Bridget Perry

Predicting Speech Intelligibility Decline in Amyotrophic Lateral Sclerosis Based on the Deterioration of Individual Speech Subsystems.

Purpose To determine the mechanisms of speech intelligibility impairment due to neurologic impairments, intelligibility decline was modeled as a function of co-occurring changes in the articulatory, resonatory, phonatory, and respiratory subsystems. Method Sixty-six individuals diagnosed with amyotrophic lateral sclerosis (ALS) were studied longitudinally. The disease-related changes in articulatory, resonatory, phonatory, and respiratory subsystems were quantified using multiple instrumental measures, which were subjected to a principal component analysis and mixed effects models to derive a set of speech subsystem predictors. A stepwise approach was used to select the best set of subsystem predictors to model the overall decline in intelligibility. Results Intelligibility was modeled as a function of five predictors that corresponded to velocities of lip and jaw movements (articulatory), number of syllable repetitions in the alternating motion rate task (articulatory), nasal airflow (resonatory), maximum fundamental frequency (phonatory), and speech pauses (respiratory). The model accounted for 95.6% of the variance in intelligibility, among which the articulatory predictors showed the most substantial independent contribution (57.7%). Conclusion Articulatory impairments characterized by reduced velocities of lip and jaw movements and resonatory impairments characterized by increased nasal airflow served as the subsystem predictors of the longitudinal decline of speech intelligibility in ALS. Declines in maximum performance tasks such as the alternating motion rate preceded declines in intelligibility, thus serving as early predictors of bulbar dysfunction. Following the rapid decline in speech intelligibility, a precipitous decline in maximum performance tasks subsequently occurred.

Surgical Optimization of Motor Recovery in Face Transplantation.

Background:Face transplantation (FT) has emerged as a viable option for treating devastating facial injuries. Most reported outcomes have demonstrated satisfactory motor and sensory restoration despite differences in technique. The authors have developed an algorithm of facial nerve management in these challenging patients. Our principles of management are illustrated by 2 specific patients. Methods:A retrospective analysis of prospectively collected data on 2 full face transplants was performed. Both patients required nerve grafting during full FT. Patient 1 due to short donor facial nerve stumps and patient 2 due to intraoperative soft tissue swelling. Patient 2 required a nerve transfer 11 months after full FT due to impaired motor recovery opposite the side of nerve grafting. Follow-up examinations consisting of manual muscle testing and Sunnybrook Facial Grading System 6 to 42 months after full FT with selected video examinations were critically reviewed. Results:Patient 1 had symmetrical motor recovery with gradual improvements noted throughout. At 6 months, Patient 2 had asymmetrically improving motor function. After nerve transfer, the patient showed gradual improvement in motor recovery, symmetry, and tone. Videos for each patient demonstrate the evolution of the patients’ ability to smile from 6 to 42 months. Discussion:The authors describe their assessment of motor recovery and management of facial nerve reconstruction as it pertains to FT. Finally, the authors illustrate the principles of nerve transfer are applicable to FT recipients.

The Effects of Lip-Closure Exercise on Lip Strength and Function Following Full Facial Transplantation: A Case Report

Purpose Facial transplantation is a relatively new option for individuals with severe facial disfigurements. Clinical case studies on existing patients indicate many instances of persistent facial motor impairment for facial expression, speech, and swallowing. These preliminary findings motivate additional research on the impact of lip-strengthening exercises following facial transplantation. Method In this study, we assessed the efficacy of an 8-week, biofeedback-driven, lip closure-strengthening exercise program in a single patient 1-year status post-full facial transplantation. Exercise was at 60% of peak strength. Outcome measures included instrumental measures of lip strength and mobility, clinical measures of speech, and patient-reported outcomes in feeding and facial expression. Results Results revealed improvements in labial strength, speed of lip movement, and range of motion during speech. A 3-point improvement in sentence speech intelligibility was also observed following strength-training exercise. The patient reported improvements in her ability to drink from a straw and communicate via facial expression. Conclusion These preliminary findings motivate additional research on the efficacy of lip-strengthening exercises following facial transplantation.

