Neuro - MND

Question 1

are sensory symptoms common?

Answer 1

no - sensory nerves unaffected

Question 2

most common subtype

Answer 2

ALS

Question 3

second most common subtype

Answer 3

progressive bulbar palsy

Question 4

what muscles does progressive bulbar palsy affect?

Answer 4

talking and swallowing muscles

Question 5

2 other types of MND

Answer 5

progressive muscular atrophy

primary lateral sclerosis

Question 6

what % are inherited?

Answer 6

5-10

Question 7

risk factors

Answer 7

smoking

exposure to heavy metals and certain pesticides

Question 8

UMN or LMN neurones affected?

Answer 8

both

Question 9

typical patient

Answer 9

late middle aged man (60 yrs) possibly with affected relative

Question 10

dysarthria

Answer 10

slurred speech

Question 11

signs of LMN disease

Answer 11

muscle wasting
reduced tone
fasciculations
reduced reflexes

Question 12

signs of UMN disease

Answer 12

increased tone or spasticity
brisk reflexes
upgoing plantars

Question 13

treatment to slow progression of disease

Answer 13

riluzole

Question 14

how long can riluzole increase survival by?

Answer 14

few months

Question 15

management

Answer 15

non-invasive ventilation
MDT
advanced directives, ACPs

Question 16

what do most patients die of?

Answer 16

resp failure

Question 17

typical presentation

Answer 17

insidious and progressive weakness of upper limbs and throughout body - trunk, face, speech
increased fatigue exercising
clumsiness, dropping things more often, dysarthria