What is Rett's syndrome?

What is Rett's syndrome?

• Rett's syndrome: Autism-like behavioral syndrome in infant girls.
• Rett's syndrome: 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.
  Source - Diseases Database

Rett's syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Rett's syndrome, or a subtype of Rett's syndrome, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Rett's syndrome as a "rare disease".
Source - Orphanet

Rett's syndrome: Introduction

Types of Rett's syndrome:

Broader types of Rett's syndrome:

• Pervasive developmental disorders
• Brain conditions
• Behavioral disorders
• Genetic Disease
• Psychological disorders
• Psychiatric disorders
• more types...»

How many people get Rett's syndrome?

Prevalance of Rett's syndrome: estimated 1 per 10,000 - 22,000 females suffer from Rett syndrome, Genetics Home Reference website
Prevalance Rate of Rett's syndrome: approx 1 in 10,000 or 0.01% or 27,200 people in USA [about data]
Incidence (annual) of Rett's syndrome: 1 in 10,000-15,000 live female births
Incidence Rate of Rett's syndrome: approx 1 in 10,000 or 0.01% or 27,200 people in USA [about data]
Prevalance of Rett's syndrome: The syndrome affects approximately 1 in every 10,000-15,000 live female births, with symptoms usually appearing in early childhood — between ages 6 and 18 months. (Source: excerpt from NINDS Rett Syndrome Information Page: NINDS)

Who gets Rett's syndrome?

Patient Profile for Rett's syndrome: Symptoms usually appear by 6-18 months

Gender Profile for Rett's syndrome: Females only. Male fetuses with the condition are aborted before birth.

Gender Ratio for Rett's syndrome: females only

How serious is Rett's syndrome?

Prognosis of Rett's syndrome: Often require lifetime supportive care.
Complications of Rett's syndrome: see complications of Rett's syndrome
Prognosis of Rett's syndrome: In spite of the severe impairments that characterize this disorder, the majority of individuals with Rett can be expected to reach adulthood, surviving at least into their 40s. However, the risk of death is increased. Sudden, unexplained death — possibly from brainstem dysfunction with respiratory arrest — often occurs. (Source: excerpt from NINDS Rett Syndrome Information Page: NINDS)

What causes Rett's syndrome?

Causes of Rett's syndrome: see causes of Rett's syndrome
Causes of Rett's syndrome: The cause of Rett syndrome is unknown. (Source: excerpt from NINDS Rett Syndrome Information Page: NINDS)

What are the symptoms of Rett's syndrome?

Symptoms of Rett's syndrome: see symptoms of Rett's syndrome

Complications of Rett's syndrome: see complications of Rett's syndrome

Onset of Rett's syndrome: 7-18 months of age

Can anyone else get Rett's syndrome?

Contagion of genetic conditions: generally not; see details in contagion of genetic diseases.
Inheritance: see inheritance of Rett's syndrome

Inheritance of genetic conditions: see details in inheritance of genetic diseases.

Rett's syndrome: Testing

Diagnostic testing: see tests for Rett's syndrome.

Misdiagnosis: see misdiagnosis and Rett's syndrome.

How is it treated?

Treatments for Rett's syndrome: see treatments for Rett's syndrome
Prevention of Rett's syndrome: see prevention of Rett's syndrome
Research for Rett's syndrome: see research for Rett's syndrome

Society issues for Rett's syndrome

Hospitalization statistics for Rett's syndrome: The following are statistics from various sources about hospitalizations and Rett's syndrome:

• 0.001% (158) of hospital consultant episodes were for Rett’s Syndrome in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 97% of hospital consultant episodes for Rett’s Syndrome required hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 68% of hospital consultant episodes for Rett’s Syndrome were for men in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 32% of hospital consultant episodes for Rett’s Syndrome were for women in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• more statistics...»

Organs Affected by Rett's syndrome:

Organs and body systems related to Rett's syndrome include:

• Mental
• Brain

Name and Aliases of Rett's syndrome

Main name of condition: Rett's syndrome

Class of Condition for Rett's syndrome: genetic

Rett Syndrome, cerebroatrophic hyperammonemia, Autism, dementia, ataxia, and loss of purposeful hand use, RTT, RTS

Cerebroatrophic hyperammonemia Source - Diseases Database

Autism, dementia, ataxia, and loss of purposeful hand use, RTS (Rett Syndrome), RTT
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Research the causes of these diseases that are similar to, or related to, Rett's syndrome:

• Neurodevelopmental disorder
• Autism
• Fragile x syndrome
• Tuberous sclerosis
• Down's syndrome
• Angelman syndrome
• Cerebral palsy
• Scoliosis
• Growth failure

 » Next page: Online Medical Textbooks for Rett's syndrome

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