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The purpose of this study was to examine the effectiveness, satisfaction, and acceptance of a low-cost Lombard-response (LR) device in a group of individuals with Parkinson’s disease (IWPD) and their communication partners (CPs).
Sixteen IWPD and hypophonia and their CPs participated in the study. The IWPD wore a LR device that included a small MP3 player (Sony Walkman) and headphones playing a multi-talker noise audio file at 80 dB during lab-based speech tasks and during their daily conversational speech over a 2-week device trial period. Outcome measures included average conversational speech intensity and scores on a questionnaire related to speech impairment, communication effectiveness, and device satisfaction.
Conversational speech intensity of the IWPD is increased by 7 to 10 dB with the LR device. Following a 2-week trial period, eight of the IWPD (50%) gave the LR device moderate-to-high satisfaction and effectiveness ratings and decided to purchase the device for long-term daily use. At the 4-month follow-up, none of the IWPDs were still using the LR device. Device rejection was related to discomfort (loudness), headaches, interference with cognition, and difficulty controlling device.
Short-term acceptance and satisfaction with the LR device was moderate, but long-term acceptance, beyond 4 months, was absent. Future studies are required to determine if other types of low-cost LR devices can be developed that improve long-term efficacy and device acceptance in IWPD and hypophonia.
Background: There is a significant need for a targeted therapy for essential tremor (ET), as medications have not been developed specifically for ET, and the ones prescribed are often not well-tolerated, so that many patients remain untreated. Recent work has shown that, unlike previous experience, kinematically guided individualized botulinum toxin type A (BoNT-A) injections provide benefit along with minimal weakness. Ours is the first long-term (96-week) safety and efficacy study of BoNT-A as monotherapy for ET using kinematically driven injection parameters. Methods: Ten ET patients were administered six serial BoNT-A treatments every 16 weeks and were assessed at 6 weeks following treatment. During each study visit, the Fahn–Tolosa–Marin (FTM) scale, the Unified Parkinson’s Disease Rating Scale, and the Quality of Life for Essential Tremor Questionnaire (QUEST) were administered along with kinematic assessment of the treated limb. Participants performed scripted tasks with motion sensors placed over each arm joint. Dosing patterns were determined using the movement disorder neurologist’s interpretation of muscles contributing to the kinematically analyzed upper limb tremor biomechanics. Results: There was a 33.8% (p<0.05) functional improvement (FTM part C) and a 39.8% (p<0.0005) improvement in QUEST score at week 96 compared to pretreatment scores at week 0. Although there was a 44.6% (p<0.0005) non-dose-dependent reduction in maximal grip strength, only 2 participants complained of mild weakness. Following the fourth serial treatment, mean action tremor score was reduced by 62.9% (p=0.001) in the treated and by 44.4% (p=0.03) in the untreated arm at week 96 compared to week 48. Conclusions: Individualized BoNT-A dosing patterns to each individual’s tremor biomechanics provided an effective monotherapy for ET as function improved without functionally limiting muscle weakness.
Background: Hereditary hemochromatosis (HH) is a genetic disorder causing pathological iron deposition and functional impairment of various organs, predominantly the liver. We assessed patients with HH for the presence of movement disorders. Methods: We reviewed the charts of 616 patients with HH who attended hemochromatosis clinic at London Health Sciences Centre, London, ON, Canada, from 1988 to 2015. Results: We found three HH patients with movement disorders, without any other major systemic manifestation. One had parkinsonism, another had chorea, and the third had tremor. All three patients had evidence of iron deposition in the brain, affecting the basal ganglia in the first two, and the dentate nucleus, red nucleus, and substantia nigra in the third patient. In addition to the C282Y homozygous mutation in the HFE gene, two of our patients had non-HFE gene mutations. Conclusion: HH should be considered in the differential diagnosis of movement disorders with pathological brain iron deposition. We report for the first time chorea in a patient with HH. Non-HFE gene mutations may predispose HH patients to iron deposition in the brain.
Background Recent studies have strongly indicated the benefits of endovascular therapy for acute ischemic stroke, but what remains a continued debate is the role for general anaesthesia versus conscious sedation (CS) for such procedures. Retrospective studies have found poorer neurological outcomes in patients who underwent general anesthesia (GA); however, some have revealed worse baseline stroke severity in these patients.Methods This study is a retrospective cohort study aimed at comparing mortality and morbidity of GA versus CS in patients treated with endovascular intervention in acute ischemic stroke. Chi-square and t-test analyses were used. Results Patients in the GA (n=42) group were more likely to be deceased than those in the CS (n=67) group at hospital discharge, 3 months, and 6 months poststroke onset. Morbidity, as defined by modified Rankin Score, was significantly greater in the GA group at hospital discharge, and a similar trend was seen in morbidity at 3 months postdischarge. Conclusion General anesthesia for endovascular intervention in acute ischemic stroke was associated with increased mortality and poorer neurological incomes compared with conscious sedation. In our study, age, gender, history of hypertension, history of diabetes, and baseline National Institute of Health Stroke Scale were not significantly different between the groups. Although the need for a randomized, prospective study on this topic is clear, our study represents further corroboration of the safety and efficacy of conscious sedation in these procedures.