Status
Not open for further replies.

Paul Wicks

Member
Joined
Feb 15, 2006
Messages
22
Reason
Loved one DX
Country
UK
State
London
Just browsing, noticed something that might need tidying up.

I see in the description it says that PMA is a genetic condition, this isn't strictly true. In fact there have been either zero or less than a handful of reported cases of genetically inherited PMA. There are a group of genetically inherited conditions, the spinal muscular atrophies, which are genetically inherited and affect only the lower motor neurones, but these are different to progressive muscular atrophy.

PMA affects only the lower motor neurones and so causes wasting/atrophy and fasciculations but in the absence of UMN signs such as spasticity, brisk reflexes, or the Babinski sign. Patients with PMA survive longer than patients with ALS and in some cases symptoms can be restricted to the arms or legs for a long time before spreading elsewhere in the body. There may be two subtypes, one with a patchy distribution and one with a leg distribution. In the latter case progression is unpredictable, whilst in the latter there is a prolonged latency period between the progression from legs to arms, and then again to the bulbar region. PMA is not rapidly progressive. If it is it's probably not PMA but ALS.

Also it's not true that PMA affects only the lower limbs. All muscles in the body, from head to neck to arms to trunk to legs to toes are ennervated by upper and lower motor neurones. So you can have LMN dysfunction which affects your tongue (atrophy) or UMN dysfunction which affects your ankles (brisk reflexes).

In contrast to patients with ALS, PMA patients are highly unlikely to have a bulbar onset of disease, do not manifest signs of emotional lability / pseudobulbar affect, and so far as I have been able to measure, show no signs of the cognitive abnormalities encounted in a proportion of patients with ALS.

Many PMA patients don't know they have it or don't know the difference from typical ALS. It's hard to diagnose and strictly speaking you need to have had LMN-only symptoms for 4 years or more to be certain, and even then about 50% of LMN-only syndromes progress to ALS (known as "PMA->ALS"), sometimes as much as 26 years later.

When I get the time I'm going to be writing an article on Wikipedia about PMA, please keep your eyes peeled for it soon!

Cheers

Paul Wicks
 
Last edited by a moderator:

David

Moderator
Joined
Apr 24, 2003
Messages
353
Reason
Diagnosis
00/0000
Country
CA
State
ON
Thank you for your insight Paul. We all appreciate the time you have taken to educate readers and to ensure information is correct.

I've taken the liberty of updating the incorrect information that was on our forum home regarding PMA - I borrowed your words - I hope you don't mind.

Want to know more about Paul Wicks?

Dr. Paul Wicks is a neuropsychologist based in the United Kingdom (London).

Paul is very active in the fight against ALS and MND, and is also an avid contributor on the WWW (World Wide Web) - helping to inform people about ALS, MND and related disease.

Paul is not only a doctor and researcher, but also contributes as an editor for ALS/MND related topics at Wikipedia. In addition to all of this, in his spare time, Dr. Wicks owns and operates an MND forum called Build-UK.

For more information or to <a href='/forum/member.php?u=344'>contact Paul click here</a>.
 
Last edited:

Al

Legendary member
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
Good to hear that he actually knows of what he speaks. I didn't bother going to his site because I mistakenly thought he was trying to be a wise guy. Ok ladies. Once again a man admits he was wrong. Don't gloat too much. LOL.
 

David

Moderator
Joined
Apr 24, 2003
Messages
353
Reason
Diagnosis
00/0000
Country
CA
State
ON
Yes, Paul knows his stuff; most of it goes over my head on first read because of the medical prowess.

Accuracy of information is obvioulsy most important and it's nice that a medical professional specializing in this area is available to correct misleading or incorrect information.

Sometimes though, I can't help thinking that the medically correct definition can be less helpful to individuals than a laymen's explanation - concerning our first time visitors, individuals learning about MND for the first time, an overview can be helpful versus a thorogh definition.

If the information is wrong, it's wrong, and that's just wrong :) Thanks for helping set it straight.
 

Al

Legendary member
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
I agree with you 100% David. If you have no medical background all the technical terms at first will overwhelm an individual. A while back I posted something verbatim from a University web site and the questioner wrote back and said WHAT? Then I explained it in layman's terms. Worked much better. Sometimes KISS works better. Keep it Simple Stupid.
 

