Motor neurone disease Flashcards
What is MND
Progressive loss of neurones in the motor cortex, CN nuclei and anterior horn cells with UMN and LMN Mix
What are UMN Signs
Everything goes up
- Hypertonia
- Spasticity
- Clonus
- Hyper-reflexia
No fasciluations
Positive babinski
What are LMN Signs
Everything goes down
- Hypotonia
- Hyporeflexia
Fasciculations
Negative babinski
What type of lesion is hypertonia
UMN
What type of lesion is Hypotonia
LMN
How is MND Managed
Riluzole (slow progression)
Non invasive ventilation
MDT/Physio
End of life care
What is ALS MND
Loss of neurones in motor cortex and anterior horn
What signs does ALS present with
UMN AND LMN
Babinski reflex
Assymetrical
BULBAR PALSY
What gene associates with ALS
SOD1 gene
What is progressive bulbar palsy
Loss of neurones in CN 9-12
Increased chance of resp fail
What signs does Bulbar palsy (CN9-12) present with
UMN AND LMN Dysphagia and chewing problem Flaccid tongue Hoarse voice No Jaw jerk
How is movement organised
Idea of moving (Pre motor cortex) UMN Activation (Motor Cortex) Impulse (Corticospinal tract) Modulation (Cerebellum to basal ganglia) Movement (Sensory tracts)
What are the RF of MND
Male
Sod1 Mutation
Age
When should we consider a patient has MND
When they have a mix of both UMN and LMS signs
Myasthaenia Gravis
UMN lesions typically are found where
Precentral cortex to anterior spinal cord
LMN lesions typically are found where
Anterior spinal cord to muscles
Describe the main motor tract (Corticsospinal)
Upper motor neurone travels from precentral gyrus and decussates (90%)
A patient suspected of MND has eye muscle disorders, what should be considered instead
Multiple scleorsis
Myaesthenia Gravis
A patient suspected of MND has sensory functional signs, what should be considered instead
Myaesthenia Gravise
Polyneuropathy
In what lesion is their negative babinski but positive fasciluations
LMN lesion
What are possible complications of MND
Resp fail
Aspirational pneumonia
Dysphagia
Progressive Muscular atrophy affects what neuerones
LMN Only
Primary lateral sclerosis affects what neurones
UMN Only
In the modulation of impulse, what controls fine tuning and what gives the sign to GO
Fine tuning by cerebellum
GO BY GANGLIA