2 Neuromascular Junction (Botulism, Myasthenia , Lambert eaton)

Question 1

What is Botulism associated with?

Answer 1

IV drug abuse, Pure motor syndrome (no sensory)

Botulism is characterized by specific clinical features related to motor dysfunction.

Question 2

What are the acute symptoms of Botulism?

Answer 2

Acute myasthenia, ptosis, dysphagia, dysarthria

These symptoms reflect muscle weakness and difficulty in swallowing and speaking.

Question 3

What eye-related signs are associated with Botulism?

Answer 3

Ptosis, restricted eye movements, dilated with no reaction pupils, 7th palsy

These signs indicate cranial nerve involvement and autonomic dysfunction.

Question 4

reflexes in Botulism?

Answer 4

Decreased DTR (Deep Tendon Reflexes)

This indicates a disorder affecting the motor nerves.

Question 5

autonom in Botulism?

Answer 5

Dry mouth, constipation, urinary retention

These signs indicate autonomic nervous system involvement.

Question 6

mechanism of Botulism?

Answer 6

Blocks acetylcholine release/pre-synaptic disorder

This mechanism leads to the characteristic symptoms of muscle weakness.

Question 7

EMG in Botulism?

Answer 7

Increment on repetitive nerve stimulation

This finding is indicative of a presynaptic neuromuscular junction disorder.

Question 8

diagnostic tool Botulism?

Answer 8

Toxin detection in serum/stool/vomit

Identifying the toxin is crucial for confirming the diagnosis.

Question 9

What are the treatments for Botulism?

Answer 9

Antitoxin, Metronidazole/Penicillin (wound)

Treatment aims to neutralize the toxin and manage infection.

Question 10

What test can be positive in Botulism but is not specific to myasthenia?

Answer 10

Tensilon test

This test can show improvement in muscle strength but is used more for myasthenia gravis.

Question 11

characteristic of Tetanus?

Answer 11

Trismus

Tetanus is characterized by muscle stiffness, particularly of the jaw.

Question 12

What treatment options are available for Tetanus?

Answer 12

Antitoxin, metronidazole, penicillin

Similar to botulism treatment.

Question 13

List common symptoms of Myasthenia gravis.

Answer 13

• Diplopia (unilateral)
• Ptosis
• Slurred/nasal speech
  *Respiratory
• Fatigue on repetitive use
• Worse in the evenings
  *Waxing and waning
• Ocular, bulbar, respiratory muscle involvement

Symptoms can be unilateral and vary in severity.

Question 14

Thymus?

Answer 14

60% Hyperplasia, 15% thymoma ( Heterogen, anti SM), Hypothyroid

Question 15

What antibodies are associated with Myasthenia gravis?

Answer 15

• Nicotin acetylcholine receptor
• Anti-MUSK
• LRP4

These antibodies contribute to the autoimmune process.

Question 16

EMG in Myasthenia?

Answer 16

Single fiber EMG is the most sensitive test, Decrease on repetitive stimulation, decremental response

Question 17

diagnostic test for Myasthenia gravis?

Answer 17

Cold compress (Edrophonium (Tensilon) outdated)

Question 18

Treatment Myasthenia

Answer 18

Pyridostigmine max 360-> Prednisolone oral in hospital! (paradoxical reaction in 50% ) , azathioprine, ciclosporine, rituximab

Question 19

What are the treatment options during a Myasthenic crisis?

Answer 19

• IVIG
• Plasmapheresis

These treatments are used for severe symptoms like dysphagia and dyspnea.

Question 20

Which medications are forbidden for patients with Myasthenia gravis?

Answer 20

• Mycin
• Floxacin
• Tetracycline
• Gentamicin
• Ampicillin
• Beta-blockers
• Calcium channel blockers
• Lithium
• Quinidine
• Penicillamine
• Phenytoin
• Magnesium
• Procainamide
• Botox
• Pethidine
• Statin

These drugs can exacerbate symptoms.

Question 21

Which medications are allowed for patients with Myasthenia gravis?

Answer 21

• Co-trimoxazole
• Cefalexin

These antibiotics are considered safe.

Question 22

What are the indications for thymectomy in Myasthenia gravis?

Answer 22

• Generalized myasthenia
• Anti-acetylcholine receptor positive
• Under 45 years old

Thymectomy is not indicated for

Question 23

Thymectomy contraindicated?

Answer 23

anti-MUSK positive , pure ocular cases.

Question 24

What triggers relapse in Myasthenia gravis?

Answer 24

Pregnancy

It is important to check B HCG during pregnancy.

Question 25

What is the normal lung function measurement for Myasthenia gravis?

Answer 25

5 cm chest expansion, can count to 20 in one breath ## Footnote These measurements help assess respiratory function.

Question 26

What drug is known to induce Myasthenia gravis?

Answer 26

Penicillamine ## Footnote This medication can trigger or worsen symptoms.

Question 27

What is the age range for patients who can develop Myasthenia gravis?

Answer 27

Young women, old men, even up to 70 years old ## Footnote The condition can affect a wide demographic.

Question 28

Lambert Eaton myasthenic syndrome associated with?

Answer 28

Small cell lung cancer, 50% Paraneoplastic syndrome ## Footnote Paraneoplastic syndromes are a group of disorders caused by the immune response to cancer.

Question 29

type of muscle weakness Lambert Eaton myasthenic syndrome?

Answer 29

Proximal weakness worse in the morning, (opposite of myasthenia), increased muscle strength with reinforcement or exercise, waddling gait ## Footnote This is opposite to the pattern seen in myasthenia gravis.

Question 30

Gait in Lambert eaton?

Answer 30

Waddling gait

Question 31

autonomic Lambert Eaton myasthenic syndrome?

Answer 31

Dry mouth, impotence ## Footnote These symptoms result from autonomic nervous system involvement.

Question 32

reflex changes Lambert Eaton myasthenic syndrome?

Answer 32

Reduced reflexes or areflexia ## Footnote Areflexia indicates a severe loss of reflexes.

Question 33

antibodies in Lambert Eaton myasthenic syndrome?

Answer 33

Anti VGCC, Calcium voltage channels ## Footnote These channels are crucial for the release of acetylcholine at the neuromuscular junction.

Question 34

What is the mechanism of action of anti-VGCC antibodies?

Answer 34

Inhibition of acetylcholine release ## Footnote This leads to the characteristic weakness seen in the syndrome.

Question 35

What are the treatments available for Lambert Eaton myasthenic syndrome?

Answer 35

* Diaminopyridine (Potassium channel blocker) * Amifampridine * Pyridostigmine * Corticosteroids (corton) * IVIG ## Footnote These treatments may help improve muscle strength and manage symptoms.