Efficacy of leuprorelin in spinal and bulbar muscular atrophy: a 3-year observational study.

OBJECTIVE: This study aimed to investigate the long-term effects and functional outcomes of androgen suppression therapy using leuprorelin among Korean patients with spinal and bulbar muscular atrophy (SBMA). METHODS: This observational study enrolled patients with genetically confirmed SBMA who provided informed consent. Leuprorelin was administered via subcutaneous injection every 12 weeks. The primary outcome measure was the change in total Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS) scores. RESULTS: A total of 48 SBMA patients were evaluated in this study. Among them, 39 patients underwent androgen suppression therapy over a 3-year period. The total SBMAFRS score decreased from 41.72 ± 5.55 to 36.74 ± 7.74 (p < 0.001) in patients who completed their treatment. The subgroup with a baseline SBMAFRS score of ≥ 42 had a significantly lower decline in SBMAFRS score than did those with a baseline SBMAFRS score of ≤ 41. We determined that at a baseline, SBMAFRS cutoff value of 41.5 could predict good prognosis, with a corresponding area under the curve of 0.689. CONCLUSION: Despite androgen suppression therapy, all enrolled participants exhibited a decrease in the overall SBMAFRS score. However, those with a baseline SBMAFRS of ≥ 42 showed a mild decrease in scores, indicating a more favorable prognosis. These findings suggest that a higher baseline motor function was a key prognostic indicator in SBMA treatment and that initiating early leuprorelin treatment in patients with high baseline function may lead to good clinical outcomes.

Effects of Alterations in Resting-State Neural Networks on the Severity of Neuropathic Pain after Spinal Cord Injury.

Neuropathic pain (NP) following spinal cord injury (SCI) is refractory to pain control strategies, and the underlying neuronal mechanisms remain poorly understood. This study aimed to determine the brain regions engaged in maintaining a spontaneous resting state and the link between those regions and the severity of NP in patients with incomplete SCI. Seventy-three subjects (41 patients and 32 age- and sex-matched healthy controls) participated in this retrospective study. Regarding the neurological level of injury, patients with incomplete SCI experienced at-level or below-level NP. The severity of NP was evaluated using a visual analog scale (VAS), and patients were divided into mild and moderate-severe NP groups based on VAS scores. Graph theory and fractional amplitude of low-frequency fluctuation (fALFF) analyses were performed to compare resting-state functional magnetic resonance imaging (fMRI) analysis results among the three groups. Graph theory analysis was performed through a region of interest (ROI)-to-ROI analysis and then fALFF analysis was performed in the brain regions demonstrating significant differences among the three groups analyzed using the graph theory. We evaluated whether the brain regions showing significant differences using graph theory and fALFF correlated with the VAS scores. Patients with moderate-severe NP showed reduced node degree and fALFF in the left middle frontal gyrus compared with those with mild NP and healthy controls. Furthermore, patients with severe NP demonstrated increased average path lengths and reduced fALFF values in the posterior cingulate gyrus. This study found that changes in intrinsic oscillations of fMRI signals in the middle frontal gyrus and posterior cingulate gyrus were significant considering the severity of NP.

Diagnostic performance of synthetic relaxometry for predicting neurodevelopmental outcomes in premature infants: a feasibility study.

OBJECTIVES: To investigate the predictability of synthetic relaxometry for neurodevelopmental outcomes in premature infants and to evaluate whether a combination of relaxation times with clinical variables or qualitative MRI abnormalities improves the predictive performance. METHODS: This retrospective study included 33 premature infants scanned with synthetic MRI near or at term equivalent age. Based on neurodevelopmental assessments at 18-24 months of corrected age, infants were classified into two groups (no/mild disability [n = 23] vs. moderate/severe disability [n = 10]). Clinical and MRI characteristics associated with moderate/severe disability were explored, and combined models incorporating independent predictors were established. Ultimately, the predictability of relaxation times, clinical variables, MRI findings, and a combination of the two were evaluated and compared. The models were internally validated using bootstrap resampling. RESULTS: Prolonged T1-frontal/parietal and T2-parietal periventricular white matter (PVWM), moderate-to-severe white matter abnormality, and bronchopulmonary dysplasia were significantly associated with moderate/severe disability. The overall predictive performance of each T1-frontal/-parietal PVWM model was comparable to that of individual MRI finding and clinical models (AUC = 0.71 and 0.76 vs. 0.73 vs. 0.83, respectively; p > 0.27). The combination of clinical variables and T1-parietal PVWM achieved an AUC of 0.94, sensitivity of 90%, and specificity of 91.3%, outperforming the clinical model alone (p = 0.049). The combination of MRI finding and T1-frontal PVWM yielded AUC of 0.86, marginally outperforming the MRI finding model (p = 0.09). Bootstrap resampling showed that the models were valid. CONCLUSIONS: It is feasible to predict adverse outcomes in premature infants by using early synthetic relaxometry. Combining relaxation time with clinical variables or MRI finding improved prediction. CLINICAL RELEVANCE STATEMENT: Synthetic relaxometry performed during the neonatal period may serve as a biomarker for predicting adverse neurodevelopmental outcomes in premature infants. KEY POINTS: • Synthetic relaxometry based on T1 relaxation time of parietal periventricular white matter showed acceptable performance in predicting adverse outcome with an AUC of 0.76 and an accuracy of 78.8%. • The combination of relaxation time with clinical variables and/or structural MRI abnormalities improved predictive performance of adverse outcomes. • Synthetic relaxometry performed during the neonatal period helps predict adverse neurodevelopmental outcome in premature infants.

