Motor neurone disease Flashcards
Steven Hawkin had what disease?
Motor neuron disease
He’s a bad example because he lived a long time but good for symptoms etc
4 types of motor neuron disease
1 - ALS amyotrophic lateral sclerosis
2- primary lateral sclerosis
3 - progressive muscular atrophy
4 - progressive bulbar palsy
Amyotrophic lateral sclerosis
Upper or lower motor neurons involved?
Amyotrophic - muscle wasting (lower motor neuron component)
lateral sclerosis - lateral corticospinal tract damage (upper motor neuron component)
Primary lateral sclerosis
Upper or lower motor neurons involved?
Upper motor component only
mainly causes weakness in the leg muscles. Some patients with this type may also develop clumsiness in the hands or develop speech problems.
Progressive muscular atrophy
Upper or lower motor neurons involved?
Lower motor component only
The small muscles of the hands and feet are usually first affected, but muscle spasticity is absent.
Progressive bulbar palsy - which nerves affected and what do they do?
CN 9, 10, 12 - speak, swallow and chew
then respiratory muscles get involved too
Diagnostic factors for MND
Asymmetric distal weakness
Brisk reflexes in wasted limb
No sensory symptoms
Relentless progression
Three things which aren’t affected in MND
Sensation
Bowels/bladder
Eyes
MND and respiration?
Type 2 respiratory failure can occur
CPAP (continuous positive airway pressure) or BiPAP (CPAP plus extra pressure on inspiration) - may be needed first at night then more often
MND other signs etc
fasciculations
foot drop and other distal muscle problems
weight loss - metabolism increases by 20%
MND management
feeding (PEG etc)
communication aids
treating aspiration etc (typically in right mid zone pneumonia)
Riluzole (adds 3 months life expectancy)
