NMJ Disorders

Question 1

Most common neuropathy

Answer 1

Diabetic neuropathy

Question 2

Days to weeks

Answer 2

GBS

Question 3

Months to one year or two

Answer 3

Diabetes

Question 4

Weeks to months

Answer 4

Nutritional deficiencies, toxin and neoplastic

Question 5

Metabolic cause of diabetic neuropathy

Answer 5

Hyperglycemia = Sorbitol + fructose in schwann cells

Question 6

Types of diabetic neuropathy

Answer 6

Polyneuropathy- mononeuropathy

Question 7

Most common type of diabetic neuropathy

Answer 7

Symmetrical Polyneuropathy

Question 8

Management of diabetic Polyneuropathy

Answer 8

• maintain BG levels.
• anti depressants.
• anti-epileptics.
• topical creams.

Question 9

Painful wasting, usually asymmetrical, of the quadriceps muscles and diminished or absent knee reflexes.

Answer 9

Diabetic amyotrophy

Question 10

The most common acute Polyneuropathy

Answer 10

GBS

Question 11

Organisms that are causative of sever GBS

Answer 11

Campylobacter jejuni + CMV

Question 12

Paralysis follows …….. weeks after the infection of GBS.

• 1-2 weeks.
• 1-3 weeks.
• 2-6 weeks.
• 3-9 weeks/

Answer 12

1-3 weeks

Question 13

The diagnosis of GBS is confirmed by ?

Answer 13

Nerve conduction study

Question 14

CSF analysis in a patient with GBS is expected to be?

Answer 14

High in protein + normal glucose levels

Question 15

How to manage a patient with GBS?

Answer 15

Ventilator support +
LMW heparin and compression stocking (to prevent the risk of venous thrombosis) + IV Immunoglobulin in the first 2 weeks ( reduces the severity and duration of paralysis)

Question 16

What is a must before doing an

IV Ig?

Answer 16

Screen for IgA deficiency to prevent any allergic reaction

Question 17

Albuminocutologic dissociation is seen in?

Answer 17

GBS (high proteins in CSF + normal WBC

Question 18

Which of the following is a post synaptic disorder?

• Acquired Myasthenia gravis.
• Lambert eaton myasthenic syndrome.
• Botulism.
• All of the above.

Answer 18

A

Question 19

How does ACh receptors antibodies damage the NMJ?

Answer 19

1- Direct blocking of the receptor.
2- Inducing endocytosis of the receptors on the surface.
3- Induce complement deposits which lead to destruction of the muscle membrane.

Question 20

When does thymectomy is done for MG patients?

Answer 20

Any patient who is seropositive for ACh receptors antibodies thymectomy must be done.

Question 21

The most common first sign of MG?

Answer 21

Diplopia + Ptosis

Question 22

Which one is more sensitive for pure ocular patients with MG?

• AChR Ab.
• Electrodiagnostic testing.

Answer 22

B

Question 23

The most sensitive test for MG?

• AChR Ab.
• Electrodiagnostic tests.
• Single fiber EMG.

Answer 23

C (but it’s not specific)

Question 24

Most common treatment for MG

Answer 24

Pyridostigmine (if prohibited use neostigmine)

Question 25

Symmetric proximal weakness developing over weeks to months associated with myalgia

Answer 25

Polymyositis