Neuromuscular Disease Supplementary Flashcards
What are 3 causes of presynaptic neuromuscular disorders?
abnormality of calcium, sodium or magnesium
botulism
lambert eaton myasthenic syndrome
What organism is present in soil & can infect through food, wounds or heroin use?
Clostridium botulinum
How does the botulinum toxin disrupt the NMJ and how does it present?
cleaves presynaptic proteins involved in vesicle formation & blocks vesicle docking
presents as rapid onset weakness without sensory loss
What is the treatment for botulism?
Supportive, will improve
Lambert Eaton Myasthenic Syndrome has a strong association with large cell carcinoma. True/false?
False - small cell carcinoma
How does Lambert Eaton Myasthenic Syndrome disrupt the NMJ?
antibodies to presynaptic calcium channels causes less vesicle release
What is the treatment for Lamber Eaton Myasthenic Syndrome?
3-4 diaminopyridine
What is the classic presentation of Myasthenia Gravis?
bilateral facial weakness
extraocular weakness, facial and bulbar weakness
limb weakness proximal
struggling to chew, hold head up
At what point do symptoms tend to start with myasthenia gravis?
When ACh receptors are reduced to about 30%
75% of Myasthenia Gravis patients have hyperplasia or thymoma. True/false?
True
What are the 2 peak ages of incidence for myasthenia gravis?
females in 3rd decase
males in 6th and 7th decades
How is diagnosis for myasthenia gravis made?
clinical: fatiguability doing task
diplopia, ptosis, head drop that comes and goes
Investigations: serum AChR antibodies and single fibre EMG & CT chest
What are the acute treatment options for myasthenia gravis?
acetylcholinesterase inhibitor (pyridosigmine) intravenous immunoglobulin plasma exchange thymectomy
What is the long term treatment for myasthenia gravis?
immunomodulating with steroids, steroid sparing agents (azathiprine/mycophenolate)
What is the drug you MUST avoid in myasthenia gravis?
Gentamicin