Motor Neurone Disease Flashcards
What is motor neurone disease?
Painless, progressive limb weakness
Which neurones does MND affect?
The motor neurones
The sensory neurones are spared
What are the different types of MND?
Amyotrophic lateral sclerosis
Primary lateral sclerosis
Progressive muscular atrophy
Progressive bulbar palsy
Which form of MND is the most common?
Amyotrophic lateral sclerosis
What signs do you get with amyotrophic lateral sclerosis?
LMN signs in arms
UMN signs in legs
What signs do you get with primary lateral sclerosis?
UMN signs only
What signs do you get with progressive muscular atrophy?
LMN signs only
Affects distal muscles before proximal
How does progressive bulbar palsy present?
Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
What are the signs of progressive bulbar palsy due to?
Loss of function of brainstem motor nuclei
Which form of MND has the worst prognosis?
Progressive bulbar palsy
Does MND present symmetrically or asymmetrically?
Asymmetrically
What are the 4 classic signs of MND?
Clumsy
Tripping
Dropping
Slurring
What upper motor neurone signs might present in the legs?
Hypertonia
Hyperreflexia
What lower motor neurone signs might present in the arms?
Hypotonia
Hyporeflexia
How does MND normally first present?
Symptoms in the arms
What are other symptoms of MND?
Fasciculations
Affects the small muscles of the hand
Are the eyes affected in MND?
No
The eyes are normally spared
How is MND initially diagnosed?
Clinically
What can you do to confirm the diagnosis of MND?
EMG
Why might you do an MRI in someone with MND?
To rule out spinal cord compression
What is the management of MND?
Riluzole
What type of drug is riluzole?
Anti-glutaminergic
How does riluzole act?
Slows the progression of the disease
What other management is there for MND?
Non-invasive ventilation at night
BIPAP
What do people with MND normally die from?
Respiratory or bulbar failure
What form of nutritional support is preferred in those presenting with MND?
PEG
What would point towards a diganosis of MND rather than anything else?
asymmetric limb weakness
mixture of LMN and UMN signs
wasting of the small hand muscles/tibialis anterior
fasciculations
the absence of sensory signs
How long does riluzole prolong life?
3 months
How long does non-invasive ventilation prolong life?
7 months
What form of dementia is associated with MND?
Frontotemporal dementia
