Motor Neurone Disease (MND)

The most commonm type of MND is Amyotrophic Lateral Sclerosis/ALS (80%), which presents with mixed UMN and LMN signs. The subtypes of this are:

• Spinal ALS – Classic MND syndrome

• Bulbar ALS – Early bulbar symptoms, and can spread to other areas

Other types of MND include:

• Primary Lateral Sclerosis (< 10%) – Only UMN signs

• Progressive Muscular Atrophy (< 10%) – Only LMN signs

Presentation

Typically presents with:

• Mix of UMN and LMN signs

  • UMN signs – Spasticity, Hyperreflexia, Upgoing plantars

  • LMS signs – Flaccidity, Wasting, Fasciculations

• No sensory involvement!

The prognosis is indicated by the onset of bulbar and respiratory muscle involvement.

Management

Eventhough the disease is progressive, Riluzole can be given to prolong survival (by a few months).

Vital Capacity has to be monitored regularly as patients will eventually develop respiratory involvement, therefore leading to neuromuscular (T2) respiratory failure and requiring NIV.