What Is Progressive Muscular Atrophy?

Muscle Atrophy

Progressive Muscular Atrophy (PMA), also known as Duchenne-Aran muscular atrophy, is a rare subtype of motor neuron disease. It is a sibling condition to amyotrophic lateral sclerosis (ALS), which affects both the upper and lower motor neurons. The distinction is important because PMA is associated with a better prognosis than classic ALS. Since its initial discovery in 1850, there has been debate in the scientific literature over whether Progressive Muscular Atrophy is a distinct disease with its characteristics or if it lies somewhere on a spectrum with ALS.

What are the symptoms of Progressive Muscular Atrophy?

Progressive Muscular Atrophy is characterized by the progressive loss of motor neurons in the spinal cord. This leads to muscle weakness and atrophy. Symptoms of Progressive Muscular Atrophy include:

  • Atrophy
  • Muscle weakness
  • Lack of reflexes
  • Lack of spasticity

Symptoms can be limited to the arms, legs, or both.

powerchair at a parking lot

How is Progressive Muscular Atrophy diagnosed?

Progressive muscular atrophy is diagnosed through the exclusion of other conditions. No specific test can conclusively establish whether a patient has the condition. Instead, several other possibilities have to be ruled out, such as multifocal motor neuropathy or spinal muscular atrophy. Tests used in the diagnostic process include MRI, clinical examination, and EMG.

The prognosis for Progressive Muscular Atrophy

The prognosis for progressive muscular atrophy is better than other similar conditions, such as ALS. There is also a longer life expectancy than those with ALS. Furthermore, those with progressive muscular atrophy do not suffer from the cognitive change identified in similar conditions. It’s also important to note that an initial diagnosis of PMA could turn out to be slowly progressive ALS many years later, sometimes even decades after the initial diagnosis.

Man in wheelchair reading laptop

Mobility for those with Progressive Muscular Atrophy

Many with Progressive Muscular Atrophy experience the progressive loss of the ability to walk and position themselves, necessitating a specialised power wheelchair. Quantum Rehab, the global leader in individualized powerchairs, emphasizes mobility technologies specific to the needs of those with Progressive Muscular Atrophy. Quantum powerchairs incorporate power-adjustable seating for user repositioning and comfort, speciality drive controls, including those requiring minimal hand strength, and a highly adaptable design to meet an individual’s current and future needs. Quantum powerchairs feature the latest advanced technologies to increase the independence of those with the condition.

iLevel seat elevation technology allows users to operate the powerchair seated or standing. Bluetooth is also integrated into Quantum’s Q-Logic 3 electronics, so those with progressive muscular atrophy can operate much of their environment with the power chair drive control. Quantum powerchairs are designed to provide optimal medical comfort and maximum independence.

ICON Rehab Powerchair

ICON Rehab Powerchair UK

Quantum Q6 Edge 2 Powerchair

Quantum Q6 Edge 2 Powerchair profile

Solution

The Quantum Edge 3 with industry-first 4.5 mph at iLevel offers the most advanced powerchair experience. Q6 Powerchair series, which includes the Edge 3 and Q6 Edge 2 powerchairs, provide highly adjustable mid-wheel drive power bases. The Q6 Edge 2 all accept our optional iLevel technology, which offers up to 12 inches of lift at 4.5 mph. The 4Front is a quiet, more responsive front-wheel drive powerchair that features automotive-grade suspension with unprecedented comfort and rides quality.

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