Question by Hayley: Does my dad have primary lateral sclerosis, amyotrophic lateral sclerosis, or Mya Gravis ?
In late 2007 my dad started having muscle weakness & leg stiffness but was still able to walk. As the weakness got worse he developed a small studder which also became progressivly worse (The studder happened over night) He went to speach therapy because he became hard to understand, After awhile the studder went away & he just talked really slowly. The muscle weakness progressed, he was falling almost everyday, once even down a flight of 15 stairs. More problems started appearing & in late 2008 is when the problems he already had became worse. As of right now he can’t barley talk at all, (he has to write down what he says), he can’t walk without a walker, & even with the walker he still falls, he’s broken his nose 4 times from the falls. He has trouble swollowing & chokes on food/liquids. He can’t breath … That may just be from the breaking of the nose. My dad went from a 5’5 200 pound muscular man, to a 130 pound man that can’t do anything on his own. He was tested for PLS & ALS .. He was positive for PLS & negitive for ALS but PLS is not fatal, ALS is. I heard PLS could turn into ALS? True or false? We don’t think he is going to make it much longer :( he is in the hospital right now because his legs were so swollen they were turning colors, & back pain from a recent fall. My dad discovered a disorder called MG which has some of his symptons but the doctor doesn’t think that’s the case. But my dad beleives that’s what he has even tho he was positive for PLS. Proboly because You can recover 50+ % with MG. Anyways my dad is in critical condition and even though he is + for PLS, that doesn’t fit his symptons. My dad is only 50 years old, he had to shut down his concrete buisness he had for 30 years because of this. & before he goes I wan’t to know what the hell is going on, so I can’t atleast try to help … I don’t know. Just if you know of any other disorders, if PLS can turn into ALS, or anything else that can help me PLEASE SEND AN ANSWER ! Thank you so much. -Hayley

Best answer:

Answer by Francis
I am sorry to hear about your father. Being a father of 3 girls I am encouraged by your interest in helping him.

I have PLS since 2003. First there is no test for PLS. They have to rule out everything and then they say PLS. PLS is rare..maybe 50 people/year get it in the US. In general, PLS progresses slowly. I started with speech problems and it took almost 5years to get to my legs. However, the speed of progression varies a lot. The median age to get it is 50 years old. I got it then. They thought I might have MG at the beginning so they tried IVIG treatment for 4 months. It did nothing so they ruled it PLS. PLS affects the upper motor neurons. These are the nerves from the brain and they connect to the lower motor neurons. ALS affects the upper and lower motor neurons. They can test the lower motor neurons with an EMG. They basically shock the nerves and observe the nerve response. If you have no lower motor neuron involvement then they say it is PLS. PLS and ALS are hard to distinguish in the early years. It took almost 3 years to get a confirmed PLS diagnosis. Basically they told me I would be dead or near death if I had ALS. PLS does not turn into ALS. However, I have heard of cases of ALS that progress slowly and look like PLS in the early years. I go to the MDA/ALS Clinic in Dallas. They are very good at diagnosing these diseases. PLS is covered by MDA. You may be able to get a referral to an MDA clinic. PLS is so rare that doctors normally know nothing about it.

In your Dad’s case he seems to have progressed quickly. I am worried on the breathing problems. Breathing is generally not a symptom of PLS. It is with ALS. He should be showing other symptoms like emotional issues (laughter/crying) or some dexterity issues in his hands. The other diseases they ruled out in my case was Wilson’s disease and Kennedy’s disease. They also ruled out Lyme disease.

Answer by Mags
I don’t think that we are going to be able to distinguish between these diseases online. My assumption is that your dad has a primary care physician and a neurologist. Your dad’s progression does sound like PLS, however, with one exception about which I don’t think we have adequate information. And it often takes a few years to tell if PLS isn’t actually ALS…if additional symptoms appear.

In looking at Myasthenia Gravis, I think your father is looking for hope. And I know that you are too. This is a terrible frightening time for you.

Take a look here – it is the easiest read. It will compare upper and lower motor neurons so that you can compare the symptoms which we haven’t heard but which you might know.
Some tests help to differentiate between ALS,PLS and MG. But many determining the results of tests is sometimes subjective rather than objective. Which means it is often a process of elimination.

http://74.125.95.132/search?q=cache:1XcRKOKv6aAJ:www.med.unc.edu/medicine/web/12.12.07%2520Honeycutt.ppt+difference+between+amyotrophic+lateral+sclerosis,+primary+lateral+sclerosis+and+myasthenia+gravis&cd=3&hl=en&ct=clnk&gl=us

The first thing I’m going to suggest is that you register (it’s free) at Patients Like Me as a caregiver. That will provide you with access to ask patients and caregivers alike what they are doing, experiencing, helpful suggestions such as setting up a bedroom on the first floor to avoid serious falls down steps.
http://www.patientslikeme.com/als/community

You don’t mention medications and the effects.
http://www.rxlist.com/rilutek-drug.htm
Riluzole – not without side effects

Some of the top treatments are : Riluzole; CoQ10, Vitamin E, Powered wheelchair. Vitamin C, Range of Motion Exercises, Non-invasive ventilator, multivitamins (careful here), Baclofen

Your dad will need physical therapy. I also suggest that your dad be taking anti-oxidants if he isn’t already. CoQ10 is a powerful anti-oxidant. Vitamin C with bioflavinoids, Vitamin E complex but only if it lists the amounts of each of the tocopherols.

