F84.2

Approximate Synonyms

• Dementia and behavioral disturbance due to rett’s syndrome

• Dementia due rett’s syndrome

• Dementia due to rett’s syndrome

• Rett syndrome

• Rett syndrome w dementia

• Rett syndrome w dementia w behavioral disturbance

• Rett’s disorder

• Retts syndrome

• Retts syndrome with dementia

Clinical Information

• A progressive neurologic disorder caused by mutations in the mecp2 gene on chromosome x. It almost exclusively affects girls. It is characterized by language and learning difficulties, poor communication skills, and repetitive hand motions. Other signs and symptoms include microcephaly, scoliosis, breathing abnormalities, and sleep disturbances.

• 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)

• Progressive disorder affecting the cerebral cortex of females; present from birth; manifested by autistic behavior, ataxia, dementia, seizures, loss of purposeful usefulness of the hands, cerebral atrophy, and mild hyperammonemia.

• Progressive encephalopathy occurring almost exclusively in females. The affected children are generally born clinically normal in most but not all cases. Their neurological and mental development begins to stagnate between the ages of 7 and 18 months with slowing down of the growth of head circumference and a rapid deterioration of brain functions marked by autistic behavior, dementia, apraxia of gait, loss of facial expression, deterioration of purposeful use of hands, ataxia, and severe dementia. There is an intermediate period of relative stable mental status, sometimes lasting several decades, which is followed by further neurological degradation, marked mainly by spasticity of the lower limbs and epilepsy.

• Rett syndrome is a rare inherited disease that causes developmental and nervous system problems, mostly in girls. It’s related to autism. Babies with rett syndrome seem to grow and develop normally at first. Between 3 months and 3 years of age, though, they stop developing and even lose some skills. Symptoms include

Details

• F84.2 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM F84.2 became effective on October 1, 2022.

• This is the American ICD-10-CM version of F84.2 – other international versions of ICD-10 F84.2 may differ.