title: Rett syndrome: spinal cord neuropathology.
abstract: The morphologic changes of the spinal cord in Rett syndrome are described in 2 young women who died at 20 and 30 years of age. Both patients had been in a severely disabled state for many years with tetraparesis and extreme muscle wasting. Degeneration and loss of spinal ganglion nerve cells, in addition to gliosis of both the white and gray matter of the spinal cord, were evident. The number of motor neurons appeared to be reduced and axonal changes suggestive of degeneration were observed in both the ascending and descending tracts.
authors.affiliation: Department of Pathology I, University of Göteborg, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Oldfors
authors.forename: B
authors.initials: B
authors.lastname: Hagberg
authors.forename: H
authors.initials: H
authors.lastname: Nordgren
authors.forename: P
authors.initials: P
authors.lastname: Sourander
authors.forename: I
authors.initials: I
authors.lastname: Witt-Engerström
mesh_terms: D000328:Adult; D001072:Apraxias / Q000473:pathology*; D001284:Atrophy; D001321:Autistic Disorder / Q000473:pathology*; D001921:Brain / Q000473:pathology; D001925:Brain Damage, Chronic / Q000473:pathology*; D002524:Cerebellar Ataxia / Q000473:pathology*; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D005911:Gliosis / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D009454:Neurofibrils / Q000648:ultrastructure; D013116:Spinal Cord / Q000473:pathology*; D013577:Syndrome
chemical_list: D005904:Glial Fibrillary Acidic Protein