Table 1. University of Rochester Batten Center's Neurobehavioral Assessment Protocol.

A. DIRECT NEUROPSYCHOLOGICAL ASSESSMENT
1. WISC-IV23 Vocabulary	Verbal Intellectual Ability
a. First 4, picture-naming items are not presented, to accommodate vision loss in JNCL. Credit for these items only given if study subject earns full credit for first 2 verbal items, otherwise the total score for the test treats the first 4 items as ‘failed’; b. Study subject is asked to recite full alphabet to evaluate retention of a well-routinized skill whose attainment likely precedes symptom onset. It is scored separately from Vocabulary raw score as ‘pass/fail.’
2. WISC-IV Digit Span*	Auditory attention, working memory
Verbal cues provided to visually impaired child, before each item is presented: “Here comes the next one”
3. WISC-IV Information	Fund of knowledge
No modifications
4. WISC-IV Similarities	Verbal abstract reasoning
No modifications
5. WRAML-245 Story Recall	Immediate, delayed, and recognition memory
No modifications
6. WRAML-2 Sentence Recall	Auditory attention, immediate recall
No modifications
7. Controlled Oral Word Association Test46	Verbal fluency
Two tasks combined – phonemic (F-A-S) and semantic (animals; food) to extend ‘floor’ of task, as phonemic items are failed earlier.

B. PARENT-PROXY RATINGS OF CHILD FUNCTION
1. PedsQL26	Child Health-related Quality of Life
No modifications
2. Family Impact Module27	Ratings of self (parental) HRQL and family QL.
No modifications
3. Child Behavior Checklist31	Omnibus assessment of child behavior and mood
No modifications
4. Scales of Independent Behavior – Revised30	Child adaptive function level
None, but use of SIB-R Short form for Visually Impaired, to reduce burden for completion

HRQL, health-related quality of life; QL, quality of life; WISC-IV, Wechsler Intelligence Scale for Children - Fourth Edition; WRAML-2, Wide Range Assessment of Memory and Learning - 2nd Edition.

Several tests were piloted for older subjects but were not retained, due to floor effects: TOMAL Digit Span⁴⁷ (2003), Wechsler Adult Intelligence Scale - 3rd Edition, Wechsler Memory Scale - 3rd Edition⁴⁸ (2004-2005), Clinical Evaluation of Language Fundamentals, Recalling Sentences⁴⁹ (2004-5).