Case report: A novel FBXW7 gene variant causes global developmental delay.

Objective: To investigate a case of neurodevelopmental disorder caused by mutation of FBXW7. Methods: Clinical data were collected from the patient, trio-WES (whole-exome sequencing) was performed on the patient and his parents (trio), and the results were verified by Sanger sequencing. RESULTS: The patient was a 2-year and 1-month old male who presented with facial dysmorphism (prominent forehead, ocular hypertelorism, and low nasal bridge), global developmental delay, language impairment, hypertonia, labial hemangioma, hydrocele, and overgrowth. The trio-WES confirmed that the child had a pathogenic de novo FBXW7 gene variant, c.1612C>T (p.G1n538*), a heretofore unreported locus. Conclusion: This case of developmental delay, hypotonia, and impaired language (OMIM: #620012) related to a mutation in FBXW7, is a rare genetic disorder, newly identified in recent years, and seldom reported. The presence of hypertonia, labial hemangioma, and hydrocele in this child suggests significant phenotypic heterogeneity of the disease, and the discovery of new mutant loci enriches the spectrum of pathogenic variants of the disease.

Longitudinal monitoring of hypertonia through a multimodal sensing glove.

As clinical evaluations of neuromuscular disorders such as hypertonia mostly rely on perception-based scales, the imprecise subjective ratings make it difficult to accurately monitor treatment progress. To promote objective evaluation, this work used a multi-modal sensing glove in a double-blind study to enable sensitive monitoring of medication effects across 19 participants. The biomechanical measurements from the sensing glove effectively distinguished patient cohorts receiving a baclofen treatment or a placebo with 95% confidence. Consistent monitoring over a two-month period was demonstrated, closely tracking variations in individual responses to treatment. The biomechanical changes were correlated to neural activities as recorded by electromyography, verifying the medication effects. The sensing glove is shown to be a reliable tool for point-of-care settings to facilitate precise evaluation of hypertonia, essential for tailoring individual treatment choices and timely management of chronic symptoms.

Belgian consensus guideline on the management of anal fissures.

Introduction: Acute and chronic anal fissures are common proctological problems that lead to relatively high morbidity and frequent contacts with health care professionals. Multiple treatment options, both topical and surgical, are available, therefore evidence-based guidance is preferred. Methods: A Delphi consensus process was used to review the literature and create relevant statements on the treatment of anal fissures. These statements were discussed and modulated until sufficient agreement was reached. These guidelines were based on the published literature up to January 2023. Results: Anal fissures occur equally in both sexes, mostly between the second and fourth decades of life. Diagnosis can be made based on cardinal symptoms and clinical examination. In case of insufficient relief with conservative treatment options, pharmacological sphincter relaxation is preferred. After 6-8 weeks of topical treatment, surgical options can be explored. Both lateral internal sphincterotomy as well as fissurectomy are well-established surgical techniques, both with specific benefits and risks. Conclusions: The current guidelines for the management of anal fissures include recommendations for the clinical evaluation of anal fissures, and their conservative, topical and surgical management.

Treatment of Acquired Deforming Hypertonia with Botulinum Toxin in Older Population: A Retrospective Study.

Acquired deforming hypertonia (ADH) affects the daily care of numerous nursing home residents. The aim of this study was to analyze the practice, aims, and effectiveness of botulinum toxin injections (BTxis) in the treatment of older patients with contractures, an indication for which BTxis are still underused. Data were extracted retrospectively from medical records regarding population, contractures, and injections. A prospective analysis was conducted to evaluate treatment goals set by goal attainment scaling (GAS) at T0 and at T1, to evaluate the therapeutic effects. We also recorded the occurrence of side effects, using a telephone questionnaire. This study included 41 patients older than 70 years who had received one or more BTxis for the first time between January 2018 and December 2021. Most of the older people we included lived in an institution (66%), manifested severe dependence, and presented significant morbi-mortality (37% of the patients died in the year after the last injection). The main objectives of these injections were purely comfort, without any functional goals. The GAS scores suggested effectiveness for comfort GAS scores. No complications were recorded. This study highlights the BTxis potential to address the needs of a larger number of older patients with ADH.

Cervicothoracic ventral-dorsal rhizotomy for treatment of brachial hypertonia in cerebral palsy.

