Cannabidiol Treatment for Neurological, Cognitive, and Psychiatric Symptoms in Sturge-Weber Syndrome.

BACKGROUND: A prior drug trial of cannabidiol for treatment-resistant epilepsy in patients with Sturge-Weber syndrome (SWS), a rare neurovascular condition, implicated improvements in neurological, quality of life (QOL), neuropsychologic, psychiatric, and motor outcomes. METHODS: Ten subjects with SWS brain involvement, controlled seizures, and cognitive impairments received study drug in this Johns Hopkins institutional review board-approved, open-label, prospective drug trial. Oral cannabidiol was taken for six months (dose ranged from 5 to 20 mg/kg/day). SWS neuroscore, port-wine birthmark score, QOL, and adverse events were recorded every four to 12 weeks. Neuropsychologic, psychiatric, and motor assessments were administered at baseline and six months' follow-up. Most evaluations were conducted virtually due to the coronavirus disease 2019 pandemic. RESULTS: Cannabidiol was generally well tolerated. Six subjects reported mild to moderate side effects related to study drug and continued on drug; one subject withdrew early due to moderate side effects. No seizures were reported. Significant improvements in SWS neuroscore, patient-reported QOL, anxiety and emotional regulation, and report of bimanual ability use were noted. Migraine QOL scores were high at baseline in these subjects, and remained high. Neuropsychologic and other QOL and motor outcomes remained stable, with some within-subject improvements noted. CONCLUSIONS: Further studies are needed to determine whether Epidiolex can improve quality of life and be beneficial for neurological, anxiety, and motor impairments in SWS independent of seizure control. Large multicentered studies are needed to extend these preliminary findings.

Improved parent self-efficacy following pediatric evaluation: Evidence for value of a telemedicine approach in psychological and neuropsychological assessment.

Objective: While considerable inquiry is currently underway into the comparability of psychological test results obtained in onsite/in-person settings versus telemedicine settings, there has been less attention given to the comparability of the impact/outcome of the assessment process across settings. The current quality improvement study conceptualized impact/outcome according to the model of Austin et al. and sought to determine whether the prior finding of increased parent self-efficacy following onsite neuropsychological assessment was also observed when psychological and neuropsychological assessment was conducted via a telemedicine modality. Method: In the course of standard care delivery, ratings from Austin et al.'s four parent self-efficacy items were obtained at time 1 prior to patients' assessment visits and then again at time 2 either (1) following their last assessment/feedback visit (the Complete Assessment group; n = 157) or (2) in the middle of the assessment process prior to the last planned visit (the Incomplete Assessment group; n = 117). Results: Analyses revealed significant findings for time and time × group. Parent self-efficacy ratings improved over time in both groups, with significantly higher ratings in the Complete Assessment group at time 2. When compared to reference means from the in-person/onsite Austin et al. study, ratings from the current study found comparable improvement in parent self-efficacy achieved via telemedicine assessment in the Complete Assessment group. Conclusions: These data support the use of telemedicine based psychological and neuropsychological evaluation and provide preliminary evidence that the impact/outcome is comparable with in-person/onsite assessment.

Pandemic intake questionnaire to improve quality, effectiveness, and efficiency of outpatient neurologic and developmental care at the Kennedy Krieger institute during the COVID-19 pandemic.

