Motor Neurone Disease Flashcards
What is MND.
Degeneration of the upper and lower motor neurons, of unknown cause.
What percentage of cases of MND follow an autosomal dominant pattern.
5-10%.
What percentage of cases of MND are sporadic.
90-95%.
Who is most affected by MND. (2)
Men are more often affected than women.
40-60 years.
What are the four most common presentations of MND. (4)
Spinal muscular atrophy.
Primary lateral sclerosis.
Progressive bulbar palsy.
Amyotrophic lateral sclerosis (ALS).
What is the most common presentation of spinal muscular atrophy.
Limb weakness due to the involvement of the spinal cord anterior horn cells.
What is the most common presentation of primary lateral sclerosis.
Spastic limb weakness due to upper motor neurone involvement of the spinal cord.
What is the most common presentation of progressive bulbar palsy. (2)
Involvement of bulbar motor neurons.
It is a progressive disease.
What is the most common presentation of ALS. (3)
A mixture of:
Limb weakness due to the involvement of the spinal cord anterior horn cells.
Spastic limb weakness due to upper motor neurone involvement of the spinal cord.
Involvement of bulbar motor neurons.
What is not involved in MND. (2)
Cardiac muscle.
Smooth muscle.
What is very rarely involved in MND.
Ocular muscles.
What is emotional lability associated with in terms of MND.
Pseudobulbar palsy.
When does autonomic dysfunction occur in MND.
Late in the disease.
What is death due to MND usually due to. (2)
Cardiac arrhythmias.
Respiratory failure.
In what time period is MND usually fatal.
3-5 years.
