Friday, 1 December 2017

Symptoms Update - Walking

So, it looks like there hasn't been many symptoms updates, and then two come along in quick succession!

Observations from the last couple of months are that my walking speed has dropped, but I'm not really sure over low long this has taken place. Certainly before I was living in Bristol I was quite a fast walker, and easily found myself at the front of groups walking. When I was visiting the Lake District (and other similar places) to go walking I know that I was able to average about 5km/hour overall, the uphill bits being the slower part of the average. I used to be able to comfortably achieve 5km/hour on other walks.

These days I notice that I am now walking slower than most people - a walk along the street will see people passing me, and I have to speed up if I want to keep up with a group of people. Recently I went to meet a friend for a couple of drinks which involved a 1.8km walk which I managed in about 23 mins, slightly downhill - about 4.7km/hr, which felt like quite a pace.

The main new thing to note, however, is that about 3/4 of the way back home at the end of the evening my legs were feeling really tired, and I suspect that I shouldnt say that my walking distance is unlimited any more. According to fitbit I had a 15km day, which is one of my busier days. You should note that I treat the distance on fitbit as a measure of activity rather than a measure of distance because fitbit counts some steps whilst I am cycling, but my average distance/busyness is about 9km per day.

Saturday, 18 November 2017

Hyperactive reflexes and Clonus

Two of the HSP symptoms which are mentioned reasonably often are hyperactive reflexes and clonus, but I wasnt sure what they are, so it was time to do some investigation. And, actually its quite tricky to work out. It appears that hyperactive reflexes and clonus are related - both are grouped together under the heading Hyperreflexia. It is very easy to find plenty of references to these as being symptoms of something else. Apologies for a very link heavy post!

Looking at HSP, I find: spasticity is an increase in muscle tone with resulting stiffness. Muscle tone refers to the mild contraction that muscles continue to exhibit even when at rest (ie, resting muscle tone). A reflex between nerve endings in the muscle and spinal cord regulates muscle tone. Normally, the corticospinal nerves control and reduce sensitivity of this reflex. Because HSP causes deterioration of the corticospinal nerves, the reflex is not reduced as it should be, the result being an exaggerated (ie, hyperactive) reflex and increased muscle tone. Essentially, HSP prevents the muscles from relaxing properly.

Clonus is relatively easy to trackdown - it is alternate involuntary muscular contraction and relaxation in rapid succession. This can also be described as rhythmic. Clonus is a larger motion than a twitch, and the muscles will contract and relax between 3 adn 8 times a second, for between a few seconds and a few minutes. The connection is that these are often caused by a reflex. gives some info.  Once more there are videos of this, for example on the ankle: and a faster one here, and for the legs and here You can also see plenty of people with their legs shaking - Here's a description: This all reminds me of things I was told back in my 2013 survey about people being misdiagnosed with restless leg syndrome.

Hyperactive reflexes are more tricky to track down, and it is difficult to know what level of reflex is considered normal (although there is a scale of 0 to 4 with 2 being normal If a reflex is hyperactive then the muscles have a larger movement than normal when they are tested on the reflexes (and the score would be more than 2). You can see that some people have both hyperactive reflexes and clonus: and

There are plenty of websites and medical papers which describe people with HSP who have either or both hyperactive reflexes and clonus, but these papers tend to assume that the reader knows what they are!

(p.s. I had previously looked up clonus myself in 2012, reflecting on my jumping legs back at school:, and borrowing the clonus text from the SP Foundation website)

Sunday, 12 November 2017

New! Fitbit data (and some symptom updates)

Earlier in the year I got myself a fitbit (part of a gift from work to celebrate my working there for 20 years). If you dont know, this is a watch with a motion detector and a heart-rate detector. It allows me to track how active I am and how well I sleep (among other things). The data tracks over time. I'm hoping to be able to use it as another monitor for the slow progress of HSP.

So, first things first, what am I tracking?

