The other names by which 'the condition' are known are Strumpell-Lorrain syndrome and Strumpell's disease.
A search on the trusty internet soon identifies that Strumpell and Lorraine are both people. Wikipedia has a page all about Adolph Strumpell, and this quickly links you here - http://www.whonamedit.com/doctor.cfm/1658.html which gives a fuller description.
One sentence directly lifted from here says: His works in neuropathology are of such fundamental importance that he must be ranked among the founders of neurology as a clinical educational discipline in Germany.
Wikipedias page on HSP notes that the condition "was first described in 1883 by Adolph Strümpell, a German neurologist, and was later described more extensively in 1888 by Maurice Lorrain, a French physician" (and this phrase seems to get repeated again and again and again)
There, appears to be very little information about Maurice Lorrain, except to note that he was born in 1867. If anyone knows of more detail here, please let me know!
It would appear that the original references for the condition are:
•A. G. G. von Strümpell:
Beiträge zur Pathologie des Rückenmarks.
Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1880, 10: 676-717.
•M. Lorrain:
Contribution à l’étude de la paraplégie spasmodique familiale.
Thesis de Paris, 1898.
These references are from here:http://www.whonamedit.com/synd.cfm/1759.html
Oh, I spot that Lorrain may have favoured the FSP acronym....
This blog records my journey to Hereditary Spastic Paraplegia (HSP, also known as Familial Spastic Paraparesis or FSP). I was diagnosed with SPG4 in 2009 when my wife became pregnant with our first child. I currently wear insoles, do daily stretches and weekly Pilates. I take medication for my bladder. I tweet about HSP, RareDisease and other things @munkee74.
Pages
▼
Thursday, 27 January 2011
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 (medical-dictionary.com).
Paraperisis: Weakness of the lower extremities (medterms.com). 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.
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 (medical-dictionary.com).
Paraperisis: Weakness of the lower extremities (medterms.com). 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.