Emery-Dreifuss muscular dystrophy: anatomical-clinical correlation (case report)

We report on a man that had weakness of humeroperoneal distribution associated with limited range of motion of the cervical spine and elbows since he was 5 years old . At age 26 he developed tachycardia episodes. A complex arrhythmia was discovered, and a nodal ablation was done with a cardiac pacemaker implanted. The patient had an arrhythmia and sudden death followed this. Emery-Dreifuss muscular dystrophy is a rare recessive X-linked muscular disorder where mixed patterns in electromyography and muscle histology (neurogenic and/or myopathic) have caused nosological confusion. The autopsy findings are here described and correlated to the clinical features in an attempt to better understand the ambiguous findings concerning the process etiology .

Bibliographic Details

Main Authors:	CARVALHO,ALZIRA ALVES DE SIQUEIRA, LEVY,JOSÉ ANTONIO, GUTIERREZ,PAULO S., MARIE,SUELY KAZUE NAGAHASHI, SOSA,EDUARDO ARGENTINO, SCANAVACA,MAURÍCIO
Format:	Digital revista
Language:	English
Published:	Academia Brasileira de Neurologia - ABNEURO 2000
Online Access:	http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2000000600023
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