Motor Neurone Disease

Question 1

Define Motor Neurone disease

Answer 1

Progressive disease of unknown aetiology causing aconal degeneration of upper and lower motor neurone neurones

Question 2

Clinical signs of MND

Answer 2

Wasting and Fasciculation - including tongue
Spasticity usually but can be flaccid
Weakness
Absent and brisk reflexes
Normal sensation
Dsyarthria

Question 3

Describe the three types of MND

Answer 3

Amyotrophic lateral sclerosis: most common, upper and lower, corticospinal tracts, spastic paraperesis
Primary lateral sclerosis: UMN
Progressive muscular atrophy: LMN, anterior horn cells, wasting/fasciculation and weakness
Progressive bulbar palsy: lower cranial nerves and suprabulbar nuclei, speech/swallow difficulty

Question 4

How would you investigate a patient with suspected MND?

Answer 4

It’s a clinical diagnosis but EMG can identify fasciculations and MRI brain/spine can exclude differentials

Question 5

How would you manage this patient?

Answer 5

MDT
Supportive measures: PEG feeding, NIV
Riluzole - slows disease progression by 3 months but no improvement in QoL

Question 6

What causes wasting of small muscles of the hand?

Answer 6

Anterior horn cell: MND, Syringomyelia, cervical cord compression, polio
Brachial plexus: cervical rib, pancpast tumour, trauma
Peripheral nerve: median and ulnar nerve lesion
Muscle: rheumatoid arthritis