Progressive muscular atrophy

Progressive muscular atrophy

Progressive muscular atrophy (PMA) is a rare subtype of Motor neurone disease (MND) which affects only the lower motor neurones. This is in contrast to the most common form of MND, amyotrophic lateral sclerosis, which affects both the upper and lower motor neurones. Famous people to suffer from it include Mike Gregory.

Symptoms

As a result of lower motor neurone degeneration, the symptoms of PMA include:

Differential diagnosis

However, in contrast to amyotrophic lateral sclerosis or primary lateral sclerosis, PMA is distinguished by the absence of:

Diagnosis

To be diagnosed with PMA one must have the lower motor neuron symptoms described above in the absence of upper motor neuron symptoms for a given period of time. Some authors recommend 3 years, others 5 years or even up to 7 years. However it is worth bearing in mind that this illness can progress into typical ALS at any time, even as many as 26 years after the initial diagnosis.

Prognosis

The significance of diagnosing progressive muscular atrophy as opposed to ALS is twofold.

  • 1) the prognosis is better. The typical prognosis for ALS is approximately 2-5 years after initial diagnosis. In PMA survival is in the order of 5-10 years.
  • 2) Patients with PMA do not suffer from the cognitive change identified in at least a subgroup of patients with ALS.

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