PURPOSE: Cervicothoracic ventral-dorsal rhizotomy (VDR) is a potential treatment of medically refractory hypertonia in patients who are not candidates for intrathecal baclofen, particularly in cases of severe upper limb hypertonia with limited to no function. A longitudinal cohort was identified to highlight our institutional safety and efficacy using cervicothoracic VDR for the treatment of hypertonia. METHODS: Retrospective data analysis was performed for patients that underwent non-selective cervicothoracic VDR between 2022 and 2023. Non-modifiable risk factors, clinical variables, and operative characteristics were collected. RESULTS: Six patients (three female) were included. Four patients underwent a bilateral C6-T1 VDR, one patient underwent a left C7-T1 VDR, and another underwent a left C6-T1 VDR. Three patients had quadriplegic mixed hypertonia, one patient had quadriplegic spasticity, one patient had triplegic mixed hypertonia, and one patient had mixed hemiplegic hypertonia. The mean difference of proximal upper extremity modified Ashworth scale (mAS) was - 1.4 ± 0.55 (p = 0.002), and - 2.2 ± 0.45 (p < 0.001) for the distal upper extremity. Both patients with independence noted quality of life improvements as well as increased ease with dressing and orthotics fits. Caregivers for the remaining four patients noted improvements in caregiving provision, mainly in dressing, orthotics fit, and ease when transferring. CONCLUSION: Cervicothoracic VDR is safe and provides tone control and quality of life improvements in short-term follow-up. It can be considered for the treatment of refractory hypertonia. Larger multicenter studies with longer follow-up are necessary to further determine safety along with long-term functional benefits in these patients.

Immediate effects of TECAR therapy on lower limb to decrease hypertonia in chronic stroke survivors: a randomized controlled trial.

PURPOSE: To analyze immediate effects of TECAR therapy (TT) to reduce lower limb hypertonia and improve functionality in chronic post-stroke. MATERIALS AND METHODS: It is a single-blind randomized controlled clinical trial. A total of 36 chronic stroke survivors were divided into two groups. The experimental group received a single 30-minute session of TT with functional massage (FM) on lower limb. The control group received a single 30-minute session sham treatment of TT plus FM. The primary outcome measure was hypertonia (Modified Ashworth Scale, MAS). Secondary outcomes were gait speed (4-Meter Walk-Test), standing knee-flexion (Fugl-Meyer Assessment Scale IV-item), change in weight bearing ankle dorsiflexion (Ankle Lunge Test, ALT), and functional lower limb strength (5-Times Sit-to-Stand Test). All measurements were performed at baseline, immediately and 30-minutes after treatment. RESULTS: There was a group-time interaction in MAS-knee (p = 0.044), MAS-ankle (p = 0.018) and ALT (p = 0.016) between T1 and T0 (p<.0001) and T2 and T0 (p<.0001) for the experimental group. There was a significant increase in ALT between T1 and T0 (p = 0.003) in the control group. CONCLUSIONS: A single session of TT performed at the same time as FM immediately reduces plantar-flexors and knee-extensor muscle hypertonia and increases change in weight bearing ankle dorsiflexion in chronic stroke survivors.

Capacitive and resistive electric transfer (TECAR) therapy may improve ankle mobility in stroke survivors.TECAR therapy may improve knee mobility in post-stroke.TECAR therapy may reduce lower limb muscle tone in stroke survivors.TECAR therapy could be used in combination with physiotherapy as a safe technique for the immediate reduction of hypertonia in stroke survivors.

Nonselective lumbosacral ventral-dorsal rhizotomy for the management of lower-limb hypertonia in nonambulatory children with cerebral palsy.

