Full facial retransplantation in a female patient-Technical, immunologic, and clinical considerations.

There is limited experience with facial retransplantation (fRT). We report on the management of facial retransplantation in a facial vascularized composite allotransplant recipient following irreversible allograft loss 88 months after the first transplant. Chronic antibody-mediated rejection and recurrent cellular rejection resulted in a deteriorated first allograft and the patient underwent retransplantation. We summarize the events between the two transplantations, focusing on the final rejection episode. We describe the surgical technique of facial retransplantation, the immunological and psychosocial management, and the 6-month postoperative outcomes. Removal of the old allograft and inset of the new transplant were done in one operation. The donor and recipient were a good immunological match. The procedure was technically complex, requiring more proximal arterial anastomoses and an interposition vein graft. During the first and second transplantation, the facial nerve was coapted at the level of the branches. There was no hyperacute rejection in the immediate postoperative phase. Outcomes 6 months postoperatively are promising. We provide proof-of-concept that facial retransplantation is a viable option for patients who suffer irreversible facial vascularized composite allograft loss.

The cumulative incidence of dysphagia and dysphagia-free survival in persons diagnosed with amyotrophic lateral sclerosis.

INTRODUCTION/AIMS: Dysphagia worsens mortality and quality of life for persons diagnosed with amyotrophic lateral sclerosis (ALS), yet our understanding of its incidence and timing remains limited. In this study we sought to estimate dysphagia incidence and dysphagia-free survival over time. METHODS: Using data from the Pooled Resource Open-Access ALS Clinical Trials Database, we compared characteristics of persons with and without dysphagia upon study entry. To account for competing mortality risk, we used Kaplan-Meier curves to estimate the cumulative incidence of dysphagia and the median number of days until the development of dysphagia or death in those without dysphagia at study entry. RESULTS: Patients with dysphagia upon study entry were more likely to have bulbar onset and had faster rates of functional decline and shorter diagnostic delays. The cumulative incidence of new-onset dysphagia was 44% at 1 year and 64% at 2 years after trial enrollment for those with spinal onset, and 85% and 92% for those with bulbar onset. The median duration of dysphagia-free survival after trial enrollment was 11.5 months for those with spinal onset and 3.2 months for those with bulbar onset. DISCUSSION: Our findings underscore the high risk for dysphagia development and support the need for early dysphagia referral and evaluation to minimize the risk of serious dysphagia-related complications.

Biomechanical Biomarkers of Tongue Impairment During Swallowing in Persons Diagnosed with Amyotrophic Lateral Sclerosis.

BACKGROUND: The impact of tongue dysfunction on deglutition in persons diagnosed with amyotrophic lateral sclerosis (ALS) is not well understood. This information is needed to improve our understanding of the mechanisms of swallowing impairment, for identifying risk factors of dysphagia, and for establishing impairment-specific treatments aimed at slowing the loss of swallow function. OBJECTIVES: The goals of this study were to determine the relation between biomechanical measures of oral tongue movements using electromagnetic articulography (EMA) and measures of swallow physiology, swallow safety and efficiency, and self-reported swallowing function. METHODS: Participants were diagnosed with ALS by a neurologist following the El Escorial Criteria from the World Federation of Neurology. Twelve participants underwent (1) EMA to derive biomechanical measures of the tongue, (2) videofluoroscopic evaluation to measure swallow physiology, safety, and efficiency, and (3) maximal tongue strength testing using the Iowa Oral Pressure Instrument (IOPI). Participants completed self-reported functional assessments. Spearman's rank correlations assessed for associations between lingual biomechanics and swallowing physiology, swallow safety and efficiency, and self-reported bulbar function. RESULTS: Results demonstrated strong associations between biomechanical and swallowing physiology, swallow safety, and self-reported measures. Notably, swallowing safety during thin liquid intake was associated with tongue speed (r = - 0.7, p < 0.05) and range of motion (r = - 0.71, p < 0.05), and swallowing safety during puree intake was associated with tongue strength (r = - 0.69, p < 0.05). CONCLUSIONS: Our findings underscore the importance of tongue movements on swallowing physiology and safety, help improve our understanding of mechanisms of swallowing impairment, and highlight a potential clinical tool to index bulbar impairment.

