Person using a wheelchair on a city sidewalk. Person using a wheelchair on a city sidewalk.

From Playground to Wheelchair: Can Early Signs Help Detect Muscular Dystrophy?

We usually don’t have any idea what’s going on within our body. How the disease affects our body and all body parts is variable and depends upon a lot of factors. Have you heard about Muscular dystrophy? Let me tell you it’s basically a whole group of diseases that are genetic and cannot be caused by injury or any activity and are not contagious.

I am talking about it because in this article I’ll be highlighting more about this disease.

First I’ll Explain to You the Muscular Dystrophy

It’s a genetic disease group that is characterized by weakness and degeneration of our muscles.

The process is ongoing and progressive and eventually affects all our skeletal muscles. Factors that differentiate between the different types include the time of onset, pattern of the affected area, and the severity observed.

What is constant across all forms is that the weakness gradually increases; so much that in some cases you might even lose the ability to walk. There can be cases where your other body parts like the heart, lungs, endocrine glands, spine, eyes, and brain are also affected.

To actually understand the effect of Muscular Dystrophy on the brain, we need to first understand what the actual problem is, which requires a correct form of diagnosis.

This implies that before jumping to any conclusion, make sure you run a proper and thorough neuropsychological evaluation of the concerned patient.

How Are Muscles Affected in Muscular Dystrophy?

Let us understand how muscles are actually affected by muscular dystrophy. So, each muscle is made up of fibers which have cells that are surrounded by an outer layer.

When an impulse is generated from the brain, a signal is sent through the spinal cord which goes through the nerves. This connects the nervous system to the organs of the body. This series of reactions is triggered by acetylcholine.

The membrane of the muscle fiber has a protein group called the dystrophin-glycoprotein complex. These help to prevent the damage caused to muscle fibers by contraction and relaxation of the fibers.

If this membrane is leaked, however, it becomes problematic as the protein creatine kinase leaks and there is excess intake of calcium; which leads to damage to the muscle fibers which eventually die. This is what is known as muscular dystrophy as it is at a stage where there is progressive muscular degeneration.

In easier terms, we can say that dystrophin is the protein that helps in the protection of the muscles. Due to mutation when the amount of dystrophin changes in the body which is the condition during Muscular Dystrophy; the muscles become more susceptible to damage.

In an interview with the Icy WHiz team, Evan Tunis, President of Florida Healthcare Insurance, talked about the relationship between muscular dystrophy and cognitive function. Here is what he said:

Evan Tunis - Featured
Evan Tunis

“Muscular dystrophy (MD) is a genetic disease that primarily affects the muscles, causing progressive weakness and degeneration. However, recent studies have shown that MD can also impact cognitive function, resulting in learning difficulties and memory problems.

There are different types of muscular dystrophy, each with varying symptoms and severity.

For example, Duchenne muscular dystrophy (DMD) is known to cause cognitive impairment, with affected individuals often experiencing learning disabilities and delayed language development.

On the other hand, facioscapulohumeral muscular dystrophy (FSHD) has been linked to changes in brain structure and function, leading to deficits in executive functioning and attention span.

The extent of cognitive involvement also depends on the stage of the disease. In the early stages, individuals with MD may have normal cognitive abilities, but as the disease progresses and muscles weaken, it can impact brain function.

This is because muscles play a crucial role in supplying oxygen and nutrients to the brain, and their dysfunction can disrupt this process.”

Muscular Dystrophy Could be of Different Types

1. Duchenne Muscular Dystrophy

This is the most common type of Duchenne MD, in the childhood form, which mostly occurs in boys because the inheritance is via the X chromosome. However, girls can also experience it if they carry the defective gene. The causal agent is protein dystrophin, the lack of which causes Duchenne MD.

