Table 1:

Scoring system for the evaluation of clinical severity of the syndrome in each patient.⁸ (Kelley TM, Hatfield LA, Lin DD, Comi AM. Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome. J Child Neurol. 2005; 20: 867–70.)

	Rating range	Meaning of assigned rating
Visual field cut	0–2	0 = no field cut 1 = partial homonymous hemianopsia 2 = full homonymous hemianopsia
Hemiparesis	0–4	0 = no weakness or posturing 1 = mild posturing intermittently 2 = fine motor impairments only 3 = significant fine and gross motor impairments 4 = severe fine and gross motor impairments, poor helper arm function, and walks with great difficulty or not at all
Seizure frequency	0–4	0 = none ever 1 = one or more seizures, currently controlled 2 = breakthrough seizures 3 = monthly seizures 4 = at least weekly seizures
Cognitive function	0–5	Infant/preschooler: 0 = normal 1 = mild speech delay but comprehends well 2 = mild delay in speech and comprehension 3 = moderately delayed speech 4 = severely delayed speech 5 = profoundly delayed speech with little or no comprehension Child: 0 = normal 1 = school difficulties, regular classes 2 = resource help needed in school 3 = special education required 4 = trainable for activities of daily living 5 = full care Adult: 0 = normal 1 = lives and works independently 2 = works in community with parental support 3 = significant difficulty maintaining employment or satisfactory social relationships 4 = trainable (i.e. group home, supervised work setting) 5 = full care
Total	0–15	normal to severely impaired