Background: The COVID-19 pandemic uniquely affects patients with neurologic and developmental disabilities at the Kennedy Krieger Institute. These patients are at increased risk of co-morbidities, increasing their risk of contracting COVID-19. Disruptions in their home and school routines, and restrictions accessing crucial healthcare services has had a significant impact. Methods: A Pandemic Intake questionnaire regarding COVID-19 related medical concerns of guardians of patients was distributed using Qualtrics. Data from May-December 2020 were merged with demographic information of patients from 10 clinics (Center for Autism and Related Disorders (CARD), Neurology, Epigenetics, Neurogenetics, Center for Development and Learning (CDL) Sickle Cell, Spinal Cord, Sturge-Weber syndrome (SWS), Tourette's, and Metabolism). A provider feedback survey was distributed to program directors to assess the effectiveness of this intervention. Results: Analysis included responses from 1643 guardians of pediatric patients (mean age 9.5 years, range 0-21.6 years). Guardians of patients in more medically complicated clinics reported perceived increased risk of COVID-19 (p < 0.001) and inability to obtain therapies (p < 0.001) and surgeries (p < 0.001). Guardian responses from CARD had increased reports of worsening behavior (p = 0.01). Providers increased availability of in-person and virtual therapies and visits and made referrals for additional care to address this. In a survey of medical providers, five out of six program directors who received the responses to this survey found this questionnaire helpful in caring for their patients. Conclusion: This quality improvement project successfully implemented a pre-visit questionnaire to quickly assess areas of impact of COVID-19 on patients with neurodevelopmental disorders. During the pandemic, results identified several major areas of impact, including patient populations at increased risk for behavioral changes, sleep and/or disruptions of medical care. Most program directors reported improved patient care as a result.

Reduction in school individualized education program (IEP) services during the COVID-19 pandemic.

Purpose: The COVID-19 pandemic created novel challenges for school systems and students, particularly students with disabilities. In the shift to remote/distance learning, this report explores the degree to which children with disabilities did not receive the special education and related services defined in their individualized education program (IEP). Methods: Patients attending an outpatient tertiary care center for neurodevelopmental disabilities in Maryland were surveyed on the impact of the pandemic on educational services provision. Results: Nearly half (46%) of respondents qualified for special education and related services through an IEP before the start of the COVID-19 pandemic. Among those with IEPs, 48% attested to reduced frequency and/or duration of special education and/or related services during the pandemic. The reduction was greatest in occupational therapy services (47%), followed physical therapy services (46%), and special education services (34%). Conclusion: This survey of children with disabilities observes a substantial reduction in IEP services reported in their completed surveys. To address the observed reduction in IEP services, we sought additional education for clinicians on the rights of students with disabilities in anticipation of students' re-entry to the classroom. A special education law attorney provided an instructional session on compensatory education and recovery services to prepare clinicians to properly inform parents about their rights and advocate for patients with unmet IEP services during the pandemic.

Parent Satisfaction With Outpatient Telemedicine Services During the COVID-19 Pandemic: A Repeated Cross-Sectional Study.

Prior to the COVID-19 pandemic, the development of hospital-based telemedicine services had been slow and circumscribed in scope due to insurance and licensure restrictions. As these restrictions were eased during the COVID-19 pandemic to facilitate ongoing patient care, the public health emergency facilitated a rapid expansion and utilization of telemedicine services across the ambulatory service sector. Objectives: The current quality improvement (QI) study utilized this unprecedented opportunity to evaluate the use of telemedicine services across a variety of clinical disciplines and patient groups. Methods: Caregivers of patients (ages 0-21) who received care through an outpatient specialty center provided experience ratings of telemedicine services delivered during the initial pandemic months (March-June 2020; N = 1311) or during the national "winter surge" in late 2020 (November 2020-February 2021; N = 1395). Questionnaires were distributed electronically following the clinical visits, and ANCOVA was employed (with patient age as the covariate) to determine if caregiver responses differed based on patient demographic characteristics. Results: Ratings of patient satisfaction with services were very strong at both time points; greater variability in scores was noted when caregivers were asked if they would use telemedicine services again. At both time points, younger patient age (i.e., age 0-5) was associated with decreased caregiver willingness to use telemedicine services in the future. Smaller effects were seen for certain "hands on" therapies (occupational, physical, and speech) during the initial months of the pandemic and for proximity to the hospital during the "winter surge." Conclusions: These data suggest a very positive overall caregiver response to telemedicine-based services during the COVID-19 pandemic. Several areas of potential improvement/innovation were identified, including the delivery of telemedicine therapies (e.g., occupational, physical, and speech) services to young patients (i.e., aged 0-5).

