Neuromuscular Disorders

Question 2

Define the term neuromuscular disorders.

Answer 2

Question 3

What is the mean age of onset of neuromuscular disorders?

Answer 3

Question 4

What are the RFS for the development of neuromuscular disorders.

Answer 4

Question 5

Describe the pathophysiology of neuromuscular disorders.

Answer 5

Question 6

List the 4 types of neuromuscular disorders? which is the most common?

Answer 6

1. Amyotrophic Lateral Sclerosis - most common
2. Progressive Bulbar Palsy
3. Progressive Muscular Atrophy
4. Primary Lateral Sclerosis

Question 7

Describe the features of ALS.

Answer 7

Question 8

Describe the features of progressive bulbar palsy.

Answer 8

Question 9

Describe the features of progressive muscular atrophy.

Answer 9

Question 10

Describe the features of primary lateral sclerosis.

Answer 10

Question 11

Symptoms of neuromuscular disorders can be separated into UMN, LMN, pseudobulbar, bulbar and respiratory symptoms.
What are the UMN symptoms?

Answer 11

Question 12

Symptoms of neuromuscular disorders can be separated into UMN, general LMN, pseudobulbar, bulbar and respiratory symptoms.
What are the general LMN symptoms (not including the pseudobulbar/bulbar palsy symptoms)?

Answer 12

Question 13

Symptoms of neuromuscular disorders can be separated into UMN, LMN, pseudobulbar, bulbar and respiratory symptoms.
What are the pseudobulbar symptoms?

Answer 13

Question 14

Symptoms of neuromuscular disorders can be separated into UMN, LMN, pseudobulbar, bulbar and respiratory symptoms.
What are the bulbar symptoms?

Answer 14

Question 15

Symptoms of neuromuscular disorders can be separated into UMN, LMN, pseudobulbar, bulbar and respiratory symptoms.
What are the respiratory symptoms?

Answer 15

Question 16

List the symptoms of neuromuscular disorders.

Answer 16

Question 18

Where does UMN become LMN?

Answer 18

Question 19

What is the pathophysiology behind dysphagia in a UMN vs LMN lesion?

Answer 19

Question 20

What is the pathophysiology behind uncoordinated movements in a UMN vs LMN lesion?

Answer 20

Question 21

What is the pathophysiology behind falls in a UMN vs LMN lesion?

Answer 21

Question 22

Are spasms UMN, LMN or both?

Answer 22

Question 23

You are asked to perform a focused exam on a patient with a neuromuscular disorder. What exams will you do? What would you expect on general inspection?

Answer 23

you would also offer speech and swallow assessment

Question 24

What cranial nerves are mostly affected by neuromuscular disorders?

Answer 24

Question 25

You are asked to perform a focused exam on a patient with a neuromuscular disorder. What would you expect on cranial nerve exam?

Answer 25

Question 26

You are asked to perform a focused exam on a patient with a neuromuscular disorder. What would you expect on motor exam?

Answer 26

weakness - decreased power and coordination. + gait - unsteady/ spastic gait vs flaccid gait

Question 27

You are asked to perform a focused exam on a patient with a neuromuscular disorder. What would you expect on sensory exam?

Answer 27

Question 28

List the ddx for neuromuscular disorders and state the differentiating features.

Answer 28

Question 29

How would you diagnose a neuromuscular disorder?

Answer 29

IT IS A Dx OF EXCLUSION!!

Question 30

List and justify the investigations you would order for a suspected neuromuscular disorder and state your expected findings.

Answer 30

Question 31

The management of a patient with neuromuscular disorders require MDT input. Who would be involved in the MDT and what are their roles?

Answer 31

Question 32

What is the disease - modifying drug used in the treatment of neuromuscular disorders? What is the MOA and the SEs? How is the drug monitored?

Answer 32

Question 33

Outline the pharmacological management of neuromuscular disorders.

Answer 33

Question 34

Outline the adjunctive/ surgical management of neuromuscular disorders.

Answer 34

Question 35

What are the complications of neuromuscular disorders?

Answer 35

Question 36

What is the average lifespan of a patient with neuromuscular disorders from dx? What are 2 good and 1 poor prognostic factor of neuromuscular disorders?

Answer 36