Clinical Study

Illness Severity, Social and Cognitive Ability, and EEG Analysis of Ten Patients with Rett Syndrome Treated with Mecasermin (Recombinant Human IGF-1)

Table 2

Positive and negative features of the novel Rett Severity Score (RSS).

Rett Severity Score features
Positive featuresNegative features

PointingHand wringing
ManipulatingBiting
Reaching for somethingRocking
Ability to mimic/imitateHitting
Indicating yes/no with head gestureIndiscriminate moaning
Reactivity to a callTongue, chewing
Reactivity to an objectVacant staring
Siming to stimulusBruxism
Deliberate VocalisationBreath holding, apnoea
AttentionValsalva Maneuver

A full description can be found in the supplementary materials of the present paper.