Comparing Cystatin C- and Creatinine-Estimated Glomerular Filtration Rates in Patients With Thoracic Versus Sacral Motor Levels of Spina Bifida.

OBJECTIVE: Patients with myelomeningocele-type spina bifida are at increased risk of developing kidney disease from neurogenic bladder. Differences between creatinine- and cystatin C-estimated glomerular filtration rates were examined in patients with thoracic versus sacral level myelomeningocele given presumed differences in muscle mass. DESIGN: A retrospective chart review (2005-2018) was performed on 57 adults with myelomeningocele [thoracic n = 44 (77%); sacral n = 13 (23%)]. Concurrently obtained creatinine and cystatin C levels were extracted and calculated creatinine- and cystatin C-estimated glomerular filtration rates were compared. RESULTS: Mean creatinine-estimated glomerular filtration rate was significantly higher for thoracic [140.8 ml/min (SD = 23.9)] versus sacral myelomeningocele [112.0 ml/min (SD = 22.6), P = 0.0003]. There was no difference in cystatin C-estimated glomerular filtration rate between sacral [116.6 ml/min (SD = 23.7)] and thoracic myelomeningocele [124.8 ml/min (SD = 17.9)]. The mean difference between creatinine- and cystatin C-estimated glomerular filtration rates in thoracic myelomeningocele [24.2 ml/min (SD = 16.3)] was significantly greater than in sacral myelomeningocele [-12.8 (SD = 15.7), P < 0.0001]. CONCLUSIONS: There was a significantly higher discrepancy between creatinine- and cystatin C-estimated glomerular filtration rates in thoracic versus sacral motor levels of myelomeningocele. These data suggest that creatinine-estimated glomerular filtration rate may overestimate kidney function in patients with thoracic myelomeningocele. Providers who manage patients with thoracic myelomeningocele should consider monitoring cystatin C to evaluate for underlying renal disease.

How much testing can a kid take? Feasibility of collecting pediatric patient experience ratings of neuropsychological and psychological assessment.

Parent-reported satisfaction is an important outcome measure in pediatric neuropsychology; however, self-report of patient experience following lengthy pediatric assessments has been under-investigated. Written at a first grade reading level and utilizing touch screen and read-aloud functionality, a set of 15 operationally-focused items were administered to evaluate pediatric patient experience of neuropsychological and psychological assessment. One-hundred ninety-seven clinically-referred patients (M age = 11.74, SD = 3.17, range = 5.86-19.02 years, 56% male) answered the post-assessment survey. The majority of patients (n = 167; 84.77%) accurately completed the initial two validity items, even though many had parent-reported ratings of reading difficulty. More than a third of patients indicated that the assessment made them tired (39%), but fewer patients reported feeling bored (13%) or worried (7%) during testing. Moreover, most patients reported having fun (66%) and many indicated that testing was preferable to other activities typically performed at that same time (i.e., school, homework). Responses to items assessing the child's interactions with the clinician and the child's effort provided little variation and were answered in a socially desirable manner. Items that focused upon the child's personal response to the assessment appointment (e.g., boredom, worry), however, elicited greater variance in patient responding. In general, patient responses did not suggest that assessment was an aversive experience. Overall, these proof of concept findings suggest that most referred pediatric patients, even those with learning issues, may be capable of independently navigating and completing self-report questionnaires while providing differential responses to items assessing clinical experience. Self-report questionnaires appear to be a feasible method for acquiring pediatric patient-reported experiences of assessment.

An abbreviated WISC-5 model for identifying youth at risk for intellectual disability in a mixed clinical sample.

