Rett Syndrome

**Definition:** 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)

**Tree numbers:** - C10.597.606.360.455.937 - C16.320.322.500.937 - C16.320.400.525.937

**Synonyms:** - Autism-Dementia-Ataxia-Loss of Purposeful Hand Use Syndrome - Rett's Syndrome - Rett Disorder - Rett's Disorder - Autism, Dementia, Ataxia, and Loss of Purposeful Hand Use - Cerebroatrophic Hyperammonemia