CMT 2A: A cardio-neurological form of laminopathy: dilated cardiomyopathy with p

[Guest guest]

Pacing Clin Electrophysiol. 2009 Mar;32(3):410-5.

A cardio-neurological form of laminopathy: dilated cardiomyopathy with permanent

partial atrial standstill and axonal neuropathy.

Duparc A, Cintas P, Somody E, Bieth E, P, Maury P, Delay M.

Unité Rythmologie et Stimulation Cardiaque, CHU Rangueil, Toulouse, France.

We present the first form of laminopathy connected with a new mutation of the

lamin A/C gene expressed by dilated cardiomyopathy and partial atrial standstill

associated with Charcot-Marie-Tooth type 2 axonal neuropathy. The rapid

development of the cardiac disease was controlled by medical treatment and

resynchronization therapy associated with a defibrillator.