OBJECTIVE: The correct "dosing" of neuropsychological assessment is of interest for the purposes of cost management and the personalization of medicine/assessment. In this context, embedded IQ screening, rather than routine comprehensive IQ testing, may be useful in identifying youth at risk for Intellectual Disability (ID) for whom further assessment is needed. This retrospective, cross-sectional study examined subtests from the Wechsler Intelligence Scale for Children-Fifth Edition (WISC-5) needed to identify youth with Full Scale IQ (FSIQ) ≤75. METHOD: Data were obtained from a large pediatric clinically referred sample (N = 4,299; Mean Age = 10.7 years; Range = 6-16y; 66% male; 54% White; 29% receiving Public Insurance), divided into training (n = 2149) and test (n = 2150) samples. RESULTS: In the training sample, sequential and additive regression-based models for predicting FSIQ comprised of one (Block Design [BD]), two (BD + Similarities [SI]), three (BD + SI + Matrix Reasoning [MR]), and four (BD + SI + MR + Digit Span [DS]) subtests of the WISC-5 explained 61.3%, 82.7%, 88.5%, and 93.0% of FSIQ variance, respectively. Using a predicted FSIQ ≤ 80 as a cut score to identify persons with observed FSIQ ≤75, the two subtest (BD + SI) model showed strong sensitivity (83.4), specificity (90.5), and negative predictive value (96.2) in the test sample; however, positive predictive value was low (65.3%). Three and four subtest models provided incremental, but modest gains in classification metrics. CONCLUSIONS: Findings suggest the first several subtests of the WISC-5 can be used to identify clinically referred youth at risk for ID who subsequently require full administration of the WISC-5 for consideration of an ID diagnosis.

Concordance of the Adaptive Behavior Assessment System, second and third editions.

BACKGROUND: Adaptive functioning is an important area of assessment with implications for differential diagnosis, educational placement, service eligibility and criminal sentencing. While periodic normative and content updates of adaptive functioning measures are necessary to keep measures relevant, knowledge of equivalence between versions is also required if adaptive measures are to be used to track the stability of adaptive functioning skills over time. METHOD: This paper presents two studies that used between-group and within-group comparison designs to examine the equivalence of the second and third editions of the Adaptive Behavior Assessment System (ABAS) in a mixed clinical sample. In study 1, ABAS-2 scores for children assessed between 2014 and 2015 (n = 1036; mean age = 10.24, SD = 3.44) were compared with ABAS-3 scores for children assessed between 2015 and 2016 (n = 1291; mean age = 10.51, SD = 3.70). Study 2 examined a separate sample of clinically referred children (n = 572) for whom parent ratings had been obtained on both the ABAS-2 (mean age = 9.65, SD = 2.80) and ABAS-3 (mean age = 13.33, SD = 2.95) in the course of repeated assessment. RESULTS: For Study 1, while no intelligence quotient score differences were observed between the ABAS-2 group (mean Verbal Comprehension Index = 93.67, SD = 16.95) and the ABAS-3 group (mean Verbal Comprehension Index = 93.08, SD = 17.42), ABAS-2 scores were lower than ABAS-3 scores on the Conceptual, Practical, and General Adaptive Composite scales. In study 2, a similar pattern was observed (ABAS-2 < ABAS-3 on the Conceptual, Practical, and General Adaptive Composite scales), and concordance correlation coefficients ranged from 0.54 [0.49, 0.58] (Practical composite) to 0.68 [0.64, 0.72] (Conceptual composite). The Practical composite had the lowest concordance correlation coefficient value and the largest mean score difference between ABAS versions. CONCLUSIONS: The ABAS-3 scores may be higher than ABAS-2 scores in clinical populations. Knowledge of these potential discrepancies will be critical when interpreting standard score changes across ABAS versions in the course of clinical, educational and forensic assessments.

Caregiver Perspectives on Informed Consent for a Pediatric Learning Healthcare System Model of Care.

