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

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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, 1 Avenue Jean

Poulhés-TSA 50032-31059 Toulouse Cedex 9, 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.