  • Steps per day (along with distance & height)
  • Exercise/activity levels
  • Heart-rate
  • Sleep levels

I've got in my personal data spreadsheet various columns which track these things on a weekly basis. I'm hoping to be able to use this data in parallel with the data from strava ( and from patientslikeme ( and see if there are any trends. Perhaps between level of activity and fatigue

After looking at the first few months worth of data I note:

Stretching time - I hadn't been accurately timing the overall duration of my stretches. As I have been going to the physio regularly various extra stretches have been added in to my routine, and I had mentally chalked this up as "10-15 mins". However, using fitbit my typical stretching session is about 8-9mins. If I'm in a rush I'm in the 5-6mins and sessions over 10mins are unusual (for now).

Heart-rate - two things to note here. Firstly, my heart rate when walking goes up higher than my heart rate when cycling, which perhaps suggests that walking requires more effort than cycling. Also my resting heart rate goes up for a few days after I've consumed a fair bit of alcohol. Resting heart rate doesn't appear to be related to my level of exercise. Overall, the walking update is that I feel a bit slower now, and walking is beginning to require a bit more effort to do. I used to be a quick walker, and now I'm heading towards the slow end of normal/typical walking speeds. I notice this the most when I'm walking with other adults rather than out with my kids.

Stair counting - My fitbit counts number of flights of stairs climbed, which it does through sensing the change in air pressure. This is one of the aspects that I find frustrating as the count is not always accurate, and so sometimes I'll have gone up more flights of stairs then the fitbit says. My 'fix' for this is to raise my hand up in the air when I get to the top of the stairs and this normally registers the climb. I find that stair climbing is beginning to get more tricky - a bit slower, and having to use the banister. I expect that it will get more tricky as time goes on, so every step counts!

Step counting - This is generally good. As the watch is worn on the wrist it gets is step count by detecting the motion of the wrist rather than actually counting steps. So, it doesnt work well when I push a supermarket trolley around a supermarket for example, which leads me to suspect that a fitbit wouldn't work work well for someone with a walking frame. On the other hand, I can get quite a bit step count from doing tasks like unloading the dishwasher or ironing clothes, so for me I'm using step count as a measure of activity level rather than specifically a count of the number of steps I've taken. Having said that I've set myself a daily step target, and if I'm close to it at the end of the day I'll just a few more steps to get to the target or to the next badge level. (

Toilet counting! Fitbit can remind/nag you take 250 steps per hour, to avoid staying stationary for long periods of time ( At work I find that if I use the toilets that are not nearest my desk then a round trip is just over 250 steps. I find that once an hour is about right for urinating. Worth noting that I'm not desperate to go after an hour, so I'm going to the toilet more frequently than my bladder demands, and sometimes I'm in meetings or on telephone calls that are more than an hour in length without a problem. but it helps to understand how often I'm going. If my calls/meetings get on for about 2 hours long then I do need to go, urgently!

When it comes to my end of year review next month I'll work out what new health tracking stats i want to add to my symptoms tracker - I have more than a full year of bike/strava data already. On this front, there are a few alterations to my usual routine as well. Back in April I began going out for more leisure/social bike rides as well as my regular commute, so the first third of the year has shorter distances than the second two thirds. I suspect I'll need a graph to see if there are any trends.

Sunday, 5 November 2017

International HSP names

Part of the promotion for my online HSP survey has been finding HSP groups on Facebook and letting them know. I spent some time translating a post about my survey into different languages. (I used Google translate). It comes as no surprise that the name HSP is different in each language. This post puts down what I have found:

Hereditary Spastic Paraplegia
Hereditary Spastic Paraparesis
Familial Spastic Paraplegia
Familial Spastic Paraparesis

French: Paraplégie Spastique Familiale (PSF)
Portuguese: Paraparesia Espástica Familiar (PEF)
Spanish: Paraparesia Espástica Familiar (PEF)
Italian: Paraparesi Spastiche Ereditarie (PSE)
Italian: Paraparesi Spastica Familiare (PSF)
Swedish: Hereditär Spastisk Parapares (HSP)
Dutch: Hereditaire Spastische Paraparese (HSP)
German: Hereditären Spastischen Spinalparalysen (HSS)
Danish: Hereditær Spastisk Paraplegi (HSP)
Danish: Hereditær Spastisk Paraparese (HSP)
Greek: Κληρονομική Σπαστική Παραπληγία (ΚΣΠ)

Strümpell-Lorrain disease
La maladie de Strümpell-Lorrain
Strümpell-Lorrain`s sygdom

If you know any more, do let me know!

