all.

Around three quarters of people are prescribed at least one form of medication for their HSP. Of the 23 who responded that they did not take any medication 10 indicated that they have not ever been on medication for HSP. The other 13 had previously been prescribed at least one medication, but no longer take any either because of side effects, because the medication was not effective or a combination of both. Naturally, readers should consult with their doctor if they wish to change their medications.

The majority of respondents included which symptoms they took their medication for, as shown for symptom experienced by at least 10 respondents;

Table 10 – Symptoms that people take medication for

Symptom	Number of medications taken
Spasticity	49
Pain	29
Bladder	22
Spasm	19
Depression	12
Nerve Pain	10

Spasticity and Spasms

Almost half of the medication being taken is used to treat spasticity and spasms, the key features of HSP. The biggest proportion of this group of medications comprises people taking Baclofen. The list of medications where at least 5 respondents are taking is shown in the following table, with descriptions of the medications taken from the UK NHS and drugs.com websites.

Table 13 – Spasticity and Spasm Medication

Medication	Description	People taking medication
Baclofen	Baclofen reduces muscle tone and unwanted muscle contractions. This helps to reduce painful muscle spasm.	43
Botulinum toxin A / Botox / OnabotulinumtoxinA	Botulinum Toxin Type A is used to treat muscle spasms. It works by stopping muscle contraction in the muscle that it is injected into. This prevents muscle spasm. It also blocks pain signals, which indirectly block the development of a migraine.	7
Diazepam	Diazepam is a medicine which helps to control feelings of anxiety. It makes people feel less agitated and less tense. It also acts as a sedative and an anticonvulsant. It can help to relieve muscle spasm.	7
Tizanidine / Zanaflex	Tizanidine reduces muscle tone. This helps to reduce painful muscle spasm. The effect of Tizanidine usually lasts for a few hours.	6

Baclofen

43 Respondents indicate that they take Baclofen for HSP symptoms. The symptoms, as described, fall into two areas, spasticity, with 31 respondents and spasms, with 7 respondents. 6 of the respondents indicate that they take their Baclofen through an intrathecal pump whilst the other 37 take tablets.

In addition there are 26 other respondents who indicated that they have tried Baclofen in the past, but no longer take it, including 1 who used use an intrathecal pump.

The 38 respondents who currently take Baclofen for spasticity and spasms cover the full range of mobilities. Daily doses range between 10mg and 180mg for oral tablets. Generally, the dose of Baclofen is higher with increased spasticity/loss of mobility, but there are exceptions to this trend both ways. 15 Respondents indicate that their dose has increased over the years, with 5 respondents indicating no change.

31 of the 38 respondents who currently take Baclofen gave details of their side effects. 10 respondents had no side effects, 11 respondents found Baclofen made them sleepy or drowsy. The other 10 respondents indicated a range of side effects including bowel/bladder issues, dizziness, dry mouth, stomach ulcers, blood pressure issues and short term memory loss.

Of the 26 respondents who indicated that they no longer take Baclofen, 19 indicated why they had stopped taking it. 9 respondents indicated that they did not perceive any benefits from the medicine, 3 indicated it made them too drowsy/sleepy with the other 7 generally noting side effects outweighing benefits.

Most respondents indicated that the benefits of Baclofen have not changed over the years whereas other respondents were not sure if there had been a change. One respondent noted a “huge” difference moving from tablets to an intrathecal pump.

Respondents showed that, on the whole, people currently perceive benefits from the medicine, it is important to them and they follow the prescribed dose. There are some respondents who take the medicine but who do not perceive any benefits. The majority of respondents who currently take Baclofen would recommend it.

Baclofen is one of the recognised treatments for HSP. The first paper to mention the use of Baclofen to treat HSP seems to date from 1989, in the Journal of the Medical Association of Thailand (PubMed ID: 2738487). Papers continue to be published on this into 2015, with just over 20 papers altogether.

  

Botulinum Toxin Type A

7 respondents indicate that they take Botulinum Toxin Type A for treatment of HSP symptoms, predominantly spasticity but also spasms. This is commonly known as Botox, although there are several different types of treatment. For ease I will refer to this treatment as Botox in the remainder of this section. One respondent indicated that they have previously taken Botox but no longer take it because it ceased to be effective.

All respondents who take Botox use mobility aids to some degree with mobility scores ranging between 3 and 9. Typically Botox is injected every three months and the effects are noted as lasting for between a few weeks and a few months. Side effects are generally not experienced, although one respondent noted weakness for around a week after injection. Apart from one respondent, Botox is perceived as having benefits and would be recommended to others.

Botox is one of the recognised treatments for HSP. The first paper to mention the use of Botox to treat HSP seems to date from 2003, in the journal Developmental Medicine & Child Neurology (PubMed ID: 14667075). Papers continue to be published on this into 2015, with 4 papers altogether.

