3: MND Flashcards
What is motor neuron disease
Neurodegenerative condition with disruption UMN and LMN
What is specific about MND
UMN and LMN signs
What. % of MND is genetic
5%
What mutation is associated with genetic MND
Superoxide dismutase (SOD)
What are the 4 types of MND
- ALS
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
What is the most common type of MND
ALS
How does ALS present
UMN and LMN loss.
Usually LMN in arms and UMN in legs
If familial what mutation is present in ALS
SOD on chromosome 21
What cranial nerves are most commonly impacted in progressive bulbar palsy
9-12
How does progressive muscular atrophy present
LMN signs in distal muscles before proximal
What muscles are affected first in progressive muscular atrophy
Distal prior to proximal
What is primary lateral sclerosis
Loss of betz cells in primary motor cortex causing UMN signs only
When is MND suspected
Over 40 with:
- Fasciculation
- UMN and LMN signs
- Hypo-reflexia
- Proximal myopathy
- Spastic gait
- Weak grip
- Wasting small muscles hand and tibialis anterior
What are never events for MND
- NO sensory signs
- NO cerebellar signs
- NO extra-ocular muscle involvement
How is MND distinguished from myasthenia gravis
No extra-ocular muscles