Also, when thinking of a list of HSP names, i know there are also names for certain types of HSP, found on OMIM

SPG1: MASA Syndrome / CRASH Syndrome
SPG3A: Strumpell Disease
SPG9A: Cataracts with Motor Neuronopathy, Short Stature, and Skeletal Abnormalities
SPG15: Kjellin Syndrome
SPG17: Silver Syndrome
SPG18: Intellectual Disability, Motor Dysfunction, And Joint Contractures; IDMDC
SPG20: Troyer Syndrome
SPG21: Mast Syndrome
SPG23: Lison Syndrome
SPG35: Fatty Acid Hydroxylase-Associated Neurodegeneration
SPG39: NTE-Related Motor Neuron Disorder
SPG47: Cerebral Palsy, Spastic Quadriplegic, 5, Formerly; CPSQ5
SPG50: Cerebral Palsy, Spastic Quadriplegic, 3, Formerly; CPSQ3
SPG51: Cerebral Palsy, Spastic Quadriplegic, 4, Formerly; CPSQ4
SPG52: Cerebral Palsy, Spastic Quadriplegic, 6, Formerly; CPSQ6

This tells me that there is no SPG22 or 40, and the list gets a bit fragmented in the high 50's and beyond. At the time of writing the last one is SPG79.

Saturday, 21 October 2017

Comments about my walking

In the last year or so i've started to get the occasional comment about my walking.

Some of these are from colleagues at work who I don't see very often. They all know about my HSP, but I have also had some comments from strangers. I wasn't expecting this to happen! It is a bit unsettling, and i've not yet worked out how I should react.

I realise that this must mean that my spasticity is beginning to be more visible, and I'll probably have a few answers lined up which I can just give. Reciting my story to strangers is not really my style, so initially I'll probably go for something like "my legs are just a bit stiff". (I realise that comment about not reciting my life story is a bit contrary to you reading my life story here!)

One person made a comment once that I should use the lift, when my foot scuffed on the way up some stairs. I automatically thought "no! I need to use the stairs to keep my muscles working, you don't understand" - but of course I didn't say this. It was just a quick comment with no context, and my mental reaction was a bit strong and I need to watch that - part of the stress management toolkit.

Saturday, 14 October 2017


Fatigue seems to be a reasonably common symptom with HSP - my 2013 survey identified that fatigue was the third most commonly occurring symptom (after loss of balance and getting more stiff in the cold). 62% of respondents reported that fatigue was significant (occuring frequently, regularly, most of the time or all of the time), 20% of respondents indicated that fatigue was minor or affected them occasionally, with 8% of respondents not being affected. Full report here:

So, what information can I found out about fatigue in HSP? - Not much.

People with HSP on patients like me also report fatigue, with 20% reporting severe, 40% moderate, 33% mild and 7%  without fatigue. Grouping severe and moderate together (60%) this is the same result I showed in 2013. Data here (although you have to be signed in to see this).

There are two papers:
One from 1999: - with no abtract here. Some hunting shows the start of the article here: This paper appears to cover the aspects of HSP as they were at the time. There is no mention of fatigue on the first page.

The other paper is from 2016: This paper compared 30 people with SPG4 HSP with 30 controls in Brazil. This paper reports that patients with HSP had higher levels of fatigue than controls (as well as more pain and more depression).

The lack of published information, and comments about fatigue from others led me to include this in my 2016 survey, I asked people to complete 3 different fatigue surveys and these results showed;

  • 11% of people had mild fatigue, 62% had moderate fatigue and 27% had severe fatigue. 
  • Fatigue is generally independent of mobility.
  • Whilst the physical factors of HSP contribute the most to fatigue there is also an important cognitive aspect. 
  • Those with SPG7 tend to have a higher level of fatigue than those with SPG4. 

