Progressive Muscular Atrophy – all you need to know
Progressive Muscular Atrophy (PMA) is a rare type of motor neuron disease (MND) that involves slow, yet progressive deterioration of lower motor neurons (present in the brainstem and spinal cord).
Getting a PMA diagnosis can be earth-shattering. However, we can take solace knowing that the deterioration of lower motor neurons is much slower than other types of MND.
At Plexus Neuro and Stem Cell Research Centre, we offer regenerative rehabilitation for PMA. As part of the best treatment for PMA in India, our rehabilitation program comprises stem cell therapy, speech and language therapy, occupational therapy, physical therapy, and other support to ensure you live your best life in spite of your PMA diagnosis.
A rare type of neuromuscular disorder, PMA affects the lower motor neurons that are located in the brainstem and spinal cord. These neurons provide muscles and glands the stimuli to function. PMA’s irreversible impact on lower motor neurons ultimately leads to loss of motor function. However, the rate of progression/deterioration is much slower than other types of MND.
PMA is alternatively called Duchenne-Aran disease or Duchenne-Aran muscular atrophy.
Symptoms of PMA
The first symptom most patients are likely to experience is muscle weakness. But this is a peculiar kind of weakness – it first starts in the hands, gradually moves to the lower body, and finally affects the chest and respiratory muscles.
Besides muscle weakness, the other symptoms of PMA include:
- Flail arm syndrome (inability to use the arms)
- Muscle pain
- Muscle cramps
- Muscle twitching
- Weight loss
- Respiratory problems
- Clumsy movements, specially hand movements
Essentially, PMA leads to muscle wasting – a condition that occurs due to the lack of use of muscle and/or disease-caused deterioration of muscle (weaking, shortening, loss).
If you experience any of the above symptoms or a combination of them, please visit your doctor immediately. Timely diagnosis and treatment can go a long way in slowing the disease’s deterioration even further,
Causes of PMA
The onset of PMA is invariably sudden and unpredictable. This is why the condition is also called ‘sporadic’, thereby making it difficult to identify its causes or triggers.
However, research indicates that environmental factors – exposure to toxins or viral diseases – and genetic factors play a significant role in the development of PMA.
What are the risk factors of PMA?
Males are more prone to develop PMA,
Unlike other types of MND, PMA is categorized as adult-onset MND with its symptoms manifesting in early adulthood itself.
How do you diagnose PMA?
Since PMA shares a lot of its symptoms with other types of MND, its diagnosis is arrived at through the process of elimination. Your doctor may suggest any or all of the below tests to identify the cause of your symptoms and provide further prognosis.
Electromyography assesses the health of muscles as well the nerves controlling them.
Computed tomography (CT) and other related scans help doctors identify and ascertain the degree of structural damage to the spinal cord and brain.
Lumbar puncture (spinal tap) measures the pressure of cerebrospinal fluid.
Nerve conduction tests measure the electrical activity of nerves.
Magnetic resonance imaging (MRI) can help identify any signs of selective muscle movement as well as give your doctor a clearer picture of the rate of progression of the disease.
Besides the above, your doctor will also ask for your medical history (to identify any genetic markers), and perform a physical examination of muscles.
Difference between ALS and PMA?
There have been several cases of PMA being misdiagnosed as amyotrophic lateral sclerosis (ALS). This is because both the conditions share some similarities. However, while both are types of MND that ultimately lead to muscle weakness and deterioration, there are some fundamental differences between the two.
PMA affects the lower motor neurons, present in the brainstem and spinal cord. The disease progresses at a very slow pace. Its symptoms typically manifest in early adulthood, hence the name adult-onset MND.
On the other hand, ALS affects both upper motor neurons (in the brain) and lower motor neurons (in the spinal cord) resulting in severe muscle loss and atrophy. Symptoms of ALS typically emerge between the ages 40 years and 70 years.
Plexus rehabilitation for PMA
Living with PMA can be physically and emotionally exhausting for both patients and their caregivers. Social interaction and communication are at a premium. Lack of movement causes unfathomable disruption to our everyday lives. Imagine not being able to do the things you love because you have a condition that always pulls you back!
This is why Plexus’ rehabilitation program for PMA is the best treatment for PMA and can significantly improve the quality of life of a PMA patient. This tailored rehabilitation program. T offers a plethora of treatments and therapies to empower the patient and encourage the caregivers to not let a PMA diagnosis deter you from living your best life.
The rehabilitation program for PMA comprises –
Stem cell therapy is a regenerative treatment that uses the body’s natural healing mechanism to treat a number of conditions. Injected stem cells slow down the rate of neurodegeneration and also have the capacity to self-renew, regenerate cells, and repair damaged tissue.
Plexus Neuro and Stem Cell Research Centre is India’s first ISO-certified stem cell research centre. Plexus uses only autologous stem cells that have been taken from the patient’s own body. The procedure is conducted by Dr. Na’eem Sadiq, India’s no. 1 stem cell specialist.
With occupational therapy (OT), the patient can perform daily activities with much more ease, OT helps in managing symptoms, restoring strength, improving endurance and hand function, and managing fatigue.
Physiotherapy helps improve posture and prevent joint immobility. The inevitable muscle weakness and atrophy are also pushed further away because the patient has not lost complete mobility just yet.
Muscle relaxants and medication
Your doctor may also recommend muscle relaxants to relieve muscle stiffness and improve muscle function.
Apart from this, the program also offers:
- Diets and nutrition plans
- Endurance training and fitness management
- Functional stretching for relief from muscle stiffness
- Hand function training
- Functional splinting
- Strengthening of muscles in the shoulder, neck, upper and lower limbs, and oral structures
- Activities for daily living training
- Energy conservation and work simplification training
- Counseling and caregiver support
Life after a PMA diagnosis
When you’re handed a PMA diagnosis you’re also told that the disease is progressive and irreversible. Your doctor will recommend a host of treatment options that can help you manage your symptoms and possibly even slow down the rate of progression.
Deterioration is inevitable. However, it doesn’t have to be the end of the road. Taking care of yourself, especially your mental health is important at such times. Below are some simple steps we recommend to buoy your spirits and remind you that life is still worth living!
- Join a support group of people who have PMA
- Add in a few minutes of meditation and quiet reflection to your daily routine
- Allow yourself to grieve; this is an important step towards accepting your condition
- Remember it’s OK to cry
- Reshift of your focus on what is more important – your health
- Exercise every day – a gush of endorphins can do wonders to your mood
- Lastly, keep track of the changes in your body, and always have your doctor on standby
You cannot pour from an empty cup. Take care of yourself.
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What age does PMA start?
PMA invariably starts in early adulthood, with most cases diagnosed even before the patient reaches 50 years of age.
What is the difference between ALS and PMA?
PMA affects lower motor neurons. ALS affects upper and lower motor neurons.
PMA’s rate of progression is much slower than that of ALS.
Who is at risk of muscular atrophy?
Studies indicate that people with a genetic predisposition (family of history) of MND are at risk of muscular atrophy.
Also, PMA affects more males than females.
What is the survival rate of PMA?
PMA is progressive and irreversible. However, with timely treatment its symptoms can be managed and its progression can be slowed even further.
Can the elderly recover from muscular atrophy?
Recovery from muscular atrophy is possible with regular exercise and diet. However, PMA is progressive and irreversible.
What vitamin deficiency can cause muscular atrophy?
Deficiency of Vitamin D has been linked to muscular atrophy.
What foods prevent muscular atrophy?
Foods rich in calcium and vitamin D have been known to reduce the risk of muscular atrophy.