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Practical Neurology Oct 2021Treating patients with progressive supranuclear palsy (PSP) is both effective and rewarding. This review aims to share our experience in the proactive management of PSP,... (Review)
Review
Treating patients with progressive supranuclear palsy (PSP) is both effective and rewarding. This review aims to share our experience in the proactive management of PSP, considering the patient, the family and the medical context in which the illness unfolds. There are many opportunities to assist your patients, ameliorate their symptoms, reduce their risks and harm, and guide them through the complex medical, social and legal minefield that characterises life with chronic neurological illness. We summarise the challenges of early diagnosis, consider PSP mimics and the role of investigations in excluding these, and discuss the available pharmacological and non-pharmacological treatment strategies to tackle the common and challenging symptoms of PSP. The best treatment will be patient centred and as part of a multidisciplinary team.
Topics: Diagnosis, Differential; Humans; Supranuclear Palsy, Progressive
PubMed: 34215700
DOI: 10.1136/practneurol-2020-002794 -
Journal of Korean Medical Science Aug 2020This study reviews recent literature on facial palsy guidelines and provides systematic reviews on related topics of interest. (Meta-Analysis)
Meta-Analysis
BACKGROUND
This study reviews recent literature on facial palsy guidelines and provides systematic reviews on related topics of interest.
METHODS
An electronic database search was performed to identify recent guidelines dealing with facial nerve palsy, systematic reviews and recent meta-analysis published between 2011 and 2019 (inclusive). The literature search used the search terms "Bell's palsy," "Ramsay-Hunt syndrome," "Facial palsy," "Facial paralysis," "Facial paresis," "Guideline," "Meta-analysis," "Systematic review," and "Randomized controlled trial." Only studies written in English were used.
RESULTS
The characteristics of treatment trends for facial palsy have been reviewed over the past decade. The most prominent change noted may be the shift from the conventional House-Brackmann facial nerve grading system to the Sunnybrook and eFACE systems. In addition, the results of serial meta-analyses indicate increasing agreement with the use of surgical decompression of the facial nerve. Beyond steroids or combined steroid-antiviral treatment, various novel drugs and treatments have been tried. For long-standing facial paralysis and postparetic synkinesis sequelae after facial palsy, facial reanimation has been highlighted and the necessity of new paradigms have been raised.
CONCLUSION
For peripheral facial paralysis, various changes have been made, not only in the facial nerve grading systems, but also in medical treatments, from surgical procedures to rehabilitation, during the last decade.
Topics: Acupuncture Therapy; Acute Disease; Decompression, Surgical; Facial Nerve; Facial Paralysis; Humans; Practice Guidelines as Topic
PubMed: 32743989
DOI: 10.3346/jkms.2020.35.e245 -
Clinical Rehabilitation Nov 2022To conduct a systematic review of the effectiveness of facial exercise therapy for facial palsy patients, updating an earlier broader Cochrane review; and to provide...
OBJECTIVE
To conduct a systematic review of the effectiveness of facial exercise therapy for facial palsy patients, updating an earlier broader Cochrane review; and to provide evidence to inform the development of telerehabilitation for these patients.
DATA SOURCES
MEDLINE, EMBASE, CINAHL, Cochrane Library, PEDro and AMED for relevant studies published between 01 January 2011 and 30 September 2020.
METHODS
Predetermined inclusion/exclusion criteria were utilised to shortlist abstracts. Two reviewers independently appraised articles, systematically extracted data and assessed the quality of individual studies and reviews (using GRADE and AMSTAR-2, respectively). Thematic analysis used for evidence synthesis; no quantitative meta-analysis conducted. The review was registered with PROSPERO (CRD42017073067).
RESULTS
Seven new randomised controlled trials, nine observational studies, and three quasi-experimental or pilot studies were identified ( = 854 participants). 75% utilised validated measures to record changes in facial function and/or patient-rated outcomes. High-quality trials (4/7) all reported positive impacts; as did observational studies rated as high/moderate quality (3/9). The benefit of therapy at different time points post-onset and for cases of varying clinical severity is discussed. Differences in study design prevented data pooling to strengthen estimates of therapy effects. Six new review articles identified were all rated critically low quality.
