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Neurology > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

what is motor neurone disease?

A

untreatable and rapidly progressive neurodenegerative condition

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2
Q

how is motor neurone disease diagnosed?

A

clinically

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3
Q

what are symptoms of MND?

A

muscle weakness and potentially problems with speech, swallow and breathing

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4
Q

what is the onset and progression of MND?

A

focal onset and continuous spread, finally generalised paresis

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5
Q

what is most common subtype of MND?

A

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

also known as Lou Gehrigs disease

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6
Q

what is median survival of MND after symptom onset?

A

3 years

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7
Q

the incidence of MND is decreasing - true or false?

A

false - it is increasing

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8
Q

MND is slightly more common in what gender?

A

male

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9
Q

what is cause of MND?

A

90% sporadic

10% familial

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10
Q

sporadic MND peaks what which age?

A

50 and 75 - then declines after age of 80

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11
Q

ALS is less common in what population?

A

non-caucasian

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12
Q

what is PLS?

A

rare form of MND - problem with upper motor neurone

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13
Q

what is PMA?

A

another rare form of MND - problem with lower motor neurone

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14
Q

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

A

bubar onset
cognitive onset
respiratory onset

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15
Q

90% of MND patients have abnormality in what protein?

A

tdp-43

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16
Q

what are 4 types of motor neurone disease?

A

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

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17
Q

what is median survival of ALS?

A

3-5 years

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18
Q

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

A

good (>5 years)

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19
Q

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

A

variable

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20
Q

what are upper motor neurone signs?

A 
increased tone 
hyper reflexia 
extensor plantar responses 
spastic gait 
exaggerated jaw jerk 
slow movements
21
Q

what are lower motor neurone signs?

A

muscle wasting
weakness
fasciculations
absent or reduced tendon reflexes

22
Q

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

A

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

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

cognitive problems (5%)

MND katabolism (40-50%)

23
Q

what is therapeutic interventions of bulbar variant MND?

A

early communicator
nutritional support
care for URT

24
Q

what does bulbar dysfunction affect?

A

tongue muscles, facial muscles and pharyngeal muscles

25
Q

what symptoms can occur in upper and lower limbs?

A

wasting (either global or split hand syndrome)

loss of tone or contractures

26
Q

what gene is associated with frontotemporal dementia?

A

C9orf72

27
Q

what are ALS variants with more benign prognosis?

note - these are not classified as MND

A 
flail arm syndrome 
flail leg syndrome 
primary lateral sclerosis 
focal distal spinal muscular dystrophy 
kennedy disease (SMA variant)
28
Q

what investigation can take place in MND to confirm diagnosis?

A

electrophysiology + clinical findings

29
Q

what is the pathology of MND?

A

motor neurone degeneration / death

30
Q

what is name of criteria used to diagnose ALS?

A

el escorial criteria

31
Q

ALS is a diagnosis of exclusion - true or false?

A

true

32
Q

what is riluzole?

A

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

33
Q

what are other interventions in MND?

A

communication needs = speech therapy, technology from tablets

nutritional needs = dieticians, gastrostomy

respiratory needs (assessment, home ventilation)

34
Q

what is the average time from symptom onset to diagnosis?

A

12 months

35
Q

who is in a MND team?

A

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

36
Q

how often are patients MND patients assessed?

A

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

37
Q

what happens to metabolic rate in MND?

A

it is doubled - weight loss expected

38
Q

what is symptomatic treatment for muscle cramps?

A

quinine

baclofen

39
Q

what is symptomatic treatment for muscle spasms?

A 
baclofen 
tizanidine 
dantrolene 
gabapentin 
medicinal cannabis
40
Q

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

A

weakness of respiratory muscles

41
Q

what is offered to support type 2 respiratory failure?

A

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

42
Q

what is symptomatic treatment for SOB / anxiety?

A

lorazepam

43
Q

what are symptomatic treatment for coughing?

A

breath stacking

cough assist

44
Q

what is the symptomatic treatment for sialorrhoea?

A

hycosine / buscopan

glycopyrronium (esp if cognitive impairment)

botox

suction / humidification / carbocisteine

45
Q

what is symptomatic treatment for malnutrition?

A

supplements / thickeners

liquid drug preparations

46
Q

what are the red flag signs of respiratory failure?

A 
breathlessness
orthopnea 
recurrent chest infection 
disturbed sleep 
non-refreshed sleep
nightmares
daytime sleepiness 
poor concentration
47
Q

what % of MND patients may have cognitive impairment?

A

50 - spectrum from mild to dementia

48
Q

what type of dementia is MND associated with?

A

frontotemporal

apathy, disinhibition, poor planning / decisions

Neurology (32 decks)

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