Severe Palsy Research Articles

ReType: Your Breath Tells Your Mind!

Neuromuscular system disease can cause severe paralysis and even speech impairments. To communicate with others, the patients can use breathing to encode information. However, traditional solutions only allow patients to output several predefined sentences. Other solutions require patients to interact with the Windows system, which are not suitable for blind paralyzed patients. To address these problems, we present a text input system based on commercial-off-the-shelf RFID devices, named Respiration-Type (ReType). A passive lightweight RFID tag is attached to the user's chest area over the clothes. By interrogating the tag continuously, an RFID reader can output phase data stream, which will be modulated by the chest fluctuation caused by respiration. Based on this phenomenon, we let normal breath and deep breath represent binary 0 and 1, respectively. To extract the feature information related to breath in real time from the noisy phase data stream, we develop a novel algorithm that is robust to noise and baseline drift. To ensure the robustness of decoding, a breath-control model is established for decoding normal breath and deep breath. Comprehensive experiments show that ReType can achieve an average recognition accuracy of 92.22% and is robust against environment changes and user diversity.

Patients' experiences and perceptions of Guillain-Barré syndrome: A systematic review and meta-synthesis of qualitative research.

Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy, with an incidence of 1-2/100,000 per year. Its severity is variable, ranging from very mild cases with brief weakness to severe paralysis, leading to inability to breathe independently, or even death. Currently there is limited evidence exploring the experiences of GBS patients. The aim of this study was to review patients' experiences and perceptions of GBS and its variants at diagnosis, discharge and during recovery, by conducting a systematic review and thematic meta-synthesis of qualitative studies of patients' experiences of GBS (and its variants). We searched twelve electronic databases, supplemented with internet searches and forward and backward citation tracking from the included studies and review articles. Data were synthesised thematically following the Thomas and Harden approach. The CASP Qualitative Checklist was used to assess the quality of the included studies of this review. Our search strategy identified a total of 5,282 citations and after removing duplicates and excluding citations based on title and abstract, and full-text screening, five studies were included in the review and meta-synthesis; all included studies were considered of acceptable quality. Through constant discussions and an iterative approach, we developed six analytical themes following a patient's journey from suspecting that they had a health problem, through to being hospitalised, experiencing ongoing difficulties, slowly recovering from GBS, adjusting to their new circumstances, and re-evaluating their lives. Despite the variety of experiences, it was evident from all included studies that being diagnosed with and surviving GBS was a life-changing experience for all participants. Protocol was registered (CRD42019122199) on the International Prospective Register of Systematic Reviews (http://www.crd.york.ac.uk/PROSPERO).

Severe oligomeric tau toxicity can be reversed without long-term sequelae.

Tau is a microtubule stabilizing protein that forms abnormal aggregates in many neurodegenerative disorders, including Alzheimer’s disease. We have previously shown that co-expression of fragmented and full-length tau in P301SxTAU62on tau transgenic mice results in the formation of oligomeric tau species and causes severe paralysis. This paralysis is fully reversible once expression of the tau fragment is halted, even though P301S tau expression is maintained. Whereas various strategies to target tau aggregation have been developed, little is known about the long-term consequences of reverted tau toxicity. Therefore, we studied the long-term motor fitness of recovered, formerly paralysed P301SxTAU62on-off mice. To assess the seeding competence of oligomeric toxic tau species, we also inoculated ALZ17 mice with brainstem homogenates from paralysed P301SxTAU62on mice. Counter-intuitively, after recovery from paralysis due to oligomeric tau species expression, ageing P301SxTAU62on-off mice did not develop more motor impairment or tau pathology when compared to heterozygous P301S tau transgenic littermates. Thus, toxic tau species causing extensive neuronal dysfunction can be cleared without inducing seeding effects. Moreover, these toxic tau species also lack long-term tau seeding effects upon intrahippocampal inoculation into ALZ17 mice. In conclusion, tau species can be neurotoxic in the absence of seeding-competent tau aggregates, and mice can clear these tau forms permanently without tau seeding or spreading effects. These observations suggest that early targeting of non-fibrillar tau species may represent a therapeutically effective intervention in tauopathies. On the other hand, the absent seeding competence of early toxic tau species also warrants caution when using seeding-based tests for preclinical tauopathy diagnostics.

