Emery-Dreifuss myopathy can be associated with a cardiomyopathy and cardiac 
dysrhythmias. The inheritance pattern of Emery-Dreifuss muscular dystrophy 
(EDMD) is X linked, whereas EDMD2 is autosomal dominant. EDMD2 is caused by 
lamin A/C gene (LMNA) mutations that produce alterations in the lamin proteins 
that are integral to nuclear and cell integrity. A 53-year-old man was brought 
to us with a right internal carotid artery dissection. Detailed work-up of the 
patient and family members revealed some unusual features, and genetic 
sequencing of the LMNA gene was undertaken. A novel mutation was identified in 
two of the samples sent for analysis. We present the first Indian family of 
EDMD2 with familial dilated cardiomyopathy and cardiac dysrhythmias in whom LMNA 
gene sequencing was performed. A novel mutation was identified and additional 
unusual clinical features were described.