Actigraphic Analysis of Treatment Response

Actigraphic measurements will be recorded every 30 seconds and will be recorded for an 11
day baseline period, followed by a 10 day period of the initial pharmacological
interventions. The non-invasive nature of watch-like actigraphy devices is particularly
attractive for use in pediatric populations. Meaningful treatment response measurements are
obtained when actigraphic data is combined with psychometric and observational repeated
measurements. Subsequent to the initial data collection the parent consented and the child
assented to extend the study and continue measurements.

The case study included baseline and repeated psychological, observational and actigraphic
measurements that were initiated prior to treatment with risperidone and repeated throughout
the treatment process with the final data collected in July 2010.

The Personality Inventory for Children (PIC) is an objective multidimensional measurement of
affect, behavior, ability and family function. The PIC was administered prior to treatment
with risperidone and repeated after 22 months of treatment. The PIC serves as both an
actuarial pre-treatment diagnostic tool as well as a post-treatment repeated measurement
indicating treatment associated change.

A primary observer (mother) was trained to report symptom percentages present since previous
observations utilizing the operationally defined and observer defined items of the
systematic observation scale throughout the treatment process.

Actigraphic measurements provide a basis by which to measure sleep and sleep onset latency
as well as periods of mobility and immobility. When programmed to record activity every
thirty seconds, thousands of measurements are quickly captured.

Actigraphic measurements were made utilizing a watch-like actigraphic device with an 11 day
baseline actigraphic measurement period and continued measurements that included the
initiation of a Once-Daily, Bedtime (q.h.s.) pharmacological intervention for 6 days,
followed by a planned adjustment to Twice a Day (b.i.d.) dosing that was measured for an
additional 4 days. Repeated actigraphic studies were subsequently conducted after 22 months
of risperidone treatment and were collected along with the repeated observational and
psychometric data.

This case study explores activity and sleep characteristics related to behavioral
characteristics and treatment response and the utility of single subject repeated
actigraphic, psychometric and observational measurements and monitoring of treatment
response.

Inclusion Criteria:

- Diagnosis of Kabuki Syndrome

- Medication Naive

- Single subject preparing to receive pharmacological intervention

Exclusion Criteria:

- Inability to wear actigraphy device