I compared my results with the Brazil results and found they were comparable.

If you have a low mood/depression and wish to do something about it, you could look here: - I found this on this podcast - plenty of other interesting similar podcasts here!

Friday, 6 October 2017

Summary of depression posts

I've been keeping an eye out on my blog statistics, and one of my posts on depression is getting quite a bit of interest in the last few weeks.

This post simply summarises the main depression posts I've put up, here together for handy reference.

Stress and depression tests (March 14)
In this post I report the Generalised Anxiety Disorder Questionnaire (GAD-7) questionnaire and the Patient Health Questionnaire (PHQ-9) tests after encountering them at my referral to the local psychology unit. The post includes links to both and a summary of how scores are interpreted.

The depression paper! (September 11)
This post is where I first found the paper describing the prevalence of depression in people with HSP from Estonia. I then use this paper in 2015 as a comparison with my own results.

Stress and mood management course notes (April 2014)
This post is a precis of my notes from the stress and mood management course I went on. The course introduced cognitive behavioural therapy, descriptions of the normal cycles of stress/anxiety and low mood/depression, and various tricks to help break out of those cycles, rules for living, communication, and problem solving.

Assessing your own depression (January 16)
This post was written after a conversation with a friend who had been suffering from depression and described an alternative approach to assessing yourself than the two questionnaires from the 2014 post. This self assessment considers well-being and aligns it with depression. You score yourself on how well you are living within your own values.

Presentation at the 2013 AGM
At the 2013 UK AGM Liz Redmond gave a talk called "Looking After Yourself" which covered low mood, another term for depression. The talk gave various techniques to help look after your mental health. (On a similar theme, see also;

Part of my 2015 survey:
In my 2015 survey I asked respondents to complete the PHQ-2 questionaire which can be used as a screening tool for depression. Overall 63% of respondents had some symptoms of depression and 37% were without those symptoms. Additionally, the results suggest that around one quarter of people with HSP may require further assessment for depression, particularly for those who are using walking frames all or most of the time to get around. There are further details in the link on my analysis of these results.

A symptom included in my 2013 survey
Depression was one of the symptoms mentioned in my 2013 survey, which showed roughly 1/3 with no depression, 1/3 with mild depression and 1/3 with significant depression.

Wednesday, 27 September 2017

Alexa as a home help?

I was having a discussion with my mum about getting a fall alarm, and there are different options available. These systems give you some kind of button to press which causes your phone to dial predetermined phone numbers so that someone can come round to help you get up.

I wondered if it might be possible to use Alexa (or one of the other similar products) as a similar system. A quick bit of online looking tells me that Alexa has a number of skills which you can enable. One of these appears to allow you to do this, using the "My Family SOS" skill: You say the phrase "Alexa open my SOS family" and it will start calling your list one number at a time.

It strikes me that you might need to test how far from the speaker you can be for it to work well, and you might need to think about moving the speaker nearer the areas where you have a higher risk of falling, or having more than one speaker around your house. This could represent a disadvantage.

On the advantage side it would mean that you wouldn't need to remember to put your fall bracelet/necklace on.

It strikes me that these voice activated speakers could be a bigger help than this. Others think so too. Here's an article where a blind person considers how Alexa could help the disabled:

Another article on how Alexa could help all kinds of people:

Just for clarity, I dont have one of these.

Update 28/9: Another, more comprehensive system:

Tuesday, 12 September 2017

2017 Survey Now Open

After the success of my previous surveys, and feedback from readers and others, I'm continuing the pattern with another survey this year.

My focus for this survey is understanding:

  • How HSP affects peoples jobs/occupations
  • Pain
  • Factors that affect walking
  • Wellbeing

There are a range of questions for each topic. I have designed my own questions for occupation and walking factors. Pain is assessed using the Short Form McGill Pain Questionnaire 2, with extra questions on where the pain is felt and how you treat it. Wellbeing is assessed using the Warwick-Edinburgh Mental Well-being Scale (WEMWBS) for  assessing positive mental health and the Patient Health Questionnaire (PHQ2) used as a screening tool for depression.