Provisional best practices guidelines for the evaluation of bulbar dysfunction in amyotrophic lateral sclerosis.

INTRODUCTION: Universally established comprehensive clinical bulbar scales objectively assessing disease progression in amyotrophic lateral sclerosis (ALS) are currently lacking. The goal of this working group project is to design a best practice set of provisional bulbar ALS guidelines, available for immediate implementation within all ALS clinics. METHODS: ALS specialists across multiple related disciplines participated in a series of clinical bulbar symposia, intending to identify and summarize the currently accepted best practices for the assessment and management of bulbar dysfunction in ALS Results: Summary group recommendations for individual speech, Augmentative and Alternative Communication (AAC), and swallowing sections were achieved, focusing on the optimal proposed level of care within each domain. DISCUSSION: We have identified specific clinical recommendations for each of the 3 domains of bulbar functioning, available for incorporation within all ALS clinics. Future directions will be to establish a formal set of bulbar guidelines through a methodological and evidence-based approach. Muscle Nerve 59:531-531, 2019.

Lingual and Jaw Kinematic Abnormalities Precede Speech and Swallowing Impairments in ALS.

Early identification of bulbar involvement in persons with ALS is critical for improving diagnosis and prognosis; however, efficacious diagnostic markers have not yet been identified. The purpose of this study was to determine whether kinematic changes of the tongue and jaw during swallowing, measured using 3D electromagnetic articulography (EMA), predate clinically identifiable symptoms of speech and swallowing impairment in persons diagnosed with ALS. Data were collected from 16 adults diagnosed with ALS and 18 neurotypical controls. Groups were aged matched. Eligible participants with ALS were tolerating an unrestricted diet (FOIS = 7), produced intelligible speech (> 97%), and had a speaking rate greater than 150 words per minute. Participants completed a 3-mL water swallow task, during which EMA recorded kinematic measures of the anterior and posterior regions of tongue including lingual speed, range of motion, duration, coordination, and efficiency. Jaw speed and range of motion were also recorded. Persons diagnosed with ALS demonstrated reduced posterior lingual range of motion (11.40 mm ± 4.01 vs. 16.07 mm ± 5.27), slower posterior lingual speeds (83.67 mm/s ± 47.96 vs. 141.35 mm/s ± 66.54), increased lingual movement duration (13.46 s ± 6.75 vs. 9.21 s ± 3.28), and reduced lingual coordination (0.04 s ± 0.11 vs. 17 s ± 0.19) during the 3-oz water swallow task compared to controls. Persons diagnosed with ALS demonstrated increased range of motion (9.86 mm ± 5.38 vs. 6 mm ± 3.78) and increased jaw speed (68.62 mm/s ± 50.13 vs. 34.72 mm/s ± 17.75) during swallowing compared to controls. The current findings suggest that changes in lingual and jaw motor performance during a simple water swallow task are present in persons with ALS who are pre-symptomatic of clinically detectable bulbar impairment.

Trismus in Face Transplantation Following Ballistic Trauma.

