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Inherited bilateral vocal cord paresis is a rare occurrence but can be a feature of certain genetic neuropathies, including those associated with TRPV4, DCTN1, SLC5A7 and GDAP1 gene mutations.We describe here a novel JAG1 c.1732T>G p.(Cys578Gly) variant in a 32-year-old man with vocal cord paresis requiring tracheostomy, facial myokymia, scoliosis and loss of ankle tendon reflexes. Symptom onset was as a teenager. The patient‘s father and paternal grandfather had similar but more mild features. Other acquired and inherited causes were ruled out. JAG1 encodes for components of the Notch signaling pathway, dysregulation of which is typically implicated in Alagille Syndrome. However, there has been one previous report of severe vocal cord paresis and hereditary axonal neuropathy associated with JAG1 mutations in two unrelated families, suggesting an alternative disease mechanism underlying the disorders. This novel Cys578Gly variant in JAG1 is absent from the gnomAD population database and in silico predictive tools support a deleterious effect on the gene.This report supports the suggestion that JAG1 has a key role in peripheral nerve function and broadens the differential of familial bilateral vocal cord paresis.Consent to publication was obtained from the patient.murva30593@hotmail.com