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Histo & Neuro Part 2 > Disorders of Neuromuscular Transmission > Flashcards

Disorders of Neuromuscular Transmission Flashcards

1
Q

Acquired Neuromuscular Disorders:

A

– Myasthenia Gravis
– Lambert-Eaton Myasthenic Syndrome
– Botulism
– Organophosphate Poisoning

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2
Q

Heritable Neuromuscular Transmission:

A

– Congenital Myasthenic Syndromes

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3
Q

What is needed for NMJ release?

A

Calcium

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4
Q

First steps in NMT:

A
  1. Action potential arrives at motor terminal
  2. Voltage-gate Ca++ channels open-
    Ca++enters
  3. Ach vesicles migrate and fuse
  4. Ach is released into the synaptic cleft
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5
Q

Last steps in NMT:

A
  1. Ach binds to Ach Receptor and Na+ enters
  2. End-Plate Potential is generated
  3. Sarcolemma depolarizes at EPP threshold
  4. Ach is degraded by Acetylcholinesterase
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6
Q

Pre-synaptic disorders:

A

– Eaton-Lambert Syndrome
– Botulism
– Congenital Deficiency Ach synthesis

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7
Q

Synaptic disorder:

A

Organophosphate poisoning

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8
Q

Post synaptic disorders:

A

– Myasthenia Gravis
– Congenital
* Abnormal AChR

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9
Q

Amount of Ach of each synaptic vesicle

A

Quantum

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10
Q

Total number of quanta release/ action potential

A

Quantal content

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11
Q

Autoimmune disease of the
neuromuscular junction

A

Myasthenia gravis

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12
Q

Can be associated with other autoimmune disorders and neoplasms (Thymoma)

A

Myasthenia gravis

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13
Q

Pathophysiology of Myasthenia gravis:

A
  1. Antibodies directed against the acetylcholine receptor or other closely- associated proteins
  2. Cross-linking or blocking of receptor
  3. Complement-mediated destruction of end- plate
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14
Q

Characterized by: droopy eyes (ptosis), double vision, nasal voice in afternoons, diffuse weakness, resiratory muscle involvement

A

Myasthenia gravis

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15
Q

What is the simpson test used for?

A

Ptosis - myasthenia gravis

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16
Q

Tests used for MG:

A

1.Tensilon test
2.Anti Ach R serum antibody
3.Anti Muscle Kinase
4.Anti thymoma
5.EMG

17
Q

What is Tensilon test?

A

Short-acting Acetylcholinestherase inhibitor (increases Acetylcholine)

18
Q

Which NMJ disease causes: Autoantibodies to pre-synaptic voltage gated calcium channels (motor nerve terminal)
Impairment of quantal release of Ach

A

Lambert Eaton Myasthenic Syndrome (LEMS)

19
Q

Characterized by:
- Weakness in proximal> distal muscles
- Proximal legs > arms
- Improved strength with brief-exercise
- Diminished or absent deep tendon reflexes
- Autonomic symptoms: dry mouth, blurred vision, impotence

A

LEMS

20
Q

Pre-synaptic block of acetylcholine release through cleavage of excitosis proteins (SNARE)

A

Botulism

21
Q

Three major presentations of botulism:

A

– Classic (Food-Borne)
– Infant Botulism
– Wound Botulism (IV drug injection)

22
Q

Characterized by:
18-38 hr incubation, diffuse weakness starting from eyes to limbs, reduced tendon reflex, dilated pupils, blured vision, GI problems

A

Botulism

23
Q

Dozen D’s

A

Botulism

24
Q

Accidental Poisoning with Insecticides and Nerve Gas (Sarin, Soman, Tabun)

A

Organophosphate toxicity

25
Q

Excess Acetylcholine Diseases = Constant Depolarization

A

Organophosphate toxicity

Inihibition of Endogenous Acetylcholinesterase

26
Q

SLUDGE

A

salivation
lacrimation
urination
diarrhea
gi
emesis

27
Q

DUMBELS

A

diarrhea
urination
miosis
bronchospasm
emesis
lacrimation
salivation

28
Q

Heterogeneous genetic disorders of neuromuscular transmission

A

Congenital Myasthenic Syndromes

29
Q

Most common- mutations in post-synaptic and synaptic proteins
*Onset in childhood

A

Congenital Myasthenic Syndromes

30
Q

Can be associated with neoplasms:

A

Thymoma
(MG) or Small Cell Lung CA (LEMS)

31
Q

Can be associated with toxins:

A

Botulism and Organophosphates

Histo & Neuro Part 2 (13 decks)

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