MND

Question 1

What is MND?

Answer 1

A group of conditions with progressive degeneration of the upper and lower motor neurones in the brain and spinal cord

Question 2

4 types of MND?

Answer 2

ALS (50%)
Bulbar palsy (second most common)
PLS
PMA

Question 3

Pathophysiology (where are lesions most commonly found?)

Answer 3

mostly affects anterior horn cells of the spinal cord and the motor cranial nerve nuclei

Question 3

Difference between MND and MS?

Answer 3

MNS has no sensory loss whereas MS has both motor and sensory

Question 4

Aetiology?

Answer 4

Unknown

Genetics, smoking, heavy metals, pesticides

Question 5

Typical presentation

Answer 5

60 yo man
Insidious, progressive weakness of muscles in trunk, limbs, face and speech/swallowing
Arms or legs typically first affected
Wrist/foot drop
Inability to exercise
Muscle wasting
Clumsy

Question 6

LMN signs?

Answer 6

Muscle wasting
Fasciculations
Hyporeflexic
Hypotonic

Question 7

UMN signs?

Answer 7

Hypertonic
Hyperreflexic
+ babinski
Spastic paralysis

Question 8

How is it diagnosed?

Answer 8

Clinical presentation
Nerve conduction studies
EMG (electromyography)

Question 9

How is it managed?

Answer 9

No cure
Riluzole- inhibits glutamate release–> slows progression + few months

Question 10

Prognosis?

Answer 10

2-4 years to live
Patients usually die of resp failure/pneumonia