Biomaterial Database

The neuropathology of Rett syndrome--overview 1994. 1995

D D Armstrong

Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA.

The syndrome which Andreas Rett and Bengt Hagberg first brought to our attention has been observed for eighteen years. It appears to be unlike any other disease of childhood. The original descriptions of Rett syndrome, as being autistic, degenerative and progressive are now, based on longitudinal observations, being questioned. In this paper the morphologic changes in the nervous system in Rett syndrome are summarized. The evidence against a degenerative process are presented, and the alternations are considered, along with the clinical features in terms of a possible arrest of brain development.

UI	MeSH Term	Description	Entries
D008543	Melanins	Insoluble polymers of TYROSINE derivatives found in and causing darkness in skin (SKIN PIGMENTATION), hair, and feathers providing protection against SUNBURN induced by SUNLIGHT. CAROTENES contribute yellow and red coloration.	Allomelanins,Melanin,Phaeomelanins
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D002395	Catecholamines	A general class of ortho-dihydroxyphenylalkylamines derived from TYROSINE.	Catecholamine,Sympathin,Sympathins
D006257	Head	The upper part of the human body, or the front or upper part of the body of an animal, typically separated from the rest of the body by a neck, and containing the brain, mouth, and sense organs.	Heads
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D015518	Rett Syndrome	An inherited neurological developmental disorder that is associated with X-LINKED INHERITANCE and may be lethal in utero to hemizygous males. The affected female is normal until the age of 6-25 months when progressive loss of voluntary control of hand movements and communication skills; ATAXIA; SEIZURES; autistic behavior; intermittent HYPERVENTILATION; and HYPERAMMONEMIA appear. (From Menkes, Textbook of Child Neurology, 5th ed, p199)	Autism-Dementia-Ataxia-Loss of Purposeful Hand Use Syndrome,Cerebroatrophic Hyperammonemia,Autism, Dementia, Ataxia, and Loss of Purposeful Hand Use,Rett Disorder,Rett's Disorder,Rett's Syndrome,Autism Dementia Ataxia Loss of Purposeful Hand Use Syndrome,Cerebroatrophic Hyperammonemias,Hyperammonemia, Cerebroatrophic,Hyperammonemias, Cerebroatrophic,Retts Syndrome,Syndrome, Rett,Syndrome, Rett's