Motor Neuron Disease

Question 1

What is it?

Answer 1

Progressive dysfunctioning of motor neurons with no sensory impairment

Question 2

What are the 4 types?

Answer 2

Amyptrophic lateral sclerosis
Progressive muscular atrophy
Primary lateral sclerosis
Bulbar presentation

Question 3

What is Amyotrophic lateral sclerosis?

Answer 3

Most common form
LMN weakness- initially affecting distal arms and extending proximally
UMN weakness- starting in lower leg
Bulbar symptoms- dysphagia and oseophageal dysmotility is often a late sign

Question 4

What is progressive muscular atrophy?

Answer 4

Just involves LMN weakness of LMN in spine
Often starts distally and extends proximally
Best prognosis

Question 5

What is Primary lateral sclerosis?

Answer 5

Rare

Affects UMN only

Question 6

What is bulbar presentation?

Answer 6

Symptoms of bulbar palsy
Dysphagia early on
Worst prognosis

Question 7

How is NMD diagnosed?

Answer 7

Clinical diagnosis

Can do EMG and MRI head to rule out other causes

Question 8

What is the prognosis of NMD?

Answer 8

Normally around 3 years

Most die from ventilatory failure or bronchopneumonia

Question 9

What is the management of NMD?

Answer 9

Resp care
-BIPAP often at night

Riluzole

• in Amyotrophic lateral sclerosis
• prolongs life for around 3 months