Motor neurone disease

Question 1

What is MND?

Answer 1

Rare condition that progressively damages parts of the nervous system. This leads to muscle weakness, often with visible wasting

Question 2

What are the 4 types of MND?

Answer 2

1) Amyotrophic lateral sclerosis
2) Primary lateral sclerosis
3) Progressive muscular atrophy
4) Progressive bulbar palsy

Question 3

What are the upper motor neuron signs?

Answer 3

weakness, spasticity (stiffen/tighten), hyperreflexia

Question 4

What are the lower motor neuron signs?

Answer 4

severe weakness, fasciculations, muscle cramps and hypotonicity, hyporeflexia

Question 5

Describe the signs of amyotrophic lateral sclerosis?

Answer 5

LMN signs in arms and UMN signs in legs

Split hand syndrome in ALS – wasting of thenar group muscles

Question 6

What are the signs of Primary lateral sclerosis?

Answer 6

UMN signs only

Question 7

What are the signs of Progressive muscular atrophy?

Answer 7

LMN signs only; weakness, fasciculations, cramps, hypotonicity, hyporeflexia,

affects distal muscles before proximal

Question 8

What are the signs of Progressive bulbar palsy

Answer 8

Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei

Question 9

Which one of the 4 MND variants carries the worst prognosis?

Answer 9

Progressive bulbar palsy

Question 10

Which one of the 4 MND variants carries the worst prognosis?

Answer 10

Progressive bulbar palsy

Question 11

What is Riluzole in MND?

Answer 11

Prevents stimulation of glutamate receptors - prolongs life by 3 months

Question 12

What other therapy apart from Riluzole is used in MND?

Answer 12

BIPAP, Speech therapy. dieticians, gastrostomy, palliative care

Question 13

What symptomatic drugs are used for muscle cramps in MND?

Answer 13

Quinine, Baclofen

Question 14

What symptomatic drugs are used for muscle SPASMS in MND?

Answer 14

Baclofen, Tizanidine, Dantrolene, Gabapentin

Question 15

What symptomatic drugs are used for shortness of breath or anxiety in MND?

Answer 15

Lorazepam

Question 16

What is the mechanism of action of baclofen

Answer 16

Baclofen is a GABA agonist, and its primary site of action is the spinal cord, where it reduces the release of excitatory neurotransmitters and substance P by binding to the GABA-B receptor.