Table 2.

Initial assignment of Rett Clinical Severity Scale scores and symptoms to Clinical Global Impression Scale Severity rating score

CLINICAL DOMAINS	CGI-S: 1	CGI-S: 2	CGI-S: 3	CGI-S: 4	CGI-S: 5	CGI-S: 6	CGI-S: 7
	(CSS =0)	(CSS <5)	(CSS 5-10)	(CSS 10-20)	(CSS 20-25)	(CSS 25-35)	(CSS 35-40)
Language/ Communication	Normal	Appropriate. May have unusual features such as perseveration/echolalia. Reading disability/dyslexia	Phrases- sentences. May have conversations or echolalia	Words (<5) Babbles Makes choices 25-50%	No Words Babbles Makes choices ≤25%	Vocalizations Occasionally screams Makes No Choices or only rarely makes choices	No Words No Vocalizations Screams No Choices
Ambulation	No impairment	Normal, may have slight evidence of dystonia/ataxia/dyspraxia on careful exam	Walks, able to use stairs/run. May ride tricycle or climb	Walks Independently, unable to use stairs or run	Walks with Assistance	Stands With Support or independently May walks with support Sits independently or with support	Cannot sit Doesn’t Stand or Walk
Hand Use	Completely normal, no impairment	Normal, may have slight fine motor issues	Bilateral Pincer grasp. May use pen to write but has some fine motor issues like tremor	Reaches for objects, raking grasp or unilateral pincer. May use utensils/cup	Reaches No Grasps	Rarely- Occasionally Reaches Out No Grasp	None
Social (Eye Contact)	Normal	Occasional eye gaze avoidance	Appropriate eye contact, >30 sec	Eye Contact <20 secs	Eye Contact <10 secs	Eye Contact, Inconsistent 5 secs	No eye Contact
Autonomic	None	Minimal	No or minimal breathing abnormalities (<5% of times observed) and Warm, pink extremities	Breathing Dysrhythmia <50% No Cyanosis Cool UE & LE Pink	Breathing Dysrhythmia 50% No Cyanosis Cool UE & LE Pink	Breathing Dysrhythmia, 50%-100%, maybe with Cyanosis Cold LE or UE, may be Blue	Breathing Dysrhythmia, Constantly with Cyanosis Cold UE & LE Mottled/Blue
Seizures	None	None or controlled	None, with or without meds	Monthly- Weekly	Weekly	Weekly-Daily	Daily
Attentiveness	Entirely normal	Occasional inattention	Attentive to conversation and follows commands	50-100% of Time	50% of Time	Less than 50% time	0%

CSS: Clinical Severity Scale. CGI-S: Clinical Global Impression Scale-Severity. UE: Upper extremity. LE: Lower extremites