Motor neurone disease Flashcards
What is it
Variety of specific diseases affecting the motor nerves
what happens to sensory neurones?
nothing, sensory symptoms suggest an alternative diagnosis
What is the most common type
Amyotrophic lateral sclerosis
What is the second most common type?
Progressive bulbar palsy
What are two other types of MND to be aware of?
Progressive muscular atrophy
Primary lateral sclerosis
explain pathophysiology
progressive degeneration of both the upper and lower motor neurones.
causes
exact cause is unclear
genetic link
5-10% cases inherited
risk factors
smoking
exposure to heavy metals
pesticides
Presentation?
Late middle aged
Progressive weakness of muscles throughout the body
Affects limbs, trunk, face and speech
Weakness often noticed first in upper limbs
Clumsiness
Slurred speech
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 diagnosis made?
Based on clinical presentation and excluding other conditions
What is treatment of ALS?
Riluzole
Can slow progression of disease and extend survival by a few months
What does management involve ?
MDT
Sx control - baclofen for muscle spasticity, antimuscarinics for excess saliva
Benzos for breathlessness worsened by anxiety
Advanced directives
NIV to support breathing
How do most MND patients die?
Respiratory failure
Pneumonia