OBJECTIVE: Children with cerebral palsy (CP) often experience medically refractory hypertonia, for which there are surgical therapies including neuromodulation and rhizotomy. Traditional surgical treatment for medically refractory mixed hypertonia or dystonia includes intrathecal baclofen pumps and selective dorsal rhizotomy. A nonselective lumbosacral ventral-dorsal rhizotomy (VDR; ventral and dorsal roots lesioned by 80%-90%) has the potential to address the limitations of traditional surgical options. The authors highlighted the institutional safety and efficacy of nonselective lumbosacral VDR for palliative tone management in nonambulatory patients with more severe CP. METHODS: The authors performed a retrospective analysis of patients who had undergone lumbosacral VDR between 2022 and 2023. Demographic factors, clinical variables, and operative characteristics were collected. The primary outcomes of interest included tone control and quality of life improvement. Secondary outcome measures included, as a measure of safety, perioperative events such as paresthesias. Postoperative complications were also noted. RESULTS: Fourteen patients (7 female) were included in the study. All patients had undergone a T12-L2 osteoplastic laminoplasty and bilateral L1-S1 VDR. Nine patients had quadriplegic mixed hypertonia, 4 had quadriplegic spasticity, and 1 had generalized secondary dystonia. Following VDR, there was a significant decrease in both lower-extremity modified Ashworth Scale (mAS) scores (mean difference [MD] -2.77 ± 1.0, p < 0.001) and upper-extremity mAS scores (MD -0.71 ± 0.76, p = 0.02), with an average follow-up of 3 months. In the patient with generalized dystonia, the lower-extremity Barry-Albright Dystonia Scale score decreased from 8 to 0, and the overall score decreased from 32 to 13. All parents noted increased ease in caregiving, particularly in terms of positioning, transfers, and changing. The mean daily enteral baclofen dose decreased from 47 mg preoperatively to 24.5 mg postoperatively (p < 0.001). Three patients developed wound dehiscence, 2 of whom had concurrent infections. CONCLUSIONS: Lumbosacral VDR is safe, is effective for tone control, and can provide quality of life improvements in patients with medically refractory lower-limb mixed hypertonia. Lumbosacral VDR can be considered for palliative tone control in nonambulatory patients with more severe CP. Larger studies with longer follow-ups are necessary to further determine safety and long-term benefits in these patients.

Safety and efficacy of continuous intrathecal baclofen via cervical catheter tip: a retrospective case series.

OBJECTIVE: Intrathecal baclofen (ITB) is an effective treatment for hypertonia in children involving the implantation of a pump and catheter system. The highest concentration of ITB is at the catheter tip. The catheter tip location is most commonly within the lumbar or thoracic spine. The cervical tip location has traditionally been avoided because of concerns of hypoventilation and pneumonia; however, these complications in cervical compared with thoracic or lumbar placement have not been reliably proven. Some studies have suggested that cervical ITB location better treats upper-extremity hypertonia. There are limited data describing the safety and efficacy of cervical ITB on hypertonia. The authors present a single-institution retrospective case series highlighting the safety and efficacy of using cervical ITB location for the treatment of hypertonia. METHODS: Retrospective data analysis was performed for children who underwent continuous dosing cervical ITB between April 2022 and October 2023. Nonmodifiable risk factors, clinical variables, operative characteristics, and adverse outcomes were collected. RESULTS: This study included 25 patients (8 female). The mean age at implantation was 12.4 years, and the mean operative duration was 90 minutes. The mean Barry-Albright Dystonia Scale score decreased by 9.5 points (p = 0.01). The mean aggregated modified Ashworth scale score in the upper extremities decreased by 2.14 points (p = 0.04), and that in the lower extremities decreased by 4.98 points (p < 0.01). One patient each (4%) had infection and baclofen toxicity. Two patients (8%) had respiratory depression requiring continuous positive airway pressure. There was no incidence of pneumonia or wound dehiscence. CONCLUSIONS: The cervical catheter tip location for ITB is safe, is effective to control tone, and should be considered for the treatment of hypertonia. Larger studies with longer follow-up are necessary to further determine upper-limit dosing safety along with long-term functional benefits in these patients.

Percutaneous needle tenotomies: indications, procedures, efficacy and safety. A systematic review.

