Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 G'day , Welcome! I guess you're the NZ Smyth from the Strength-L list which seems to have basically died lately?? Smyth wrote: Hi Mark and all,This is my first post on this list, I have been reading the posts for awhile though. I am on the HSP list and PLS has been talked about alot due to symptoms being similar. I too asked myself about whether PLSers and HSPers should get together. I am 28 yrs and use a electric wheelchair for my mobility. I got my first symptoms when I was 4yrs old. I tip toed when I walked. I used crutches then a walker then a manual wheelchair. My legs are more affected. I have spasitiy in my thigh muscles whcih cause my legs to go in a spasm on excitment, effort etc and stick out straight, then they become really hard to bend at the knee. I have to lean forward in my chair to release the spasm. My upper body is weaker than normal. I go swimming and to the gym during the week. What are the main PLS symptoms???HSP main symptoms are spasm/weaking of lower body, Babinski sign (run your finger under the bottom of my foot, it curls right up), tip toeing, funny walk. -- . ,-._|\ Covington / Oz \ \_,--.x/ v Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Hi , Welcome to PLS-Friends. You are the first PLSer I know of (if you are a PLSer, not an HSPer--if there is a difference) who has experienced symptoms at such a young age--although there are several reports in the medical literature of HSPers developing symptoms while still babies/toddlers. Does anyone else in your family have similar symtoms? Mark --- Smyth wrote: > Hi Mark and all, > This is my first post on this list, I have been > reading the posts for awhile though. I am on the > HSP list and PLS has been talked about alot due to > symptoms being similar. I too asked myself about > whether PLSers and HSPers should get together. I am > 28 yrs and use a electric wheelchair for my > mobility. I got my first symptoms when I was 4yrs > old. I tip toed when I walked. I used crutches > then a walker then a manual wheelchair. My legs are > more affected. I have spasitiy in my thigh muscles > whcih cause my legs to go in a spasm on excitment, > effort etc and stick out straight, then they become > really hard to bend at the knee. I have to lean > forward in my chair to release the spasm. My upper > body is weaker than normal. I go swimming and to > the gym during the week. > > What are the main PLS symptoms??? > HSP main symptoms are spasm/weaking of lower body, > Babinski sign (run your finger under the bottom of > my foot, it curls right up), tip toeing, funny walk. > > > > > > > > <1) some PLS and HSP are actually the same disorder, > and, if so, > <2) whether PLSers and HSPers should get together. > > <It should be an interesting day. > > <Mark> > __________________________________________________ Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Hi , and dear Mark, Welcome to the group, . All PLS friends are the best, what can be. All are so warm and nice. My name is Sonja and I am also 28 years old. I come from Switzerland. I habe my disease since more than 10 years. My first symptoms started perhaps at age of 14 years. I am wheelchair bounded, have a spasticity paresis at upper and lower limbs and thrunk. I have problems with speech, swallowing and breathing. I recived my diagnosis just a year ago. All physicians did not think that a so young girl or woman can have a such diagnose like PLS or ALS. My breathing and swallowing problems are very progressed and now I have the same treatment like ALS patients. A very good friend of me has HSP. His brother and father too. Theyr lower limbs are affected. They are in a wheelchair and can go with two canes. But they haven't some speech and swallowed problems. I know them very good. But they can't english. I am a litle bit confuced about the possibility that some PLS patients have really perhaps HSP? Take care and best wishes Sonja > > Hi Mark and all, > > This is my first post on this list, I have been > > reading the posts for awhile though. I am on the > > HSP list and PLS has been talked about alot due to > > symptoms being similar. I too asked myself about > > whether PLSers and HSPers should get together. I am > > 28 yrs and use a electric wheelchair for my > > mobility. I got my first symptoms when I was 4yrs > > old. I tip toed when I walked. I used crutches > > then a walker then a manual wheelchair. My legs are > > more affected. I have spasitiy in my thigh muscles > > whcih cause my legs to go in a spasm on excitment, > > effort etc and stick out straight, then they become > > really hard to bend at the knee. I have to lean > > forward in my chair to release the spasm. My