Motor neuron disease

Question 1

Define motor neuron disease

Answer 1

Cluster of degenerative disease characterised by
axonal degeneration of neurones in the motor cortex,
CN nuclei and anterior horn cells.

Question 2

Are UMN or LMN affected in motor neuron disease?

Answer 2

both are affected

Question 3

What are the investigations for MND?

Answer 3

brain/cord MRI to exclude structural lesions
LP to exclude inflammatory cause
EMG- electromyography/nerve studies

Question 4

What are the signs and symptoms of MND?

Answer 4

 UMN: spasticity, ↑ reflexes, ↑ plantars
 LMN: wasting, fasciculation of tongue / abdo / thigh
 Speech or swallowing impairment
 Fronto-temporal dementia

Question 5

What is the management of MND?

Answer 5

MDT- neurologist, OT, physio, dietician

Antiglutamatergic drug

Supportive- NG/PEG feeding, analgesia, botulinum for spasticity