BACKGROUND: Percutaneous needle tenotomies constitute a promising approach that enables direct access to tendons through minimally invasive interventions. They can be performed rapidly without need for large incisions or general anaesthesia. However, the reported procedures are heterogeneous and currently conducted without guidelines. OBJECTIVES: We aimed to determine the indications for percutaneous needle tenotomies described in the current literature. Our secondary aim was to identify the different procedures reported, as well as their efficacy and their safety. METHODS: A systematic review following PRISMA guidelines was conducted to identify original articles that mentioned percutaneous needle tenotomy in humans and reported its application, description, effectiveness or adverse events. Non-percutaneous tendinous surgical procedures and ineligible designs were excluded. The Downs and Black checklist was used to assess the risk of bias. RESULTS: A total of 540 studies were identified from the MEDLINE, Embase, Cochrane Library, and PEDro databases. Fourteen clinical studies met the inclusion criteria and were found to have an acceptable quality (674 individuals, 1664 tenotomies). Our results indicated a wide variety of indications for percutaneous needle tenotomies in children and in adults. We highlighted 24 tendons as eligible targets in the upper and lower limbs. Tenotomies were performed with either 16- or 18-Ga needles, lasted from 1 to 30 min, and were performed using various procedures. Their efficacy was mainly assessed through clinical outcomes highlighting tendon discontinuity on palpation after the procedure. Passive range-of-motion gains after tenotomy were reported for both upper and lower limbs with an estimated 5 % complication rate. CONCLUSION: This is the first review to systematically synthesize all the available evidence on the indications, procedures, efficacy and safety of percutaneous tenotomies exclusively performed with needles. Current evidence suggests that procedures are safe and effective for treating various deformities. PROSPERO REGISTRATION: CRD42022350571.

Hypertonia of the Big Toe Revealing Parkinson's Disease: A Case Report.

Despite being less commonly discussed than other motor symptoms such as tremors and bradykinesia, hypertonia of the hallux holds diagnostic and prognostic significance in Parkinson's disease (PD). This motor anomaly is dissected within the context of the broader clinical spectrum of PD symptoms, emphasizing its importance alongside its cardinal symptoms. This case report underscores the importance of accurate clinical assessment especially thorough neurological evaluation in discerning hallux hypertonia, potentially enabling early disease recognition and intervention. By synthesizing these clinical insights, we trust that this case report contributes to an enhanced understanding of hypertonia of the hallux as a distinctive clinical presentation in PD fostering improved diagnostic precision.

The effect of suboccipital muscle dysfunction on the biomechanics of the upper cervical spine: a study based on finite element analysis.

OBJECTIVE: Muscle dysfunction caused by repetitive work or strain in the neck region can interfere muscle responses. Muscle dysfunction can be an important factor in causing cervical spondylosis. However, there has been no research on how the biomechanical properties of the upper cervical spine change when the suboccipital muscle group experiences dysfunction. The objective of this study was to investigate the biomechanical evidence for cervical spondylosis by utilizing the finite element (FE) approach, thus and to provide guidance for clinicians performing acupoint therapy. METHODS: By varying the elastic modulus of the suboccipital muscle, the four FE models of C0-C3 motion segments were reconstructed under the conditions of normal muscle function and muscle dysfunction. For the two normal condition FE models, the elastic modulus for suboccipital muscles on both sides of the C0-C3 motion segments was equal and within the normal range In one muscle dysfunction FE model, the elastic modulus on both sides was equal and greater than 37 kPa, which represented muscle hypertonia; in the other, the elastic modulus of the left and right suboccipital muscles was different, indicating muscle imbalance. The biomechanical behavior of the lateral atlantoaxial joint (LAAJ), atlanto-odontoid joint (ADJ), and intervertebral disc (IVD) was analyzed by simulations, which were carried out under the six loadings of flexion, extension, left and right lateral bending, left and right axial rotation. RESULTS: Under flexion, the maximum stress in LAAJ with muscle imbalance was higher than that with normal muscle and hypertonia, while the maximum stress in IVD in the hypertonic model was higher than that in the normal and imbalance models. The maximum stress in ADJ was the largest under extension among all loadings for all models. Muscle imbalance and hypertonia did not cause overstress and stress distribution abnormalities in ADJ. CONCLUSION: Muscle dysfunction increases the stress in LAAJ and in IVD, but it does not affect ADJ.

Cervicothoracic ventral-dorsal rhizotomy for bilateral upper-extremity hypertonia in cerebral palsy: illustrative case.

