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Neurology > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

What is another name for MND?

A

ALS

Lou Gehrig’s Disease

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2
Q

What is MND?

A

Untreatable rapidly progressive neurodegenerative condition

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3
Q

How common is MND?

A

200 cases per year in Scotland

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4
Q

What is the median survival for a patient diagnosed with MND?

A

3 years

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5
Q

What causes MND?

A

90% sporadic

10% familial

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6
Q

Name the four classifications of MND

A
  • Primary lateral sclerosis
  • Progressive muscular atrophy
  • Amyotrophic lateral sclerosis
  • Bulbar onset
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7
Q

Describe primary lateral sclerosis

A

Very rare, motor dysfunction but upper motor neuron phenotype - no muscle wasting and very tight muscles, good prognosis

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8
Q

Describe progressive muscular atrophy

A

Very rare, lower motor neuron phenotype, muscle wasting, weakness and spasticity - variable prognosis

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9
Q

Describe amyotrophic lateral sclerosis

A

Mixed upper and lower motor neuron signs - muscle wasting, fasciculations, brisk reflexes and increased tone. Most common type, poor prognosis.

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10
Q

Describe bulbar onset MND

A

No peripheral involvement, arms and legs are fairly well preserved. Wasted fasciculated tongue, hypertonic nasal speech. Confined to the mouth, more common in women and usually generalises to ALS.

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11
Q

What interventions can be used in bulbar onset MND?

A

Speech and language therapy
URT management
Eating and nutritional advice

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12
Q

State the upper motor neuron signs

A
  • increased tone
  • hyper-reflexia
  • extensor plantar responses
  • spastic gait
  • exaggerated jaw-jerk
  • slowed movements
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13
Q

State the lower motor neuron signs

A
  • muscle wasting or contractures
  • weakness
  • fasciculations
  • absent/reduced tendon reflexes
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14
Q

Do patients more commonly have upper or lower motor neuron signs?

A

90% have lower motor neuron signs

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15
Q

What is a bad survival sign in MND?

A

MND katabolism - increased metabolic function and weight loss

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16
Q

What is split hand syndrome?

A

Wasting of the thenar group, typical pattern of atrophy seen in ALS and preservation of the abductor digit minmi

17
Q

What indicates a benign prognosis?

A

ALS variant with flail arm or leg syndrome
Primary lateral sclerosis
Focal distal spinal muscular dystrophy
Kennedy’s disease

18
Q

What is the name of the classification system used for the diagnosis of MND?

A

El Escorial Criteria - includes EMG and other neuroimaging to rule out other diseases

19
Q

What is the only drug currently that can be used in MND?

A

Riluzole - prolongs life by 3 months at most disabled but causes severe liver damage

20
Q

Describe the communication aids that can help patients when they are unable to communicate properly

A 
Ipad 
Eye gaze
Pen and paper 
Word board
Voice bank may be created early on in the disease
21
Q

What can be done for patients who are struggling to eat?

A

PEG inserted endoscopically
RIG inserted radiologically
NG tube
All have a narrow window of efficacy

22
Q

Define sialorrhoea

A

Excess saliva

23
Q

How can excess saliva be managed?

A

Buscopan, glycopyrronium, botox into glands, suction or humification, carbocistein thickens saliva

24
Q

How can a dry mouth be managed?

A

Fluids

Artificial salivation

25
Q

As patient’s disease progresses how can nutrition and swallowing difficulties be managed?

A

Liquid drug preparations

Supplements and thickeners

26
Q

How can muscle cramps be managed?

A

Quinine, baclofen

27
Q

How can muscle spasms be managed?

A

Baclofen, gabapentin, dentrolene, cannabis therapy spray

28
Q

What is one of the main causes of MND death?

A

Weakness of respiratory muscles

29
Q

What are the red flag respiratory symptoms?

A 
Breathlessness 
Orthopnoea 
Recurrent chest infections
Disturbed sleep 
Nightmares
Poor concentration
Type 2 respiratory failure
30
Q

What is used in MND patients with respiratory problems?

A

Non-invasive ventilation - start at night and evolve into daytime use - mask needs to be tight and this is sometimes not well tolerated

31
Q

How can SOB and anxiety be managed?

A

Lorazepam

Neurology (29 decks)

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