CONCLUSION
The findings of this targeted review reinforce those of the earlier more general Cochrane review. New research studies strengthen previous conclusions about the benefits of facial exercise therapy early in recovery and add to evidence of the value in chronic cases. Further standardisation of study design/outcome measures and evaluation of cost-effectiveness are recommended.
Topics: Bell Palsy; Exercise Therapy; Facial Nerve; Facial Paralysis; Humans; Physical Therapy Modalities
PubMed: 35787015
DOI: 10.1177/02692155221110727 -
Parkinsonism & Related Disorders Apr 2020Progressive supranuclear palsy (PSP) is a complex clinicopathologic disease with no current cure or disease modulating therapies that can only be definitively confirmed... (Review)
Review
Progressive supranuclear palsy (PSP) is a complex clinicopathologic disease with no current cure or disease modulating therapies that can only be definitively confirmed at autopsy. Growing understanding of the phenotypic diversity of PSP has led to expanded clinical criteria and new insights into etiopathogenesis that coupled with improved in vivo biomarkers makes increased access to current clinical trials possible. Current standard-of-care treatment of PSP is multidisciplinary, supportive and symptomatic, and several trials of potentially disease modulating agents have already been completed with disappointing results. Current ongoing clinical trials target the abnormal aggregation of tau through a variety of mechanisms including immunotherapy and gene therapy offer a more direct method of treatment. Here we review PSP clinicopathologic correlations, in vivo biomarkers including MRI, PET, and CSF biomarkers. We additionally review current pharmacologic and non-pharmacologic methods of treatment, prior and ongoing clinical trials in PSP. Newly expanded clinical criteria and improved specific biomarkers will aid in identifying patients with PSP earlier and more accurately and expand access to these potentially beneficial clinical trials.
Topics: Drug Therapy; Genetic Therapy; Humans; Immunotherapy; Supranuclear Palsy, Progressive
PubMed: 32487421
DOI: 10.1016/j.parkreldis.2020.04.014 -
Ideggyogyaszati Szemle Jul 2022The incidence of brachial plexus palsy (BPP) has decreased recently, but the indivi-d-ual's quality of life is endangered. To provide better chan-ces to BPP neonates and... (Review)
Review
BACKGROUND AND PURPOSE
The incidence of brachial plexus palsy (BPP) has decreased recently, but the indivi-d-ual's quality of life is endangered. To provide better chan-ces to BPP neonates and infants, the Department of Developmental Neurology worked out, introduced, and applied a complex early therapy, including nerve point stimulation.
METHODS
After diagnosing the severity of BPP, early intensive and complex therapy should be started. Appro-x-imately after a week or ten days following birth, the slightest form (neurapraxia) normalizes without any intervention, and signs of recovery can be detected around this period. The therapy includes the unipolar nerve point electro-stimulation and the regular application of those elemen-tary sensorimotor patterns, which activate both extremities simultaneously.
RESULTS
With the guideline worked out and applied in the Department of Developmental Neurology, full recovery can be achieved in 50% of the patients, and even in the most severe cases (nerve root lesion), functional upper limb usage can be detected with typically developing body-scheme.
CONCLUSION
Immediately starting complex treatment based on early diagnosis alters the outcome of BPP, providing recovery in the majority of cases and enhancing the everyday arm function of those who only partially benefit from the early treatment.
Topics: Brachial Plexus; Brachial Plexus Neuropathies; Early Diagnosis; Humans; Infant; Infant, Newborn; Neonatal Brachial Plexus Palsy; Paralysis; Quality of Life
PubMed: 35916611
DOI: 10.18071/isz.75.0247 -
Nature Nov 2022A spinal cord injury interrupts pathways from the brain and brainstem that project to the lumbar spinal cord, leading to paralysis. Here we show that spatiotemporal...
