Motor Neurone Disease Flashcards
What is motor neurone disease?
Motor neurone disease is a progressive, eventually fatal condition where the motor neurones stop functioning
Does it affect sensory neurones?
No
What is the most common MND?
Amyotrophic lateral sclerosis
What is the second most common?
Progressive bulbar palsy
What does Progressive bulbar palsy affect?
Muscles of talking and swallowing
What is the pathophysiology of MND?
Progressive degenerative diseases of both upper and lower motor neurones
Sensory neurones are spared
Family history is important in around 5-10% of cases.
What can increase risk?
Smoking
Heavy metals
Certain pesticides
What is the presentation?
Late Middle aged man
Possible affected relative
Insidious, progressive weakness of muscles throughout the body, affecting limbs, trunk, face and speech.
Increased fatigue when exercising
Complain of clumsiness, drop things more or tip over
Can develop slurred speech
Where is weakness first noticed>
Upper limbs
What are signs of lower motor neurone disease?
Muscle wasting
Reduced tone
Fasciculations (twitches in the muscles)
Reduced reflexes
What are signs of upper motor neurones disease?
Increased tone or spasticity
Brisk reflexes
Upgoing plantar reflex
How is a diagnosis made?
Based on clinical presentation after excluding other conditions
Should only be made by a specialist
What is the management of MND?
Riluzole
Non-invasive ventilation
Support
What does riluzole do?
Slow the progression of the disease and extend survival by several months in ALS
What can be used in symptom control?
Baclofen for muscle spasticity
Antimuscarinics for excessive saliva
What can help breathlessness worsened by anxiety?
Benzodiazepines
How do people with MND tend to die?
Respiratory failure
Pneumonia
