Assessment/intervention
Month 1
Month 2
Month 3
Month 4
Month 12
Month 15
Month 21
Tumor resection
x
Polysomnography sleep study
x
x
Multiple sleep latency testing
x
x
x
Sleep questionnaires
x
x
x
Proton beam therapy
x
x
x
Research cognitive assessment
xa
x
Clinical cognitive assessment
x
Modafinil intervention initiation
xb
Henry was followed with serial neurocognitive and sleep assessments as part of his enrollment on a clinical research trial. He was then referred for a clinical (non-research) psychological assessment in month 12 due to concerns related to weaknesses in intellectual functioning and verbal learning and memory noted during his research-based assessment prior to initiating proton therapy (month 2), as well as because of poor academic performance in spite of academic supports in place. See Table 9.1 for an outline of his assessment and intervention schedule.
History
At the time of Henry’s diagnosis, concerns were denied regarding problematic sleep and fatigue. His mother noted mild daytime sleepiness that was not described as problematic prior to the initiation of medical treatment. He was reportedly obtaining adequate sleep (i.e., approximately 9 h per night), and there were no reported concerns historically or at the time of diagnosis regarding sleep hygiene, sleep onset, sleep maintenance, tonsillar hypertrophy, or sleep-disordered breathing. There were also no concerns regarding cataplexy, hypnagogic or hypnopompic hallucinations, or sleep paralysis historically and across all assessment time points.
Henry received special education services for reading and speech difficulties that predated his tumor diagnosis, though it was unclear when the tumor first presented relative to the academic concerns, due to the often delayed recognition of craniopharyngioma [1]. Henry’s mother reported that he continued to be “just getting by” academically despite his relative improvements overall since proton therapy. She also stated that his EDS interfered with his alertness and attention in both the home and classroom settings. Henry’s mother described concerns with his attention, distractibility, organization, learning and memory, and speed of task completion. Family history was significant for Attention-Deficit/Hyperactivity Disorder (ADHD) and undiagnosed difficulties with attention.
Diagnostic Studies
At the time of his diagnosis, Henry had experienced a notable change in his mental status, and an MRI revealed the presence of a left subdural hematoma and large suprasellar mass (2.2 cm ×2.4 cm × 1.3 cm) with obstructive hydrocephalus that was consistent with craniopharyngioma (month 1). He experienced recurrent subdural hematomas of varied degrees of thickness (i.e., up to 6 mm) in the left frontal and parietal regions of the brain during the first 6 months following resection. In month 2 following his tumor resection and prior to his research-driven cognitive evaluation, an MRI revealed mass effect on the hypothalamus, resolution of the hydrocephalus, and a continued subdural hematoma and moderately enlarged third and lateral ventricles. A PET scan in month 2 indicated decreased uptake in the left frontal region of the brain that was suggestive of possible hypoperfusion. His MRI in month 6 revealed decreased size of the mass, midline shift, and distorted ventricles related to his hematoma, as well as mass effect on the superior sagittal sinus and left frontal and parietal lobes. There were no noted hematomas in the month 9 MRI or thereafter. Henry’s most recent MRI at the time of his psychological assessment (month 12) indicated a decrease in the size of his tumor and an improved appearance of the subdural collections.
Initial Sleep Study and Subjective Sleep Ratings (Month 2)
In month 2 during his baseline assessment prior to proton therapy, Henry and his mother completed subjective sleep questionnaires, and Henry underwent a nocturnal polysomnography (PSG ) that was followed by daytime multiple sleep latency testing (MSLT; see Table 9.2). He received an adequate quantity of sleep during his PSG (500.0 min), with a sleep onset latency (SOL) of 2.0 min and a sleep efficiency of 97.1%. The distribution of his sleep stages was atypical due to the decreased proportion of time spent in REM sleep (i.e., 10.5%). Henry exhibited 20 spontaneous arousals, as well as 11 respiratory-related arousals. His apnea/hypopnea (AHI) index was 2.6 per hour of sleep, which included five obstructive apneas and 16 hypopneas with no central or mixed apneas. Henry’s minimum oxygen saturation (SpO2) was 95%, and seven desaturations occurred over the course of the PSG. There were no periodic limb movements during the study. On the MSLT, Henry fell asleep at each nap opportunity. His mean sleep onset latency was 2.6 min (Range = 0.5–4.0 min), and he exhibited sleep onset REM (SOREM) at each opportunity (Range = 4.5–8.0 min), indicating clinically significant hypersomnia.
