Muscular Dystrophy so close to ALS

[Clearwater AL]

Often when we try to help serious “Could this be ALS’ers” there is a neuromuscular disease we may miss which also has many mimics of ALS.

Something interesting I came across… so close but not.

Muscular Dystrophy (MD) is a neuromuscular disease. There is no cure.

Muscular dystrophy (MD) is a group of disorders that gradually weakens and damages the muscles. Multiple sclerosis (MS) is an immune-mediated disorder of the central nervous system that disrupts communication between the brain and body and within the brain itself.

Many individuals eventually lose the ability to walk. Some types of MD also affect the heart, gastrointestinal system, endocrine glands, spine, eyes, brain, and other organs. Respiratory and cardiac diseases may occur, and some people may develop a swallowing disorder.

Patients exhibit symptoms of ventilatory insufficiency, the first to be noticed are those associated with CO2 retention: dyspnea, nightmares, increased heart rate, and increased blood pressure. Many patients with late stage Duchenne muscular dystrophy have been supplied with mechanical aid for ventilatory assistance.

In some types of muscular dystrophy, weakness in the facial and oral muscles that control the use of the tongue, lips, soft palate, cheeks, and diaphragm results in problems with speech quality (dysarthria) and voice quality (dysphonia).

It can affect anyone from teenagers to adults in their 40s. Distal muscular dystrophy affects the muscles of the arms, legs, hands, and feet. It usually comes on later in life, between ages 40 and 60. Oculopharyngeal muscular dystrophy starts in a person's 40s or 50s.

Muscular dystrophy (MD) is diagnosed through a physical exam, and tests.

These might include:

A muscle biopsy (the removal and exam of a small sample of muscle tissue)
DNA (genetic) testing.
*Electromyography or nerve conduction tests (which use electrodes to test muscle and/or nerve function).

To me, how close it is but… it is not ALS.

 

[lgelb]

There are indeed similarities -- it's always good to remember. I have worked with MD patients.

But the EMG will show myopathy -- a primary problem with the muscles -- not the nerves. Their motor neurons are fine -- but the muscles can't respond and atrophy from disuse. So you often see a kind of stick figure look all over, instead of in limited areas as you see with the flail arm or flail leg variants of ALS.

The age of onset is also younger, of course, with many diagnosed in childhood, though there are also adult onset forms.

BTW, current experience is that even people with MDs other than Duchenne can often benefit from BiPAP, progressive forms of MS as well.

Best,
Laurie

 

[nona]

Why is ALS considered part of the MD community? I got my rollator from the MDA at my ALS clinic a while back.

 

[Nikki J]

Their mission says MD and related neuromuscular diseases. I am happy they include us for whatever reason. The MDA was founded more than 30 years before ALSA so it may have been that they extended their umbrella to those who had nowhere else.

the MDA sponsors patient symposia every year. The ALS one is May 12 and 13 and is virtual. MDA Engage: Amyotrophic Lateral Sclerosis (ALS) Symposium | Muscular Dystrophy Association
I attended an in person one a couple of years ago. MGH is an MDA clinic and I think they get some financial support on a per capita basis or at least used to.

 

[vickim]

I haven't posted here is a long time. I am glad to see that some are starting to see the similarities in these diseases. I am 14 years into mitochondrial myopathy complex 1 and 3 adult onset. It is robbing me of muscle, eye sight, hearing and energy. I have nerve damage too. There is no cure. There are no real medications. Just suggested supplements called a mito cocktail. I used to go to a MDA/ALS clinic but the neuro there said there is nothing he can do for me.....so I just deal with what comes.

 

[affected]

{{{{{{{{{{{{{{{{{{{VICKIM}}}}}}}}}}}}}}}}}} I think of you so often

 

[vickim]

Well life has gotten in the way. Trying to stay safe. Got vaccinated so I am a little more inclined to get out. Still wear a mask and my cane makes it easy to keep others at a distance! LOL. Still camping and fishing though. I think of all you all the time. I pray for all of you. I will try and check in more often.