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Current Opinion in Neurology Aug 2023Since the original description of progressive supranuclear palsy (PSP) by Steele, Richardson and Olszewski, the clinical spectrum of PSP has expanded and now includes... (Review)
Review
PURPOSE OF REVIEW
Since the original description of progressive supranuclear palsy (PSP) by Steele, Richardson and Olszewski, the clinical spectrum of PSP has expanded and now includes multiple phenotypic variants linked by a common disease. In this review, we discuss the evolution of the PSP syndrome and clinical criteria, with a particular focus on the 2017 Movement Disorders Society PSP criteria, its application and limitations. We also discuss our current approach to diagnosis and treatment.
RECENT FINDINGS
There is a significant overlap between the different variants of PSP and multiple phenotypes that may be applied to the same patient simultaneously. Variant severity and predominance also evolve throughout the course of the disease. Each variant and level of certainty is associated with different specificity and sensitivity for underlying disease. The differential diagnosis of PSP is continuously evolving and includes other tauopathies, neurodegenerative, genetic, autoimmune and infectious disorders. MRI measurements can aid in the diagnosis. The first guidelines to help with clinical management of those patients have been recently published.
SUMMARY
Although much improved, clinical PSP criteria alone remain insufficient and emphasize the need for improved biomarkers to identify patients at early stages to direct appropriate therapeutic strategies and target potential research.
Topics: Humans; Supranuclear Palsy, Progressive; Movement Disorders; Tauopathies; Diagnosis, Differential; Phenotype
PubMed: 37381926
DOI: 10.1097/WCO.0000000000001163 -
Nature Reviews. Neurology Sep 2023Cerebral palsy is a clinical descriptor covering a diverse group of permanent, non-degenerative disorders of motor function. Around one-third of cases have now been... (Review)
Review
Cerebral palsy is a clinical descriptor covering a diverse group of permanent, non-degenerative disorders of motor function. Around one-third of cases have now been shown to have an underlying genetic aetiology, with the genetic landscape overlapping with those of neurodevelopmental disorders including intellectual disability, epilepsy, speech and language disorders and autism. Here we review the current state of genomic testing in cerebral palsy, highlighting the benefits for personalized medicine and the imperative to consider aetiology during clinical diagnosis. With earlier clinical diagnosis now possible, we emphasize the opportunity for comprehensive and early genomic testing as a crucial component of the routine diagnostic work-up in people with cerebral palsy.
Topics: Humans; Cerebral Palsy; Neurodevelopmental Disorders; Intellectual Disability; Causality; Paralysis
PubMed: 37537278
DOI: 10.1038/s41582-023-00847-6 -
Auris, Nasus, Larynx Feb 2024This study aimed to reveal the efficacy of physical therapy for patients with peripheral facial palsy. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
This study aimed to reveal the efficacy of physical therapy for patients with peripheral facial palsy.
METHODS
A literature search was conducted using PubMed, Ichushi-Web, and Cochrane Central Register of Controlled Trials. Published randomized controlled trials comparing the physical therapy versus placebo/non-treatment for peripheral facial palsy such as Bell's palsy, Ramsay Hunt syndrome, and traumatic facial palsy were included for meta-analysis. The primary outcome was non-recovery at the end of the follow-up. Non-recovery was defined according to the authors' definition. The secondary outcomes were the composite score of the Sunnybrook facial grading system and sequelae (presence of synkinesis or hemifacial spasm) at the end of the follow-up. Data was analyzed using Review Manager software and pooled risk ratio (RR) or mean difference (MD) with 95% confidence intervals (CI) were calculated.
RESULTS
Seven randomized controlled trials met the eligible criteria. The data on non-recovery from four studies was obtained and included 418 participants in the meta-analysis. Physical therapy might reduce non-recovery (RR = 0.51 [95% CI = 0.31-0.83], low quality). Pooling the data of composite score of the Sunnybrook facial grading system from three studies (166 participants) revealed that physical therapy might increase the composite scores (MD = 12.1 [95% CI = 3.11-21.0], low quality). Moreover, we obtained data on sequelae from two articles (179 participants). The evidence was very uncertain about the effect of physical therapy on reduction of sequelae (RR = 0.64 [95% CI = 0.07-5.95], very low quality).
CONCLUSION
The evidence suggested that physical therapy reduces non-recovery in patients with peripheral facial palsy and improves the composite score of the Sunnybrook facial grading system, whereas the efficacy of physical therapy in reducing sequelae remained uncertain. The included studies had high risk of bias, imprecision, or inconsistency; therefore, the certainty of evidence was low or very low. Further well-designed randomized controlled trials are needed to confirm its efficacy.
Topics: Humans; Anti-Inflammatory Agents; Facial Paralysis; Bell Palsy; Physical Therapy Modalities; Drug Therapy, Combination
PubMed: 37149416
DOI: 10.1016/j.anl.2023.04.007 -
Nature Aug 2023Speech brain-computer interfaces (BCIs) have the potential to restore rapid communication to people with paralysis by decoding neural activity evoked by attempted speech...
