Motor Neurone Disease (3)

Question 1

What is the most common type?
→ What are the subtypes of this?

What are the other types?

Answer 1

➊ Amyotrophic Lateral Sclerosis (80%) – Mixed UMN and LMN signs
• Spinal ALS – Classic MND syndrome
• Bulbar ALS – Early bulbar symptoms, and can spread to other areas

➋ • Primary Lateral Sclerosis (< 10%) – Only UMN signs
• Progressive Muscular Atrophy (< 10%) – Only LMN signs

Question 2

How does it typically present?

What indicates the prognosis?

Answer 2

➊ • Mix of UMN and LMN signs
‣ UMN signs – Spasticity, Hyperreflexia, Upgoing plantars
‣ LMS signs – Flaccidity, Wasting, Fasciculations
• No sensory involvement!

➋ Onset of bulbar and respiratory muscle involvement

Question 3

Eventhough the disease is progressive, what can be given to prolong survival?

What needs to be monitored regularly? Why?

Answer 3

➊ Riluzole (only prolongs survival by a few months)

➋ • VC as pt will eventually develop respiratory involvement, therefore leading to neuromuscular (T2) RF
• May require NIV