Motor Neurone Disease F035

Current RMA Instruments

Changes from previous SOPs

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 generally 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 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.  Disease modifying treatment that slightly slows disease progression and prolongs life in some patients is available in Australia.