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Year 3 > Motor neuron disease (N) > Flashcards

Motor neuron disease (N) Flashcards

1
Q

What is motor neurone disease?

A

Neurodegenerative disorder with UMN and LMN dysfunction, characterised by progressive muscle weakness

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2
Q

What are the different types of motor neurone disease? (4)

A
  • amyotrophic lateral sclerosis (ALS) - most common
  • progressive muscular atrophy
  • primary lateral sclerosis
  • progressive bulbar palsy variant
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3
Q

What types of signs are there in amyotrophic lateral sclerosis (MND)?

A

Both UMN and LMN signs

Combined degeneration of UMN&LMN

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4
Q

What types of signs are there in progressive muscular atrophy (MND)?

A

Only LMN signs - carries best prognosis

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5
Q

What types of signs are there in primary lateral sclerosis (MND)?

A

Only UMN signs

(Loss of Betz cells in motor cortex)

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6
Q

What types of signs are there in progressive bulbar palsy variant (MND)?

A

Affects CNIX-XII –> tongue and bulbar involvement (dysarthria, dysphagia) = worst prognosis

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7
Q

What is the aetiology of MND?

A
  • unknown aetiology
  • positive Fx of ALS in 5-10% of cases
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8
Q

What demographics is MND most common in?

A
  • male
  • age >65
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9
Q

What type of dementia are those with MND at increased risk of developing?

A

Frontotemporal dementia

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10
Q

Describe the pathology of MND.

A
  • progressive motor neurone degeneration and death
  • gliosis replacing lost neurones
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11
Q

What is ALS (MND) characterised by?

A

Progressive muscle weakness that can start in limb, axial, bulbar or respiratory muscles and then generalises relentlessly, causing progressive disability and ultimately death, usually from respiratory failure

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12
Q

What are the clinical features of MND? (10)

A
  • progressive muscle weakness + proximal myopathy (asymmetrical)
  • speech disturbance - slurring, reduction in volume
  • dysphagia
  • behavioural changes - disinhibition, emotional lability
  • stiffness with poor coordination and balance
  • spastic, unsteady gait
  • painful muscle spasms
  • dyspnoea/SOB
  • wasting of thenar muscles and tongue base
  • fasciculations
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13
Q

What signs are seen in MND?

A

Combination of UMN and LMN signs

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14
Q

What UMN signs are seen in MND? (9)

A

Primarily in legs

  • weakness (spastic)
  • hyperreflexia - brisk limb and jaw reflexes
  • hypertonia
  • Babinski sign
  • dysarthria - strained slow speech
  • dysphagia
  • loss of dexterity
  • spasticity
  • extensor plantar response
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15
Q

What LMN signs are seen in MND? (8)

A

Primarily in arms

  • weakness (flaccid)
  • hyporeflexia
  • hypotonia
  • muscle wasting
  • tongue fasciculations
  • dysphagia
  • nasal speech
  • foot drop, head drop
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16
Q

What is the most common presentation of ALS (MND)?

A

Asymmetric limb weakness

17
Q

What does MND not affect? (4)

A
  • no sensory signs/symptoms
  • does not affect external ocular (eye) muscles
  • no cerebellar signs
  • abdominal reflexes usually preserved (and sphincter dysfunction if present is a late feature)
18
Q

What are some risk factors for MND? (2)

A
  • Fx
  • age >40y
19
Q

How is MND diagnosed?

A

Clinical diagnosis by assessing Hx and examination

20
Q

What scans can be done for MND? (3)

A
  • electromyography (EMG) - show signs of denervation (reduced APs with increased amplitude)
  • normal nerve conductions studies (exclude neuropathy)
  • MRI - exclude cord compression, myelopathy, brainstem lesions
21
Q

What investigation can be used to help monitor respiratory muscle weakness in MND?

A

Spirometry

22
Q

What are some differential diagnoses for MND? (9)

A
  • cervical spondylosis
  • multifocal motor neuropathy
  • inclusion body myositis
  • monomelic amyotrophy (LMN, upper extremity)
  • myasthenia gravis (ocular Sx e.g. ptosis, diplopia, extraocular muscle dysfunction, absent UMN/LMN signs, mimics bulbar-onset ALS, AChR antibodies)
  • benign fasciculations
  • post-polio syndrome (LMN)
  • progressive muscular atrophy (LMN)
  • primary lateral sclerosis (UMN)
23
Q
A
24
Q

What is the first-line treatment for MND?

A

Riluzole (glutamate antagonist) - prolongs life by 3 months

(+ supportive care)

25
Q

What do we monitor every 3 months in MND patients on riluzole?

A

LFTs and RBC

Riluzole can cause neutropenia

26
Q

How do we manage respiratory symptoms in MND?

A

Non-invasive ventilation (BiPAP) at night for type II respiratory failure

(Survival benefit of 7 months)

27
Q

How do we support nutrition (and swallowing difficulties) in MND?

A

Percutaneous gastronomy (PEG) tube - preferred way to support nutrition

28
Q

What are some complications of MND? (5)

A
  • respiratory failure
  • nutritional deficit
  • aspiration pneumonia
  • frontotemporal dementia
  • riluzole-related neutropenia
29
Q

Describe the prognosis of MND.

A

Very poor - most patients die within 3-5y (50% die within 3y)

Year 3 (208 decks)

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