Motor Neurone Disease Flashcards Preview

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Flashcards in Motor Neurone Disease Deck (48):
1

what is motor neurone disease?

untreatable and rapidly progressive neurodenegerative condition

2

how is motor neurone disease diagnosed?

clinically

3

what are symptoms of MND?

muscle weakness and potentially problems with speech, swallow and breathing

4

what is the onset and progression of MND?

focal onset and continuous spread, finally generalised paresis

5

what is most common subtype of MND?

amyotrophic lateral sclerosis (ALS) - mixed upper and lower signs

also known as Lou Gehrigs disease

6

what is median survival of MND after symptom onset?

3 years

7

the incidence of MND is decreasing - true or false?

false - it is increasing

8

MND is slightly more common in what gender?

male

9

what is cause of MND?

90% sporadic
10% familial

10

sporadic MND peaks what which age?

50 and 75 - then declines after age of 80

11

ALS is less common in what population?

non-caucasian

12

what is PLS?

rare form of MND - problem with upper motor neurone

13

what is PMA?

another rare form of MND - problem with lower motor neurone

14

65% of patients present with spinal / limb onset. What else could patients present with?

bubar onset
cognitive onset
respiratory onset

15

90% of MND patients have abnormality in what protein?

tdp-43

16

what are 4 types of motor neurone disease?

PLS - primary lateral sclerosis
ALS - amyotrophic lateral sclerosis
PBS - progressive bulbar palsy
PMA - progressive muscular atrophy

17

what is median survival of ALS?

3-5 years

18

what is median survival of PLS (1-3% of cases)?

good (>5 years)

19

what is median survival of PMA (10% of cases)?

variable

20

what are upper motor neurone signs?

increased tone
hyper reflexia
extensor plantar responses
spastic gait
exaggerated jaw jerk
slow movements

21

what are lower motor neurone signs?

muscle wasting
weakness
fasciculations
absent or reduced tendon reflexes

22

what signs may you see when you examine someone with MND?

extremities (70%) (upper > lower)
bulbar (25%)
thoracic (2%)

upper motor neurone (10%)
lower motor neurone (90%)

cognitive problems (5%)

MND katabolism (40-50%)

23

what is therapeutic interventions of bulbar variant MND?

early communicator
nutritional support
care for URT

24

what does bulbar dysfunction affect?

tongue muscles, facial muscles and pharyngeal muscles

25

what symptoms can occur in upper and lower limbs?

wasting (either global or split hand syndrome)

loss of tone or contractures

26

what gene is associated with frontotemporal dementia?

C9orf72

27

what are ALS variants with more benign prognosis?

note - these are not classified as MND

flail arm syndrome
flail leg syndrome
primary lateral sclerosis
focal distal spinal muscular dystrophy
kennedy disease (SMA variant)

28

what investigation can take place in MND to confirm diagnosis?

electrophysiology + clinical findings

29

what is the pathology of MND?

motor neurone degeneration / death

30

what is name of criteria used to diagnose ALS?

el escorial criteria

31

ALS is a diagnosis of exclusion - true or false?

true

32

what is riluzole?

drug which can be given in end stages of MND to prolong life for 3 months

33

what are other interventions in MND?

communication needs = speech therapy, technology from tablets

nutritional needs = dieticians, gastrostomy

respiratory needs (assessment, home ventilation)

34

what is the average time from symptom onset to diagnosis?

12 months

35

who is in a MND team?

1 consultant and 2 nurse specialist delivering care and advice at home and at clinic

36

how often are patients MND patients assessed?

4-6 weeks - not always in person, can do phone and email

37

what happens to metabolic rate in MND?

it is doubled - weight loss expected

38

what is symptomatic treatment for muscle cramps?

quinine
baclofen

39

what is symptomatic treatment for muscle spasms?

baclofen
tizanidine
dantrolene
gabapentin
medicinal cannabis

40

what is one of the main causes of death in MND?

weakness of respiratory muscles

41

what is offered to support type 2 respiratory failure?

non-invasive ventilation - BiPAP mask, commenced at night and gradually increased depending on need

42

what is symptomatic treatment for SOB / anxiety?

lorazepam

43

what are symptomatic treatment for coughing?

breath stacking
cough assist

44

what is the symptomatic treatment for sialorrhoea?

hycosine / buscopan

glycopyrronium (esp if cognitive impairment)

botox

suction / humidification / carbocisteine

45

what is symptomatic treatment for malnutrition?

supplements / thickeners

liquid drug preparations

46

what are the red flag signs of respiratory failure?

breathlessness
orthopnea
recurrent chest infection
disturbed sleep
non-refreshed sleep
nightmares
daytime sleepiness
poor concentration

47

what % of MND patients may have cognitive impairment?

50 - spectrum from mild to dementia

48

what type of dementia is MND associated with?

frontotemporal

apathy, disinhibition, poor planning / decisions