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Emery‐dreifuss muscular dystrophy
Author(s) -
Rowland L. P.,
Fetell M.,
Olarte M.,
Hays A.,
Singh N.,
Wanat F. E.
Publication year - 1979
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410050203
Subject(s) - muscular dystrophy , confusion , medicine , electromyography , weakness , muscle weakness , paralysis , physical medicine and rehabilitation , anatomy , psychology , surgery , psychoanalysis
A man had weakness of humeroperoneal distribution associated with limited range of motion of the cervical spine and elbows. At age 25 he developed permanent atrial paralysis, and a cardiac pacemaker was inserted. Although this case was sporadic, most others have been transmitted as an X‐linked recessive trait. Mixed patterns in electromyography and muscle histology have caused nosological confusion, but the unique clinical signs seem to define a distinct form of muscular dystrophy, warranting the designation “Emery‐Dreifuss” type.