You know more of his symptoms so I am providing a list of sites which list symptoms for each of the three diseases:

Myasthenia Gravis:
http://www.hsc.virginia.edu/uvahealth/adult_neuro/myasgr.cfm
I read this at the site above:
“A primary characteristic of MG results in the response of an affected person to certain medications. When given an anticholinesterase medication, such as neostigmine (Prostigmin) or edrophonium (Tensilon), muscle weakness often dramatically improves for a brief time. This provides strong support for the diagnosis of MG.”
What it suggests is that if your dad isn’t responsive to this medication, then he doesn’t have MG. You might want to show that to him or to his doctor.

Primary Lateral sclerosis:
http://www.ninds.nih.gov/disorders/primary_lateral_sclerosis/primary_lateral_sclerosis.htm

ALS:
http://www.hsc.virginia.edu/uvahealth/adult_neuro/als.cfm
“There is no proven treatment for ALS. However, the US Food and Drug Administration (FDA) approved Rilutek®, the first drug that has prolonged the survival of persons with ALS”

Since there can be genetic aspects to these diseases, it might be a god idea if both you and your father were tested. You can talk to his doctor about that.

It sounds as if your father’s condition has been seriously compromised by the falls. Please do everything you can to fall proof the house. Don’t forget the shower chair, rearranging furniture for easier pathways, removing loose throw rugs…although I suspect that you’ve already done most of this.

I’m going to post this answer and then do some more research – because I write about Parkinson’s disease and there are some pipeline projects which may also benefit ALS patients – and then I’ll return. But right now I have to take my husband to exercise therapy for his PD. Just want to make sure he’s taken his COQ10.

Breathe – you can help more when you are calmer and empowered. Just remembrt that many symptoms for diseases are not textbook. So this isn’t going to work like a geometry problem. The human body is very complicated. But a daughter’s love can really help alot. The calmer you can be, the better your father will feel because he will know what a good job he has done with you.

addendum
the differences between pls and als:
:PLS is caused primarily by degeneration of the upper motor neurons in the brain and spinal cord, which results in increasing spasticity and weakness of voluntary muscles. It is often referred to as a benign variant of Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s disease).
The primary difference between the two is that in PLS the spinal motor neurons or lower motor neurons stay intact. Thus, there is no muscle wasting (amyotrophy), which is the symptom that ultimately causes fatal complications in ALS.”
“However, there are some slowly progressing forms of ALS that are difficult to distinguish from PLS. In fact, up to 10% of ALS patients survive more than ten years. In addition, improved medical care is resulting in longer and more productive lives for all ALS patients”
“The hallmark of PLS is progressive weakness and spasticity of voluntary muscles.
The first symptoms are often tripping or difficulty lifting the legs. Other people may be the first to notice a change in the affected person’s gait.
Occasionally, speaking (dysarthria) and swallowing (dysphasia) difficulties, or arm weakness are the first symptoms. Speech problems can begin with hoarseness, a reduced rate of speaking, excessive clearing of the throat, or slurred speech when a person is tired. In some cases, speech becomes so slurred that others cannot understand it. Drooling can be a problem as well due to weakened bulbar muscles”
http://www.sp-foundation.org/pls.htm

I found a study of Blink Reflex which was done that indicated that the BR remained unchanged in patients with PLS but disappeared over a few years in ALS. That might be a marker to ask your dad’s doctors about. It might help to distinguish.

One last reference:
http://archneur.ama-assn.org/cgi/reprint/64/2/232.pdf

I know this is a lot to read and that’s not what you want to do. But there are differences between the two forms and the tests try to determine that. Note the progression above and the progression which you described. Do they sound similar?
The only difference is the wasting. But that might be subscribed to lack of exercise and depression.

At this point, your dad’s real issue is to find treatment and supplements that work for him.

In the pipeline, among other treatment hopes, is Cogane a GDNF, glial derived neurotropic factor which may indeed be of benefit for ALS patients if it ever gets through clinical trials. (there have been $ $ $ $ $ $ issues)
http://parkinsonsfocustoday.blogspot.com/2009/05/cogane-future-treatment-with-hope-for.html

There may also be stem cell treatments in research that will be effective but they are a few years away.

Diagnosis are not warranties. They can be wrong. The sites above can be printed and provided to doctors. We do that with my husband’s PD and it has been effective – more than words.

Have you dad ask his doctor about low dosage Naltrexone for ALS although it may be off-label usage.

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