BACKGROUND: Trismus can be a challenging consequence of ballistic trauma to the face, and has rarely been described in the setting of face transplantation. Almost half of all current face transplant recipients in the world received transplantation to restore form and function after a ballistic injury. Here we report our experience and challenges with long standing trismus after face transplantation. METHODS: We reviewed the medical records of our face transplant recipients whose indication was ballistic injury. We focused our review on trismus and assessed the pre-, peri- and postoperative planning, surgery and functional outcomes. RESULTS: Two patients received partial face transplantation, including the midface for ballistic trauma. Both patients suffered from impaired mouth opening, speech intelligibility, and oral competence. Severe scarring of the temporomandibular joint (TMJ) required intraoperative release in both patients, and additional total condylectomy on the left side 6 months posttransplant for 1 patient. Posttransplant, both patients achieved an improvement in mouth opening; however, there was persistent trismus. One year after transplantation, range of motion of the jaw had improved for both patients. Independent oral food intake was possible 1 year after surgery, although spillage of liquids and mixed consistency solids persisted. Speech intelligibility testing showed impairments in the immediate postoperative period, with improvement to over 85% for both patients at 1 year posttransplant. CONCLUSIONS: Ballistic trauma to the face and subsequent reconstructive measures can cause significant scarring and covert injuries to structures such as the TMJ, resulting in long standing trismus. Meticulous individual planning prior to interventions such as face transplantation must take these into account. We encourage intraoperative evaluation of these structures as well as peri- and postoperative treatment when necessary. Due to the nature of the primary injury, functional outcomes after face transplantation in these patients may differ substantially from those of other indications.

Alcohol Use and Problems in Daily and Non-Daily Coffee Drinking College Females.

BACKGROUND: For more than a decade, a large proportion of research on caffeine use in college students has focused on energy drinks (ED), demonstrating an association between ED consumption and heavy/problem alcohol use. The present study examined the relationship between daily coffee consumption and varied measures of alcohol use and problems in a sample of college women. METHODS: Participants were undergraduate females (N=360) attending an urban university in 2001-02 and prior to the rise in ED popularity on college campuses. Analyses compared women who reported drinking coffee daily (DC; 16.9%), to women who did not (NDC; 83.1%) on standardized measures of alcohol use and problems. RESULTS: For both past month and year of drinking, DC women generally reported consuming more alcohol and were 2.1-2.6 times more likely to screen at risk for alcohol problems than their NDC counterparts. DC women were also more likely than NDC women to report problems related to drinking (e.g., experiencing blackouts, inability to stop drinking after they had started). CONCLUSIONS: Findings support potential benefits of health education and screening that goes beyond EDs, focusing on varied forms of caffeine consumption.

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.

Individualized white matter connectivity of the articulatory pathway: An ultra-high field study.

In current sensorimotor theories pertaining to speech perception, there is a notable emphasis on the involvement of the articulatory-motor system in the processing of speech sounds. Using ultra-high field diffusion-weighted imaging at 7 Tesla, we visualized the white matter tracts connected to areas activated during a simple speech-sound production task in 18 healthy right-handed adults. Regions of interest for white matter tractography were individually determined through 7T functional MRI (fMRI) analyses, based on activations during silent vocalization tasks. These precentral seed regions, activated during the silent production of a lip-vowel sound, demonstrated anatomical connectivity with posterior superior temporal gyrus areas linked to the auditory perception of phonetic sounds. Our study provides a macrostructural foundation for understanding connections in speech production and underscores the central role of the articulatory motor system in speech perception. These findings highlight the value of ultra-high field 7T MR acquisition in unraveling the neural underpinnings of speech.

Oral diadochokinetic markers of X-linked dystonia-parkinsonism.

INTRODUCTION: X-linked dystonia-parkinsonism (XDP) is a neurodegenerative disorder that may result in severe speech impairment. The literature suggests that there are differences in the speech of individuals with XDP and healthy controls. This study aims to examine the motor speech characteristics of the mixed dystonia-parkinsonism phase of XDP. METHOD: We extracted acoustic features representing coordination, consistency, speed, precision, and rate from 26 individuals with XDP and 26 controls using Praat, MATLAB, and R software. Group demographics were compared using descriptive statistics. A one-way analysis of variance (ANOVA) with Tukey's post hoc test was used to test for acoustic differences between the two groups. RESULTS: The XDP group had significantly lower consistency, speed, precision, and rate than controls (p < 0.05). For coordination, the XDP group had a smaller ratio of pause duration during transitions when compared to controls. DISCUSSION: To our knowledge, this study is the first to describe the motor speech characteristics of the mixed dystonia-parkinsonism phase of XDP. The motor speech of mixed dystonia-parkinsonism XDP is similar to prior characterizations of mixed hyperkinetic-hypokinetic dysarthria with noted differences in articulatory coordination, consistency, speed, precision, and rate from healthy controls. Identifying the motor speech components of all three phenotypes of XDP (i.e., dystonia-dominant phase, parkinsonism-dominant phase, and mixed dystonia-parkinsonism phase) is needed to establish markers of speech impairment to track disease progression.

