Friday, 14 January 2011

Whats in a name?

I have always wondered the difference between HSP and FSP, and have done a little research into the difference. Firstly, a look at the different names by which 'the condition' is known:

Hereditary Spastic Paraplegia
Hereditary Spastic Paraperisis
Familial Spastic Paraplegia
Familial Spastic Paraperisis
Strumpell-Lorraine syndrome
Strumpell's disease

Of course, if you look hard enough on the internet you can find other variations on this. The purpose of this post is to understand the difference between Hereditary and Familial, and the difference between Paraplegia and Paraperisis, with a passing look at Spastic. Strumpell and Lorraine will feature in another post.

Firstly, Spastic: My initial dictionary look ups end up going circular with all the definitions including the word spastic. However, I find how this word is derived - via Latin from the Greek spastikos meaning "drawing in", and first used in 1753 (Merriam Webster dictionary). From then it turns out to be more useful to look up spasticity rather than spastic.

Spasticity: a feature of altered skeletal muscle performance. There is a loss inhibitory control from the brain, such that muscles become overactive. This can cause an ongoing level of contraction, with decreased ability for the individual to control muscle contraction, and feel increased resistance on passive stretches. (paraphrased from Wikipedia)

Familial vs Hereditary: Familial - tending to occur in more members of a family than expected by chance alone. Hereditary - genetically transmitted or transmittable from parent to offspring (both Merriam Webster). Various other dictionaries have similar meanings for both, with the word Heredity popping up quite frequently - the passing of traits to offspring.

Paraplegia: Paralysis of the lower half of the body with involvement of both legs (Merriam Webster). Impairment in motor or sensory function of the lower extremities (Wikipedia). Paralysis of the legs and lower part of the body (

Paraperisis: Weakness of the lower extremities ( There do not seem to be so many definitions here.

Conclusion - Hereditary and Familial are interchangeable. Spastic Paraplegia refers to paralysis of the lower half of the body due to over-active muscles. In my humble opinion, Paraperisis is a red herring and Paraplegia describes this much better, although I accept that paralysis could well give rise to a weakness.

As always, comments welcome.

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