MND Flashcards
1
Q
What is it?
A
A progressive, ultimately fatal disease where motor neurones stop functioninng. Sensory neurons are not involved.
2
Q
Subtypes?
A
Amyotrophic Lateral Sclerosis (ALS)- most common type
Progressive Bulbar Palsy- second most common type. This primarily affects speech and swallowing muscles
Primary lateral sclerosis
Progressive muscular atrophy
3
Q
Pathophysiology?
A
degeneration of both upper and lower motor neurones
4
Q
Aetiology /Risk factors?
A
- Genetic component - 5-10% of cases found to be inherited
- increased risk w/ smoking, expsoure to heavy metals and certain pesticides
5
Q
What is the main feature of MND?
A
progressive muscle weakness throughout whole body
- trunk
- limbs
- face
- speech
6
Q
How does it present initially?
A
- muscle weakness noticed first in upper limbs
- pts notice increased fatigue when exercising, clumsiness, tripping, dropping things more often and slurred speech
7
Q
Signs?
A
Upper and lower motor neuron signs
- UMN
- hyperreflexia (brisk reflexes)
- spasticity
- Babinski sign
- LMN
- reduced reflexes
- reduced tone
- muscle wasting
- fasciculations
8
Q
what occurs rarely in MND, and later on
A
- involvement of ocular muscles
- smooth muscle and cardiac involvement doesnt occur
- autonomic dysfunction occurs later on
9
Q
Mx?
A
ALS–> Riluzole (glutamate antagonist)
- can slow the progression of the disease and increase life expectancy by a couple months
NIV to support breathing at night
MDT approach
