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Neurology > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

What is the commonest MND in adults?

A

ALS- lou Gehrig’s disease

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2
Q

How does MND present?

A

Problems with speech, swallow and breathing

Upper and/or lower MD signs without sensory problems

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3
Q

Describe the progression of MND?

A

Focal onset and continuous spread, finally generalised paresis

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4
Q

What is the median survival after symptom onset?

A

3 years

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5
Q

90% of MND is ______

A

90% of MND is sporadic

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6
Q

A genetic aetiology has been identified in up to __% of sporadic and __% of genetic familial cases

A

A genetic aetiology has been identified in up to 20% of sporadic and 60% of genetic familial cases

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7
Q

Sporadic MND peaks at the ages of __-__ years and declines after age __

A

Sporadic MND peaks at the ages of 50-75 years and declines after age 80

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8
Q

ALS is less common in which population?

A

Non-caucasuan populations

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9
Q

What is included in MND?

A

Amyotrophic Lateral Sclerosis
Progressive muscular atrophy
Progressive bulbar palsy
Primary lateral sclerosis

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10
Q

List the UMN signs in MND

A 
Increased tone
Hyper-reflexia
Extensor plantar gait
Exaggerated Jaw-jerk
Slows movements
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11
Q

List the LMN signs in MND

A

Muscle wasting
Weakness
Fasciculations
Absent or reduced deep tendon reflexes

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12
Q

What percentage of patients have primary bulbar onset MND? Which gender and age is affected?

A

25%

Women > Men (60-80)

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13
Q

What is the prognosis of primary bulbar MND?

A

Generalisation into ALS

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14
Q

What are the therapeutic interventions for primary bulbar ALS?

A

Early communicator
Nutritional support
Care for URT

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15
Q

What is affected by bulbar dysfunction?

A

Tongue muscles, facial muscles and pharyngeal muscles

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16
Q

What is seen in spinal dysfunction in NMD?

A

Muscle wasting

Loss of tone or contractures

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17
Q

What is split hand syndrome?

A

Preferential wasting of the thenar group is typical in ALS

Wasting of first dorsal interosseous and abductor policies brevis and preservation of abductor digits minimi

18
Q

What are the ALS variants with more benign prognosis?

A 
Flail arm syndrome
Flail leg syndrome
Primary Lateral Sclerosis
Focal distal spinal muscular dystrophy
Kennedy’s disease (SMA variant)
19
Q

What is the pathology of MND?

A

Motor neurone degeneration and death

20
Q

What is the investigation for NMD?

A

Electrophysiology

21
Q

Describe clinically definite ALS

A

Upper and lower motor neurone signs in bulbar and at least two spinal (lumbosacral, thoracic, or cervical) regions
OR
Upper and Lower motor neurone signs in three spinal regions

22
Q

Describe clinically probable ALS

A

Upper and lower motor neurone signs in at least two regions (bulbar or spinal) with some upper motor neurone signs rostral to the lower motor neurone signs

If lab supported= EMG findings of lower motor neurone involvement in at least two body regions

23
Q

What are frequent misdiagnosis of MND?

A

Carpal tunnel syndrome, stroke, neuropathy

24
Q

What are frequent false positive diagnosis of MND?

A

Multifocal motor neuropathy, Kennedy’s disease, myopathy (inclusion body myopathy), cervical spondylotic radiculomyelopathy

25
Q

Describe on-going management in MND?

A
  • Key worker assessing needs & coordination of care
  • Communication needs (Speech therapy, technology from tablets to ‘voice banking’)
  • Nutritional needs (dieticians, gastrostomy)
  • Respiratory needs (assessment, Home ventilation)
  • Riluzole
26
Q

What is riluzole?

A

Riluzole delays the onset of ventilator-dependence or tracheostomy in some people and may increase survival by two to three months

27
Q

Why is nutrition important in MND?

A

Metabolic need is doubled

A weight loss of >10% at diagnosis is a poor survival indicator

28
Q

How should nutrition be managed?

A
  1. give small high energy supplements regularly
  2. consideration of upper limb (hand to mouth action) is important, carers may need to help
  3. early insertion of gastrostomy tubes should be clinical priority
29
Q

What variations of gastrostomy can be used?

A

PEG- Percutaneous endoscopic gastrostomy

RIG- Radiologically Inserted Gastrostomy

PIGG- per-oral image guided gastrostomy
NG- Nasogastric tube

30
Q

What is sialorrhoea?

A

Hypersalivation

31
Q

What are the treatment options for sialorrhoea?

A
  • Hyoscine/Buscopan
  • Glycopyrronium (especially if cognitive impairment)
  • Botox
  • Suction/Humidification/Carbocisteine
32
Q

What are the treatments for muscle cramps?

A

Quinine

Baclofen

33
Q

What are the treatments for muscle spasms?

A

Baclofen
Tizanidine
Dantrolene
Gabapentin

34
Q

What are the red flags of respiratory failure

A 
Breathlesness
Orthopnea
Recurrent chest infection
Disturbed sleep
Non-refreshed sleep
Nightmares
Daytime sleepiness
Poor concentration
35
Q

What means of non-invasive ventilation can be used?

A

BiPAP mask

Commenced at night initially and gradually increases

36
Q

What are the symptomatic treatments for SOB/anxiety?

A

Lorazepam

37
Q

What are the symptomatic treatments for coughing?

A

Breath stacking

Cough assist

38
Q

What is breath stacking?

A

Sit upright in a comfortable position
Take a deep breath in and hold it
Try to take another deep breath in on top of the previous breath
Aim to take another 1-3 breaths in the same way (3-5 breaths in total)
Breathe out or cough
Repeat this process 3-5 times

39
Q

What must be up to date to withdraw NIV?

A

DNACPR, AWI, POA

40
Q

What is emotional lability?

A

Inappropriate crying or laughing
Part of UMN signs
Sometimes treated with antidepressants

41
Q

What percentage of MND patients have cognitive impairment?

A

50%

Neurology (41 decks)

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