Speech brain-computer interfaces (BCIs) have the potential to restore rapid communication to people with paralysis by decoding neural activity evoked by attempted speech into text or sound. Early demonstrations, although promising, have not yet achieved accuracies sufficiently high for communication of unconstrained sentences from a large vocabulary. Here we demonstrate a speech-to-text BCI that records spiking activity from intracortical microelectrode arrays. Enabled by these high-resolution recordings, our study participant-who can no longer speak intelligibly owing to amyotrophic lateral sclerosis-achieved a 9.1% word error rate on a 50-word vocabulary (2.7 times fewer errors than the previous state-of-the-art speech BCI) and a 23.8% word error rate on a 125,000-word vocabulary (the first successful demonstration, to our knowledge, of large-vocabulary decoding). Our participant's attempted speech was decoded at 62 words per minute, which is 3.4 times as fast as the previous record and begins to approach the speed of natural conversation (160 words per minute). Finally, we highlight two aspects of the neural code for speech that are encouraging for speech BCIs: spatially intermixed tuning to speech articulators that makes accurate decoding possible from only a small region of cortex, and a detailed articulatory representation of phonemes that persists years after paralysis. These results show a feasible path forward for restoring rapid communication to people with paralysis who can no longer speak.
Topics: Humans; Amyotrophic Lateral Sclerosis; Brain-Computer Interfaces; Cerebral Cortex; Microelectrodes; Paralysis; Speech; Vocabulary; Neural Prostheses
PubMed: 37612500
DOI: 10.1038/s41586-023-06377-x -
Nature Aug 2023Speech neuroprostheses have the potential to restore communication to people living with paralysis, but naturalistic speed and expressivity are elusive. Here we use...
Speech neuroprostheses have the potential to restore communication to people living with paralysis, but naturalistic speed and expressivity are elusive. Here we use high-density surface recordings of the speech cortex in a clinical-trial participant with severe limb and vocal paralysis to achieve high-performance real-time decoding across three complementary speech-related output modalities: text, speech audio and facial-avatar animation. We trained and evaluated deep-learning models using neural data collected as the participant attempted to silently speak sentences. For text, we demonstrate accurate and rapid large-vocabulary decoding with a median rate of 78 words per minute and median word error rate of 25%. For speech audio, we demonstrate intelligible and rapid speech synthesis and personalization to the participant's pre-injury voice. For facial-avatar animation, we demonstrate the control of virtual orofacial movements for speech and non-speech communicative gestures. The decoders reached high performance with less than two weeks of training. Our findings introduce a multimodal speech-neuroprosthetic approach that has substantial promise to restore full, embodied communication to people living with severe paralysis.
Topics: Humans; Cerebral Cortex; Clinical Trials as Topic; Communication; Deep Learning; Face; Gestures; Movement; Neural Prostheses; Paralysis; Speech; Vocabulary; Voice
PubMed: 37612505
DOI: 10.1038/s41586-023-06443-4 -
The Journal of Craniofacial SurgeryLifting the temporal and mid-face areas creates a very dynamic change in the facial appearance and different planes of dissection were described before. In this article,...
Lifting the temporal and mid-face areas creates a very dynamic change in the facial appearance and different planes of dissection were described before. In this article, a new plane of dissection is described which allows the surgeon to perform a very quick and safe dissection in both the temporal and mid-face regions. Patients were operated on using the presented technique and brow lift, cantopexy, and mid-facelift were performed. The outcome of the surgery was analyzed by the authors from standardized photos before and 6 months after the surgery with measurements of the brow and lateral canthus. The plane of dissection is just over the subgaleal fascia which is actually the sub-superficial musculoaponeurotic system layer in the mid-face. This plane of dissection is easy and quick and creates a very mobile mid-face and temporal flap. Forty female patients were operated using the presented technique with a mean follow-up period of 15 months, the longest follow-up was 26 months. No major complication was encountered in these patients. In 5 patients, transient unilateral frontal branch palsy was encountered. In 1 patient, bilateral temporal area depression was observed 8 months after the surgery and treated with a fat injection under local anesthesia. One patient had alopecia in the temporal suture line which can be covered with hair. Temporal facelift is a versatile method with long-lasting results due to its important features; creating a very mobile flap for suspension dissection area, a large surface for adherence, and numerous sutures sharing the tension on the key sutures. Despite its slightly difficult early healing period, the technique should be kept in mind for the rejuvenation of the periorbital area and mid-face. Level of Evidence: Level I.
Topics: Humans; Female; Rhytidoplasty; Superficial Musculoaponeurotic System; Wound Healing; Eyelids; Hair; Paralysis
PubMed: 37449577
DOI: 10.1097/SCS.0000000000009525 -
Facial Plastic Surgery Clinics of North... Feb 2024Pediatric facial palsy is rare but severely debilitating and results in profound functional, developmental, psychosocial, and esthetic consequences. Identifying the... (Review)
Review
Pediatric facial palsy is rare but severely debilitating and results in profound functional, developmental, psychosocial, and esthetic consequences. Identifying the specific cause of the palsy is important in directing the treatment course. The most common etiologies of pediatric facial palsy are distinct from those of adults. Facial reanimation interventions are targeted to address the zones of the face, with oral/smile rehabilitation the most common region requiring intervention in pediatric patients. Gracilis microneurovascular free tissue transfer is safe and highly effective in the pediatric population, providing significant functional, psychosocial, and esthetic benefits.
Topics: Adult; Humans; Child; Facial Paralysis; Esthetics
PubMed: 37981412
DOI: 10.1016/j.fsc.2023.07.003 -
Laryngo- Rhino- Otologie Sep 2023
Topics: Humans; Vocal Cord Paralysis
PubMed: 37657426
DOI: 10.1055/a-2049-3846 -
Zhongguo Zhen Jiu = Chinese Acupuncture... Dec 2023
Topics: Humans; Supranuclear Palsy, Progressive; Acupuncture Therapy
PubMed: 38092549
DOI: 10.13703/j.0255-2930.20230925-k0008 -
Laryngo- Rhino- Otologie Sep 2023
Topics: Humans; Vocal Cord Paralysis
PubMed: 37657427
DOI: 10.1055/a-2049-3900