Quantitative Measurement of Communication Ability in Children with Angelman Syndrome.

BACKGROUND: Angelman syndrome is a rare disorder in which most individuals do not develop speech. Testing of communication ability using traditional neuropsychological measures reveals a performance level at or near the floor of the instrument resulting in an inability to detect change when experimental therapeutics are applied. METHODS: Nine individuals, with molecularly confirmed AS, ranging in age from 34 to 126 months, and a single healthy control child (age 16 months) were audio and video-recorded while interacting with a licensed speech-language pathologist in an attempt to elicit vocalization and non-verbal communication. Thirty-minute audio recordings were transcribed and categorized per the Stark Assessment of Early Vocal Development-Revised and a phonetic inventory was created. Using video recordings, gestures were classified by function, either behavioral regulation or social interaction and further categorized as deictic or representational (i.e., behavioral regulation) and joint attention or shared engagement (i.e., social interaction). RESULTS: The range of vocalizations produced by the children with AS was characteristic of children between 0-6 months and none of the children with AS used advanced forms of vocalizations. The mean frequency of reflexive vocalizations, control of phonation and expansion far exceeded the number of uses of canonical syllables, consistant with the characteristics of children around 12 months of age. Most vocalizations were either laughter or isolated vowels, only three children with AS produced consonant-vowel combinations. Children with AS tended to use central and low vowels with few producing high vowels, suggesting the presence of childhood apraxia of speech. CONCLUSION: Our results show the utilization of video-recorded behavioral observations provides a feasible and reliable alternative for quantification of communication ability in this patient population and may be employed during future clinical studies of potential therapeutics.

Awake High-Flow Extracranial to Intracranial Bypass for Complex Cerebral Aneurysms: Institutional Clinical Trial Results.

OBJECTIVE: Assess the potential added benefit to patient outcomes of "awake" neurological testing when compared with standard neurophysiologic testing performed under general endotracheal anesthesia. METHODS: Prospective study of 30 consecutive adult patients who underwent awake high flow extracranial to intracranial (HFEC-IC) bypass. Clinical neurological and neurophysiologic findings were recorded. Primary outcome measures were the incidence of stroke/cerebrovascular accident (CVA), length of stay, discharge to rehabilitation, 30-day modified Rankin scale score, and death. An analysis was also performed of a retrospective control cohort (n = 110 patients who underwent HFEC-IC for internal carotid artery (ICA) aneurysms under standard general endotracheal anesthesia). RESULTS: Five patients (16.6%) developed clinical awake neurological changes (4, contralateral hemiparesis; 1, ipsilateral visual changes) during the 10-minute ICA occlusion test. These patients had 2 kinks in the graft, 1 vasospasm, 1 requiring reconstruction of the distal anastomosis, and 1 developed blurring of vision that reversed after the removal of the distal permanent clip on the ICA. Three of these 5 patients had asynchronous clinical "awake" neurological and neurophysiologic changes. Two patients (7%) developed CVA. Median length of stay was 4 days. Twenty-eight of 30 patients were discharged to home. Median modified Rankin scale score was 1. There were no deaths in this series. Absolute risk reduction in the awake craniotomy group (n = 30) relative to control retrospective group (n = 110) was 7% for CVA, 9% for discharge to rehabilitation, and 10% for graft patency. CONCLUSIONS: Temporary ICA occlusion during HFEC-IC bypass for ICA aneurysms in conjunction with awake intraoperative clinical testing was effective in detecting a subset of patients (n = 3, 10%) in whom neurological deficit was not detected by neurophysiologic monitoring alone.

Biopsychosocial influence on shoulder pain: Rationale and protocol for a pre-clinical trial.

BACKGROUND: Chronic musculoskeletal pain conditions are a prevalent and disabling problem. Preventing chronic musculoskeletal pain requires multifactorial treatment approaches that address its complex etiology. Prior cohort studies identified a high risk subgroup comprised of variation in COMT genotype and pain catastrophizing. This subgroup had increased chance of heightened pain responses (in a pre-clinical model) and higher 12month post-operatives pain intensity ratings (in a clinical model). This pre-clinical trial will test mechanisms and efficacy of personalized pain interventions matched to the genetic and psychological characteristics of the high-risk subgroup. METHODS: Potential participants will be screened for high risk subgroup membership, appropriateness for exercise-induced muscle injury protocol, and appropriateness for propranolol administration. Eligible participants that consent to the study will then be randomized into one of four treatment groups; 1) personalized pharmaceutical and psychological education; 2) personalized pharmaceutical and general education; 3) placebo pharmaceutical and psychological education; 4) placebo pharmaceutical and psychological education. Over the 5-day study period participants will complete an exercise-induced muscle injury protocol and receive study interventions. Pain and disability assessments will be completed daily, with primary outcomes being duration of shoulder pain (number of days until recovery), peak shoulder pain intensity, and peak shoulder disability. Secondary outcomes include inflammatory markers, psychological mediators, and measures of pain sensitivity regulation. CONCLUSION: This pre-clinical trial builds on prior cohort studies and its completion will provide foundational data supporting efficacy and mechanisms of personalized interventions for individuals that may be at increased risk for developing chronic shoulder pain. TRIAL REGISTRATION: ClinicalTrials.gov registry, NCT02620579 (Registered on November 13, 2015).

Optimism Moderates the Influence of Pain Catastrophizing on Shoulder Pain Outcome: A Longitudinal Analysis.

Study Design Secondary analysis of prospectively collected data. Background An abundance of evidence has highlighted the influence of pain catastrophizing and fear avoidance on clinical outcomes. Less is known about the interaction of positive psychological resources with these pain-associated distress factors. Objective To assess whether optimism moderates the influence of pain catastrophizing and fear avoidance on 3-month clinical outcomes in patients with shoulder pain. Methods Data from 63 individuals with shoulder pain (mean ± SD age, 38.8 ± 14.9 years; 30 female) were examined. Demographic, psychological, and clinical characteristics were obtained at baseline. Validated measures were used to assess optimism (Life Orientation Test-Revised), pain catastrophizing (Pain Catastrophizing Scale), fear avoidance (Fear-Avoidance Beliefs Questionnaire physical activity subscale), shoulder pain intensity (Brief Pain Inventory), and shoulder function (Pennsylvania Shoulder Score function subscale). Shoulder pain and function were reassessed at 3 months. Regression models assessed the influence of (1) pain catastrophizing and optimism and (2) fear avoidance and optimism. The final multivariable models controlled for factors of age, sex, education, and baseline scores, and included 3-month pain intensity and function as separate dependent variables. Results Shoulder pain (mean difference, -1.6; 95% confidence interval [CI]: -2.1, -1.2) and function (mean difference, 2.4; 95% CI: 0.3, 4.4) improved over 3 months. In multivariable analyses, there was an interaction between pain catastrophizing and optimism (ß = 0.19; 95% CI: 0.02, 0.35) for predicting 3-month shoulder function (F = 16.8, R2 = 0.69, P<.001), but not pain (P = .213). Further examination of the interaction with the Johnson-Neyman technique showed that higher levels of optimism lessened the influence of pain catastrophizing on function. There was no evidence of significant moderation of fear-avoidance beliefs for 3-month shoulder pain (P = .090) or function (P = .092). Conclusion Optimism decreased the negative influence of pain catastrophizing on shoulder function, but not pain intensity. Optimism did not alter the influence of fear-avoidance beliefs on these outcomes. Level of Evidence Prognosis, level 2b. J Orthop Sports Phys Ther 2017;47(1):21-30. Epub 5 Nov 2016. doi:10.2519/jospt.2017.7068.