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Prognosis

Authoring team

Prognosis varies depending on the type of MND and factors such as the age of onset

  • PLS progress slowly
  • ALS and some forms of spinal muscular atrophy (SMA) are fatal

  • ALS is a fatal disease
    • median survival is 3 years from clinical onset of weakness (1)
      • note though that about 15% of patients with ALS live 5 years after diagnosis, and about 5% survive for more than 10 years
      • longer survival is associated with:
        • a younger age at onset
        • male sex
        • limb (rather than bulbar) symptom onset

  • slower progession occurs in regionally limited forms of motor neuron disease (ie, brachial biplegia, lumbosacral biplegia, and progressive bulbar palsy [PBP])

  • progressive muscular atrophy (PMA), distinct from classic ALS because of lack of upper motor neuron (UMN) findings, progresses at the same rate as classic ALS

  • a slower rate of progression is seen in upper motor neuron -predominant ALS

  • primary lateral sclerosis (PLS) - survival rate is much longer than ALS (measured in decades)

From the differential prognoses seen in motor neurone disease it seems that the loss of LMNs is a significant prognostic determinant.

Reference:

  • Kiernan MC. Motor neurone disease: a Pandora's box. m ed J Aust 2003;178:311-2
  • Armon C. Amyotrophic Lateral Sclerosis (ALS), Medscape, May 2014.

 


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