A spinal cord injury interrupts pathways from the brain and brainstem that project to the lumbar spinal cord, leading to paralysis. Here we show that spatiotemporal epidural electrical stimulation (EES) of the lumbar spinal cord applied during neurorehabilitation (EES) restored walking in nine individuals with chronic spinal cord injury. This recovery involved a reduction in neuronal activity in the lumbar spinal cord of humans during walking. We hypothesized that this unexpected reduction reflects activity-dependent selection of specific neuronal subpopulations that become essential for a patient to walk after spinal cord injury. To identify these putative neurons, we modelled the technological and therapeutic features underlying EES in mice. We applied single-nucleus RNA sequencing and spatial transcriptomics to the spinal cords of these mice to chart a spatially resolved molecular atlas of recovery from paralysis. We then employed cell type and spatial prioritization to identify the neurons involved in the recovery of walking. A single population of excitatory interneurons nested within intermediate laminae emerged. Although these neurons are not required for walking before spinal cord injury, we demonstrate that they are essential for the recovery of walking with EES following spinal cord injury. Augmenting the activity of these neurons phenocopied the recovery of walking enabled by EES, whereas ablating them prevented the recovery of walking that occurs spontaneously after moderate spinal cord injury. We thus identified a recovery-organizing neuronal subpopulation that is necessary and sufficient to regain walking after paralysis. Moreover, our methodology establishes a framework for using molecular cartography to identify the neurons that produce complex behaviours.
Topics: Animals; Humans; Mice; Neurons; Paralysis; Spinal Cord; Spinal Cord Injuries; Walking; Electric Stimulation; Lumbosacral Region; Neurological Rehabilitation; Sequence Analysis, RNA; Gene Expression Profiling
PubMed: 36352232
DOI: 10.1038/s41586-022-05385-7 -
JAMA Otolaryngology-- Head & Neck... Nov 2020Facial paralysis (FP) after surgery has substantial functional, emotional, and financial consequences. Most iatrogenic FP is managed by watchful waiting, with the...
IMPORTANCE
Facial paralysis (FP) after surgery has substantial functional, emotional, and financial consequences. Most iatrogenic FP is managed by watchful waiting, with the expectation of facial function recovery. A potential treatment is physical therapy (PT).
OBJECTIVE
To investigate whether noninvasive PT compared with no PT or other intervention improves facial nerve outcomes in adults with iatrogenic FP.
EVIDENCE REVIEW
Patients with noniatrogenic FP, facial reanimation surgery, and invasive adjunctive treatments (acupuncture or botulinum toxin injection) were excluded. A systematic review was conducted for records discussing iatrogenic FP and PT; a search for these records was performed using Ovid MEDLINE (1946-2019), Embase (1947-2019), Scopus (1823-2019), Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, World Health Organization International Clinical Trials Registry Platform (2004-2019), and ClinicalTrials.gov (1997-2019). The references of all the included articles were also assessed for eligible studies. All human participant, English-language study designs with at least 2 cases were included. Quality assessment was performed using the Methodological Index for Non-randomized Studies (MINORS) and the revised Cochrane Risk of Bias 2 (RoB 2) tool for randomized controlled trials. All search strategies were completed on May 16, 2019, and again on October 1, 2019.
FINDINGS
Fifteen studies (7 of which were retrospective cohort studies) and 313 patients with iatrogenic FP were included in the systematic review. Most iatrogenic FP (166 patients [53%]) was associated with parotidectomy; traditional PT (ie, facial massage) was the most common intervention (196 patients [63%]). The use of various facial grading systems and inconsistent reporting of outcomes prevented direct comparison of PT types.
CONCLUSIONS AND RELEVANCE
Because of heterogeneity in reported outcomes of facial nerve recovery, definitive conclusions were unable to be made regarding the association between PT and outcomes of iatrogenic FP. Physical therapy probably has benefit and is associated with no harm in patients with iatrogenic FP.
Topics: Facial Paralysis; Humans; Iatrogenic Disease; Physical Therapy Modalities
PubMed: 32970128
DOI: 10.1001/jamaoto.2020.3049 -
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 -
JAMA Neurology Mar 2023Brain-computer interface (BCI) implants have previously required craniotomy to deliver penetrating or surface electrodes to the brain. Whether a minimally invasive... (Clinical Trial)
Clinical Trial
Assessment of Safety of a Fully Implanted Endovascular Brain-Computer Interface for Severe Paralysis in 4 Patients: The Stentrode With Thought-Controlled Digital Switch (SWITCH) Study.
IMPORTANCE
Brain-computer interface (BCI) implants have previously required craniotomy to deliver penetrating or surface electrodes to the brain. Whether a minimally invasive endovascular technique to deliver recording electrodes through the jugular vein to superior sagittal sinus is safe and feasible is unknown.
