Review Article

Rett Syndrome: Coming to Terms with Treatment

Table 3

Rett syndrome (consensus criteria—2010) (atypical or variant).

Regression followed by recovery or stabilization
Meet at least 3 of 6 main criteria
Meet at least 5 of 11 supportive criteria

Main criteria
Partial or complete loss of purposeful hand skills
Partial or complete loss of spoken language
Gait abnormalities: dyspraxic or absent
Stereotypic hand movements

Supportive criteria
Awake breathing problemsGrowth retardation
BruxismSmall hands and feet
Impaired sleepScoliosis and/or kyphosis
Abnormal muscle toneIncreased pain tolerance
Peripheral vasomotor abnormalitiesIntense eye contact
Inappropriate laughter or screaming