Do somatosensory deficits predict efficacy of neurorehabilitation using neuromuscular electrical stimulation for moderate to severe motor paralysis of the upper limb in chronic stroke?

Background:Various neurorehabilitation programs have been developed to promote recovery from motor impairment of upper extremities. However, the response of patients with chronic-phase stroke varies greatly. Prediction of the treatment response is important to provide appropriate and efficient rehabilitation. This study aimed to clarify whether clinical assessments, such as motor impairments and somatosensory deficits, before treatment could predict the treatment response in neurorehabilitation.Methods:The data from patients who underwent neurorehabilitation using closed-loop electromyography (EMG)-controlled neuromuscular electrical stimulation were retrospectively analyzed. A total of 66 patients with chronic-phase stroke with moderate to severe paralysis were included. The changes from baseline in the Fugl-Meyer Assessment–Upper Extremity (FMA-UE) and the Motor Activity Log-14 (MAL-14) of amount of use (AOU) and quality of movement (QOM) were used to assess treatment response, and multivariate logistic regression analysis was performed using the extracted candidate predictors, such as baseline clinical assessments, to identify predictors of FMA-UE and MAL-14 improvement.Results:FMA-UE and MAL-14 scores improved significantly after the intervention (FMA-UE p < 0.01, AOU p < 0.01, QOM p < 0.01). On multivariate logistic regression analysis, tactile sensory (p = 0.043) and hand function (p = 0.030) were both identified as significant predictors of FMA-UE improvement, tactile sensory (p = 0.047) was a significant predictor of AOU improvement, and hand function (p = 0.026) was a significant predictor of QOM improvement. The regression equations explained 71.2% of the variance in the improvement of FMA-UE, 69.7% of AOU, and 69.7% of QOM.Conclusion:Both motor and tactile sensory impairments predict improvement in motor function, tactile sensory impairment predicts improvement in the amount of paralytic hand use, and motor impairment predicts improvement in the quality of paralytic hand use following neurorehabilitation treatment in patients with moderate to severe paralysis in chronic-phase stroke. These findings may help select the appropriate treatment for patients with more severe paralysis and to maximize the treatment effect.

The Pathology of Poliomyelitis and the Vaccines and Nonvaccine Therapy

Poliomyelitis is an exclusively human disease that mainly affects children. Clinical features of poliomyelitis can be varied, from mild illness to the most severe paralysis, and the factor why poliomyelitis has different performances in individuals has been proved strongly correlated with membrane protein CD155. The nervous system shows a special protecting phenomenon against the invasion of poliovirus, and the mechanism is not very clear at present. Vaccines are the main means of preventing and controlling polio, and many different vaccines have been invented in the process of fighting polio. Inactivated polio vaccine (IPV) and oral polio vaccine (OPV) are the two main vaccines. IPV is known for its safety while OPV is widely used in developing countries because of its relatively low cost. This usage also leads to some side effects: vaccine-associated paralytic polio (VAPP) and vaccine-derived poliovirus (VDPV). Now, for polio eradication, the elimination of these two diseases has become particularly important. Thus, a new type of vaccine was created: sequential IPV-OPV with the safety of IPV and the low cost of OPV. This paper will talk about the different polio vaccines and their effects. An enormous difference between people who have gotten the vaccine and people who have not got the vaccine. Comparing the two kinds of people, people who get normal poliovirus, and people who get poliovirus after taking a vaccine, known as VAPP (vaccine-associated paralytic poliomyelitis), the former cannot get full recovery whole life and the latter has a very low possibility. In conclusion, people should take vaccines if it is affordable for them.

Combined upper approaches (electrical stimulation, orthotics, robotics, and CI therapy) and the daily use of paralyzed hand under hand orthosis for a sub-acute stroke patient with severe upper limb paralysis:

Combined upper approaches (electrical stimulation, orthotics, robotics, and CI therapy) and the daily use of paralyzed hand under hand orthosis for a sub-acute stroke patient with severe upper limb paralysis:

Effectiveness of a knee-ankle-foot orthosis with a knee extension aid in gait training for stroke patients.

