Neuromuscular Disorders Flashcards
Describe the pathway that occurs over the neuromuscular junction
- AP
- Opening of voltage gated calcium channels
- Calcium influx
- Vesicles of ACh are released
- ACh diffuses across
- Binds to ACh receptors
- Permeable to sodium and potassium
What is the name of the enzyme that breaks down ACh
Acetylcholinesterase
How does curare work?
Occupies the same position as ACh receptor but does not open ion channel - no muscle contraction leads to no respiration
How does novichok work?
Inhibits cholinesterase
Name two presynapic disorders
- Clostridium botulinum
- Lambert Eaton Myasthenic Syndrome
Describe clostridium botulinum
Organism present in soil and food that can infect wounds. Exotoxin inhibits ACh release by cleavage of pre-synaptic proteins involved in vesicle formation and blocks vesicle docking on presynaptic membrane - rapid onset weakness without sensory loss
What can botox be used for medically?
To relax muscles
Describe Lambert Eaton Myasthenic syndrome
Antibodies to presynaptic Ca2+ channels leads to less vesicle release
What is Lambert Eaton associated with?
Small cell carcinoma
State the treatment for Lambert Eaton syndrome?
Pyridostigmine
3.4- diaminopyridine
What is Myasthenia Gravis?
Autoimmune disorder where antibodies to acetyl choline receptors are produced, reducing the number of functioning receptors leading to muscle weakness and fatigue
Describe the pathophysiology of Myasthenia Gravis
- T cells are activated by unfolded AChR subunits
- B cells are stimulated to produce anti-AChR IgG antibodies
- Thymic cells are attacked by these antibodies and release anti-AChR immune complexes
- These activate antigen presenting cells
- Antibodies block the binding of ACh
- Ultimately causing destruction of muscle end plate folds
When do females tend to present with MG?
20s
When do males tend to present with MG?
70/80s
State the clinical features of MG
Weakness typically fluctuating and worse through the day
Most common - extraocular, facial, bulbar and proximal limb weakness