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Neurology finals > Motor neuron disease > Flashcards

Motor neuron disease Flashcards

1
Q

Define motor neurone disease

A

A cluster of neurodegenerative diseases
Characterized by selective neuron loss in motor cortex, cranial nerve nuclei and anterior horn cells
Upper and lower motor neurons can be affected

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2
Q

How is MND distinguished from myasthenia gravis?

A

MND does not affect eye movements

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3
Q

How is MND distinguished from MS and polyneuropathies?

A

In MND there is no sensory loss or sphincter disturbance

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4
Q

Name the 4 clinical patterns of MND

A

ALS/amyotrophic lateral sclerosis
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis

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5
Q

Describe amyotrophic lateral sclerosis

A

Most common type of MND

Loss of motor neurons in the motor cortex and anterior horn of the cord

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6
Q

What signs are present in amyotrophic lateral sclerosis

A

Combined UMN and LMN signs

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7
Q

Describe factors causing a worse prognosis of amyotrophic lateral sclerosis

A

Bulbar onset
Increased age at diagnosis
Decreased FVC

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8
Q

What does progressive bulbar palsy affect?

A

Cranial nerve nuclei 9-12 in the medulla

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9
Q

Where is the lesion in progressive muscular atrophy

A

Anterior horn cell lesion

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10
Q

What signs are present in progressive muscular atrophy

A

LMN signs only

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11
Q

Which muscles are most affected in progressive muscular atrophy

A

Distal muscles

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12
Q

Describe the prognosis of progressive muscular atrophy in comparison to ALS

A

Progressive muscular atrophy has the better prognosis compared to ALS

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13
Q

Describe progressive lateral sclerosis

A

Loss of betz cells in the motor cortex

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14
Q

What signs are present in progressive lateral sclerosis?

A

UMN
Marked spastic leg weakness
Pseudobulbar palsy
No cognitive decline

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15
Q

When should you consider a diagnosis of MND?

A

Patient aged >40 yo (median age of onset = 60yo)
Stumbling, spastic gait, foot drop, proximal myopathy, weak grip and shoulder abduction
Aspiration pneumonia

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16
Q

What signs may be present in someone with MND?

A

UMN - spasticity, brisk reflexes, upgoing plantars

LMN - wasting, fasciculations of tongue, abdomen, back and thigh

17
Q

Which dementia is associated with MND (ALS)?

A

Frontotemporal dementia

18
Q

Which gene is thought to be linked to MND and frontotemporal dementia?

A

C0orf72

19
Q

Which diagnostic criteria is used for ALS?

A

El Escorial

20
Q

What is Brain/cord MRI useful for?

A

Excluding structural pathology

21
Q

What is LP useful for?

A

Excluding inflammatory causes

22
Q

What is neurophysiology useful for?

A

Detecting subclinical denervation and help exclude mimicking motor neuropathies

23
Q

Describe the prognosis of MND

A

Poor - 3 years post onset in half of patients

24
Q

Describe the management of MND

A

MDT approach
Riluzole can help improve survival - inhibitor of glutamate release and NMDA receptor antagonist
Excess saliva - positioning, oral care, suctioning, antimuscarinic (propantheline or Glycopyrronium bromide), botulinum toxin A
Dysphagia - blend food, gastrostomy
Spasticity - exercise, orthotics
Communication - augmentative and alternative communication equipment
End of life care - involve palliative care services from diagnosis
Breathlessness - opioids and NIV

25
Q

What are the signs of bulbar palsy

A

LMN lesion of the tongue and muscles of talking and swallowing
Flaccid, fasciculating tongue
Jaw jerk normal or absent
Speech is quiet, hoarse or nasal

26
Q

What can cause a bulbar palsy?

A

MND, Guillain-Barre, polio, myasthenia gravis, Syringobulbia, brainstem tumours, central pontine myelinolysis

27
Q

Describe corticobulbar palsy

A

UMN lesion of muscles of swallowing and talking due to bilateral lesions above the mid-pons
Commoner than bulbar palsy

28
Q

What are the signs of corticobulbar palsy

A

Slow tongue movements with slow deliberate speech
Increased jaw jerk
Increased pharyngeal and palatal reflexes
Pseudobulbar affect- weeping unprovoked by sorrow or mood incongruent giggling

29
Q

Which conditions also present with emotional incontinence without mood change?

A 
MS
Wilsons
Parkinson's
Dementia
Nitrous oxide use
Head injury

Neurology finals (32 decks)

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