To evaluate the effectiveness of a knee-ankle-foot orthosis (KAFO) with a newly developed knee extension aid in gait training for stroke patients with severe leg paralysis. The participants were 7 recovering stroke patients prescribed a KAFO. With the KAFO knee joint set to allow free flexion, knee flexion angle, lower limb muscle activity, and time of plantar ground contact were measured during assisted walking with the knee extension aid or a conventional support loop. When first using the knee extension aid, knee flexion angle at initial ground contact was significantly decreased and time from heel strike on the paralyzed side to forefoot strike was prolonged compared with the support loop. After using the knee extension aid for 1 week, in addition to the two parameters above, maximum knee flexion angle during the swing phase, knee extension displacement from the maximum flexion angle to initial ground contact, and the muscle activity ratio of the biceps femoris during the swing phase were significantly increased compared with the support loop. This knee extension aid may be indicated for patients who have difficulty in initial heel strike due to excessive knee flexion at the end of the swing phase.

Post-discharge effectiveness of combined robotic and modified constraint-induced movement therapies for post-stroke convalescent patients with moderate to severe upper limb paralysis

Post-discharge effectiveness of combined robotic and modified constraint-induced movement therapies for post-stroke convalescent patients with moderate to severe upper limb paralysis

Motor neuroprosthesis implanted with neurointerventional surgery improves capacity for activities of daily living tasks in severe paralysis: first in-human experience

BackgroundImplantable brain–computer interfaces (BCIs), functioning as motor neuroprostheses, have the potential to restore voluntary motor impulses to control digital devices and improve functional independence in patients with severe paralysis due...

小脳・脳幹梗塞後に重度球麻痺，中枢性低換気を呈した患者に対する包括的リハビリテーションの訓練経過 ～摂食嚥下リハビリテーションを中心に～

小脳・脳幹梗塞後に重度球麻痺，中枢性低換気を呈した患者に対する包括的リハビリテーションの訓練経過 ～摂食嚥下リハビリテーションを中心に～

Anti-tau scFvs Targeted to the Cytoplasm or Secretory Pathway Variably Modify Pathology and Neurodegenerative Phenotypes

Immunotherapies designed to treat neurodegenerative tauopathies that primarily engage extracellular tau may have limited efficacy as tau is primarily intracellular. We generated tau-targeting single-chain variable fragments (scFvs) and intrabodies (iBs) from the phosphorylated tau-specific antibodies CP13 and PHF1 and the pan-tau antibody Tau5. Recombinant adeno-associated virus (rAAV) was utilized to express these antibody fragments in homozygous JNPL3 P301L tau mice. Two iBs (CP13i, PHF1i) and one scFv (PHF1s) abrogated tau pathology and delayed time to severe hindlimb paralysis. In a second tauopathy model (rTg4510), CP13i and PHF1i reduced tau pathology, but cognate scFvs did not. These data demonstrate that (1) disease-modifying efficacy does not require antibody effector functions, (2) the intracellular targeting of tau with phosphorylated tau-specific iBs is more effective than extracellular targeting with the scFvs, and (3) robust effects on tau pathology before neurodegeneration only resulted in modest disease modification as assessed by delay of severe motor phenotype.

Deep learning the sound of light to guide surgeries

Photoacoustic imaging utilizes light and sound to make images by transmitting laser pulses that illuminate regions of interest, which subsequently absorb the light, causing thermal expansion and the generation of sound waves that are detected with conventional ultrasound transducers. The Photoacoustic and Ultrasonic Systems Engineering (PULSE) Lab is developing novel methods that use photoacoustic imaging to guide surgeries with the ultimate goal of eliminating surgical complications caused by injury to important structures like major blood vessels and nerves that are otherwise hidden from a surgeons immediate view. This invited presentation will summarize our recent work to learn from the physics of sound propagation in tissue and develop acoustic beamforming algorithms that improve image quality, using state-of-the-art deep learning methods. These deep learning methods hold promise for robotic tracking tasks, visualization and visual servoing of surgical tool tips, and assessment of relative distances between the surgical tool and nearby critical structures (e.g., major blood vessels and nerves that if injured will cause severe complications, paralysis, or patient death). Impacted surgeries and procedures include neurosurgery, spinal fusion surgery, hysterectomies, cardiac interventions, and biopsies.

O12 Pulseless electrical activity arrest in a young woman: could renal tubular acidosis due to Sjögren’s syndrome be the underlying cause?

