motor neurone disease

Question 1

what is it

Answer 1

neurodegenerative condition
affects brain and spinal cord

Question 2

what does it involve and what are the symptoms

Answer 2

degeneration of motor neurones leads to progressive muscle weakness
symptoms include muscle cramps, wasting, stiffness, loss of dexterity, reduced respiratory function and cognitive dysfunction

Question 3

pt suspected of having developed motor neurone disease…

Answer 3

refer to neurologist without delay

Question 4

most common form

Answer 4

amyotrophic lateral sclerosis

Question 5

any cure

Answer 5

no

Question 6

non drug treatment includes the following

Answer 6

nutrition, psychosocial support, physiotherapy, exercise programmes and use of special equipment or mobility aids.

Question 7

1st line for muscle cramps

Answer 7

quinine (unlicensed indication)

Question 8

2nd line for muscle cramps if quinine contraindicated or not tolerated

Answer 8

baclofen (unlicensed)

Question 9

3rd line treatment options quinine (first line) and baclofen (second line) not tolerated or CI for muscle cramps

Answer 9

tizanidine
dantrolene
gabapentin

all unlicensed indications

Question 10

4 options to help with symptoms of muscle stiffness, spasticity or increased tone

Answer 10

baclofen
tizanidine
dantrolene
gabapentin (unlicensed)

Question 11

what to do if pt has severe muscle spasticity

Answer 11

may need specialist referral

Question 12

saliva problems - what type of drug can be considered for excessive drooling of saliva

Answer 12

trial of antimuscarinic drug (unlicensed indication)

Question 13

what drug is recommended in pt who excessive drooling of saliva & have cognitive impairment? and why?

Answer 13

glycopyrronium bromide
unlicensed
as it has fewer CNS SE

Question 14

if treatment with glycopurronium bromide for saliva problems if ineffective, not tolerated or CI, what is the next step?

Answer 14

may require referral for specialist admin of botulinum toxin type A

Question 15

3 things that can be used to treat patients with thick, tenacious saliva

Answer 15

humidification
nebulisers
carbocisteine

Question 16

what to do in pt with respiratory symptoms (3)

Answer 16

• should reversible causes of worsening respiratory impairment (E.g. RTI Or secretion problems) before considering other options
• breathlessness: can give opioids, or BZDPNs (both unlicensed indications) if the pt symptoms are exacerbated by anxiety
• consider non invasive ventilation in pt with respiratory impairment

Question 17

these two drug classes may be given, unlicensed indications, for patients experiencing breathlessness

Answer 17

opioids
BZDPNs if symptoms exacerbated by anxiety

Question 18

when would you choose a BZDPN instead of opioid for patients experiencing breathlessness?

Answer 18

if their symptoms are exacerbated by anxiety

Question 19

which drug is licensed for use in pt with amyotrophic lateral sclerosis to extent life or to extend the time to mechanical ventilation

Answer 19

riluzole

Question 20

riluzole is licensed for…

Answer 20

use in pt with amyotrophic lateral sclerosis to extent life or to extend the time to mechanical ventilation

Question 21

baclofen is contraindicated with oral use when the pt has …

Answer 21

active peptic ulceration

Question 22

common SE of baclofen

Answer 22

• constipation
• diarrhoea
• depression
• confusion
• drowsy
• dry mouth
• euphoric mood, hallucination
• headache
• nausea, vomiting

Question 23

why should baclofen not be withdrawn abruptly?
and how should you reduce the dose?

Answer 23

• risk of hyperactive state, may exacerbate spasticity, and precipitate autonomic dysfunction including hyperthermia, psychiatric reactions and convulsions
• to minimise risk, discontinue by gradual dose reduction over at least 1-2 weeks, longer if symptoms occur

Question 24

carbocisterine is contraindicated if pt has

Answer 24

active peptic ulceration

Question 25

dose of carbocisteine for reduction of sputum viscosity

Answer 25

initially 2.25g daily in 3 divided doses
reduce to 1.5g daily in 2-4 divided; dose to be reduced as conditions improve