Neuropediatrics, Table of Contents Neuropediatrics 1995; 26(2): 105-108DOI: 10.1055/s-2007-979737 Neuropathology © Hippokrates Verlag GmbH StuttgartMicroscopic Observations of the Brain in Rett SyndromeM. L. Bauman1 , Th. L. Kemper2 , D. M. Arin3 1The Children's Neurology Service, Massachusetts General Hospital, Boston, MA 02114, USA, 2Department of Neurology, Boston City Hospital, Boston, MA 02118, USA, and 3Massachusetts Institute of Allied Health Professionals, Boston, MA 02114, USA Recommend Article Abstract PDF Download(opens in new window) Buy Article(opens in new window) Abstract Rett syndrome (RS) is a clinically defined disorder which appears to be unique to females and which is associated with apparent loss of cognitive and motor skills early in life. Using the technique of gapless serial section, microscopic analysis of the brains from three cases of RS and identically processed age-matched controls was conducted to determine the nature and extent of cerebral abnormality in this disorder. Small neuronal cell size and increased cell packing density were observed throughout the brain in all three cases, without evidence of gliosis or active degeneration. These findings are consistent with a curtailment of brain development which may begin before birth. Further, the brain abnormalities in RS appear to be more diffuse than previously appreciated and are in accord with the widespread neurological symptoms characteristic of this disorder. Key words Rett syndrome - Neuropathology - Brain development PDF (1408 kb)