Duchenne muscular dystrophy is caused by the presence of :

This comes out right up in toddler stages and leads to muscular weakness and wasting, among other symptoms like waddling gait, impaired breathing, loss of some reflexes, heart problems, and frequent falls and clumsiness. Children suffering from this might not be able to run or jump, or even have cognitive and behavioral problems.

It has been observed that children suffering from this specifically might even have learning disabilities or some degree of mental impairment. Also, they are not very good with any form of oral information that they receive.

However, recently the U.S. Food and Drug Administration approved a new drug to treat Duchenne Muscular Dystrophy for patients between 4 to 5 years of age, which is one of the first gene therapies approved to treat this fatal disease.

2. Becker Muscular Dystrophy

It is less severe as compared to Duchenne MD, but related to it. It surfaces around the age of 10-11 years to 25 years. Its effects are variable, some people can retain the strength for more years as compared to others.

Early symptoms include frequent falls, difficulty in getting up from the floor and walking on toes.

As compared to Duchenne MD the problems in cognition and behavioral impacts are pretty less. Although comparatively less than Duchenne MD, people with Becker MD also suffer from some kinds of psychological disorders like attention-deficit/hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD).

3. Distal Muscular Dystrophy

A type of disease that basically affects the distant muscles, like the forearms or feet. This usually occurs between the ages of 40 and 60 years.

Distal muscular dystrophy (Medical Condition)

The muscle degeneration is comparatively lesser and slower but it has the scope of spreading out. It can impact the respiratory muscles in a pretty intense manner, so you might need a ventilator in case of distal MD. It is usually an autosomal disorder, but in some cases, it can have an infant-stage onset as well.

4. Myotonic Dystrophy

Another type of common MD is also known as Myotonia. It is basically the body’s inability to relax after a sudden muscle contraction. Usually, it occurs between the ages of 20 and 30, but there are also cases of childhood-onset and even congenital onset of the same.

It can affect the central nervous system, heart, adrenal glands, and even the gastrointestinal glands. Symptoms of Myotonic MD include cardiac complications, cataracts, poor vision, weight loss, and mild cognitive impairment. You can also experience difficulty in swallowing, early frontal baldness, and droopy eyelids.

Dr. Caskey discusses myotonic muscular dystrophy

Although research has been going on for a very long time, there have been various new developments from time to time. To date, we can say that there are more than 50 types of muscular dystrophy. These are distinguished based on how they affect you and which muscles or organs are specifically affected.

I hope now you understand that muscular dystrophy affects the brain but not in a very significant manner. Although the actual effect will vary from individual to individual as no concrete or generalized opinion can be formed, based on currently present data and research.

Guest Author: Saket Kumar

Last Updated on May 17, 2024 by Pragya

Authors

Anushree Khandelwal
Pragya
  1. As muscles are the main body part, no one can be even able to stand without the support from muscles. This article is very helpful i think everyone should read it once.

  2. After reading about Muscular Dystrophy and its effects on the brain, I learned a lot. It’s interesting to see how muscles and the brain are connected. Knowing more about how it affects the brain helps us understand it better. This shows why it’s important to study it from different angles. We need to look at the whole picture to figure out how to deal with Muscular Dystrophy.

  3. I found the article to be an enlightening exploration. Unraveling the genetic nature of this group of diseases, I learned that Muscular Dystrophy isn’t caused by injury, activity, or contagion. The article delves into the intricate details, shedding light on potential impacts on various body parts, including the heart, lungs, endocrine glands, spine, eyes, and brain. It further explores how muscles are affected and introduces the diverse types of Muscular Dystrophy, numbering over 50, each distinct in its impact on specific muscles or organs. A truly informative read.

  4. Muscular dystrophy, a genetic disease, impacts muscles progressively. The brain’s involvement varies, necessitating thorough evaluations. Muscle fiber membrane damage, due to dystrophin deficiency, characterizes muscular dystrophy. Duchenne MD, common in boys, reveals in toddlers, affecting muscles and often cognitive functions. Becker MD, less severe, appears later.

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