3(E): Peripheral Polyneuropathies

Question 1

What is a peripheral neuropathy

Answer 1

Damage to multiple peripheral nerves

Question 2

Give 3 features of neuropathies (compared to myopathies)

Answer 2

• Distal muscles affected first
• Early muscle waisting
• Diminished or absent reflexes

Question 3

What genetic condition can cause poly neuropathies

Answer 3

Charcot marie tooth

Question 4

What are acquired causes of polyneuropathy

Answer 4

Inflammatory: Gullian Barre, Chronic Demyelinating Polyneuropathy
Toxin: Alcohol, Lead

Question 5

What toxins can cause polyneuropathy

Answer 5

Alcohol, Lead

Question 6

What causes alcoholic neuropathy

Answer 6

Direct toxin effect of alcohol

Vitamin B12 deficiency

Question 7

How does alcoholic neuropathy present

Answer 7

Loss sensation prior to motor symptoms

Question 8

What does vitamin B12 deficiency cause

Answer 8

Subacute combined degeneration spinal cord

Question 9

What tract is impacted first in subacute combined degeneration spinal cord

Answer 9

DCP

Question 10

How does diabetic neuropathy present

Answer 10

Loss of sensation in glove and stocking distribution. Burning in the feet worse at night

Question 11

What is the inheritance pattern of Charcot Marie Tooth

Answer 11

Autosomal dominant

Question 12

What is predominantly lost in Charcot Marie Tooth

Answer 12

Motor symptoms

Question 13

What are 3 distinct features of CMT

Answer 13

Pes cavus = high arch foot
Hammer toes
Stork legs = atrophy calf muscles

Question 14

What is a common presenting story for CMT

Answer 14

Recurrent sprained ankles

Question 15

What is ordered for CMT

Answer 15

NCS

Nerve Biopsy

Question 16

What is seen on NCS in CMT

Answer 16

reduced conduction velocity

Question 17

What is seen on nerve biopsy in CMT

Answer 17

myelination and then demyelination = onion peel appearance

Question 18

What is Gullian Barre Syndrome

Answer 18

Immune-mediated demyelinating peripheral polyneuropathy

Question 19

What do patients experience before neuronal symptoms in GBS

Answer 19

URTI or GI Infection 1-4W prior

Question 20

What pathogens cause GBS

Answer 20

• Campylobacter Jejuni
• CMV
• EBV

Question 21

Explain symptoms of Gillian barre

Answer 21

Progressive weakness in all four limbs to cause paralysis. It is an ascending paralysis - meaning it starts in the legs and progresses to the arms. Proximal muscles are affected first.

Areflexia
Autonomic Dysfunction - arrhythmia, gastroparesis and voting dysfunction

Question 22

Describe reflexes in Gullian Barre

Answer 22

Areflexia

Question 23

What autonomic dysfunction may occur in gullian barre

Answer 23

• Arrhythmia
• Gastroparesis
• Voiding dysfunction

Question 24

What is a late sign of Guillian barre

Answer 24

Sensory Loss

Landry Paralysis

Question 25

What is Landry paralysis

Answer 25

Involvement of respiratory muscles - medical emergency

Question 26

What is a variant of GBS

Answer 26

Miller-Fisher

Question 27

What is Miller-Fisher Syndrome

Answer 27

Ophthalmoplegia and ataxia

Question 28

What muscles are affected first in Miller-Fisher

Answer 28

Extra-occular muscles

Question 29

Describe muscle involvement in Miller-Fisher

Answer 29

In contrast to GBS, it is a descending paralysis. Affects arms before legs. Usually starts with eye muscles

Question 30

What antibodies are present in Miller-Fisher

Answer 30

anti-GQ1b antibodies

Question 31

What investigations are ordered in Gillian barre

Answer 31

- NCS - EMG - Vital Capacity monitoring (anti-GQ1b - for miller fisher)

Question 32

What phenomenon is seen on CSF in Gullian Barre

Answer 32

Albumincytologic dissociation

Question 33

What is albuminocytologic dissociation in Gullian Barre

Answer 33

- Raised albumin | - Normal WCC

Question 34

Explain conduction velocity in GBS

Answer 34

Decreased

Question 35

What needs to be monitored in GBS

Answer 35

Vital Capacity

Question 36

How is GBS managed

Answer 36

IVIg

Question 37

What is ineffective in GBS

Answer 37

Glucocorticoids

Question 38

What is complication of GBS

Answer 38

Respiratory Failure