Neurology 5 - Motor Neurone Disease

Question 1

define motor neurone disease

Answer 1

umbrella term for multiple diseases

progressive + fatal condition where all types of motor neurone stop functioning

Question 2

4 types of motor neurone disease

Answer 2

ALS - amylotrophic lateral sclerosis

PBP - progressive bulbar palsy, primarily affects muscles of talking and swallowing

Progressive muscular atrophy

Primary lateral sclerosis

Question 3

presentation of MND

Answer 3

signs of BOTH upper and lower motor neurone disease

lower - muscle wasting, twitching, reduced tone, reduced reflexes

upper - increased tone, spasticity, brisk reflexes, upgoing plantars

Question 4

diagnosing MND

Answer 4

often delayed diagnosis

specialist diagnosis excludes other conditions and accounts for clinical picture

Question 5

management of MND

Answer 5

no treatments to halt it yet

riluozole, edaravone, NIV

key management is support + good patient-centred care

should arrange advance directives and end of life planning