OBJECTIVE
To assess the safety of an endovascular BCI and feasibility of using the system to control a computer by thought.
DESIGN, SETTING, AND PARTICIPANTS
The Stentrode With Thought-Controlled Digital Switch (SWITCH) study, a single-center, prospective, first in-human study, evaluated 5 patients with severe bilateral upper-limb paralysis, with a follow-up of 12 months. From a referred sample, 4 patients with amyotrophic lateral sclerosis and 1 with primary lateral sclerosis met inclusion criteria and were enrolled in the study. Surgical procedures and follow-up visits were performed at the Royal Melbourne Hospital, Parkville, Australia. Training sessions were performed at patients' homes and at a university clinic. The study start date was May 27, 2019, and final follow-up was completed January 9, 2022.
INTERVENTIONS
Recording devices were delivered via catheter and connected to subcutaneous electronic units. Devices communicated wirelessly to an external device for personal computer control.
MAIN OUTCOMES AND MEASURES
The primary safety end point was device-related serious adverse events resulting in death or permanent increased disability. Secondary end points were blood vessel occlusion and device migration. Exploratory end points were signal fidelity and stability over 12 months, number of distinct commands created by neuronal activity, and use of system for digital device control.
RESULTS
Of 4 patients included in analyses, all were male, and the mean (SD) age was 61 (17) years. Patients with preserved motor cortex activity and suitable venous anatomy were implanted. Each completed 12-month follow-up with no serious adverse events and no vessel occlusion or device migration. Mean (SD) signal bandwidth was 233 (16) Hz and was stable throughout study in all 4 patients (SD range across all sessions, 7-32 Hz). At least 5 attempted movement types were decoded offline, and each patient successfully controlled a computer with the BCI.
CONCLUSIONS AND RELEVANCE
Endovascular access to the sensorimotor cortex is an alternative to placing BCI electrodes in or on the dura by open-brain surgery. These final safety and feasibility data from the first in-human SWITCH study indicate that it is possible to record neural signals from a blood vessel. The favorable safety profile could promote wider and more rapid translation of BCI to people with paralysis.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT03834857.
Topics: Aged; Humans; Male; Middle Aged; Brain; Brain-Computer Interfaces; Cerebral Cortex; Paralysis; Prospective Studies
PubMed: 36622685
DOI: 10.1001/jamaneurol.2022.4847 -
Brazilian Journal of Physical Therapy 2022Facial palsy (FP) is defined as an injury of the seventh cranial nerve pair, partial or total, which can be classified as central or peripheral. Proprioceptive... (Review)
Review
BACKGROUND
Facial palsy (FP) is defined as an injury of the seventh cranial nerve pair, partial or total, which can be classified as central or peripheral. Proprioceptive neuromuscular facilitation (PNF) is primarily used in the functional recovery of upper and lower limb conditions, however the technique has also been used for FP.
OBJECTIVE
To analyze the effect of PNF in the treatment of dysfunctions in FP.
METHODS
Ten databases including BVS, CENTRAL Cochrane, CINAHL, PEDro, PubMed, Scielo, ScienceDirect, SCOPUS, Web of Science, and Google Scholar were comprehensively searched for dates prior to April 2021. Randomized controlled trials of PNF in individuals with dysfunctions caused by facial paralysis were eligible. Outcomes measures were recovery rate and clinical recovery, both measured by using the House Brackmann Scale. Recovery time was measured in days and synkinesis assessed with the Synkinesis Assessment Questionnaire.
RESULTS
A total of 184 patients were included. In general, the included studies have low methodological quality. None of the five studies used PNF as the sole intervention. In all of the included studies PNF was used in combination with other interventions. Our findings show very low evidence that PNF is more effective than minimal intervention for treating FP.
CONCLUSION
We conclude that given the limited number of studies included and the low methodological quality presented, recommendations based on these studies should be interpreted with caution. The effects of PNF on facial paralysis are not clear.
Topics: Humans; Facial Paralysis; Muscle Stretching Exercises; Synkinesis; Recovery of Function
PubMed: 36279766
DOI: 10.1016/j.bjpt.2022.100454