PEDIATRICS Vol. 46 No. 6 December 1970, pp. 891-899
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Lebenthal, E.
Right arrow Articles by Matoth, Y.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Lebenthal, E.
Right arrow Articles by Matoth, Y.

ARTHROGRYPOSIS MULTIPLEX CONGENITA: TWENTY-THREE CASES IN AN ARAB KINDRED

Emanuel Lebenthal M.D.1, Solly Barnard Shochet M.B., M.P.H.1, Avinoam Adam M.D.1, Moshe Seelenfreund M.D.1, Amnon Fried M.D.1, Theodore Najenson M.D.1, Uriel Sandbank M.D.1, and Yehuda Matoth M.D.1

1 Departments of Pediatrics, Orthopedic Surgery and Traumatology, Electrodiagnosis, and Pathology, Beilinson Hospital and Medical Center, Petah-Tikva, and the Government Health Center, Tira, Department of Family Medicine, the Department of Genetics, Tel-Hashomer Hospital, and the Rehabilitation Department, Lowenstein Hospital (all departments associated with the Tel Aviv University Medical School, Tel Aviv, Israel)

An Arab kindred is described in which 17 males and six females had clinical manifestations of arthrogryposis multiplex congenita (AMC). The syndrome, which is present at birth, is expressed mainly by flexion and extension contractures of the knees and elbows, with muscle weakness around the involved joints. Six patients had associated congenital heart disease which caused death in five. One patient was mentally retarted.

Electromyographic studies failed to reveal any participation of the lower motor neurons. Histological and biochemical findings suggest a myogenicetiology.

The pedigree data, being characterized by a very high rate of consanguinity among the parents of the affected and the existence of 20 normal off-spring of three affected males, demonstrate the autosomal recessive mode of inheritance, which is in contrast to the dominant pattern observed in neurogenic AMC.

Submitted on December 29, 1970
Accepted on June 9, 1970