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Sunday, April 20, 2014
Congenital myasthenic syndrome - postsynaptic
is there neuromuscular transmission if you have the postsynaptic kind of congenital myasthenia?
Autoimmune myasthenia gravis is a post-synaptic process, just like Post-synaptic congenital myasthenia gravis. There is neuromuscular transmission; it is just impaired. If there was no transmission, then there would be complete paralysis so we know there has to be some. In some forms of post-synpatic failure, the transmission across the neuromuscular cleft is good but the way the receptor reacts to it is bad (slow channel or endplate AChE deficiency) so the receptor stays open too long and becomes less able to respond normally.
Robert W Neel, IV, MD
Assistant Professor of Neurology
College of Medicine
University of Cincinnati