BACKGROUND: Data is needed to provide insight into the issue of preference around consent for use of pediatric clinical data for research. This study evaluated caregivers' preferences concerning use of their child's clinical information. METHODS: Caregivers of children (n = 101; response rate 81.5% of n = 124) presenting for psychological evaluation at an urban medical center viewed a video regarding how the information contained in their child's medical record could be used for research. RESULTS: An anonymous survey following the video indicated that: 1) >90% of caregivers felt comfortable with their child's information being used; 2) >90% of caregivers felt their child's privacy would be adequately protected; 3) 98% of caregivers reported themselves to be as or more likely to return to the institution after viewing the video; 4) 60% of caregivers felt no additional consent procedures beyond viewing the video were needed, while 20% preferred an opt-out and 20% preferred a traditional consent procedure. Caregiver demographic variables were largely unrelated to consent preferences. DISCUSSION: Overall, caregivers reported strong support for use of their child's clinical data for research purposes.

Pre-appointment online assessment of patient complexity: Towards a personalized model of neuropsychological assessment.

Recent events such as the global pandemic of COVID-19 have challenged neuropsychologists to scale up their capacity to conduct portions of their assessment remotely. While more complex patients will likely continue to require on-site, office-based interaction and assessment, the current emergency-based expansion of online and telehealth evaluation practices may ultimately lay the groundwork for more routine, online assessment of patients with less complex presentations in the future. To this end, the current study evaluated a pre-appointment, online methodology for differentiating referred pediatric patients based upon the scope and severity of their caregiver-reported adaptive, academic, attentional, behavioral, and emotional impairment. Prior to on-site assessment, parents/caregivers of 2197 children (Mean age = 10.0y, range = 4-19y, 62% male) completed an online developmental history form screening for symptoms of adaptive, attentional, learning, affective, and behavioral impairment; 71% of those children eventually underwent assessment. Using latent class analysis, the data supported a reproducible 4-class model consisting of groups of children at increased risk for: 1) severe multi-domain dysfunction; the "High Complexity" group, 30%, 2) behavioral-affective (but not academic) dysregulation; the "Behavioral Focus" group, 13%, 3) academic (but not behavioral-affective) problems; the "Academic and Inattention" group, 37%, and 4) patients with minimal clinical complexity; the "Low Complexity" group, 20%. Comparison of pre-visit classification with day-of-assessment standardized test scores supported the validity of patient subtypes. Moving forward, pre-appointment clarification of patient complexity may support efficient patient triage with regard to assessment modality (e.g., on-site or online) and length of appointment (e.g., comprehensive or targeted).

Sirolimus Treatment in Sturge-Weber Syndrome.

BACKGROUND: Sturge-Weber syndrome is a rare neurovascular disorder associated with capillary malformation, seizures, cognitive impairments, and stroke-like episodes (SLEs), arising from a somatic activating mutation in GNAQ. Studies suggest this mutation may cause hyperactivation of the mammalian target of rapamycin pathway. Sirolimus is an mammalian target of rapamycin inhibitor studied in other vascular anomalies and a potentially promising therapy in Sturge-Weber syndrome. METHODS: Ten patients with Sturge-Weber syndrome brain involvement and cognitive impairments were enrolled. Oral sirolimus was taken for six months (maximum dose: 2 mg/day, target trough level: 4-6 ng/mL). Neuropsychological testing, electroencephalography, and port-wine score were performed at baseline and after six months on sirolimus. Neuroquality of life, adverse events, and Sturge-Weber Syndrome Neurological Score (neuroscore) were recorded at each visit. RESULTS: Sirolimus was generally well tolerated; one subject withdrew early. Adverse events considered related to sirolimus were mostly (15/16) grade 1. A significant increase in processing speed was seen in the overall group (P = 0.031); five of nine patients with available data demonstrated statistically rare improvement in processing speed. Improvements were seen in the neuroquality of life subscales measuring anger (P = 0.011), cognitive function (P = 0.015), and depression (P = 0.046). Three subjects experiencing SLEs before and during the study reported shortened recovery times while on sirolimus. CONCLUSIONS: Sirolimus was well tolerated in individuals with Sturge-Weber syndrome and may be beneficial for cognitive impairments, especially in patients with impaired processing speed or a history of SLE. A future, randomized, placebo-controlled trial of sirolimus in patients with Sturge-Weber syndrome is needed to further understand these potentially beneficial effects.

