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Neurology > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

What is motor neuron disease?

A

A group of progressive and fatal conditions where motor neurons stop functioning.

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2
Q

Is sensation affected in MND?

A

No - only motor is affected

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3
Q

What is the most common form of MND?

A

ALS - amyotrophic lateral sclerosis

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4
Q

What are the other forms of MND?

A

Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral scleorosis

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5
Q

Which motor neurons does ALS affect?

A

Upper and lower motor neurons

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6
Q

What is the aetiology of MND?

A

Most cases are sporadic but there are genetic components.

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7
Q

What are 3 risk factors for developing MND?

A

Smoking
Pesticides
Heavy metal exposure

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8
Q

Which motor neurons does progessive bulbar palsy affect?

A

Upper and lower motor neurons

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9
Q

Which motor neurons does progressive muscular atrophy affect?

A

Only lower motor neurons

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10
Q

Which motor neurons does primary lateral sclerosis affect?

A

Only upper motor neurons

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11
Q

What type of patient typically presents with MND?

A

Middle to late middle aged male patient

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12
Q

What are the classical symptoms of MND at presentation?

A

Asymmetrical weakness - typically in the upper limb first
Slurred speech
Fatiguability
Tripping over
Clumsiness
Wasting of muscles

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13
Q

What are the UMN signs?

A

Hypertonia
Hyperreflexia
Spasticity
Positive babinski sign

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14
Q

What are the LMN signs?

A

Hypotonia
Hyporeflexia
Fasciculations
Muscle atrophy

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15
Q

What investigations can be performed in the diagnosis of MND?

A

EEG
EMG
MRI brain and spine
ACh and MuSK antibodies
Lumbar puncture
Blood tests

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16
Q

What types of cells die in ALS?

A

Anterior horn cells and motor cortex neuronal cells

17
Q

What features on history would point away from MND?

A

Sensory loss
Involvement of ocular muscles

18
Q

What is the only disease modifying treatment for MND?

A

Riluzole

19
Q

What does riluzole do?

A

Slows down the progression of ALS and prolongs life by a few months

20
Q

What other management options are involved in MND?

A

Analgesia
Botox injections
Baclofen injections
Anticholinergics for drooling
NG/PEG tube
Advance care planning
Psychological support
Cough assist machines
Voice banking

21
Q

What are the differentials of MND?

A

Myasthenia Gravis
Malignancy
Stroke
Motor neuropathy
MS
Disc prolapse

22
Q

Which type of MND has the best prognosis?

A

Progressive muscular atrophy

23
Q

Which type of MND has the worst prognosis?

A

Progressive bulbar palsy

24
Q

What type of dementia is associated with motor neuron disease?

A

Fronto-temporal dementia

Neurology (35 decks)

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