O12 Pulseless electrical activity arrest in a young woman: could renal tubular acidosis due to Sjögren’s syndrome be the underlying cause?

A case series of distal renal tubular acidosis, Southeast Asian ovalocytosis and metabolic bone disease

BackgroundFamilial distal renal tubular acidosis (dRTA) associated with mutations of solute carrier family 4 membrane − 1 (SLC4A1) gene could co-exist with red cell membrane abnormality, Southeast Asian ovalocytosis (SAO). Although this association is well described in Southeast Asian countries, it is less frequently found in Sri Lanka.Case presentationWe describe six patients who had dRTA co-existing with SAO. All of them initially presented with severe hypokalemia and paralysis. They presented within a period of six months to the Teaching Hospital Anuradhapura, Sri Lanka.All had metabolic acidosis indicated by low serum bicarbonate. Three of them were having underlying chronic kidney disease as well. Those three patients had mixed high and normal anion gap metabolic acidosis indicated by low delta ratio. In all dRTA was confirmed by presence of normal anion gap, hyperchloraemia, high urine pH and positive urine anion gap.Examination of blood films of all of them revealed presence of stomatocytes and macro-ovalocytosis compatible with SAO.In relation to complications of dRTA, two patients had medullary nephrocalcinosis. Three patients had biochemical evidence of osteomalacia, with two of them having radiological evidence of diffuse osteosclerosis. One patient had secondary hyperparathyroidism and a pathological fracture.ConclusionsErythrocyte in SAO is exceptionally rigid and this abnormality is said to be evolved as it protects against Plasmodium vivax malaria and cerebral malaria cause by Plasmodium falciparum. Although two families of SAO was described earlier, SAO and dRTA combination was reported only once in a patient from Anuradhapura district.Distal renal tubular acidosis, SAO combination and its related complications including nephrocalcinosis, chronic kidney disease and metabolic bone disease was not described in Sri-Lankan literature. This case series emphasize the importance of investigating recurrent/ chronic hypokalemia to diagnose dRTA and its associations, as early correction of acidosis could prevent development of chronic kidney disease and metabolic bone disease.

The alteration of gut microbiome and metabolism in amyotrophic lateral sclerosis patients

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease accompanied with severe paralysis or even death, while the pathogenesis of ALS is still unclear and no effective therapy exists. The accumulating evidence has indicated the association between gut microbiota and various neurological diseases. Thus, to explore the potential role of gut microbiome in ALS, 20 patients diagnosed with probable or definite ALS and 20 healthy controls were enrolled and their fecal excrements were collected. The analysis of fecal community diversity with 16S rDNA sequencing showed an obvious change in microbial structure of ALS patients, where Bacteroidetes at the phylum level and several microbes at the genus level were up-regulated, while Firmicutes at the phylum level and Megamonas at the genus level were down-regulated compared to healthy controls. Additionally, decreased gene function associated with metabolic pathways was observed in ALS patients. The metagenomics further demonstrated the discrepancies in microflora at the species level and relevant metabolites thereof were also revealed when combined with metabolomics. In conclusion, the altered composition of the gut microbiota and metabolic products in ALS patients provided deeper insights into the pathogenesis of ALS, and these biomarkers might be established as potential therapeutic targets which deserve further exploration.

Non-recovery animal model of severe facial paralysis induced by freezing the facial canal

ObjectiveSome cases of peripheral facial paralysis are resistant to treatment, thus, a non-recovery model of facial paralysis is needed to develop new treatment strategies for this condition. The purpose of the current study was to develop an animal model of which facial palsy was severe and prolonged. MethodsTen 8-week-old female Hartley guinea pigs weighing between 400 and 500 g were used for the animal model. The vertical segment of the facial canal was accessed via the otic bulla, without removing the bony wall of the facial canal. The canal was then frozen for 5 s using freeze spray. Facial movements, electroneurography (ENoG), histology, and changes in temperature were evaluated. ResultsAll animals exhibited complete facial paralysis immediately after the procedure and recovered gradually, however, not all of them had recovered completely 15 weeks after freezing. The ENoG values one week after freezing for all animals (10/10) were 0%. Histological examination one week after freezing revealed that most of the vertically placed myelinated nerve fibers which had been frozen were remarkably affected and denatured. The number of vertically placed myelinated nerve fibers increased 15 weeks after freezing, but the nerve fibers were smaller than normal nerve fibers and were distorted in shape. ConclusionComplete facial paralysis was induced in Hartley guinea pigs by freezing the facial canal. The behavioral, ENoG, and histopathological data suggest that the facial paralysis was severe and prolonged. This model may assist in developing novel treatment for severe facial palsy and facilitate basic research on facial nerve regeneration.

