Overview

• Neurodegenerative disease with upper and lower motor neurone dysfunction
• M>F, mean age of onset is 65 years old
• Aetiology → unknown, several different theories
• Positive family history of ALS in 5-10% of cases
• Different Types
  • Amyotrophic Lateral Sclerosis (Most Common) → UMN + LMN signs
  • Progressive Bulbar Palsy → early tongue and bulbar involvement (carries worst prognosis)
  • Primary Lateral Sclerosis → UMN signs only
  • Progressive Muscular Atrophy → LMN signs only (carries best prognosis)

Making Diagnosis

Clinical Features:

• Progressive Muscle Weakness
• Fasiculations
• Dysphagia → bulbar onset ALS may present with difficulties swallowing initially
• Shortness of Breath
• Eye Movements Spared and Normal Sensation
• Signs → wasting of thenar muscles and wasting of tongue base
• UMN Signs → spasticity, hyper-reflexia, clonus, positive babinski sign
• LMN Signs → hypotonia, hyporeflexia, muscle atrophy, fasiculations and fibrillations
  • Mixture of UMN and LMN signs
• Asymmetrical Symptoms (unlike other neurological conditions)