BACKGROUND: Management of medically refractory limb-specific hypertonia is challenging. Neurosurgical options include deep brain stimulation, intrathecal baclofen, thalamotomy, pallidotomy, or rhizotomy. Cervical dorsal rhizotomy has been successful in the treatment of upper-extremity spasticity. Cervical ventral and cervical ventral-dorsal rhizotomy (VDR) has been used in the treatment or torticollis and traumatic hypertonia; however, the use of cervicothoracic VDR for the treatment of upper-extremity mixed hypertonia is not well described. OBSERVATIONS: A 9-year-old girl with severe quadriplegic mixed hypertonia secondary to cerebral palsy (CP) underwent cervicothoracic VDR. Modified Ashworth Scale scores, provision of caregiving, and examination improved. Treatment was well tolerated. LESSONS: Cervicothoracic VDR can afford symptomatic and quality of life improvement in patients with medically refractory limb hypertonia. Intraoperative positioning and nuances in surgical techniques are particularly important based on spinal cord position as modified by scoliosis. Here, the first successful use of cervicothoracic VDR for the treatment of medically refractory upper-limb hypertonia in a pediatric patient with CP is described.

Case report: Intrathecal baclofen therapy improved gait pattern in a stroke patient with spastic dystonia.

Background: Intrathecal baclofen (ITB) therapy, a viable alternative for unsuitable candidates of conventional spasticity medications, is a preferred method of administration over the oral route. Owing to its enhanced bioavailability, ITB ensures a more effective delivery at the target site. Objective: There is a lack of conclusive evidence regarding the use of ITB treatment in managing ambulatory patients with spastic dystonia. Before ITB pump implantation, patients commonly undergo an ITB bolus injection trial to rule out potential adverse reactions and verify the therapeutic effects on hypertonic issues. In this report, we highlight a case of spastic dystonia, particularly focusing on an ambulatory patient who demonstrated significant improvement in both the modified Ashworth scale (MAS) score and gait pattern following the ITB injection trial. Case report: This case report outlines the medical history of a 67-year-old male diagnosed with left-side hemiplegia and spastic dystonia, resulting from his second episode of intracranial hemorrhage in the right thalamus. An ITB injection trial was initiated because the patient was not suitable for continued botulinum toxin injections and oral medications. This was due to the persistent occurrence of spastic dystonia in both the upper and lower extremities. The patient underwent a four-day ITB injection trial with progressively increasing doses, resulting in improved MAS scores and gait parameters, including cadence, step length, step time, stride length, and stride time were increased. Particularly, kinematic gait analysis demonstrates a substantial improvement of increased knee flexion in the swing phase in stiff knee gait pattern. These findings indicated a gradual reduction in spasticity-related symptoms, signifying the positive effect of the ITB injection trial. The patient eventually received an ITB pump implantation. Conclusion: In this post-stroke patient with spastic dystonia, ITB therapy has demonstrated effective and substantial management of spasticity, along with improvement in gait patterns.

Variants in tropomyosins TPM2 and TPM3 causing muscle hypertonia.

Patients with myopathies caused by pathogenic variants in tropomyosin genes TPM2 and TPM3 usually have muscle hypotonia and weakness, their muscle biopsies often showing fibre size disproportion and nemaline bodies. Here, we describe a series of patients with hypercontractile molecular phenotypes, high muscle tone, and mostly non-specific myopathic biopsy findings without nemaline bodies. Three of the patients had trismus, whilst in one patient, the distal joints of her fingers flexed on extension of the wrists. In one biopsy from a patient with a rare TPM3 pathogenic variant, cores and minicores were observed, an unusual finding in TPM3-caused myopathy. The variants alter conserved contact sites between tropomyosin and actin.

Safety and efficacy of intrathecal baclofen trials for the treatment of hypertonia: a retrospective cohort study.

