Neurology Exam Prep Podcast Episode 6 - Neuromuscular Disorders Part II
Jan 23, 2020
Dr. Jeffrey Dewey, a neurologist with expertise in neuromuscular disorders, returns to discuss a range of fascinating topics. He dives into myasthenia gravis, highlighting antibody mechanisms and clinical symptoms such as fatigable weakness. The conversation covers diagnostic techniques like the ice pack test and EMG. Furthermore, he sheds light on congenital myasthenic syndromes, inflammatory myopathies, and conditions like muscular dystrophies. Dewey also examines glycogenosis and lipid metabolism disorders with engaging anecdotes for better understanding.
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Myasthenia Gravis Basics
- Myasthenia gravis is the prototypical antibody-mediated disorder affecting postsynaptic neuromuscular transmission.
- It mainly involves antibodies against acetylcholine receptors causing fatigable, fluctuating weakness typically worse in evenings.
Use Bedside Tests for Diagnosis
- Use bedside tests like sustained gaze and ice pack test to detect fatigable weakness and ptosis in myasthenia gravis.
- These tests transiently improve ptosis by affecting acetylcholine breakdown or uptake.
Repetitive Nerve Stimulation Testing
- Perform low frequency (2-5 Hz) repetitive nerve stimulation to detect decrements in muscle response amplitude in neuromuscular junction disorders.
- A decrement >10% in motor potential amplitude supports diagnosis.