motor neurone disease Flashcards
what is it
neurodegenerative condition
affects brain and spinal cord
what does it involve and what are the symptoms
degeneration of motor neurones leads to progressive muscle weakness
symptoms include muscle cramps, wasting, stiffness, loss of dexterity, reduced respiratory function and cognitive dysfunction
pt suspected of having developed motor neurone disease…
refer to neurologist without delay
most common form
amyotrophic lateral sclerosis
any cure
no
non drug treatment includes the following
nutrition, psychosocial support, physiotherapy, exercise programmes and use of special equipment or mobility aids.
1st line for muscle cramps
quinine (unlicensed indication)
2nd line for muscle cramps if quinine contraindicated or not tolerated
baclofen (unlicensed)
3rd line treatment options quinine (first line) and baclofen (second line) not tolerated or CI for muscle cramps
tizanidine
dantrolene
gabapentin
all unlicensed indications
4 options to help with symptoms of muscle stiffness, spasticity or increased tone
baclofen
tizanidine
dantrolene
gabapentin (unlicensed)
what to do if pt has severe muscle spasticity
may need specialist referral
saliva problems - what type of drug can be considered for excessive drooling of saliva
trial of antimuscarinic drug (unlicensed indication)
what drug is recommended in pt who excessive drooling of saliva & have cognitive impairment? and why?
glycopyrronium bromide
unlicensed
as it has fewer CNS SE
if treatment with glycopurronium bromide for saliva problems if ineffective, not tolerated or CI, what is the next step?
may require referral for specialist admin of botulinum toxin type A
3 things that can be used to treat patients with thick, tenacious saliva
humidification
nebulisers
carbocisteine
what to do in pt with respiratory symptoms (3)
- 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
these two drug classes may be given, unlicensed indications, for patients experiencing breathlessness
opioids
BZDPNs if symptoms exacerbated by anxiety
when would you choose a BZDPN instead of opioid for patients experiencing breathlessness?
if their symptoms are exacerbated by anxiety
which drug is licensed for use in pt with amyotrophic lateral sclerosis to extent life or to extend the time to mechanical ventilation
riluzole
riluzole is licensed for…
use in pt with amyotrophic lateral sclerosis to extent life or to extend the time to mechanical ventilation
baclofen is contraindicated with oral use when the pt has …
active peptic ulceration
common SE of baclofen
- constipation
- diarrhoea
- depression
- confusion
- drowsy
- dry mouth
- euphoric mood, hallucination
- headache
- nausea, vomiting
why should baclofen not be withdrawn abruptly?
and how should you reduce the dose?
- 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
carbocisterine is contraindicated if pt has
active peptic ulceration
dose of carbocisteine for reduction of sputum viscosity
initially 2.25g daily in 3 divided doses
reduce to 1.5g daily in 2-4 divided; dose to be reduced as conditions improve