Electrophysiologic Patterns of Symptomatic Vincristine-Induced Peripheral Neuropathy in Children with Acute Lymphocytic Leukemia.

Acute lymphocytic leukemia is one of the most common cancers in children. Multi-drug chemotherapy is used for treatment, and the representative drug is vincristine. Although various side effects may occur due to vincristine, the association with peripheral neuropathy is high compared to that of other drugs. This study focused on children under the age of 18 years of age with ALL who received chemotherapy containing vincristine. We retrospectively analyzed the results of a nerve conduction study and a cumulative dose of vincristine in 30 children diagnosed with peripheral neuropathy. The average cumulative dose until diagnosis of vincristine-induced peripheral neuropathy was 14.99 ± 1.21 mg/m2, and motor nerves were predominantly involved. Additionally, a marked decrease in average amplitude was also observed in motor nerves. In addition, when the relationship between the incidence of peripheral neuropathy and the cumulative dose was analyzed through the survival curve, about 50% of children developed peripheral neuropathy at a dose of 15.5 ± 1.77 mg/m2. Based on the electrophysiological characteristics of pediatric vincristine-induced peripheral neuropathy, as well as the relationship between the incidence rate and the cumulative dose, it is possible to observe more closely the vincristine-induced peripheral neuropathy occurrence in children with ALL at an appropriate time.

Factor Analysis Affecting Degree of Depression in Family Caregivers of Patients with Spinal Cord Injury: A Cross-Sectional Pilot Study.

This study was conducted to determine the degree of depression in family caregivers of spinal-cord-injury patients and to identify factors influencing family caregivers' depression. The final study subjects were 30 (family caregivers: 6 males and 24 females). The CES-D of family caregivers; general characteristics of spinal-cord-injury patients and family caregivers; and information on physical health, household income, leisure, social activity, family relationship, and life-in-general status of family caregiver were collected. A frequency analysis, normality test, Mann-Whitney test, Kruskal-Wallis test, Spearman Correlation analysis, hierarchical regression analysis, and spider network through a path model analysis were performed. As for the general characteristics, when the patient was economically active, the caregiver's depression was mean ± SD; 2.04 ± 0.71; otherwise, it was mean ± SD 2.86 ± 0.74, indicating that the caregiver of the non-economic activity patient was more depressed (p = 0.013). In Model 1 of the multiple regression analysis to understand the effect on the depression of the caregiver, it was confirmed that the depression of the caregiver decreased as the family caregiver had more leisure and social activities (B = -0.718, p = 0.001). In Model 2, it was found that the depression of caregivers increased when the patient did not engage in economic activity (B = 0.438, p = 0.016). In the spider-web form through the path model analysis, as the family's economic level increased, physical health increased by B = 0.755 (p < 0.001), and the increase in physical health (B = 0.424, p = 0.042) was, in turn, a factor in the increase of engagement in leisure and social activities. Various policies will be needed for the successful return to society of spinal-cord-injury patients by ensuring that their leisure and social activities and establishing measures to support their economical income.

Effect of computerized cognitive rehabilitation in comparison between young and old age after traumatic brain injury.