Diazepam

7 respondents indicate that they take Diazepam for treatment of HSP symptoms, predominantly spasticity but also spasms. No respondents indicated that they have previously taken Diazepam but no longer take it.

All respondents who take Diazepam use mobility aids to some degree with mobility scores ranging between 3 and 8. Typically one dose is taken daily, often at night, although some take more frequently and less frequently than this. The principal side effect noted is sleepiness, by 4 respondents. Diazepam is generally perceived as having benefits and would be recommended to others.

Diazepam is mentioned in one HSP paper from 1989, in the Japanese journal No to Shinkei - Brain and Nerve (PubMed ID: 2803825).

Tizanidine / Zanaflex

6 respondents indicate that they take Tizanidine for treatment of HSP symptoms, for both spasticity and spasms. 10 respondents indicated that they have previously taken Tizanidine but no longer take it, either due to side effects or to no perceived benefits.

Most of the respondents who take Tizanidine are able to walk, with 4 having a mobility score 3 or less, with one respondent scoring 5 and one scoring 8. The principal side effect noted is tiredness or fatigue, by 5 respondents.  Those who currently take Tizanidine generally perceive it to have benefits. There are some reservations recommending it to others.

Tizanidine is reported as having benefits for spastic paresis in 1982, in the Journal of the Neurological Sciences (PubMed ID: 7035623).

Other Treatments (Fewer than 5 respondents)

Of the other treatments for spasms and spasticity taken by respondents, only Levodopa is described as a treatment for HSP in the Movement Disorders Journal in 2006 (PubMed ID: 16463348). It is interesting to note, however, that Nabiximols/Sativex is reported to also control pain and has benefits on bladder issues, that Dalfampridine/Ampyra is reported to reduce fatigue and that Levodopa is reported to improve the control of muscles. One respondent indicated that they had a spinal cord stimulator implanted which was the biggest benefit they had of all.

Pain

Many people are taking a range of painkillers, some are over-the-counter medicines like paracetamol right through to strong opioid medication like morphine.  At least 5 respondents are taking antidepressant or anticonvulsant medication as shown in the following table;

Table 16 – Pain Medication

Medication	Description	People taking medication
Amitriptyline / Elavil	Amitriptyline is a tricyclic antidepressant. It is also a medication that is used in management of long term (chronic) pain, especially nerve pain. It can also be used for improving sleep. It is prescribed in low doses for managing pain and is not addictive. Amitriptyline is also used for the treatment of bedwetting in children	6
Gabapentin / Neurontin	Gabapentin is an anti-epileptic medication, also called an anticonvulsant. It affects chemicals and nerves in the body that are involved in the cause of seizures and some types of pain. It is also used to treat restless legs syndrome (RLS).	6

Amitriptyline / Elavil

6 respondents indicate that they take Amitriptyline for treatment of HSP symptoms including nerve pain, pain and restless legs. 1 respondent indicated that they have previously taken Amitriptyline but no longer take it,  due to trying a different medication at the moment.

The respondents who take Amitriptyline include most of the full range of mobility, having a mobility score between 2 and 9. In terms of side effects, 4 respondents note no side effects with 2 noting tiredness. Based on the 6 respondents currently taking Amitriptyline, it is perceived as having benefits and would be recommended to others. None of the respondents indicated that they take Amitriptyline for bladder issues, but this medicine can be prescribed to treat bedwetting in children by helping the bladder wall to relax.

Amitriptyline is not reported in any HSP papers.

Gabapentin / Neurontin

6 respondents indicate that they take Gabapentin for treatment of HSP symptoms, predominantly nerve pain and also pain. 7 respondents indicated that they have previously taken Gabapentin but no longer take it, 2 indicating because of side effects and 2 because of lack of perceived benefit.

The respondents who take Gabapentin use mobility aids to some degree with mobility scores ranging between 3 and 8. In terms of side effects, all respondents note side effects including tiredness/drowsiness (5 respondents), dry mouth (1 respondent) and unpleasant dreams/thoughts (1 respondent). Based on the 6 respondents currently taking Gabapentin, it is generally perceived as having benefits and would generally be recommended to others.

Gabapentin is reported in one HSP paper from 2007, in the European Journal of Neurology (PubMed ID: 17539946) although this indicates no difference in tests between Gabapentin and a placebo.

Supplements and Diet

Respondents were asked to give details of supplements that they take. 58 respondents answered this question. 6 of these indicated that they did not take any supplements and 52 indicated that they took at least one. Generally people take up to 3 supplements with a small number taking more than this.

8 respondents are certain that they get benefits from taking magnesium to reduce spasms/cramps and to relax muscles, including bladder muscles. There is one paper which reports using magnesium glycerophosphate to reduce spasticity in paraplegia caused by MS (PubMed ID: 11136367). There were two respondents who were certain that taking cranberry tablets was beneficial in maintaining a healthy bladder.

