Congenital slow channel syndrome (Q92750): Difference between revisions

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Latest revision as of 07:52, 16 August 2026

In slow channel syndrome, a congenital myasthenic syndrome subtype with a postsynaptic defect, there is selectively severe involvement of cervical and wrist and finger extensor muscles in most cases. Cranial muscles only mildly affected; slowly progressive course. Worsened by long-term pyridostigmine therapy; little or no response to edrophonium. Dominant inheritance in nearly all cases. Repetitive nerve stimulation shows decrement and repetitive compound muscle action potentials.

Language	Label	Description	Also known as
default for all languages	ID_339968706
English	Congenital slow channel syndrome	In slow channel syndrome, a congenital myasthenic syndrome subtype with a postsynaptic defect, there is selectively severe involvement of cervical and wrist and finger extensor muscles in most cases. Cranial muscles only mildly affected; slowly progressive course. Worsened by long-term pyridostigmine therapy; little or no response to edrophonium. Dominant inheritance in nearly all cases. Repetitive nerve stimulation shows decrement and repetitive compound muscle action potentials.