This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Harvey B. Sarnat, MD; Timothy O'Connor, MB, BCh; Paul A. Byrne, MD

Arch Neurol. 1976;33(7):459-465.

Abstract
• The article describes pregnancies and labors of five women with myotonic muscular dystrophy and their four severely involved infants, and reviews the pertinent literature. Three of the four neonatal cases died by 3 weeks of age of respiratory failure or aspiration; the fourth infant is now 4 months old and has respiratory and swallowing difficulties. The symptoms of myotonic dystrophy worsen during pregnancy. A high rate of fetal loss occurs due to spontaneous abortion, prematurity, and neonatal involvement with the disease. Prolonged labor has been described as a consistent complication, but the evidence does not justify this conclusion. Although many neonates with myotonic dystrophy are asymptomatic, severely affected newborns have a recognizable disorder unrelated to the severity of the maternal disease. The most common clinical manifestations in the neonate are arthrogryposis involving predominantly the lower extremities, generalized hypotonia and weakness, and pharyngeal weakness. Less constant features include polyhydramnios, facial diplegia, diaphragmatic paralysis, respiratory failure, decreased motility of the gastrointestinal tract, congenital cataracts, and electrocardiographic abnormalities.

Author Affiliations
From the departments of neurology (Dr Sarnat), obstetrics and gynecology (Dr O'Connor), and pediatrics (Drs Sarnat and Byrne), St Louis University School of Medicine, and Cardinal Glennon Memorial Hospital for Children. Dr Sarnat is now with Princess Margaret Hospital for Children, Perth, Western Australia.

Footnotes
Accepted for publication Feb 9, 1976.

Reprint requests to Section of Neurology, Princess Margaret Hospital for Children, Perth, Western Australia 6001 (Dr Sarnat).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Relative Disease Severity in Siblings With Myotonic Dystrophy
Andrews and Wilson
J Child Neurol 1992;7:161-167.
ABSTRACT  

Magnetic Resonance Imaging and Clinical Correlates of Intellectual Impairment in Myotonic Dystrophy
Huber et al.
Arch Neurol 1989;46:536-540.
ABSTRACT  

Dysmaturation in Infants of Mothers With Myotonic Dystrophy
Young et al.
Arch Neurol 1981;38:716-719.
ABSTRACT  

Congenital Myotonic Dystrophy: Fiber Type Abnormalities in Two Cases
Argov et al.
Arch Neurol 1980;37:693-696.
ABSTRACT  

The Muscular Dystrophies and Related Disorders: II. Diseases Simulating Muscular Dystrophies
Furukawa and Peter
JAMA 1978;239:1654-1659.
ABSTRACT  

Maturational Arrest of Fetal Muscle in Neonatal Myotonic Dystrophy: A Pathologic Study of Four Cases
Sarnat and Silbert
Arch Neurol 1976;33:466-474.
ABSTRACT