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Motor Neurone Disease F035

Document
Last amended 
1 November 2018
Current RMA Instruments

Reasonable Hypothesis SOP

67 of 2013 as amended

Balance of Probabilities SOP

68 of 2013

Changes from previous SOPs

SOP Bulletin 205

ICD Coding
  • ICD-9-CM Codes: 335.2
  • ICD-10-AM Codes: G12.2
Brief description

This is a chronic progressive neurological disease involving the motor pathways of the body in the brain, spinal cord and peripheral nerves.  This condition normally presents with both upper and lower motor neurone signs involving more than one region, i.e. cranial, cervical, thoracic, lumbosacral. Clinical signs are muscle weakness (paresis, paralysis), muscle atrophy, hyperreflexia, and spasticity.

Confirming the diagnosis

This is a complex diagnosis requiring specialist opinion, appropriate investigations (e.g. MRI scans of the brain and spinal cord, spinal puncture and electrophysiological studies of the nerves and muscles), assessment against appropriate diagnostic criteria and the exclusion of other pathologies.

The relevant medical specialist is a neurologist.

Additional diagnoses covered by SOP
  • Amyotrophic lateral sclerosis
  • Lou Gerhig’s disease
  • Motor neuron disease
  • Primary lateral sclerosis
  • Progressive bulbar palsy
  • Progressive spinal muscular atrophy
Conditions excluded from SOP
  • Cervical radiculomyelopathy (may be covered by cervical spondylosis SOP)
  • Hereditary myopathies
  • Inflammatory myopathy# (polymyositis, dermatomyositis)
  • Multifocal motor neuropathy#
  • Myasthenia gravis*
  • Paraneoplastic diseases
  • Post-polio syndrome#

* another SOP applies

# non-SOP condition

Clinical onset

Clinical onset will be based on when progressive symptoms consistent with upper and lower motor neuron dysfunction first appeared.

Clinical worsening

The normal course for the condition would be for it to progress at a steady rate, leading to death after a median time of approximately three to five years.  The only SOP worsening factor is for inability to obtain appropriate clinical management.  Currently the only disease modifying treatment available in Australia is the drug riluzole.  This drug may slightly slow disease progression and prolong life in some patients.