Facial Expression after Face Transplant: An International Face Transplant Cohort Comparison.

BACKGROUND: Assessment of motor function restoration following face transplant (FT) is difficult, as standardized, bilateral tests are lacking. This study aims to bolster support for software-based analysis through international collaboration. METHODS: FaceReader (Noldus, Wageningen, The Netherlands), a facial expression analysis software, was used to analyze posttransplant videos of eight FT patients from Boston, Massachusetts (range, 1 to 9 years after transplant), two FT patients from Helsinki, Finland (range, 3 to 4 years after transplant), and three FT patients from Antalya, Turkey (range, 6.5 to 8.5 years after transplant). Age-matched healthy controls from respective countries had no history of prior facial procedures. Videos contained patients and controls performing facial expressions evaluated by software analysis using the Facial Action Coding System. Facial movements were assigned intensity score values between 0 (absent) and 1 (fully present). Maximum values were compared with respective healthy controls to calculate percentage restoration. RESULTS: Of 13 FT patients, eight patients were full FT, five patients were partial FT, and two patients were female patients. Compared with healthy controls, the median restoration of motor function was 36.9% (interquartile range, 28.8% to 52.9%) for all patients with FT ( P = 0.151). The median restoration of smile was 37.2% (interquartile range, 31.5% to 52.7%) for all patients with FT ( P = 0.065). When facial nerve coaptation was performed at the distal branch level, average motor function restoration was 42.7% ± 3.61% compared with 27.9% ± 6.71% at the proximal trunk coaptation level ( P = 0.032). Use of interpositional nerve grafts had no influence on motor outcomes. CONCLUSIONS: Software-based analysis is suitable to assess motor function after FT. International collaboration strengthens outcome data for FT. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Establishing a natural history of X-linked dystonia parkinsonism.

X-linked dystonia parkinsonism is a neurodegenerative movement disorder that affects men whose mothers originate from the island of Panay, Philippines. Current evidence indicates that the most likely cause is an expansion in the TAF1 gene that may be amenable to treatment. To prepare for clinical trials of therapeutic candidates for X-linked dystonia parkinsonism, we focused on the identification of quantitative phenotypic measures that are most strongly associated with disease progression. Our main objective is to establish a comprehensive, quantitative assessment of movement dysfunction and bulbar motor impairments that are sensitive and specific to disease progression in persons with X-linked dystonia parkinsonism. These measures will set the stage for future treatment trials. We enrolled patients with X-linked dystonia parkinsonism and performed a comprehensive oromotor, speech and neurological assessment. Measurements included patient-reported questionnaires regarding daily living activities and both neurologist-rated movement scales and objective quantitative measures of bulbar function and nutritional status. Patients were followed for 18 months from the date of enrollment and evaluated every 6 months during that period. We analysed a total of 87 men: 29 were gene-positive and had symptoms at enrollment, seven were gene-positive and had no symptoms at enrollment and 51 were gene-negative. We identified measures that displayed a significant change over the study. We used principal variables analysis to identify a minimal battery of 21 measures that explains 67.3% of the variance over the course of the study. These measures included patient-reported, clinician-rated and objective quantitative outcomes that may serve as endpoints in future clinical trials.

Predicting dysphagia onset in patients with ALS: the ALS dysphagia risk score.

