What is progressive muscular atrophy?

Progressive muscular atrophy (PMA), also known as Duchenne-Aran muscular atrophy, is a rare subtype of motor neuron disease. Progressive muscular atrophy only affects the lower motor neurons. 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?

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.

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. Those with progressive muscular atrophy have 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.

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 specialized 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 progressive muscular atrophy. 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 for those with progressive muscular atrophy.

Solution

The Quantum Edge 3 with industry-first 4.5 mph at iLevel offers the most advanced power chair 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.