Classification of Facial Expressions for Intended Display of Emotions Using Brain-Computer Interfaces.

Facial expressions are important for intentional display of emotions in social interaction. For people with severe paralysis, the ability to display emotions intentionally can be impaired. Current brain–computer interfaces (BCIs) allow for linguistic communication but are cumbersome for expressing emotions. Here, we investigated the feasibility of a BCI to display emotions by decoding facial expressions. We used electrocorticographic recordings from the sensorimotor cortex of people with refractory epilepsy and classified five facial expressions, based on neural activity. The mean classification accuracy was 72%. This approach could be a promising avenue for development of BCI‐based solutions for fast communication of emotions. ANN NEUROL 2020;88:631–636

Otitis Externa

Key Points Otitis externa most commonly caused by Pseudomonas aeruginosa or Staphylococcus aureus bacteria.Examination noted to have swelling of ear canal, possible discharge, pain on movement of pinna or tragus.TreatmentFor intact tympanic membrane, can use neomycin, polymyxin B, or trimethoprim sulfate.For non-intact tympanic membrane or tympanostomy tube–associated otorrhea, use fluoroquinolones (eg, ciprofloxacin, ofloxacin).Malignant otitis externa (ie, rapid soft tissue and bone involvement) is characterized by severe pain, copious discharge, and possible facial paralysis and requires admission with imaging and intravenous antibiotics.

Anti-N-methyl-D-aspartate receptor encephalitis associated with peripheral nerve injury: A case report

A patient with Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis presented with quadriplegia and multiple peripheral neuropathy with axonal lesion, confirmed by electrophysiological examination. The muscle strength in the limbs of the patient gradually recovered almost completely, accompanied by the reversal of neuroelectrophysiological symptoms, and the improvement of clinical manifestations, including consciousness, respiration and cognitive function. It was revealed that the neuropathy in NMDAR encephalitis involved motor or sensorimotor nerves more than pure sensory nerves. No autoantibodies were detectable, in contrast to other anti-NMDAR overlapping syndromes. Although the underlying mechanism remains unclear, it may be associated with autoimmune generalization. In conclusion, when patients with NMDAR encephalitis present with severe limb paralysis, the possibility of peripheral nerve damage should be considered.

Muscular Activity Modulation During Post-operative Walking With Hybrid Assistive Limb (HAL) in a Patient With Thoracic Myelopathy Due to Ossification of Posterior Longitudinal Ligament: A Case Report.

Disorders of the central nervous system sometimes cause severe sensory motor paralysis accompanied by gait impairment. Recently, there are several reports on the effectiveness of robot-assisted gait training for patients experiencing these issues. The purpose of this case report was to assess the neuromechanical effect of a wearable robot suit HAL (Hybrid Assistive Limb) during post-operative gait training in a patient with gait impairment due to compressive myelopathy caused by ossification of the posterior longitudinal ligament (OPLL). For this purpose, we compared lower limb muscular activities while the patient was walking with and without the robot through a course of treatment sessions by (i) gait phase-dependent muscle usage analysis, (ii) muscle synergy analysis, and (iii) muscle network analysis. The results show (i) enhanced activity of the extensor muscles for weight-bearing in the initial sessions by using HAL and reduced knee extensor and increased hip extensor activations for achieving larger steps and faster gait in the later sessions; (ii) involvement of a greater number of synergies during walking with HAL than without HAL; and (iii) modulated muscle network property during walking with HAL remaining until the next HAL session. The patient's gait was improved after completing HAL sessions, acquiring close to normal joint profile with greater range of joint movement, faster walking speed, and larger step length. We discuss that the muscular activity modulation during walking with HAL suggests altered control of the muscles by the central nervous system during post-operative walking. Activity-dependent sensorimotor augmentation by HAL is discussed in the context of recovery of gait control by the central nervous system. The relationship between the altered control and the achieved gait recovery requires further investigation.