Purpose: For patients diagnosed with ALS, dysphagia can result in aspiration, malnutrition, and mortality. The purpose of this study was to develop a clinical prediction model capable of identifying patients with ALS at imminent risk for developing swallowing complications. Methods: A retrospective cohort study using the Pooled Resource Open-Access ALS Clinical Trials Database (PRO-ACT) was conducted. After dividing the PRO-ACT database into development and validation cohorts with dysphagia defined from the ALS Functional Rating Scale (ALSFRS), a multivariable Cox proportional hazards regression model estimated the probability of dysphagia at 3, 6, and 12-months with subsequent evaluation of model discrimination and calibration. Results: With 2057 participants in the development cohort and 1891 in the validation cohort, the Cox model included 7 clinical variables: spinal-onset; bulbar, fine and gross motor ALSFRS subscale scores; respiratory impairment; functional progression rate; and time from diagnosis. The cumulative incidence of dysphagia was 18% at 3-months, 29% at 6-months, and 45% at 12-months. The mean predicted probability of dysphagia development ranged from 4.5% in the bottommost risk decile to 40% in the topmost decile at 3 months, 10%-72% at 6 months, and 25%-93% at 12 months. In the validation cohort, the model had good discrimination and calibration with an optimism corrected c-statistic of 0.70 and calibration slope of 0.96. Conclusions: The ALS dysphagia risk score can be used to identify patients with ALS at high risk for self-reported dysphagia development who would benefit from a comprehensive swallowing assessment and proactive dysphagia management strategies.

Licit Substance Use and Premenstrual Syndrome Symptom Severity in Female College Students.

Introduction: Premenstrual syndrome (PMS) affects the majority of women and is characterized by physical, behavioral, and mood symptoms, which can have a profound impact on quality of life. PMS symptoms have also been linked to licit substance use. This study examined the relationships between daily/problem use (DPU) of caffeine (Caf+), alcohol (Alc+), and tobacco (Cig+) and PMS symptomology in a sample of college women. Methods: Participants (N = 196) completed an anonymous one-time health survey. Demographic, PMS symptomatology, and DPU of licit substance variables were examined. Independent t-tests compared PMS symptom scores in women with and without Caf+, Cig+, and Alc+ use. One-way analysis of variances examined the associations between PMS symptom severity and number of DPU-positive substances. Results: PMS subscale severity (pain [F(2,190) = 4.47, p = 0.013], affective [F(2,192) = 8.21, p < 0.001], and water retention [F(2,191) = 13.37, p < 0.001]) and total PMS symptom severity [F(2,189) = 10.22, p < 0.001] showed a dose response effect, with the number of licit substances with DPU significantly associated with PMS symptom severity. Conclusions: This study findings provide important new information about the relationship between PMS symptoms and at-risk substance use. These are cross-sectional data, however, and affirm a need for longitudinal research to better understand the associations, with a focus on potential benefits of education and intervention.

Longitudinal Recovery of Speech Motor Function Following Facial Transplantation: A Prospective Observational Study.

OBJECTIVES: Although facial transplantation is considered effective for restoring facial appearance, research on speech outcomes following surgery is limited. More research is critically needed to inform patients of expected rates and extent of recovery, and to develop interventions aimed at improving speech outcomes. METHODS: Four patients in early recovery (3 weeks-24 months postsurgery) and three patients in late recovery (36-60 months postsurgery) were included. Clinical measures of speech recovery, including speech intelligibility measured using the Sentence Intelligibility Test, a lip strength testing device (Iowa Oral Performance Instrument), and kinematic measures of lip and jaw function measured using high-resolution 3D optical motion capture were used to describe the rate and extent of functional speech and lip recovery, describe and compare the rate of functional speech recovery and kinematic lip and jaw changes in early and late stages of recovery, and explore the association between kinematic measures and functional speech. RESULTS: Speech intelligibility, speaking rate, and lip strength were below normative values in the first 2 years of postsurgery. Participants in the first 2 years of recovery demonstrated steeper slopes of improvement in clinical and kinematic measures than participants in the later stages of recovery (36-64 months). Gains in jaw range of movement and gains in lip speed and range of movement were significantly correlated with rates of sentence intelligibility improvement. Gains in lip strength were not associated with functional speech improvement. CONCLUSIONS: These findings motivate ongoing work aimed at developing interventions for improving motor speech function in this population. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:2359-2367, 2022.

