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Neurology > Motor neurone disease > Flashcards

Motor neurone disease Flashcards

1
Q

What is motor neurone disease

A

A devastating condition causing degeneration of the motor neurones, causing progressive weakness and death

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2
Q

What usually causes death in MND?

A

Respiratory failure or aspiration

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3
Q

Describe the aetiology of MND

A

50-75 y/o
ALS more common in non-Caucasian populations

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4
Q

What is the median survival after symptom onset in MND?

A

3 years

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5
Q

What causes MND?

A

The reason for it is unknown

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6
Q

What conditions are found under the term MND?

A

ALS
PLS
Progressive bulbar palsy
PMA

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7
Q

What are some benign variants of ALS?

A

Flail arm syndrome
Flail leg syndrome
(These will eventually progress into ALS)

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8
Q

What is the most common form of MND?

A

ALS - Amyotrophic Lateral Sclerosis

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9
Q

What does ALS stand for?

A

Amyotrophic Lateral Sclerosis

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10
Q

What does PLS stand for?

A

Primary Lateral Sclerosis

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11
Q

What does PMA stand for?

A

Progressive muscular atrophy

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12
Q

Describe the pattern of disease in ALS

A

Classic paraneoplastic presentation with simultaneous involvement of upper and lower motor neurones, usually in one limb, spreading gradually to other limbs and trunk muscles

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13
Q

What is split hand syndrome?

A

Preferential wasting of thenar group - a typical pattern of atrophy seen in ALS

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14
Q

Describe the pattern of disease in in progressive muscular atrophy (PMA)

A

Pure lower motor neurone presentation with weakness, muscle wasting and fasciculations, usually starting in one limb and gradually spreading to involve other adjacent spinal segments

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15
Q

Describe the pattern of disease in progressive bulbar and pseudo bulbar palsy

A

The lower cranial nerve nuclei and their supranuclear connections are initially involved, affecting the tongue, facial and pharyngeal muscles

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16
Q

How does progressive bulbar and pseudo palsy present?

A

Dysarthria
Dysphagia
Nasal regurgitation of fluids
Choking
Hypo/Hyper sialorrhoea
Wasting of the tongue and pharynx

17
Q

Describe the pattern of disease in primary lateral sclerosis (PLS)

A

Confined to upper motor neurones, causing a slowly progressive tetraparesis and pseudobulbar palsy

18
Q

What are some common presentations of MND?

A

Muscle weakness and possible speech and swallow problems
UMN and LMN signs without sensory signs
Focal onset and continuous spread

19
Q

What is MND catabolism?

A

Loss of weight in MND despite normal eating - Cause unknown

20
Q

What are some UMN signs of MND?

A
  • Weakness or paralysis
  • Spasticity
  • Increased muscle tone (Hypertonia)
  • Over-responsive reflexes (Hyperreflexia)
  • Emotional lability
  • Positive Babinski sign
21
Q

What is meant by a positive Babinski sign?

A

When the bottom of the foot is stroked, instead of the toes curling in, the big toe extends up and the other toes fan out

22
Q

Wha are some LMN signs of MND?

A
  • Weakness or paralysis
  • Decreased or absent muscle tone (Hypotonia or atonia)
  • Decreased or absent reflexes (Hyporeflexia or areflexia)
  • Involuntary muscle twitches (Fasiculations)
  • Muscle atrophy
23
Q

What are some red flag symptoms of respiratory failure in MND?

A
  • Breathlessness
  • Orthopnea
  • Recurrent chest infection
  • Disturbed sleep
  • Non-refreshed sleep
  • Nightmares
  • Daytime sleepiness
  • Poor concentration
24
Q

What is meant by emotional lability in UMN MND?

A

Inappropriate crying or laughing

25
Q

How is a diagnosis of MND made?

A

Mainly clinical diagnosis
Electrophysiology
Possible neuroimaging and lab studies

26
Q

What are some on-going management strategies required in MND?

A
  • Assessment and coordination of care
  • Communication - SALT, AAC
  • Nutrition - Gastrostomy, dieticians
  • Respiratory - Assessment, home ventilation
27
Q

What drug is the only drug licensed for MND?

A

Riluzole

28
Q

What is riluzole?

A

A sodium-channel blocker that inhibits glutamate release, giving patients an extra 3 months of life

29
Q

Why do most patients not choose to take riluzole?

A
  • This will give the patient an extra 3 months of life, however, this will be during the end of their life, when they are in their worst state
  • It also causes lethargy, sickness and possible cognitive impairment
30
Q

Symptomatic treatment - Sialorrhoea

A

Hyoscine/buscopan
Glycopyrronium
Botox
Suction

31
Q

Symptomatic treatment - Nutritional support

A

Supplements/thickeners
Liquid drug preparations

32
Q

Symptomatic treatment - Bulbar dysfunction

A

Early communicator
Nutritional support
Care for upper respiratory tract

33
Q

Symptomatic treatment - Limb dysfunction

A

Washing
Dressing
Feeding
Turning in bed
Mobility aids/hoists often necessary

34
Q

Symptomatic treatment - Muscle cramps

A

Quinine
Baclofen

35
Q

Symptomatic treatment - Muscle spasms

A

Baclofen
Tizanidine
Dantroline
Gabapentin

36
Q

Symptomatic treatment - Emotional lability

A

Sometimes treated with antidepressants

Neurology (28 decks)

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