Barbie4

Distinguished member
Joined
Mar 26, 2006
Messages
139
Country
CA
State
Ontario
great to know

David:

Thanks for letting us know who Paul was. I was kinda wondering myself who he was as well. It is great to know a professional is on here along with the real pros. I understand more when Al writes it though. ha ha.
Paul thanks for all of your efforts with ALS/MND. Anychance you could find a cure soon? The sooner the better. :)

Barb :)
 

BWk

Distinguished member
Joined
Jun 6, 2006
Messages
113
Reason
PALS
Country
grant
State
new mexico
Paul or anyone else, What is a babinski sign mean and are some als persons being affected mentally also,noticed some changes in myself,forgetting more than usual, misspelling words more than I used to. It's not just stress either. I forget to pay credit card bills, etc. My mental state just is not up to par anymore.
 

Al

Legendary member
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
Babinski's sign is an abnormal reflex in the toes. When they scratch your foot with the pointer your toes should go one way and they don't do that with the Babinski reflex. Can't remember which way they are supposed to go but if they don't go the right way it is not a good sign and you usually are tripping over your toes. There is research being done with ALS dementia and it used to be believed that it was not present with ALS. Present day findings are that it is occurring more often and it may be that we are living longer and they are noticing it more or that there is something else going on. The ALSA newsletter a couple of months ago had an article about some research going on.
 

BWk

Distinguished member
Joined
Jun 6, 2006
Messages
113
Reason
PALS
Country
grant
State
new mexico
Al Thanks for info! Did you or anyone else out there have problems with eye pain? Have had pain in the eyes since sept.2005 worried it may be in the eye muscles?Still can move eyes ok, but it is a big concern of mine. I don't have glaucoma. Barry
 

MaggyQ

Member
Joined
Jun 30, 2006
Messages
24
Country
US
State
california
Some general information

I noticed the question regarding the Babinski sign.

This is a good site that explains all aspects of the neuro exam (which I'm sure most here have experienced):

http://endeavor.med.nyu.edu/neurosurgery/

This one contains the basic description of the Babinski reflex:

http://www.nlm.nih.gov/medlineplus/ency/article/003294.htm

(The big toe goes UP):)

I've done so much research over the past 2 years that I actually prefer the medical terminology over a too simplified lay version. Actually, I much prefer the technical
followed by a simplified synopsis to ensure that I got it. I find that if you're not given the technical terms that it's more difficult to do follow up research.

I've still not been diagnosed. One set of doctors think it's probably monomelic amyotrophy. Others aren't ready to attach the ALS label, but want to monitor me to see
whether I have progression. All I know is that I've been limping for over 2 years, am using an AFO, and have atrophy in my calf and thigh. I'm at the moment where I don't even know whether I have other symptoms because I've become so hyper-aware.

My problems all began with a severe wound from what I believe was a spider bite.
While doctors admit that they don't know the cause of these lower motor syndromes, they're all quick to say it's not the bite. I find that hard to accept because I had no problems until that wound, which was necrotic, took a year to heal, and was in the very
area where I had my initial symptoms.

Maybe someone here has some ideas?

TIA
 

Al

Legendary member
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
Thanks TIA. Good information. We try to not be too complex here but it is good if someone has a link to the technical websites. We're more about support and general information here. See you're a new member. Welcome. AL.
 

BWk

Distinguished member
Joined
Jun 6, 2006
Messages
113
Reason
PALS
Country
grant
State
new mexico
Maggy, Have you had any cramping? That seems to be the one thing different from me and others with this illness.I don't wish for cramps but most others with this disease I've noticed complain of cramps and I have not had one cramp so far. I'm about two yrs. into this illness.I do have much flaucidity in all the muscles of my body. Your case sounds a little different also, but I'am not sure what to make of it,not much help I guess.Bwk
 

MaggyQ

Member
Joined
Jun 30, 2006
Messages
24
Country
US
State
california
Al, thanks for the welcome. The reason I like the more technical explanations is because I find it easier to research and then to interpret test results, etc. I'm also used to reading medical charts and discoursing with doctors and technical experts since I'm a litigator. I figure knowledge is power. :-D But I can also understand the desire to focus
on support and less technical data. (btw, "TIA" was meant as shorthand for "thanks in advance". I guess you can use a Ronald Reagan line :"there (s)he goes again!":-D )


BWK: Yes, I had cramping in my right calf for a good 6 months before the foot drop really manifested. Before then I experienced a dragging sensation from time to time. Oddly, after the foot drop became obvious, the cramping basically stopped. I still get the occasional cramp, sometimes in the leg leg and once in a while in the abdomen. But
nothing like the daily cramps I had in the right calf.
 

Al

Legendary member
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
Hi Maggy. Always good to have a lawyer in the neighbourhood. Never know when you might need some advice. Hope you're not putting the forum in as billable hours. LOL.
 

MaggyQ

Member
Joined
Jun 30, 2006
Messages
24
Country
US
State
california
Hope you're not putting the forum in as billable hours. LOL

Well, not yet. :-D
 
Status
Not open for further replies.
Top