Motor neurone disease

Question 1

motor neurone disease definition

Answer 1

a progressive degenerative disease of the upper and lower motor neurones resulting in progressive disability and death

Question 2

epidemiology of MND

Answer 2

1. occurs in 2/100,000 adults per year with mean age of onset around 65 years

Question 3

MND clinical features

Answer 3

1. progressive weakness of one or more limbs over months
   - dysarthia : difficult articulation of speech
   - dysphagia : difficulty swallowing
   - tongue wasting + weakness
   - wasting of small hand muscles/tibialis anterior is common

Question 4

MND on examination

Answer 4

mixed upper and lower motor neuron features

1. muscle wasting + fasiculation
2. brisk reflexes
3. no sensory involvement

Question 5

Clinical features of MND in advanced disease

Answer 5

1. respiratory muscle weakness causes breathlessness when lying flat (orthopnea)
   - eventually causing respiratory failure
2. minority develop frontotemporal dementia

Question 6

4 types of motor neurone disease

Answer 6

1. Amyotrophic lateral sclerosis
2. Primary lateral sclerosis
3. Progressive muscular atrophy
4. Progressive bulbar palsy

Question 7

ALS diagnosis

Answer 7

1. based on presence of upper and lower motor neurone signs

2. Overall is a clinical diagnosis