The role of face transplantation in the self-inflicted gunshot wound

BACKGROUND Facial self-inflicted gunshot wounds (SIGSWs) cause a devastating midfacial defect and pose a challenging problem to the reconstructive surgeon. Face transplantation (FT) has the potential for near-normal restoration in otherwise non-reconstructible defects. Two out of 7 FT recipients at Brigham and Womens Hospital (BWH) sustained SIGSWs. In this study, we illustrate the role of FT in the management of SIGSWs through an aesthetic, functional, and psychosocial examination of outcomes. METHODS We performed a retrospective analysis of individuals with SIGSWs who were screened at BWH between 2008 and 2015. We then collected data of the injuries, modes of conventional reconstruction (CR), and deficits. For the FT recipients, we critically reviewed the psychosocial screening process and post-transplantation aesthetic, functional, and psychosocial outcomes. RESULTS A total of six individuals post-SIGSWs were screened for FT. All of them had undergone CR, with five receiving loco-regional flaps and free tissue transfers, and one undergoing serial debridement and primary soft-tissue repair. Following CR, all suffered from residual functional and aesthetic deficits. Two underwent partial FT and one is currently undergoing FT screening. We describe the pre-transplant psychosocial screening process and the aesthetic, functional, and psychosocial outcomes of the SIGSW FT recipients. CONCLUSIONS We examined the facial SIGSW injury, outcomes of CR, and the mechanism of FT to offer a potential solution to the shortcomings of CR. More importantly, we highlight the critical nature of the psychosocial component of the multidisciplinary evaluation given the history of mental illness and suicidal behavior in this subset of patients.

Follow-Up on Functional Outcomes of Face Transplantation.

Material and Methods: Between 2005 and 2015, 32 FTs were performed worldwide. We defined functional outcomes of FT as the ability to smell, breathe, eat, speak, generate facial expressions and experience facial sensation. We performed physical examination, phography/video recordings and imaging-based airway volume measurements on the 7 FT recipients at our institution. We quote functional outcomes of the remaining 25FT from peer-reviewed literature.

Best practices protocol for the evaluation of bulbar dysfunction: summary recommendations from the NEALS bulbar subcommittee symposium

Abstract Objective: The aim of this Symposium was to develop a consensus based, bulbar assessment protocol for implementation within NEALS clinics. Methods: A one-day symposium, held in April 2017, was organized into Speech and Swallowing sections to establish summary recommendations for the assessment of bulbar dysfunction within each group. Results: Summary recommendations included speech referrals and AAC evaluations at initial visit, CNS-BFS, maximum sustained phonation, and speaking rate. Dysarthria evaluation included the speech subsystem involvement of respiration, phonation, resonance, and articulation. Specific recommendations for swallowing were established for each of the following domains: dietary/oral intake, airway defense physiologic capacity, swallow safety screen, patient-reported swallow-related outcomes, oral sensorimotor exam, and pulmonary function. Practice parameters focused upon patient education and unresolved questions included the use of videofluoscopy, monitoring diet progression, and swallow safety screening. Conclusions: The working goal is to establish a clinical bulbar protocol, designed to be incorporated within ALS clinics and ultimately to formulate a best practice set of bulbar ALS guidelines, available for implementation throughout the international ALS community.

2579: Face transplantation and the reconstructive management of the self-inflicted gunshot wound: An aesthetic, functional, and psychosocial report of 6 clinical cases

2579: Face transplantation and the reconstructive management of the self-inflicted gunshot wound: An aesthetic, functional, and psychosocial report of 6 clinical cases Harriet Kiwanuka, Mario A. Aycart, MD, David Gitlin, MD, Elaine Devine, LICSW, Bridget Perry, MS, Thet Su Win, MD, PhD, Ericka M. Bueno, Muayyad Alhefzi, Nicco Krezdorn, and Bohdan Pomahac, MD Brigham and Women’s Hospital, Boston, MA, USA; Mass General Institute of Health Professions, Boston, MA, USA Background Self-inflicted gunshot wounds to the face cause devastating midfacial defects, and pose a challenge to the reconstructive surgeon Face transplantation has the potential to provide near-normal restoration in otherwise non-reconstructable defects Two out of 7 face transplant recipients at our institution had sustained self-inflicted gunshot wounds We illustrate the role of face transplantation in the management of self-inflicted gunshot wounds through an aesthetic, functional, and psychosocial examination of outcomes. Methods We performed a retrospective analysis of individuals with self-inflicted gunshot wounds who were screened for inclusion in our face transplantation protocol between 2008 and 2015. We collected data describing the patients’ injuries, modes of conventional reconstruction, and deficits thereafter For those who received face transplants, we critically reviewed the psychosocial screening process and post-transplantation aesthetic, functional, and psychosocial outcomes. Results Six individuals postself-inflicted gunshot wounds were screened for face transplantation All had undergone conventional reconstruction, with 5 receiving loco-regional flaps and free tissue transfers, and one undergoing serial debridement and primary soft tissue repair All suffered from residual functional and aesthetic deficits Two underwent partial face transplantation at our center and one is currently undergoing screening We describe the pre-transplant psychosocial screening process and the aesthetic, functional, and psychosocial outcomes of the self-inflicted gunshot wound transplant recipients. Conclusions We examined the injury imparted by self-inflicted gunshot wounds to the face, outcomes of conventional reconstruction, and how face transplantation offers a potentially superior solution. More importantly, we highlight the critical nature of the psychosocial component of the multidisciplinary evaluation given the history of mental illness and suicidal behavior in this subset of patients CONTACT Harriet Kiwanuka hkiwanuka@partners.org Color versions of one or more of the figures in the article can be found online at www.tandfonline.com/kvca.