Sunday, 7 September 2014

The ALS Ice Bucket Challenge

Many people have seen or heard of the ALS Ice Bucket Challenge. I watched this through facebook and it circled closer and closer to me with friends and family being challenged and then nominating on. [Feb 17 - realised I hadn't included the video, now at the bottom]

I had spotted that ALS was a mis-diagnosis for HSP in my survey last year, and so I thought about the similarities and differences between them.

So, I start with a definition:
Motor Neuron Disease can refer either to the most commonly occurring form - ALS (Amyotrophic Lateral Sclerosis) also known as Lou Gherig's disease, or to the broader spectrum of conditions.

Motor Neuron Diseases (MND) are a group of neurological disorders that affect motor neurons in adults and in children.  Motor neurons are specific types of cells that control voluntary muscle activities such as speaking, walking, and breathing. 

Everyone who has looked up HSP on the internet must have read "Hereditary Spastic Paraplegia (HSP) is a group of rare, inherited neurological disorders" (today, copied from the SPF website). HSP is a neurological disease because the long nerves in the spine are gradually degenerating - the nerves are the neurological system (paraphrased from HSP Research Foundation).


The one paragraph description of HSP (with parts borrowed from both previous links) is:
The primary features of HSP are spasticity and weakness in the legs, varying between individuals. There is progressive difficulty walking and symptoms worsen over time. Initial symptoms are typically difficulty with balance, stubbing the toe or stumbling. Changes begin gradually. As the disease progresses, canes, walkers and eventually wheelchairs may become needed, although some people never require assistive devices. A wide variety of symptoms are observed across cases and over time, including balance, fatigue, bladder and back pain. The majority of individuals with HSP have a normal life expectancy. [Jan 2015 update - this refers to people with uncomplicated HSP]


Whereas ALS describes as (NHS:

The symptoms of motor neurone disease begin gradually over weeks and months. Common early symptoms are: weakened grip, weakness at the shoulder, "foot drop", dragging of the leg, slurred speech.  As damage progresses, symptoms spread to other parts of the body and the condition becomes more debilitating. Eventually, a person with motor neurone disease may be unable to move. They may also find communicating, swallowing and breathing difficult. The condition is not usually painful. ALS is a severely life-shortening condition for most people. Life expectancy for about half of those with the condition is three to four years from the start of symptoms.


Primary lateral sclerosis (PLS) slowly gives rise to progressive weakness in voluntary muscle movement. The first symptoms are often tripping or difficulty lifting the legs. The disorder often affects the legs first, followed by the body, trunk, arms and hands, and, finally the bulbar muscles (muscles that control speech, swallowing, and chewing). PLS progresses gradually over a number of years, or even decades.  Life expectancy is normal.


I wont go further and list any other types of motor neuron disease, but note the similarities:
  • Early symptoms for all three are similar.
  • All three cause upper motor neurons to degenerate (those from the brain down into the spine)
And the differences:
  • ALS and PLS progress to more parts of the body
  • Life expectancy for ALS is reduced.

It depends where you read to see if HSP is included within the motor neurone disease list or not. I certainly found some websites which did and others which didnt.

I take the view that all this ALS ice bucket challenge activity raises awareness of diseases caused by degeneration of the nervous system, and that with all such causes it is down to the participants to personalise their message to encourage others to donate money. On this basis when I was nominated I donated to the UK HSP support group, and several friends and family who were nominated at around the same time did so as well.

Naturally, I write this with my own diagnosis safely under my belt, but having read up more about these other diseases, I can picture the trauma which people go through before getting their own diagnosis.

If there are any readers who were not lucky enough to be nominated for the ice bucket challenge and would like to make a donation, then I encourage you to do so, even if you dont empty a bucket of icy water over your own head. If you're stuck to find a relevant charity which you would like to donate to, then why not choose the UK HSP support group? 

You can read Wikipedia's ice bucket challenge story here too: it would seem that golfer Chris Kennedy was the first to nominate/donate for ALS on July 15 2014, and former basketball player Pete Frates who has ALS and is a patient advocate began spreading the ALS word through Twitter. 


I think it is worth pointing out here as well, the other major mis-diagnoses for HSP in my survey were ataxias (MS, ataxia, Spinocerebellar Ataxia)

( These are diseases which affect co-ordination, balance and speech, usually as a result of damage to the brain. The symptoms of ataxia can affect every part of the body and cause difficulties with: walking, balance, speaking, vision, swallowing or tasks that require a high degree of physical control, such as writing and eating. There are currently more than 50 recorded types of ataxia, which fall in three broad categoriess.

  • acquired ataxia – from trauma, stroke, multiple sclerosis (MS), brain tumour etc.
  • hereditary ataxia – develops slowly over many years from underlying genetics 
  • idiopathic late onset cerebellar ataxia (ILOCA) – progressively damaged over time for reasons that are still unclear

The key difference from HSP is that Ataxias affect the brain rather than the nerves, even though some of the symptoms appear the same. I note, of course, that complex forms of HSP affect the structure or functioning of the brain, and I can picture the trauma associated with difficulties getting a clear diagnosis.

It is also clear that there is a fair degree of cross-over here - some of the HSP groups on Facebook are for HSP and PLS people, and indeed that is the focus of the US SP Foundation: Also the SPATAX network groups together researchs in spastic paraplegia and ataxia:

Feb 2017 Video Addition

So, you want to see me getting iced? - here it is:

1 comment:

  1. Thanks for the interesting article, I am currently under the excellent care of Prof Pamela Shaw and her team ( and note that she says "My research group consists of a multidisciplinary team of basic and clinical scientists investigating molecular mechanisms of neurodegeneration in disorders of the human motor system, particularly motor neurone disease (MND), spinal muscular atrophy and hereditary spastic paraparesis (HSP)."