upper > > body is weaker than normal. I go swimming and to > > the gym during the week. > > > > What are the main PLS symptoms??? > > HSP main symptoms are spasm/weaking of lower body, > > Babinski sign (run your finger under the bottom of > > my foot, it curls right up), tip toeing, funny walk. > > > > > > > > > > > > > > > > <1) some PLS and HSP are actually the same disorder, > > and, if so, > > <2) whether PLSers and HSPers should get together. > > > > <It should be an interesting day. > > > > <Mark> > > > > > __________________________________________________ > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Hi , Welcome, Hello Mark and others. I have my NIH appointment in latter July. Will any of the testing differentiate PLS vs. HSP? I am under the impression that the testing done at NIH should either reconfirm a PLS diagnosis, or come up with a new one. Is that right? I still haven't heard of any others going to NIH this summer. Where are you? Take care all, and God bless. Laurel --- Mark Weber wrote: > Hi , > > Welcome to PLS-Friends. You are the first PLSer I > know of (if you are a PLSer, not an HSPer--if there > is > a difference) who has experienced symptoms at such a > young age--although there are several reports in the > medical literature of HSPers developing symptoms > while > still babies/toddlers. > > Does anyone else in your family have similar > symtoms? > > Mark > > --- Smyth wrote: > > Hi Mark and all, > > This is my first post on this list, I have been > > reading the posts for awhile though. I am on the > > HSP list and PLS has been talked about alot due to > > symptoms being similar. I too asked myself about > > whether PLSers and HSPers should get together. I > am > > 28 yrs and use a electric wheelchair for my > > mobility. I got my first symptoms when I was 4yrs > > old. I tip toed when I walked. I used crutches > > then a walker then a manual wheelchair. My legs > are > > more affected. I have spasitiy in my thigh > muscles > > whcih cause my legs to go in a spasm on excitment, > > effort etc and stick out straight, then they > become > > really hard to bend at the knee. I have to lean > > forward in my chair to release the spasm. My > upper > > body is weaker than normal. I go swimming and to > > the gym during the week. > > > > What are the main PLS symptoms??? > > HSP main symptoms are spasm/weaking of lower body, > > Babinski sign (run your finger under the bottom of > > my foot, it curls right up), tip toeing, funny > walk. > > > > > > > > > > > > > > > > <1) some PLS and HSP are actually the same > disorder, > > and, if so, > > <2) whether PLSers and HSPers should get together. > > > > <It should be an interesting day. > > > > <Mark> > > > > > __________________________________________________ > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Hi , PLS comes in different flavors--just like HSP. Some PLSers have symptoms from the waist down. The symptoms are: -spasticity/weakness in the legs (and sometimes trunk), -drop foot, -hyperreflexia in the legs (and sometimes mild hyperflexia in the arms), -sometimes urinary urgency, and -sometimes loss of vibration or pin prick sensation in the lower legs. In other cases, the upper body is also affected. Symptoms can then also include: -spasticity/weakness in the hands/arms, and/or -difficulty speaking and/or swallowing. In yet other PLS cases,symptoms can start in the mouth with difficulty speaking and/or swallowing. Age of onset and rate of progression all vary. HSPers with the first set of symptoms have " pure " or " uncomplicated " HSP. HSPers with the 2nd set have " complicated " HSP. But unlike PLSers, HSPers have a family history of the symptoms. Mark Weber --- Smyth wrote: > Hi Mark and all, >snip< > What are the main PLS symptoms??? > HSP main symptoms are spasm/weaking of lower body, > Babinski sign (run your finger under the bottom of > my foot, it curls right up), tip toeing, funny walk. > > > > <1) some PLS and HSP are actually the same disorder, > and, if so, > <2) whether PLSers and HSPers should get together. > > <It should be an interesting day. > > <Mark> > __________________________________________________ Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Laurel, The testing at NIH may confirm you PLS diagnosis. Then again, it may not. For me, it opened new possibilities which have since been ruled out. The object of the study has something to do with further defining PLS in comparison to ALS--I think(??). Mark --- Laurel Aasland wrote: > Hi , Welcome, Hello Mark and others. > I have my NIH appointment in latter July. Will any > of > the testing differentiate PLS vs. HSP? I am under > the > impression that the testing done at NIH should > either > reconfirm a PLS diagnosis, or come up with a new > one. > Is that right? > I still haven't heard of any others going to NIH > this > summer. Where are you? > Take care all, and God bless. > Laurel > > --- Mark Weber wrote: > > Hi , > > > > Welcome to PLS-Friends. You are the first PLSer I > > know of (if you are a PLSer, not an HSPer--if > there > > is > > a difference) who has experienced symptoms at such > a > > young age--although there are several reports in > the > > medical literature of HSPers developing symptoms > > while > > still babies/toddlers. > > > > Does anyone else in your family have similar > > symtoms? > > > > Mark > > > > --- Smyth wrote: > > > Hi Mark and all, > > > This is my first post on this list, I have been > > > reading the posts for awhile though. I am on > the > > > HSP list and PLS has been talked about alot due > to > > > symptoms being similar. I too asked myself > about > > > whether PLSers and HSPers should get together. > I > > am > > > 28 yrs and use a electric wheelchair for my > > > mobility. I got my first symptoms when I was > 4yrs > > > old. I tip toed when I walked. I used crutches > > > then a walker then a manual wheelchair. My legs > > are > > > more affected. I have spasitiy in my thigh > > muscles > > > whcih cause my legs to go in a spasm on > excitment, > > > effort etc and stick out straight, then they > > become > > > really hard to bend at the knee. I have to lean > > > forward in my chair to release the spasm. My > > upper > > > body is weaker than normal. I go swimming and > to > > > the gym during the week. > > > > > > What are the main PLS symptoms??? > > > HSP main symptoms are spasm/weaking of lower > body, > > > Babinski sign (run your finger under the bottom > of > > > my foot, it curls right up), tip toeing, funny > > walk. > > > > > > > > > > > > > > > > > > > > > > > > <1) some PLS and HSP are actually the same > > disorder, > > > and, if so, > > > <2) whether PLSers and HSPers should get > together. > > > > > > <It should be an interesting day. > > > > > > <Mark> > > > > > > > > > __________________________________________________ > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Sonja, I'm glad to see that you're back. We all missed you. I hope that your pneumonia clears up soon. The PLS/HSP matter is based solely on a hunch that I have, that I have been slowly sharing with PLSers (and HSPers) over the past several months. Here are my ideas/questions. (And please remember--I am neither a physician nor a scientist. A real scientist might easily tear apart my ideas. (But some agree with my questions.) Because of my lack of science background, when you read the following, TREAD WITH CAUTION.) Hereditary spastic paraparesis (HSP) is strikingly similar to PLS. Since we're all familiar with PLS, I'll begin by describing HSP. (The next 3 paragraphs are taken from http://hspinfo.org/HSP.htm .) " Hereditary Spastic Paraplegia or Hereditary Spastic Paraparesis is a name used to represent a group of inherited degenerative spinal cord disorders characterized by a slow, gradual, progressive weakness and spasticity (stiffness) of the legs. Symptoms may be first noticed in early childhood, or at any age through adulthood. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. Rate of progression and the severity of symptoms is quite variable even among members of the same family. Anticipation may occur in some families, with symptoms of the disorder beginning earlier and more severely in successive generations. HSP rarely results in complete loss of lower limb mobility, although mobility devices such as canes, walkers, or wheelchairs may be necessary. In some patients, the symptoms continue to increase throughout their life. For others, symptoms may begin in early childhood, worsen for a few years, then level off after adolescence. " " At the present time, diagnosis of HSP is generally a process of exclusion of other disorders, and observation of family history. However, a few of the gene locations have been found, and more are close to being identified. This will lead to the possibility (hopefully in the near future) of blood tests to confirm diagnosis. Blood testing for certain forms of the disorder can already be performed. " " HSP is generally classified as " uncomplicated " and " complicated " . In uncomplicated HSP, symptoms are generally limited to a gradual weakening in the legs, urinary bladder disturbance, and sometimes impaired sensation in the feet. " In complicated HSP, there are additional symptoms. These can include upper body weakness and spasticity, as well as dysarthria--speech problems. I have been on the HSP discussion group (info at http://hspinfo.org/hspl.htm) since 