1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-6-42-b 10.15844/pedneurbriefs-6-6-3 Neuromuscular Diseases Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Myotonic Dystrophy: Severity and Maternal Age http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 06 1992 01 07 2016 6 6 42 43 Copyright: © 1992 The Author(s) 1992 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Relative disease severity in siblings with myotonic dystrophy

The severity of myotonic dystrophy in 17 affected sibling pairs from 15 families in which 2 or more affected children were born to mothers with myotonic dystrophy is reported from the Hospital for Sick Children, London and the Prince of Wales Children’s Hospital, Sydney, Australia.

 Myotonic Dystrophy Maternal Age Scoliosis

The severity of myotonic dystrophy in 17 affected sibling pairs from 15 families in which 2 or more affected children were born to mothers with myotonic dystrophy is reported from the Hospital for Sick Children, London and the Prince of Wales Children’s Hospital, Sydney, Australia. In 13 of 17 sibling pairs the younger child was more severely affected than the older child. Increasing age difference between the affected siblings paralleled increasing age for each mother and showed a positive correlation with the difference in disease severity between siblings. Infants born to older mothers suffered more severe myotonic dystrophy. Maternal age at delivery correlated with the age at which the infant sat alone and walked alone. In addition, the incidence of neonatal feeding difficulties, neonatal respiratory difficulties, surgery for talipes, and scoliosis were directly related to maternal age at delivery. [1]

COMMENT. These findings have relevance in genetic counseling and suggest that maternal age and birth order are important in the relative severity of childhood onset myotonic dystrophy. The authors note that a normal infant born after a neonatally affected child is unlikely to develop myotonic dystrophy.

An increased risk of spontaneous abortion, cesarian section and neonatal death for women with myotonic dystrophy is reported from the Chicoutimi Hospital, Quebec Canada [2]. The risk estimate that women with myotonic dystrophy will have a congenitally effected child is 3-9%. Among children surviving the neonatal period, 7.3% had congenital myotonic dystrophy and 7.3% had childhood onset myotonic dystrophy.

 Andrews PI Wilson J Relative disease severity in siblings with myotonic dystrophy J Child Neurol 1992 Apr 7 2 161 167 10.1177/088307389200700205 1573233 Prevost C Mathieu J Pregnancy outcome of women with myotonic dystrophy Neurology 1992 April 42 Suppl 3 226