Rate of speech decline in individuals with amyotrophic lateral sclerosis.

Although speech declines rapidly in some individuals with amyotrophic lateral sclerosis (ALS), longitudinal changes in speech have rarely been characterized. The study objectives were to model the rate of decline in speaking rate and speech intelligibility as a function of disease onset site, sex, and age at onset in 166 individuals with ALS; and estimate time to speech loss from symptom onset. We also examined the association between clinical (speaking rate/intelligibility) measures and patient-reported measures of ALS progression (ALSFRS-R). Speech measures declined faster in the bulbar-onset group than in the spinal-onset group. The rate of decline was not significantly affected by sex and age. Functional speech was still maintained at 60 months since disease onset for most patients with spinal onset. However, the time to speech loss was 23 months based on speaking rate < 120 (w/m) and 32 months based on speech intelligibility < 85% in individuals with ALS-bulbar onset. Speech measures were more responsive to functional decline than were the patient-reported measures. The findings of this study will inform future work directed toward improving speech prognosis in ALS, which is critical for determining the appropriate timing of interventions, providing appropriate counseling for patients, and evaluating functional changes during clinical trials.

Escherichia coli Survival on Strawberries and Unpacked Romaine Lettuce Washed Using Contaminated Water.

A number of foodborne outbreaks have occurred in the past decade, with higher incidences associated with romaine lettuce and strawberries. Contaminated agricultural water has been reported as the source of microbial contamination in most of these outbreaks. Maintaining the adequate and sanitary quality (0 E. coli/100 mL) of agricultural water can be challenging during post-harvest operations such as washing. The study focused on the attachment of generic E. coli (Rifampicin resistant) onto romaine lettuce and strawberries, mimicking the produce wash step. The produce was washed with contaminated water, air-dried, and stored in display units for 7 days. The produce was sampled randomly each day and analyzed for the surviving E. coli count. The results indicated that E. coli can survive in both lettuce and strawberries over extended periods. A survival population of 2.3 log CFU/cm2 (day 8) was observed on lettuce with an initial population of 2.8 log CFU/cm2 (day 0). On strawberries, the population reduced from 3.0 (day 0) to 1.7 log CFU/cm2 (day 7), with an initial E. coli concentration of approx. 6 log CFU/mL in the wash water. Strawberry leaves had a higher attachment of E. coli than the fruit (p < 0.05). In conclusion, romaine lettuce and strawberries washed with contaminated water can cause an outbreak affecting consumers and public health.

Determining the Potential Food Safety Risks Associated with Dropped Produce on Floor Surfaces in Grocery Stores.

ABSTRACT: Grocery stores handle fresh produce in large quantities daily. According to the Food and Drug Administration Food Code, food is to be stored at least 15 cm above the floor, and all foods shall be protected from any source of contamination or otherwise discarded. It is reported in the literature that dropped produce could be a potential source of microbial contamination. Both consumers and employees often drop produce on the floor and then place it back into a display case or bin, which could potentially serve as a source of contamination. This study aims to determine the bacterial transfer rate on different produce types when dropped for various contact times onto floor surfaces contaminated with Listeria monocytogenes. Apples, peaches, and romaine lettuce were dropped separately onto carpet and tile surfaces from a distance of 1 m and held for 5 s, 1 min, 10 min, 1 h, and 4 h. Results showed that transfer from all produce types occurred from both the carpet (10.56%) and tile (3.65%) surfaces. Still, percent transfer was not statistically significant among different times used in this study (P > 0.05). Dropped romaine lettuce had the most transfer (28.97%) from both the surfaces combined, followed by apples (8.80%) and peaches (7.32%) with minimal transfer. Even with a low transfer level, grocery stores should include signage to alert consumers not to pick up dropped produce and should train their employees accordingly.