Motor neurone disease

Question 1

What is MND

Answer 1

Progressive loss of neurones in the motor cortex, CN nuclei and anterior horn cells with UMN and LMN Mix

Question 2

What are UMN Signs

Answer 2

Everything goes up
- Hypertonia
- Spasticity
- Clonus
- Hyper-reflexia
No fasciluations
Positive babinski

Question 3

What are LMN Signs

Answer 3

Everything goes down
- Hypotonia
- Hyporeflexia
Fasciculations
Negative babinski

Question 4

What type of lesion is hypertonia

Answer 4

UMN

Question 5

What type of lesion is Hypotonia

Answer 5

LMN

Question 6

How is MND Managed

Answer 6

Riluzole (slow progression)
Non invasive ventilation
MDT/Physio
End of life care

Question 7

What is ALS MND

Answer 7

Loss of neurones in motor cortex and anterior horn

Question 8

What signs does ALS present with

Answer 8

UMN AND LMN
Babinski reflex
Assymetrical
BULBAR PALSY

Question 9

What gene associates with ALS

Answer 9

SOD1 gene

Question 10

What is progressive bulbar palsy

Answer 10

Loss of neurones in CN 9-12
Increased chance of resp fail

Question 11

What signs does Bulbar palsy (CN9-12) present with

Answer 11

UMN AND LMN
Dysphagia and chewing problem
Flaccid tongue
Hoarse voice
No Jaw jerk

Question 12

How is movement organised

Answer 12

Idea of moving (Pre motor cortex)
UMN Activation (Motor Cortex)
Impulse (Corticospinal tract)
Modulation (Cerebellum to basal ganglia)
Movement (Sensory tracts)

Question 13

What are the RF of MND

Answer 13

Male
Sod1 Mutation
Age

Question 14

When should we consider a patient has MND

Answer 14

When they have a mix of both UMN and LMS signs

Myasthaenia Gravis

Question 15

UMN lesions typically are found where

Answer 15

Precentral cortex to anterior spinal cord

Question 16

LMN lesions typically are found where

Answer 16

Anterior spinal cord to muscles

Question 17

Describe the main motor tract (Corticsospinal)

Answer 17

Upper motor neurone travels from precentral gyrus and decussates (90%)

Question 18

A patient suspected of MND has eye muscle disorders, what should be considered instead

Answer 18

Multiple scleorsis
Myaesthenia Gravis

Question 19

A patient suspected of MND has sensory functional signs, what should be considered instead

Answer 19

Myaesthenia Gravise
Polyneuropathy

Question 20

In what lesion is their negative babinski but positive fasciluations

Answer 20

LMN lesion

Question 21

What are possible complications of MND

Answer 21

Resp fail
Aspirational pneumonia
Dysphagia

Question 22

Progressive Muscular atrophy affects what neuerones

Answer 22

LMN Only

Question 23

Primary lateral sclerosis affects what neurones

Answer 23

UMN Only

Question 24

In the modulation of impulse, what controls fine tuning and what gives the sign to GO

Answer 24

Fine tuning by cerebellum
GO BY GANGLIA