Glossary / Lexicon

Rett Syndrome refers to a progressive neurologic developmental disorder and one of the most common causes of mental retardation in females. The key features of this syndrome are:

(1) loss of purposeful use of the hand which is replaced with stereotyped hand-wringing;

(2) screaming fits and inconsolable crying;

(3) loss of speech;

(4) behavior like that of autism;

(5) panic-like attacks;

(6) grinding of teeth (bruxism); and

(7) microcephaly, among others

Rett Syndrome is also called Rett's Disorder.

Description

Rett Syndrome is a rare neurological disorder that predominantly affects females, characterized by a slowing of head growth, loss of purposeful hand skills, development of repetitive hand movements, and difficulties with walking. Individuals with Rett Syndrome often experience severe cognitive and communication impairments, as well as stereotypical behaviors such as hand-wringing. The syndrome is caused by mutations in the MECP2 gene on the X chromosome and typically presents in early childhood, around 6-18 months of age. It is classified as a pervasive developmental disorder, placing it within the realm of psychological conditions that impact behavior, social interaction, and communication.

Application Areas

• Educational psychology in relation to learning difficulties and cognitive impairments
• Behavioral psychology in understanding and managing stereotypical behaviors
• Developmental psychology for studying early childhood development and milestones
• Clinical psychology for providing interventions and support for individuals with Rett Syndrome

Treatment and Risks

• Behavioral therapy to address stereotypical behaviors and improve social interactions
• Speech therapy to support communication skills
• Physical therapy to enhance motor skills and mobility
• There is a risk of developing seizures and gastrointestinal issues in individuals with Rett Syndrome

Examples

• An individual with Rett Syndrome may exhibit hand-wringing behavior when feeling anxious or overwhelmed
• A child with Rett Syndrome may struggle with maintaining eye contact and responding to social cues
• A teenager with Rett Syndrome may have difficulties with balance and coordination while walking

Similar Concepts and Synonyms

• MECP2 Duplication Syndrome
• Autism Spectrum Disorder with Regression
• X-Linked Rett Syndrome

Summary

Rett Syndrome is a rare neurological disorder that primarily affects females, causing a range of physical and cognitive impairments. It is characterized by a regression in skills, development of repetitive hand movements, and difficulties with communication and social interaction. Psychological interventions such as behavioral therapy, speech therapy, and physical therapy are often utilized to support individuals with Rett Syndrome. Despite the challenges posed by the syndrome, early diagnosis and appropriate interventions can improve quality of life for affected individuals and their families.

--