1999. (I'm mostly a lurker.) This year, I started tracking HSPers with speech problems. So far I've found 9 such HSPers who have spontaneously mentioned that they have speech problems. I would classify them all as having complicated HSP. Three of those 9 HSPers mentioned having a family history of HSP with speech problems. I do not know how many of the other approximately 210 HSPers in the HSP discussion group have complicated HSP with speech problems. Obviously, HSP is strikingly similar to PLS. So what are the differences? So far, the only discussion of the differences between HSP and PLS that I have found has compared PLS to uncomplicated HSP. (Video of November, 2000 HSP Symposium.) In the comparison, uncomplicated HSPers have symptoms only from the waist down--no arm/hand/speech problems. (But complicated HSPers can have these symptoms.) Uncomplicated HSPers also may have a loss of vibration sensation in their feet lower legs, as well as bladder symptoms. But some PLSers also experience these symptoms. Most important, HSPers have a family history of similar symptoms. PLSers have no family history. This seems to be the major difference. Pathology studies in HSP show that the motor neurons degenerate near their end--near the bottom of the spine. Not so in PLS. In PLS there is a loss of neurons in the precentral gyrus in the head, as well as a loss of neurons in the spine. But these differences might be reconciled. Only uncomplicated HSP cases were examined in the HSP pathology cases. They all had symptoms only below the waist. The autopsied PLS cases were all of PLSers with lower and upper extremity symptoms--including dysarthria--speech difficulties. Could the pathological differences between these two groups be because of the differences in symptoms between the two groups? Could a PLSer with no family history, and symptoms only below the waist have no more cell damage than an uncomplicated HSPer? Could a PLSer with lower and upper body symptoms, including dysarthria, have the same cell damage as a complicated HSPer with the same symptoms? No one knows (to my knowledge). In addition to being classified as complicated and uncomplicated, HSP can also be classified by the mode of inheritance: autosomal dominant, autosomal recessive, or X-linked. (For a good discussion of these terms, go to http://genetics.miningco.com/science/genetics and click on " Basic Genetics " in the box on the left. Then click on " How genetic disorders are inherited " at the end of the list in the middle of the page.) Notice that in autosomal recessive inheritance of HSP, both parents need to be carriers of the same defective HSP gene in order to have a child with HSP. Each child from such parents would have a 25% chance of inheriting the mutant gene and developing the symptoms. So far, only a few HSP genes have been identified. But several gene loci have been discovered. Scientists are working to find the HSP genes at those locations. For a great listing and discussion of the genes and gene loci responsible for HSP, go to the OMIN web site at http://www3.ncbi.nlm.nih.gov/Omim/searchomim.html, and search for the phrase " spastic paraplegia " . Start by clicking on the article for SPG7--one of the two HSP genes discovered to date. SPG7 is an autosomal recessive gene. Notice that the article mentions slowed speech in 2 of the HSPers studied. See also the article on the OMIN site about the SPG5A gene--another autosomal recessive gene where the HSPers with a defect in that gene presented with dysarthria--speech difficulties. Also go to http://www.geneclinics.org/profiles/hsp and see the sections on complicated, autosomal recessive HSP and uncomplicated, autosomal recessive HSP. The point of all this is only to question whether some PLS may in fact be autosomal recessive HSP--or a new mutation in an autosomal recessive HSP gene. (Some is also probably ALS--but not yet developed enough to properly diagnose as ALS. Some may also be a very slowly progressive form of ALS.) Autosomal recessive HSP could be diagnosed as PLS due to a lack of family history. A family history would not be present because one's ancestors never mated with anyone else with the same autosomal recessive HSP gene. There is of course no proof of this. Until the molecular research on HSP and PLS is completed this is just a hunch by a non-science type. YIKES! MUCH CAUTION needed here. Mark --- sonja.balmer@... wrote: >snip< > I am a litle bit confuced about the possibility that > some PLS > patients have really perhaps HSP? > >snip< > > Sonja > __________________________________________________ Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Hi Laurel, If you haven't been before the testing is to get a