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Lower Limb Neuro
 
 

Motor Neurone Disease

 
 
 
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Overview

  • 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
Motor neurone disease does not present with sensory involvement or ocular palsy.
  • Clinical Phenotypes of Motor Neurone 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
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