Suicide Screening in Sturge-Weber Syndrome: An Important Issue in Need of Further Study.

BACKGROUND: Sturge-Weber syndrome is a neurocutaneous disorder associated with epilepsy, glaucoma, cognitive impairments, and a port-wine birthmark. Although individuals with Sturge-Weber syndrome are vulnerable to known risk factors for suicide, including chronic illness and physical differences (port-wine birthmark), frequency of suicidal ideation and attempts, and the clinical factors associated with suicide risk, in patients with Sturge-Weber syndrome is unknown. METHODS: As a part of routine hospital practice, all outpatients aged eight years and older underwent suicide risk screening during nursing triage using a standardized suicide screening tool. Suicide risk screening results, demographic variables, and medical history (as available) for patients with Sturge-Weber syndrome (N = 34; median age = 15.5; range = 8 to 47 years, 44% male) and other neurological conditions seen at the same institution (N = 369; median age = 14; range = 8 to 78 years, 66% male) were used for retrospective within- and between-group analysis. RESULTS: In the combined sample of Sturge-Weber syndrome and neurologically involved patients, a positive suicide risk screen was related to Sturge-Weber syndrome diagnosis (P = 0.043); analysis by sex showed increased risk of Sturge-Weber syndrome diagnosis in males (P = 0.008), but not in females. Within the Sturge-Weber syndrome group, use of a selective serotonin reuptake inhibitor (P = 0.019) was related to a positive risk screen. CONCLUSION: People with Sturge-Weber syndrome may be at greater risk of suicidal thoughts or behaviors than those with other neurological conditions. Further study of suicide risk in patients with Sturge-Weber syndrome is needed.

Utility of the ImPACT test with deaf adolescents.

OBJECTIVE: The goals of the study included empirical examination of the utility of the Immediate and Post-Concussion Assessment and Cognitive Testing (ImPACT) test with adolescents who are deaf or hard-of-hearing and to investigate patterns of performance at baseline that may arise in the assessment of this population. Baseline assessment of student-athletes has been conducted on a widespread scale with focus on performance of typically developing student-athletes and some clinical groups, though to date no studies have examined adolescents who are deaf or hard-of-hearing. METHOD: Retrospective and de-identified ImPACT baseline test used with deaf and hard-of-hearing high-school student-athletes (N = 143; 66% male, mean age = 16.11) was examined. RESULTS: Review indicated significant differences in some composite scores between the deaf and hard-of-hearing group and hearing normative comparisons. A possible marker of task misunderstanding was identified to occur more frequently within the deaf and hard-of-hearing sample (13% in deaf sample vs. .31% in hearing sample). CONCLUSIONS: Results may provide support for the consideration and use of additional measures to ensure comprehension of task demands when considering this tool for use with deaf and hard-of-hearing adolescents.

[Formula: see text]Intellectual and adaptive functioning in Sturge-Weber Syndrome.

The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.

AlGaAs/GaAs/InGaAs pnp-type vertical-cavity surface-emitting transistor-lasers.

We report on the design, fabrication and analysis of vertical-cavity surface-emitting transistor-lasers (T-VCSELs) based on the homogeneous integration of an InGaAs/GaAs VCSEL and an AlGaAs/GaAs pnp-heterojunction bipolar transistor (HBT). Epitaxial regrowth confinement, modulation doping, intracavity contacting and non-conducting mirrors are used to ensure a low-loss structure, and a variety of design variations are investigated for a proper internal biasing and current injection to ensure a wide operating range. Optimized devices show mW-range output power, mA-range base threshold current and high-temperature operation to at least 60°C with the transistor in its active mode of operation for base currents well beyond threshold. Current confinement schemes based on pnp-blocking layers or a buried tunnel junction are investigated as well as asymmetric current injection for reduced extrinsic resistances.

Depressive symptoms in adults with spina bifida.

