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Neuroscience > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

Define Motor Neurone Disease?

A

A progressive neurodegenerative disorder of cortical, brainstem and spinal motor neurons (lower and upper motor neurons)

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2
Q

What are the subtypes of Motor Neurone Disease?

A

Amyotrophic Lateral Sclerosis (ALS)
Progressive Muscular Atrophy Variant
Progressive Bulbar Palsy Variant
Primary Lateral Sclerosis Variant

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3
Q

What is Amyotrophic Lateral Sclerosis (ALS)?

A

AKA Lou Gehrig’s disease

Combined generation of upper and lower motor neurones resulting a mix of LMN and UMN signs

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4
Q

What is Progressive Muscular Atrophy Variant?

A

Only LMN signs

Better prognosis

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5
Q

What is Progressive Bulbar Palsy Variant?

A

Dysarthria
Dysphagia
Wasted fasciculating tongue
Brisk jaw jerk reflex

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6
Q

What is the Primary Lateral Sclerosis Variant?

A

UMN pattern of weakness
Brisk reflexes
Extensor plantar responses
NO LMN signs

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7
Q

What is the aetiology of Motor Neurone Disease?

A

Free radical damage and glutamate excitotoxicity have been implicated

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8
Q

What is the pathology of Motor Neurone Disease?

A

Progressive motor neurone degeneration and death

Gliosis replacing lost neurones

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9
Q

What are the associations of Motor Neurone Disease?

A

Frontotemporal lobar dementia

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10
Q

What is the epidemiology of Motor Neurone Disease?

A

RARE
Incidence: 2/100,000
Mean age of onset: 55 yrs
5-10% have a family history with autosomal dominant inheritance

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11
Q

What are the presenting symptoms of Motor Neurone Disease?

A 
Weakness of limbs 
Speech disturbance (slurring or reduction in volume)
Swallowing disturbance (e.g. choking on food)
Behavioural changes (e.g. disinhibition, emotional lability)
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12
Q

What are the signs of Motor Neurone Disease?

A

Combination of UMN and LMN signs

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13
Q

What are the LMN features in Motor Neurone Disease?

A

Muscle wasting
Fasciculations
Flaccid weakness
Hyporeflexia

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14
Q

What are the UMN features in Motor Neurone Disease?

A

Spastic weakness
Extensor plantar response
Hyperreflexia

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15
Q

What would Motor Neurone Disease be on a sensory examination?

A

Should be NORMAL

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16
Q

What investigations do we do for Motor Neurone Disease?

A 
Bloods 
Electromyography (EMG)
Nerve conduction studies
MRI
Spirometry
17
Q

What bloods do we do for Motor Neurone Disease?

A

Mild elevation in CK
ESR
Anti-GM1 ganglioside antibodies

18
Q

Why do we do a MRI for Motor Neurone Disease?

A

Exclude cord compression and brainstem lesions

19
Q

Why do we do Spirometry for Motor Neurone Disease?

A

Assess respiratory muscle weakness

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