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Neuromuscular & Muscular
Neuromuscular & Muscular

Motor Neuron Disease

On this page:DiagnosisManagement


  • Clinial Phenotypes of Motor Neuron Disease

  • Amyotrophic lateral sclerosis (ALS) - mixed upper and lower motor neuron involvement
  • Primary lateral sclerosis - mainly upper motor neuron involvement
  • Progressive muscular atrophy - mainly lower motor neuron involvement
  • Progressive bulbar palsy - mainly bulbar involvement


  • Signs of Motor Neurone Disease

  • Bulbar palsy (LMN) - nasal speech, absent gag reflex, tongue wasting and fasciculations, absent palate rise, absent (i.e. normal) jaw jerk
  • Pseudobulbar palsy (UMN) - dysarthria, increased / normal gag reflex, tongue spasticity, absent palate rise, increased jaw jerk
  • Upper motor neuron signs - increased tone, clonus, reduced power, hyperreflexia, upgoing plantar reflex
  • Lower motor neuron signs - muscle wasting, fasciculations, reduced tone, reduced power, hyporeflexia, downgoing plantar reflex


  • Management Options for Motor Neuron Disease

  • Non-Pharmacologic

  • Physiotherapy
  • Non-invasive ventilation (NIV)
  • Speech pathology assessment
  • Consideration of PEG feeding
  • Psychological sypport
  • Pharmacologic

  • Rulizole
  • Anti-spasmodics - baclofen, diazepam, botox
  • Neuropathic agents - pregabalin, amitriptyline
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