baseline and then see how your motor cortex is doing (at least that's what was done to us last year). The exam is just to be sure the docs in NIH agree with yours but it will not repeat all the testing. It's more a pass on what has already been done than starting from square one. Lavon At 07:49 AM 05/09/2001 -0700, you wrote: >Hi , Welcome, Hello Mark and others. >I have my NIH appointment in latter July. Will any of >the testing differentiate PLS vs. HSP? I am under the >impression that the testing done at NIH should either >reconfirm a PLS diagnosis, or come up with a new one. >Is that right? >I still haven't heard of any others going to NIH this >summer. Where are you? >Take care all, and God bless. >Laurel Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 Mark & Lavon: Thanks for the info. I am just so curious. Where did you all stay when you were there last year? The rooms are quite expensive, and quite a few dollars more than the $75.00 NIH allowance. I am quite broke right now, as I am newly single, and self-supporting. I am hoping to find a place for close to the $75.00. Thanks again. Laurel Re: new to list/pls/hsp > Hi Laurel, If you haven't been before the testing is to get a baseline and > then see how your motor cortex is doing (at least that's what was done to > us last year). The exam is just to be sure the docs in NIH agree with > yours but it will not repeat all the testing. It's more a pass on what has > already been done than starting from square one. Lavon > > At 07:49 AM 05/09/2001 -0700, you wrote: > >Hi , Welcome, Hello Mark and others. > >I have my NIH appointment in latter July. Will any of > >the testing differentiate PLS vs. HSP? I am under the > >impression that the testing done at NIH should either > >reconfirm a PLS diagnosis, or come up with a new one. > >Is that right? > >I still haven't heard of any others going to NIH this > >summer. Where are you? > >Take care all, and God bless. > >Laurel > > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 9, 2001 Report Share Posted May 9, 2001 One of our group stayed at NIH itself- seemed if you traveled alone you could- I'd ask Joy about that. I stayed at the Park Inn one night and would not recommend it. I can't remember the one I stayed at (would recognize the name as it is a major chain). The best deal was where Sue Niquette stayed can't remember that one either! If you travel alone the allowance is less than accompanied. I'm sorry I remember so few details. Call Joy and good luck! Let us know what you do! Lavon At 07:14 PM 05/09/2001 -0500, you wrote: >Mark & Lavon: >Thanks for the info. I am just so curious. Where did you all stay when you >were there last year? The rooms are quite expensive, and quite a few >dollars more than the $75.00 NIH allowance. I am quite broke right now, as >I >am newly single, and self-supporting. I am hoping to find >a place for close to the $75.00. >Thanks again. >Laurel Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 10, 2001 Report Share Posted May 10, 2001 Lavon: I got the best deal at the Park Inn, and they were the most courteous. It is $90+ tax. All the others were a minimum of $119, and the folks athe AmericanInn were rude. I booked a room with 2 dbls, for the $90, for 5 nights. My friend and I will stay an extra day and a half to pop over to DC and spend Saturday. I don't have the flight booked yet, but will today when I get home. Thanks for your input. Laurel P.S. I don't usually have to make all my own arrangements, so this is a new experience for me. And, being newly single, I usually travel with my husband, and this time it will be just us 2 gals. --- Mark and Lavon wrote: > One of our group stayed at NIH itself- seemed if you > traveled alone you > could- I'd ask Joy about that. I stayed at the Park > Inn one night and > would not recommend it. I can't remember the one I > stayed at (would > recognize the name as it is a major chain). The > best deal was where Sue > Niquette stayed can't remember that one either! If > you travel alone the > allowance is less than accompanied. I'm sorry I > remember so few > details. Call Joy and good luck! Let us know what > you do! Lavon > > At 07:14 PM 05/09/2001 -0500, you wrote: > >Mark & Lavon: > >Thanks for the info. I am just so curious. Where > did you all stay when you > >were there last year? The rooms are quite > expensive, and quite a few > >dollars more than the $75.00 NIH allowance. I am > quite broke right now, as > >I > >am newly single, and self-supporting. I am hoping > to find > >a place for close to the $75.00. > >Thanks again. > >Laurel > > > > > > Quote Link to comment Share on other sites More sharing options...
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