OBJECTIVE: Intrathecal baclofen (ITB) is an effective treatment for refractory hypertonia in children. ITB has long been effective for the treatment of spasticity, and indications have naturally evolved to include dystonia and mixed pediatric movement disorders (PMDs). The established uses for ITB trials are insurance prerequisite, mixed tone, and family request. Despite agreement for ITB therapy by a multidisciplinary group of subspecialists in a complex PMD program, insurance companies often require an ITB trial be performed. A longitudinal cohort was identified to determine the safety and efficacy of ITB trials and to determine the utility of test dosing in this population. METHODS: Retrospective data analysis was performed for patients with hypertonia who underwent ITB bolus trials at the authors' institution between 2021 and 2023. Nonmodifiable risk factors and clinical variables were collected. RESULTS: Thirty-one patients (11 female) underwent 32 ITB trials. Of these patients, 67.7% had a diagnosis of mixed hypertonia, 32.3% pure spasticity, and 9.1% secondary dystonia. The mean age at test dose was 12.8 years, and 58.1% of patients were born premature. The mode Gross Motor Function Classification System score was 5. The mean difference in Barry-Albright Dystonia Scale (BADS) scores was -7.33 points (p = 0.01) at 2.5 hours postoperatively. The mean difference in upper-extremity modified Ashworth Scale (mAS) scores was -5.36 points (p = 0.003), and that for lower-extremity mAS scores was -6.61 (p < 0.001). In total, 21.9% of patients developed a post-dural puncture headache. Conversion to a permanent baclofen pump was performed in 22/32 (68.8%) patients. Of those who did not pursue pump placement, 1 patient had high surgical risk, 1 had an ineffective response, 1 had a bad reaction to the test dose and cited both regression and increased discomfort, and 2 declined despite an effective trial owing to family preferences. CONCLUSIONS: ITB trials require hospitalization in some form and carry risks of procedural complications. The decision to pursue a trial should be made on a case-by-case basis by clinicians and should not be determined by insurance companies. The complication rate of ITB trials is high, and a test dose is unnecessary in this fragile population.

From one to many: Hypertonia in schizophrenia spectrum psychosis an integrative review and adversarial collaboration report.

Different types of resistance to passive movement, i.e. hypertonia, were described in schizophrenia spectrum disorders (SSD) long before the introduction of antipsychotics. While these have been rediscovered in antipsychotic-naïve patients and their non-affected relatives, the existence of intrinsic hypertonia vs drug-induced parkinsonism (DIP) in treated SSD remains controversial. This integrative review seeks to develop a commonly accepted framework to specify the putative clinical phenomena, highlight conflicting issues and discuss ways to challenge each hypothesis and model through adversarial collaboration. The authors agreed on a common framework inspired from systems neuroscience. Specification of DIP, locomotor paratonia (LMP) and psychomotor paratonia (PMP) identified points of disagreement. Some viewed parkinsonian rigidity to be sufficient for diagnosing DIP, while others viewed DIP as a syndrome that should include bradykinesia. Sensitivity of DIP to anticholinergic drugs and the nature of LPM and PMP were the most debated issues. It was agreed that treated SSD should be investigated first. Clinical features of the phenomena at issue could be confirmed by torque, EMG and joint angle measures that could help in challenging the selectivity of DIP to anticholinergics. LMP was modeled as the release of the reticular formation from the control of the supplementary motor area (SMA), which could be challenged by the tonic vibration reflex or acoustic startle. PMP was modeled as the release of primary motor cortex from the control of the SMA and may be informed by subclinical echopraxia. If these challenges are not met, this would put new constraints on the models and have clinical and therapeutic implications.

Spatial mapping of posture-dependent resistance to passive displacement of the hypertonic arm post-stroke.

BACKGROUND: Muscles in the post-stroke arm commonly demonstrate abnormal reflexes that result in increased position- and velocity-dependent resistance to movement. We sought to develop a reliable way to quantify mechanical consequences of abnormal neuromuscular mechanisms throughout the reachable workspace in the hemiparetic arm post-stroke. METHODS: Survivors of hemiparetic stroke (HS) and neurologically intact (NI) control subjects were instructed to relax as a robotic device repositioned the hand of their hemiparetic arm between several testing locations that sampled the arm's passive range of motion. During transitions, the robot induced motions at either the shoulder or elbow joint at three speeds: very slow (6°/s), medium (30°/s), and fast (90°/s). The robot held the hand at the testing location for at least 20 s after each transition. We recorded and analyzed hand force and electromyographic activations from selected muscles spanning the shoulder and elbow joints during and after transitions. RESULTS: Hand forces and electromyographic activations were invariantly small at all speeds and all sample times in NI control subjects but varied systematically by transport speed during and shortly after movement in the HS subjects. Velocity-dependent resistance to stretch diminished within 2 s after movement ceased in the hemiparetic arms. Hand forces and EMGs changed very little from 2 s after the movement ended onward, exhibiting dependence on limb posture but no systematic dependence on movement speed or direction. Although each HS subject displayed a unique field of hand forces and EMG responses across the workspace after movement ceased, the magnitude of steady-state hand forces was generally greater near the outer boundaries of the workspace than in the center of the workspace for the HS group but not the NI group. CONCLUSIONS: In the HS group, electromyographic activations exhibited abnormalities consistent with stroke-related decreases in the stretch reflex thresholds. These observations were consistent across repeated testing days. We expect that the approach described here will enable future studies to elucidate stroke's impact on the interaction between the neural mechanisms mediating control of upper extremity posture and movement during goal-directed actions such as reaching and pointing with the arm and hand.

