Neurology - Motor Neurone Disease Flashcards
What is motor neurone disease?
A term that encompasses a variety of specific diseases affecting the motor nerves
Progressive, eventually fatal condition where motor neurones stop working
What type of neurones are affected by motor neurone disease?
Motor neurones
Sensory neurones not affected- no sensory symptoms
What are the different types of MND?
Amyotrophic lateral sclerosis (ALS) (most common)
Progressive bulbar palsy (second most common)
Progressive muscular atrophy
Primary lateral sclerosis
What muscles are primarily affected by progressive bulbar palsy?
The bulbar muscles (muscles of talking and swallowing)
What is the pathophysiology of motor neurone disease?
Progressive degeneration of both upper and lower motor neurones
Sensory neurones not affected
No clear cause
What percentage of motor neurone disease cases are inherited?
5-10%
List some risk factors associated with motor neurone disease
- Family history
- Smoking
- Exposure to heavy metals
- Certain pesticides
What is the typical MND patient?
Late middle-aged man (e.g., 60)
Possibly affected relative
What are common initial symptoms of motor neurone disease?
- Insidious, progressive weakness of muscles
- Increased fatigue when exercising
- Clumsiness and dropping things
- Tripping over
- Slurred speech (dysarthria)
What are signs of lower motor neurone disease?
- Muscle wasting
- Reduced tone
- Fasciculations
- Reduced reflexes
What are signs of upper motor neurone disease?
- Increased tone or spasticity
- Brisk reflexes
- Upgoing plantar reflex
How is the diagnosis of motor neurone disease made?
Based on clinical presentation after excluding other conditions
Specialist diagnosis only where there is certainty
What is a key challenge in diagnosing motor neurone disease?
Diagnosis is often delayed, causing stress.
What medication can slow the progression of ALS?
Riluzole
What is non-invasive ventilation (NIV) used for in motor neurone disease?
To support breathing when respiratory muscles weaken.