Table 9.2
Results of objective and subjective sleep assessments
PSG | Month 2 | Month 15 | Month 21 |
|---|---|---|---|
Total time in bed | 500.0 | 485.5 | 547.5 |
Total sleep time (TST) | 485.5 | 429.5 | 527.5 |
Sleep onset latency | 2.0 | 7.0 | 9.0 |
REM latency | 225.5 | 245.0 | 188.0 |
Sleep efficiency (%) | 97.1% | 88.5% | 96.3% |
Sleep stages (percent of sleep time) | |||
Stage N1 sleep | 3.3% | 1.9% | 0.3% |
Stage N2 sleep | 50.8% | 50.4% | 36.9% |
Stage N3 Sleep | 35.4% | 38.9% | 52.1% |
REM sleep | 10.5% | 8.8% | 10.7% |
Number of spontaneous arousals | 31.0 | 71.0 | 65 |
Apnea/hypopnea index (AHI, events per hour) | 2.6 | 2.0 | 0.2 |
SpO2 minimum (%) | 95.0% | 93% | 86% |
PLM index | 0.0 | 0.0 | 0.0 |
MSLT | Month 2 | Month 15 | Month 21 |
|---|---|---|---|
Mean sleep onset latency (minutes) | 2.6 | 4.75 | 15.0 |
REM onset (number per opportunity) | 4/4 | 0/4 | 0/4 |
Nap 1 (minutes) | |||
Sleep onset latency | 0.5 | 1.5 | 16.5 |
Sleep onset REM | 4.5 | N/A | N/A |
Nap 2 (minutes) | |||
Sleep onset latency | 2.0 | 2.5 | 17.5 |
Sleep onset REM | 6.5 | N/A | N/A |
Nap 3 (minutes) | |||
Sleep onset latency | 4.0 | 9.0 | 17.5 |
Sleep onset REM | 5.5 | N/A | N/A |
Nap 4 (minutes) | |||
Sleep onset latency | 4.0 | 6.0 | 8.5 |
Sleep onset REM | 8.0 | N/A | N/A |
Sleep questionnaires | Month 2 | Month 15 | Month 21 |
|---|---|---|---|
Excessive daytime sleepinessa | 12.0 | 4.0 | 4.0 |
Self-reported fatigueb | |||
General fatigue | 100 | 79.2 | 83.3 |
Sleep/rest fatigue | 100 | 83.3 | 91.7 |
Cognitive fatigue | 100 | 100 | 100 |
Total fatigue | 100 | 87.5 | 91.7 |
Parent-proxy fatigueb | |||
General fatigue | 50.0 | 66.6 | 100 |
Sleep-rest fatigue | 37.5 | 25.0 | 100 |
Cognitive fatigue | 12.5 | 50.0 | 75 |
Total fatigue | 33.3 | 47.2 | 91.7 |
Consistent with MSLT findings, Henry endorsed significant EDS on the modified Epworth Sleepiness Scale (ESS = 12; see Table 9.2). He did not endorse concerns regarding fatigue across any of the domains of the Pediatric Quality of Life Multidimensional Fatigue Scale (PedsQL MFS ) ; however, parent-proxy report indicated significant fatigue across the general, sleep/rest, cognitive, and overall fatigue domains (see Table 9.2). Henry’s mother indicated that he “often” or “almost always” felt tired, felt physically weak, slept and rested a lot, and experienced difficulties with sustained attention, remembering what people told him or what he just heard, remembering more than one thing at a time, and thinking quickly. No pharmacological intervention was initiated due to the limited presence of and interference from EDS in the home and school settings and his plan to initiate proton therapy, with recommendations that symptoms be monitored.
