Motor Neurone Disease Flashcards

1
Q

MND is untreatable. TRUE/FALSE?

A

TRUE

the treatments we have are not effective at improving progosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Do patients with MND have Upper motor neuron symptoms, lower motor neuron symptoms or sensory symptoms?

A

Can have both UMN and LMN symptoms

NO SENSORY SYMP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What si the most common subtype of MND?

A

Amyotrophic lateral sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the median prognosis of MND after prognosis?

A

3 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

MND is becoming more common. TRUE/FALSE?

A

TRUE

mechanism unknown

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Is MND slightly more common in males or females?

A

Males

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Are most cases of MND sporadic or familial?

A

90% sporadic

10% Familial

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

The diagnosis of sporadic MND peaks at what ages?

A

50-75

if not diagnosed by 80, likelihood goes down

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

ALS is less common in non-Caucasian populations. TRUE/FALSE?

A

TRUE

=> more common in caucasians

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How is MND usually classified?

A

UMN - Primary lateral sclerosis
LMN - Primary Muscular Atrophy
Mix of the two = ALS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

If patients with ALS exhibit signs of upper and lower motor neuron dysfunction, what symptoms can they have?

A
Brisk reflexes (UMN)
Muscle wasting
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

If MND has a bulbar onset, what activities are affected?

A

Speech

Swallowing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the prognosis of ALS compared to other types of MND?

A

ALS - poor prognosis (3-5 years)
Primary Lateral Sclerosis (>5yrs)
Progressive Muscular atrophy (variable)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What gene should you test for in ALS with frontotemporal dementia (FTD-MND)

A

C9 ORF 72

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Give examples of UMN signs seen in MND?

A
Increased tone
Hyper-reflexia
Extensor plantar responses
Spastic gait
Slow movement
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What LMN signs could be seen in MND?

A

Muscle wasting
Weakness
Fasiculation
Absent/reduced reflexes

17
Q

What is MND katabolism and what percentage of patients does this affect?

A
  • patients are cahectic and struggle to maintain body weight despite eating normal amounts
  • due to progressive muscle break down
  • 40-50% of patients have this
18
Q

What tasks do MND patients with limb dysfunction often complain they are struggling to complete?

A

Writing
Holding objects
Turning keys in the door

19
Q

What is the largest complication risk in MND patients with bulbar onset?

A

aspiration due to poor swallow

=> respiratory complications

20
Q

What patient group normally gets primary bulbar onset and what does this progress to?

A

Woman > Men (60-80 years of age)

Always generalises into ALS

21
Q

What early interventions are given to patients with bulbar involvement?

A
  • Early communicator => voice banking
  • nutritional support
  • care for upper respiratory tract
22
Q

Other than muscles in the tongue, what does bulbar onset MND affect?

A

facial muscles and pharyngeal muscles

23
Q

What symptoms are seen in spinal dysfunction MND?

A
  • Muscle wasting
  • Loss of tone or contractures
  • Problems with washing, dressing, feeding, turning in bed
24
Q

MND “Split hand syndrome” indicates what group of muscles wastes more than the other?

A

Thenar muscle wasting (1st dorsal interosseous and abductor pollicis brevis)

hypothenar = spared (adbductor digiti minimi)

25
Q

How is a diagnosis of MND made?

A
  • clinical criteria UMN/LMN signs and no sensory symptoms
  • Limb/bulbar/spinal onset +/- cognitive symptoms
  • El-Escorial criteria and EMG findings
  • Imaging/ lab studies to rule out other diseases
26
Q

Why are a lot of patients with MND referred to another speciality first?

A

Bulbar onset can mimic potential ENT tumour

=> referred urgently there first

27
Q

What can cause a false positive diagnosis of MND?

A

Multifocal motor neuropathy (MMN)
Kennedy’s disease
cervical spondylotic radiculomyelopathy

28
Q

What drug can be used in MND and what does it do?

A

Riluzole - prolongs life by 3 months in very late stage of illness
many pts choose not to take the drug for this reason

29
Q

What interventions can be used to help with breathing and nutrition in MND?

A

BiPAP - non-invasive ventilation

PEG tube

30
Q

Why can BiPAP ventilation be a problem for patients who have reduced upper limb function?

A

If mask falls off during the night, patient cant use their hand to fix this

31
Q

How many months are usually between symptom onset and diagnosis?

A

12 months

32
Q

Most MND care is delivered in hospital. TRUE/FALSE?

A

FALSE

Most in community/ at patients homes

33
Q

Bulbar dysfunction can also cause problems with saliva. How can these be treated?

A

Over salivation- Tx: buscopan, botox

Under salivation- Tx: Salivation pastilles

34
Q

What are the different methods of augmentative and alternative communication (AAC)?

A

Low-tech: Paper and pen, Word Boards

High-tech: iPads, Lightwriter, Eye-Gaze

35
Q

Weight loss is a red flag symptom in MND prognosis. TRUE/FALSE?

A

TRUE

- 6 months left after patient begins to become cachexic

36
Q

What drugs can be given to patients to help with muscle cramps and spasms?

A

Cramps

  • Quinine (not widely used now)
  • Baclofen

Spasms

  • Baclofen
  • Tizanidine
  • Dantrolene
  • Gabapentin
37
Q

What are the red flags of respiratory failure in MND?

A
  • SOB
  • Orthopnoea
  • Recurrent chest infection
  • Disturbed sleep/nightmares
  • Poor concentration
38
Q

If patients breathlessness cannot be controlled by non-invasive ventilation, how are they managed?

A
  • Sedation - lorazepam

- coughing techniques

39
Q

Certain MND patients can be emotionally labile. What does this mean?

A
  • Inappropriate crying or laughing

- Part of the disease (Upper Motor Neuron sign)