Proof-of-concept for characterization of neurodegenerative disorders utilizing two non-REM sleep biomarkers.

Study objective: This proof-of-concept study aimed to determine whether the combined features of two non-rapid eye movement (NREM) sleep biomarkers acquired predominantly in-home could characterize different neurodegenerative disorders. Methods: Sleep spindle duration and non-REM hypertonia (NRH) were evaluated in seven groups including a control group (CG = 61), and participants with isolated REM sleep behavior disorder (iRBD = 19), mild cognitive impairment (MCI = 41), Parkinson disease (PD = 16), Alzheimer disease dementia (ADem = 29), dementia with Lewy Bodies or Parkinson disease dementia (LBD = 19) and progressive supranuclear palsy (PSP = 13). One-way analysis of variance (ANOVA), Mann-Whitney U, intra-class (ICC) and Spearman ranked correlations, Bland-Altman plots and Kappa scores, Chi-square and Fisher exact probability test, and multiple-logistic regression were focused primarily on spindle duration and NRH and the frequencies assigned to the four normal/abnormal spindle duration/NRH combinations. Results: ANOVA identified group differences in age, sleep efficiency, REM, NRH (p < 0.0001) and sleep time (p = 0.015), Spindle duration and NRH each demonstrated good night-to-night reliabilities (ICC = 0.95 and 0.75, Kappa = 0.93 and 0.66, respectively) and together exhibited an association in the PD and LBD groups only (p < 0.01). Abnormal spindle duration was greater in records of PSP (85%) and LBD (84%) patients compared to CG, MCI, PD and ADem (p < 0.025). Abnormal NRH was greater in PSP = 92%, LBD = 79%, and iRBD = 74% compared to MCI = 32%, ADem = 17%, and CG = 16% (p < 0.005).The combination biomarker normal spindle duration/normal NRH was observed most frequently in CG (56%) and MCI (41%). ADem most frequently demonstrated normal spindle duration/normal NRH (45%) and abnormal spindle duration/normal NRH (38%). Normal spindle duration/abnormal NRH was greatest in iRBD = 47%, while abnormal spindle duration/abnormal NRH was predominant in PSP = 85% and LBD = 74%. Conclusion: The NREM sleep biomarkers spindle duration and NRH may be useful in distinguishing patients with different neurodegenerative disorders. Larger prospective cohort studies are needed to determine whether spindle duration and NRH can be combined for prodromal assessment and/or monitoring disease progression.

Positional Change Used to Manage Postextubation Respiratory Failure in a Child With Cerebral Palsy.

Dental treatment for patients with cerebral palsy (CP) is often performed under general anesthesia due to involuntary movements that can render dental treatment difficult. Since CP is often accompanied by spasticity, care must be taken when positioning patients during general anesthesia. We report the management of a 14-year-old girl with CP and epilepsy undergoing general anesthesia for dental treatment who experienced respiratory failure due to acute thoracoabdominal muscle hypertonia after extubation. She had a history of cardiac arrest due to respiratory failure caused by acute muscle hypertonia and successful resuscitation. General anesthesia was induced after careful positioning of the patient to prevent spastic muscle stretching, and the dental treatment was completed without complications. However, upon awakening after extubation, the patient developed respiratory failure due to acute muscle hypertonia. The patient was resedated and repositioned from a supine to a sitting position, and her symptoms improved. There was no recurrence of muscle hypertonia, and she recovered fully without complications. In this case, respiratory failure associated with acute muscle hypertonia was successfully managed by position change after initial treatment with positive-pressure ventilation and propofol. It is important to be prepared for the possibility of respiratory failure associated with acute muscle hypertonia and its countermeasures when providing general anesthesia for patients with CP.