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.  https://emedicine.medscape.com/article/306713-clinical

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. https://en.wikipedia.org/wiki/Clonus gives some info.  Once more there are videos of this, for example on the ankle: https://www.youtube.com/watch?v=UX75k8s5QUE and a faster one here https://www.youtube.com/watch?v=XVlo1IB-DAc, and for the legs https://www.youtube.com/watch?v=AnxbuKNYY-c and here https://www.youtube.com/watch?v=fdas2LZ_X7Q. You can also see plenty of people with their legs shaking - https://www.youtube.com/watch?v=vwgnCyGqZ5M https://www.youtube.com/watch?v=VW6kWT92xrs. Here's a description: https://www.youtube.com/watch?v=yXugT6hzCXk 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 https://www.ncbi.nlm.nih.gov/books/NBK396/). 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: https://www.youtube.com/watch?v=PPPgTq3L6k4&t=12s and https://www.youtube.com/watch?v=cC7uDy2DnQw&t=76s

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: http://hspjourney.blogspot.co.uk/2012/01/clonus_21.html, and borrowing the clonus text from the SP Foundation website)

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 (http://hspjourney.blogspot.co.uk/2016/11/survey-update.html) and from patientslikeme (http://hspjourney.blogspot.co.uk/2013/12/another-outlet-for-my-message.html) 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. (https://blog.fitbit.com/fitbit-badges/)

Toilet counting! Fitbit can remind/nag you take 250 steps per hour, to avoid staying stationary for long periods of time (https://help.fitbit.com/articles/en_US/Help_article/1878). 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.

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:

English:
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 https://www.omim.org/phenotypicSeries/PS303350

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.