Time Between Scheduled Sleep Assessments (Months 6–12)
During medical follow-up at month 6, Henry’s mother indicated that he was experiencing increased sleep problems since diagnosis, including fatigue, worsened daytime sleepiness, and late afternoon napping. At the time of cognitive testing (month 12), Henry was experiencing persistent EDS that resulted in his falling asleep in school, and he was taking daily naps from 4–6 pm despite a full night’s sleep (i.e., approximately 9 h, with no reported difficulties with sleep initiation or maintenance). His neurologist also recommended a follow-up sleep study (scheduled month 15) and consideration of stimulants given Henry’s persistent EDS.
One Year Follow-Up Sleep Study and Subjective Sleep Ratings (Month 15)
In month 15, Henry and his mother completed subjective sleep questionnaires, and Henry underwent a repeat PSG followed by MSLT (see Table 9.2). He received an adequate quantity of sleep during his PSG (429.5 min), with a SOL of 7.0 min and a sleep efficiency of 88.5%. The distribution of his sleep stages continued to be atypical due to the decreased proportion of REM sleep (i.e., 8.8%). Henry exhibited 66 spontaneous arousals and five respiratory-related arousals. His AHI index was 2.0 per hour of sleep, which included three obstructive apneas and 11 hypopneas. Henry’s minimum oxygen saturation (SpO2) was 93%, and 38 desaturations occurred. There were no periodic limb movements. On the MSLT, Henry initiated sleep at each nap opportunity. His mean sleep onset latency was 4.75 min (Range = 1.5–6.0 min), and there were no episodes of SOREM. Although the results of his MSLT indicated SOREM improvements compared to month 2, Henry continued to exhibit clinically significant daytime sleepiness.
Henry did not endorse significant EDS on the modified ESS at month 15 (ESS = 4; see Table 9.2). He did endorse increased concern on the PedsQL MFS regarding fatigue across the general, sleep/rest, and total fatigue domains. For example, Henry endorsed that he “sometimes” felt tired, slept a lot, and spent a lot of time in bed, and that he “often” felt too tired to spend time with his friends. Henry’s mother continued to express significant concerns regarding Henry’s fatigue. She endorsed concerns on similar items as she did in month 2, with the exception of her also endorsing in month 15 that he “often” felt tired when waking up in the morning.
Cognitive Testing
Behavioral Observations
During his research-based cognitive assessment prior to proton therapy (month 2), Henry was described as exhibiting some lapses in attention and motivation as well as frequent fidgeting, but he otherwise appeared to be adequately engaged throughout testing. He put forth strong effort throughout his cognitive testing in month 12, and he did not exhibit outward signs of inattention, distractibility, or impulsive behaviors. There were also no observable signs of sleepiness (e.g., dozing off, “zoning out”). Henry displayed poor speech articulation, and he frequently exhibited difficulties with word finding and expressive language (i.e., poor coherence of sentence construction and often talking around the point he was making). He did not demonstrate difficulties following directions (i.e., receptive language). The results were thought to reflect valid estimates of his functioning at the time of administration.
Intellectual Functioning
Henry was administered the Wechsler Intelligence Scale for Children, 4th edition (WISC-IV) in month 2 during his baseline research-based cognitive assessment. In month 12, given the recent WISC-IV administration, Henry was administered the Wechsler Abbreviated Scale of Intelligence, 2nd Edition (WASI-II), as a brief re-assessment of cognitive functioning as part of his clinical assessment. The results in months 2 and 12 indicated variable and generally underdeveloped cognitive abilities. His performance fell 1.33 to 2.75 standard deviations (SDs) below age-based norms across domains, including verbal reasoning, nonverbal reasoning, and working memory. Henry exhibited a significant personal strength in his Average range processing speed skills. During his month-15 research-based follow-up assessment, Henry continued to exhibit impairments on verbal reasoning and working memory tasks. He exhibited a significant improvement in his performance on nonverbal reasoning tasks (i.e., nearly 2 SD increase), which fell within expected age limits. The variability between domains and across time points called into question the stability of his cognitive functioning. See Table 9.3 for results.
Table 9.3
Intellectual, verbal fluency , and academic functioning
Measure/scalea | Month 2 | Month 12b | Month 15 |
|---|---|---|---|
WISC-IV/WASI-II | |||
Full scale IQ
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