Following the previous pattern, I will collect results until early 2018, then analysing these in time to publish the results here on rare disease day, 28th Feb 2018.

Also like before, all questions are optional (apart from your name and country). If you have taken part in any of my surveys before, I'd appreciate you using the same name to allow tracking.

I would appreciate any readers with HSP to complete this survey:

Mid October update: I've just over 100 responses so far. Quick highlights: 

  • 80% consider themselves disabled. 
  • 80% get pain from HSP. 
  • 80% cannot walk as far as they want. 
  • 40% are in work, 30% do not work. (other 30% retired/student/carer). 
People are spending ~25mins to complete the survey, and I'd be really pleased for some more responses. Thanks to everyone!

Early December update: I've now got over 150 responses, and am looking into the results in a little more detail. I'm still collecting answers for another month or so, so if you have HSP and a spare half an hour, please take part.

As more complete the survey the proportion considering themselves disabled has gone up. Now 85%.

80% of people with HSP suffer from pain. 50% have mild/discomforting pain whereas 30% have more severe pain.

For people who have walking effects, the biggest factors affecting walking are all types of uneven/sloping/stepped ground, carrying things, needing the toilet or being tired.

85% of people who do not consider themselves disabled are working or studying, whereas for those who consider themselves disabled it is 40% working or studying.

Around 2/3 of people have had to change jobs or stop working early as a result of HSP. A similar proportion expect that they will have to change jobs or stop working early as a result of HSP in the future.

Thursday, 24 August 2017

AGM2017: Living with the enemy - Robin Paijmans

The last presentation at the AGM was called Living With The Enemy: Psychology of Chronic Conditions, by Robin Paijmans. Robin is a psychologist looking at human behaviour, and how changes in behaviour affect life.

With chronic conditions there are both physical changes and mental changes. There are often different tools which can help to cope with the physical changes, however the issues around changes in mental are that these require a change in the way that we think. With chronic conditions there are three questions:
How do I cope?
How does my family cope?
How do professionals cope.

Robin observes that medical professionals are oftem compulsive problem solvers, they want to fix things, and often with chronic conditions there are no cures or solutions, which presents a problem for the problem solver.

Generally, people deal with problems either by moving towards the problem or moving themselves away from the problem - the approach/avoid.

When we visit healthcare professionals we ask questions like: Will they know about my condition? Can I trust what they say? Will they help me? Are they behaving appropriately (listening/giving attention/etc.)?

The healthcare professional may have questions of their own: Will the patient know how I feel? What will I do? What if I dont know what to do?

Robin then described a "brain hack" which people may be able to use at times that they are not feeling happy. It is a mindfulness technique. I'll write the points as a list of bullets:

  • Pick something which is worrying you
  • Choose a number between 1 and 10 to represent how much this worries you (1 is perfectly OK)
  • Imagine the issue as an object in the room/space that you are in. Think how it looks:
    • What colour is it?
    • What shape is it?
    • What size is it?
    • What texture does it have? (e.g. rough/smooth)
    • What temperature is it?
    • How heavy is it?
    • Where is it in the room/space that you are in?
  • Now imagine moving the object to a place outside the room/space.
  • Now imagine moving it a couple of miles away.
  • Choose a number between 1 and 10 to represent how much this worries you (1 is perfectly OK)
The second number should be smaller than the first number, and you have mentally shrunk the problem.

Note - if you cannot mentally move the object away from you then try changing its colour/size/weight/texture instead.

Robin discussed values, in that these values are a compass heading to guide you towards things that you want to do/achieve/have. The values themselves are not the destination. However, some things that we do to move away from discomfort can also move us away from our values. Once you have identified your values and being working towards them this can give you the strength to face threats. There are lots of things which we can do every day to reinforce our values.

Robin mentioned two books:

I've ordered the second one from my library. I'll post a review when I've read it!