MND

Question 1

Common clinical features

Answer 1

• asymmetric limb weakness
• mixture of UMN and LMN signs
• wasting of the small hand muscles/tibialis anterior
• fasciculations

Question 2

What parts of the body are not affected by MND?

Answer 2

• external ocular muscles
• cerebellum
• abdominal reflexes
• sphincter dysfunction not affected till v late

Question 3

MND diagnosis is clinical, but what investigations can be used to rule out other causes

Answer 3

Nerve conduction studies show normal motor conduction
=> exclude neuropathy

Electromyography shows a reduced number of action potentials with increased amplitude

MRI can exclude cervical myelopathy

Question 4

After what age does MND normally present?

Answer 4

> 40y

Question 5

Name the 3 common patterns of MND

Answer 5

Amyotrophic Lateral Sclerosis (ALS)
Progressive Muscular Atrophy
Bulbar palsy

Question 6

What oral medication is used for MND?

Answer 6

Riluzole
- prevents stimulation of glutamate receptors
- used mainly in ALS
- prolongs life by ~3 months

Question 7

What respiratory care is important for MND patients

Answer 7

non-invasive ventilation (usually BIPAP) is used at night

Question 8

How is nutrition delivered to MND patients with dysphagia?

Answer 8

percutaneous gastrostomy tube (PEG)

Question 9

3 Year prognosis in MND

Answer 9

50% of patients die within 3 years

Question 10

LMN signs in arms and UMN signs in legs

gene responsible lies on chromosome 21 and codes for superoxide dismutase

Answer 10

Amyotrophic Lateral Sclerosis

Question 11

What pattern of MND has only UMN signs

Answer 11

Primary lateral sclerosis

Question 12

What pattern of MND has only LMN signs, affecting distak muscles first before proximal?

Answer 12

Progressive muscular atrophy

Question 13

Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei

This subtype carries the worst prognosis

Answer 13

Progressive bulbar palsy