OBJECTIVE: To examine the prevalence of depressive symptoms in adults with spina bifida and identify contributing factors for depressive symptomatology. METHOD: Retrospective Cohort Study. Data collection was conducted at a regional adult spina bifida clinic. A total of 190 charts from adult patients with spina bifida were included. The main outcome measures were the Beck Depression Inventory-II (BDI-II) and the mobility domain of the Craig Handicap Assessment Reporting Technique-Short Form (CHART-SF). RESULTS: Of the 190 participants, 49 (25.8%) had BDI-II scores (14+) indicative of depressive symptomatology. Sixty-nine (36.3%) were on antidepressants to treat depressive symptoms, and 31 (63.3%) of those with clinical symptoms of depression were on antidepressants. Participants with a history of depressive symptoms may be as high as 45.7% if both participants with BDI-II scores 14+ and those with antidepressant use specifically for the purposes of depression treatment are combined. In this population, lower CHART-SF mobility score, expressing "emotional concerns" as a reason for the visit on an intake sheet, and use of antidepressant medications were significantly associated with depressive symptoms. CONCLUSIONS: Depressive symptomatology appears to be common and undertreated in this cohort of adults with spina bifida, which may warrant screening for emotional concerns in routine clinic appointments. Significant depressive symptoms are associated with fewer hours out of bed and fewer days leaving the house. Additional research is needed to assess the impact of interventions directed toward mobility on depression and in the treatment of depression in this patient population.

Stimulant use in patients with sturge-weber syndrome: safety and efficacy.

BACKGROUND: Sturge-Weber syndrome is characterized by a facial port-wine birthmark, vascular eye abnormalities, and a leptomeningeal angioma. Attention and behavioral issues are common in Sturge-Weber syndrome. However, literature evidence for stimulant treatment is minimal. This study evaluates stimulant medication safety and efficacy in individuals with Sturge-Weber syndrome. METHODS: The research database of the Hunter Nelson Sturge-Weber Center (n = 210 subjects in the database) was reviewed for stimulant use. Twelve patients (mean age 10.5 years, age range 4 to 21 years) on stimulants were seen between 2003 and 2012. A retrospective chart review obtained comorbid diagnoses, stimulant type and dosage, medication side effects, vital signs, and medication efficacy. RESULTS: All 12 patients had brain involvement (unilateral, nine; bilateral, three). Additional comorbidities included epilepsy (twelve), hemiparesis (eight), headaches (eight), and vision deficits (six). Eight patients reported side effects, primarily appetite suppression (four) and headaches (three). There were no statistically significant changes in weight or blood pressure 6 months after medication initiation. Medication efficacy was subjectively reported in 11 patients. Seven patients remained on stimulants at their most recent follow-up visit. CONCLUSIONS: This study preliminarily evaluates stimulant medication use in a small group of Sturge-Weber syndrome patients. Stimulants were tolerated and effective in most subjects. Side effects were mostly minor and medication did not negatively affect growth or vital signs. Stimulant medication may be a safe and effective intervention for Sturge-Weber syndrome children with attention issues/attention deficit hyperactivity disorder. Further studies with larger sample sizes are needed.

To ID or not to ID? Changes in classification rates of intellectual disability using DSM-5.

The Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition (DSM-5) diagnostic criteria for intellectual disability (ID) include a change to the definition of adaptive impairment. New criteria require impairment in one adaptive domain rather than two or more skill areas. The authors examined the diagnostic implications of using a popular adaptive skill inventory, the Adaptive Behavior Assessment System-Second Edition, with 884 clinically referred children (ages 6-16). One hundred sixty-six children met DSM-IV-TR criteria for ID; significantly fewer (n  =  151, p  =  .001) met ID criteria under DSM-5 (9% decrease). Implementation of DSM-5 criteria for ID may substantively change the rate of ID diagnosis. These findings highlight the need for a combination of psychometric assessment and clinical judgment when implementing the adaptive deficits component of the DSM-5 criteria for ID diagnosis.