There was also a general question about dietary changes. 39 respondents have not made dietary changes and 32 people had made changes. There were 7 respondents who made changes due to bowel issues, with some adding more or cutting down on fibre and/or fruit/vegetables. One respondent avoids caffeine and alcohol due to bladder issues. Low/No gluten and carbohydrate diets are mentioned as giving benefits to several respondents, with one noting improved mobility on a gluten free diet and another noting reduced inflammation with a low carb diet. One respondent limits alcohol to assist with balance, and another notes caffeine and sugar make their symptoms worse. 7 respondents have just made general health improvements in their diet, including two partly as a result of weight gain from reduced mobility.

It is worth noting that the Spastic Paraplegia Foundation website indicates that “There are no known studies indicating that vitamins or food supplements are an effective treatment for HSP.” (https://sp-foundation.org/understanding-hsp-pls/treatments-and-therapies/). Any readers wishing to make changes to their diet or to change any supplements being taken may wish to consult their doctor.

Exercise and Relaxation

The final section of the questionnaire looked at respondents exercise and relaxation routines. Overall 90 respondents answered this question. The following table shows the distribution of answers given.

Table 17 – Exercise and Relaxation

	Stretches	Yoga etc.	Weights	Power plate etc.	Cardiovascular	Walk/Run/Cycle	Swimming	Massage	Aromatherapy	Acupuncture	Physiotherapy	Chiropractic
I do this several times a day	8	0	0	1	1	1	0	0	0	0	1	0
I do this daily	38	8	8	2	1	15	2	1	0	0	5	0
I do this weekly	9	5	13	4	13	17	14	9	3	2	13	1
I do this monthly	4	2	0	1	0	1	0	10	1	2	10	1
I do this occasionally	17	3	13	2	8	20	23	13	3	4	10	5
I dont do this	11	55	42	61	50	24	35	42	59	63	34	62
Total Answers	79	73	76	70	72	77	74	75	66	71	72	69

This shows that stretches are the most common form of exercise being undertaken with over 85% of respondents doing this. Other activities which are commonly undertaken include weights, cardiovascular machines, walking/running/cycling, swimming and physiotherapy.

Of the 90 respondents only 1 did not undertake any of the activities or indicate other activities. Respondents who undertake activities weekly or more frequently are grouped together as undertaking that activity “regularly”. Overall 71 respondents undertake at least 1 activity regularly.

To look at this in more detail Yoga/Tai-Chi/Pilates, Weights, Power plate/circulation booster, walking/running/cycling, swimming and physiotherapy are grouped together as “exercise”. Overall 81 respondents undertake at least one exercise activity, and 66 of these undertake at least one of those activities regularly (weekly or more often). Grouping the other activities together shows that these activities are undertaken regularly, but the general take-up of the other activities is lower than the exercise activities.

This information shows that for people who do not use mobility aids at all (mobility score 0 or 1) over 80% undertake at least one activity regularly. Activities are likely to include walking/running/cycling. Many respondents also do stretches and take part in swimming.  Other activities undertaken include physiotherapy, massage, yoga/tai-chi/pilates, weights and cardiovascular.

Almost 80% of people who use mobility aids some of the time (mobility score 2 or 3) take part in at least one activity regularly. Activities are likely to include stretches and walking/running/cycling. Many respondents also take part in weights, swimming and physiotherapy.  Other activities undertaken include cardiovascular, massage and yoga/tai-chi/pilates.

           

Three quarters of people who use sticks most or all of the time (mobility score 4 or 5) take part in at least one activity regularly. Activities are likely to include stretches. Many respondents also take part in physiotherapy and walking/running/cycling. Other activities undertaken include massage, swimming and weights.

Two thirds of people who use walking frames most or all of the time (mobility score 6 or 7) take part in at least one activity regularly. Activities are likely to include stretches. Many respondents also take part in walking/running/cycling. Other activities undertaken include physiotherapy and weights.

Almost 90% of people who use wheelchairs most or all of the time (mobility score 8 or 9) take part in at least one activity regularly. Many respondents take part in stretches. Other activities undertaken include swimming, physiotherapy, massage, weights, power plate/circulation booster and aromatherapy.

Like this? in other years:
Overview of all my surveys: http://hspjourney.blogspot.co.uk/p/my-on-line-resarch.html
2016: Fatigue, bladder, bowel & information: http://hspjourney.blogspot.co.uk/2017/02/2016-survey-results.html
2015: Modifications at home, depression, quality of life: http://hspjourney.blogspot.co.uk/2016/02/2015-survey-results.html
2013: Symptoms and misdiagnosis: http://hspjourney.blogspot.co.uk/2014/02/hsp-survey-results.html