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ARS Home » Plains Area » Houston, Texas » Children's Nutrition Research Center » Research » Publications at this Location » Publication #101010
Title: ORGAN GROWTH IN RETT SYNDROME: A POSTMORTEM EXAMINATION ANALYSIS

Author
item ARMSTRONG, DAWNA - BAYLOR COLL OF MEDICINE
item DUNN, J - BAYLOR COLL OF MEDICINE
item SCHULTZ, REBECCA - BAYLOR COLL OF MEDICINE
item HERBERT, DAVID - BAYLOR COLL OF MEDICINE
item GLAZE, DANIEL - BAYLOR COLL OF MEDICINE
item Motil, Kathleen

Submitted to: Pediatric Neurology
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 8/28/1998
Publication Date: N/A
Citation: N/A

Interpretive Summary: Rett syndrome is a disorder specific to females that strikes early in life. Girls with Rett syndrome display both mental and motor retardation within a general pattern of altered growth. We analyzed 39 postmortem examination studies to further document and understand the growth pattern of Rett girls, and compared the organ weights to those of healthy females of comparable age and height. The results suggest that the pattern of growth failure in Rett syndrome may be unique, compared to other mental disorders such as Down syndrome. The rate of brain growth slows after birth. The increment in normal brain weight after 4 years of age (that of the first postmortem exam) is not observed in the Rett brain. However, the heart, kidneys, liver and spleen grow normally until 8-12 years of age, when the growth rate slows, although the organs continue to gain weight. The results suggest that despite generally slower growth in Rett syndrome, the brain may be particularly affected. These results will be helpful in better understanding and dealing with Rett syndrome, which has no known cause.

Technical Abstract: Rett syndrome is a disorder of unknown etiology in females that manifests as severe mental and motor retardation during the first years of life. A postnatal pattern of altered growth is its earliest clinical expression. Head growth decelerates during the first year of age and is followed by a decline in somatic (height/weight) growth. The decreased occipitofrontal circumference (OFC) is reflected in decreased brain size, and measurements of the dendrites of cortical neurons suggest that a developmental and growth arrest have occurred. To further document growth in Rett syndrome, measurements of organ weights, as recorded in 39 postmortem examination studies, were compared with normal organ weights for females of comparable age and height. These organ weights suggest that the pattern of growth failure in Rett syndrome, as compared with other forms of mental handicap, such as Down syndrome and Turner's syndrome, may be unique. In Rett syndrome the rate of brain growth, as derived from OFC, decelerates after birth. The increment in normal brain weight after 4 years of age, the age of the first postmortem examinations, is not observed in the Rett brain. The heart, kidneys, liver, and spleen grow at the normally defined rate until 8-12 years of age, when their growth rate decelerates, but their growth continues achieving organ weights that are appropriate for the height of the female. Adrenal weights are normal. These observations suggest that despite a generalized decreased growth in Rett syndrome the brain may be preferentially affected in this syndrome.