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Charis & Ellie VS Neuro > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

What is MND?

A

Untreatable and rapidly progressive neurodegenerative condition
-Mainly clinical diagnosis

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2
Q

What does MND cause?

A

Progressive weakness and eventually death usually as a result of respiratory failure or aspiration

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3
Q

What is death from MND usually as a result of?

A

Respiratory failure

Aspiration

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4
Q

Most common type of MND?

A

Amyotrophic lateral sclerosis

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5
Q

What is MND AKA?

A

ALS

Lou Gehrig’s disease

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6
Q

Who gets MND?

A
  • Males > females
  • 90% sporadic and 10% familial
  • Sporadic MND peaks at 50-75 y/o
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7
Q

Aetiology of MND?

A

Thought to be links with some genes such as
-C90RF72 gene
(which is linked to FTD suggesting conditions are interlinked)

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8
Q

Which MND is least common in non-Caucasians?

A

ALS

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9
Q

How does MND present?

A
  • Muscle weakness and potentially problems with speech, swallow and breathing
  • Upper and/or lower motor neurone signs without sensory problems
  • Focal onset and continuous spread, finally generalized paresis
  • Cognitive impairment
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10
Q

What are upper motor neurone signs?

A
  • Pseudobulbar affect
  • Increased tone
  • Hyper-reflexia
  • Extensor plantar responses
  • Spastic gait
  • Exaggerated jaw jerk
  • Slowed movements
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11
Q

Lower motor neurone signs?

A
  • Muscle wasting
  • Weakness
  • Fasciculations
  • Absent or reduced deep tendon reflexes
  • Muscle cramps
  • Muscle hypotonicity
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12
Q

What is bulbar dysfunction?

A

Affects tongue muscles, facial muscles and pharyngeal muscles

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13
Q

What is spinal dysfunction?

A
  • Variable
  • Muscle wasting
  • Loss of tone or contractures
  • Consider: washing, dressing and feeding
  • Mobility aids/hoists often necessary
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14
Q

Presentation of ALS?

A
  • Flail arm syndrome
  • Flail leg syndrome
  • Focal distal spinal muscular dystrophy
  • Kennedys disease (SMA Variant)
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15
Q

Presentation of emotional lability?

A

Inappropriate crying or laughing

Sometimes treated with anti-depressants

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16
Q

Types of MND?

A
  • PLS: Primary Lateral Sclerosis
  • ALS: Amyotrophic Lateral sclerosis
  • PMA: Progressive muscular atrophy
  • PBP: Progressive bulbar palsy:
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17
Q

What is PLS?

A

Primary Lateral Sclerosis
- UMN variant
- 1-3% of MND cases (less common)
- Good survival (>5 years)

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18
Q

What physiologically is PLS?

A

-Confined to UMN causing a slow progressive tetraparesis and pseudobulbar palsy

(Tetraparesis: 4 limbs muscle weakness)

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19
Q

What is ALS?

A
  • Most common MND phenotype
  • Upper and lower motor neurone features
  • Poor survival (median 3-5 years)
  • Muscle wasting and fasciculations
  • Brisk reflexes
20
Q

Presnetation of ALS?

A

Progressive Focal Muscle Weakness
- Muscle twitching
-Weakness in arm
-Wasting with muscle fasciculations due to spontaneous firing of abnormality large motor units formed by surviving axons branching to innervate muscle fibres that have lost their nerve supply

21
Q

Initial presentation of ALS?

A

Weakness often starts in one limb and the spreads to other parts of body

-Cramps are common

22
Q

What is PMA?

A

Progressive muscular atrophy

  • Pure LMN presentations
  • 10% of MND cases (Rare)
23
Q

Presnetation of PMA?

A
  • Weakness
  • Muscle wasting
  • Fasciculations that start in one limb and spread
24
Q

What is progressive bulbar palsy?

A
  • 1-2% MND cases
  • Women > men (60-80 y/o)
  • Limbs are preserved but symptoms in mouth
25
Q

Mixed UMN and LMN but confined to the mouth is?

A

Progressive Bulbar Palsy

26
Q

Progressive bulbar palsy mouth symptoms?

A
  • Bulbar and wasted tongue
  • Affected speech
  • Tongue fasciculations with slow stiff tongue movements
  • Choking
  • Nasal regurgitation
  • Dysarthria
  • Dysphagia
27
Q

Therapeutic interventions in progressive bulbar palsy?

A
  • Early communicator
  • Nutritional support
  • Care for respiratory tract
28
Q

How is MND diagnosed?

A
  • Electrophysiology

- ALS: EI Escorial criteria

29
Q

How is MND treated?

A

-Access to specialist MND services

30
Q

Treatment plans of MND?

A
  • Keyworker assessing needs and coordination of care
  • Communication needs
  • Nutritional needs
  • Resp needs
  • Riluzole
  • Muscle cramps/spasms
31
Q

Drug treatment for muscle cramps?

A

Quinine

Baclofen

32
Q

Drug treatment for muscle spasms?

A

Baclofen
Tizanidine
Dantrolene
Gabapentin

33
Q

what are the consequences of respiratory dysfunction?

A
  • Weakness of resp muscles is one of the main causes of death in MND
  • Resp management from diagnosis is vital
34
Q

MND Resp red flags?

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

When is non-invasive ventilation offered in resp dysfunction?

A

To support T2 Resp failure

36
Q

Prognosis of MND?

A

3 years after symptom onset

37
Q

Presentation of MND?

A
  • Functional effects of muscle weakness eg loss of dexterity, falls or trips
  • Speech/swallowing problems or tongue fasciculations
  • Muscle problems: weakness, wasting, twitching, cramps, stiffness
  • Breathing Problems: SOB, resp symptoms
  • XSive daytime sleepiness, fatigue, early morning headache, SOB lying down
  • Cognitive symptoms: Frontotemporal dementia, behavioural changes and emotional lability
  • UMN and or LMN signs on examination
38
Q

Diagnosis of MND?

A

Largely clinical

-No specific diagnostic tests but investigations can exclude other conditions

39
Q

What does an EMG confirm? And what is that?

A

-Electromyography

Denervation of muscles due to degeneration of LMN

40
Q

How long do most MND survive?

A

Most cases don’t survive past 3 years but some variants do better than others

41
Q

What drug slows progression of MND?

A

Riluzole

42
Q

What is riluzole?

A

Na+ channel blocker

-Inhibits glutamate release and slows progression slighlty by 3-4 months

43
Q

Is riluzole popular?

A

NO

-Extra time added to life tends to be when you are most disabled

44
Q

WHat is increased in MND?

A

Metabolic rate (wt loss expected)

45
Q

What treats muscle spasms?

A

Baclofen

46
Q

Management of MND?

A
  • MDT approach
  • Communication aids
  • Dietician advice
  • Gastrostomy
  • Physiotherapy
  • Baclofen for muscle spasms
  • Non-invasive ventilatory support- BiPAP at night

Charis & Ellie VS Neuro (49 decks)

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