INTRODUCTION: Cognitive dysfunction reduces patients' quality of life and social participation with traumatic brain injury (TBI). Computerized cognitive rehabilitation is increasingly being used for cognitive therapy in TBI patients. The purpose of this study was to investigate the influence of age on the effect of computerized cognitive rehabilitation in cognitive dysfunction after TBI. MATERIAL AND METHODS: A total of 34 patients with cognitive dysfunction after TBI were enrolled. Participants performed 30 sessions of computerized cognitive rehabilitation (Comcog) for 6 weeks. A cognitive evaluation was performed before and after treatment with Mini-Mental State Examination (MMSE) and Computerized Neurophyschologic Test (CNT). RESULTS: There were no cognitive tests that differed between the young group and the old group at baseline. However, after computerized cognitive rehabilitation, the young group showed significant improvement compared to the old group in verbal memory, visual memory, attention, and visuo-motor coordination tests. The young group showed improvement in MMSE, verbal and visual memory, and visuo-motor coordination tests after computerized cognitive rehabilitation. In contrast, the old group showed significant improvement only in MMSE and visual learning test, one of the visual memory tests. CONCLUSION: Our findings demonstrate that age may be an important factor related to the effect of computer cognitive rehabilitation on cognitive dysfunction after TBI. Methodologically more ordered studies with larger sample sizes are needed in the future.

Analysis of the physiological load on lumbar vertebrae in patients with osteoporosis: a finite-element study.

This study aims to investigate the difference in physiological loading on the spine in three different motions (flexion-extension, lateral bending, and axial rotation) between osteoporotic and normal spines, using finite element modelling. A three-dimensional finite element (FE) model centered on the lumbar spine was constructed. We applied two different material properties of osteoporotic and normal spines. For the FE analysis, three loading conditions (flexion-extension, lateral bending, and axial rotation) were applied. The von Mises stress was higher on the nucleus pulposus at all vertebral levels in all movements, in the osteoporosis group than in the normal group. On the annulus fibrosus, the von Mises stress increased at the level of L3-L4, L4-L5, and L5-S in the flexion-extension group and at L4-L5 and L5-S levels in the lateral bending group. The values of two motions, flexion-extension and lateral bending, increased in the L4 and L5 cortical bones. In axial rotation, the von Mises stress increased at the level of L5 of cortical bone. Additionally, the von Mises stress increased in the lower endplate of L5-S and L4-L5 in all movements, especially lateral bending. Even in the group with no increase, there was a part that received increased von Mises stress locally for each element in the three-dimensional reconstructed view of the pressure distribution in color. The von Mises stress on the lumbar region in the three loading conditions, was greater in most components of osteoporotic vertebrae than in normal vertebrae and the value was highest in the nucleus pulposus. Considering the increase in the measured von Mises stress and the local increase in the pressure distribution, we believe that these results can contribute to explaining discogenic pain and degeneration.

Correlation Between Maximal Tongue Pressure and Swallowing Function in Spinal and Bulbar Muscular Atrophy.

Background: Spinal and bulbar muscular atrophy (SBMA) is an X-lined motor neuron disease characterized by progressive muscle weakness, bulbar palsy, and dysphagia. Dysphagia is associated with tongue weakness, which is a common manifestation of SBMA. This study aimed to investigate the correlations between tongue pressure and dysphagia in patients with SBMA. Materials and Methods: Thirty-nine genetically confirmed SBMA patients underwent a videofluoroscopic swallowing study (VFSS) and tongue pressure assessment. Then, we analyzed the maximal tongue pressure (MTP), oral transit time, penetration-aspiration scale (PAS), videofluoroscopic dysphagia scale (VDS), amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R), and 6-min walk test (6MWT). Pearson and Spearman correlation coefficients were calculated to analyze the association of the MTP with clinical, swallowing, and functional parameters. Results: In the correlation analysis, MTP was negatively correlated with disease duration (r = -0.396, p = 0.013) and VDS (r = -0.426, p = 0.007), and positively correlated with ALSFRS-R (r = 0.483, p = 0.002) and 6MWT (r = 0.396, p = 0.013). The bulbar (r = 0.367, p = 0.022) and gross motor (r = 0.486, p = 0.002) domains of the ALSFRS-R were correlated with MTP. Conclusion: Tongue pressure assessment can be used as a safe and easy tool to assess swallowing function in SBMA patients. Moreover, MTP reflects functional states, including activities of daily living and gait performance, showing it to be a potential biomarker for physical performance in SBMA.

Effect of leuprorelin in bulbar function of spinal and bulbar muscular atrophy patients: observational study for 1 year.

BACKGROUND: This study aimed to investigate the effect of androgen suppression therapy using leuprorelin focused on the bulbar function of patients with spinal and bulbar muscular atrophy (SBMA). METHODS: Genetically confirmed SBMA patients who consented to participate in this observational study were enrolled. Leuprorelin was subcutaneously injected every 12 weeks. Videofluoroscopic swallowing study was performed at baseline and after androgen suppression therapy for 1 year. The primary outcome measures were the changes in the vallecular residue and pyriform sinus residue. The videofluoroscopic swallowing study data were analyzed and interpreted by two experienced physiatrists. RESULTS: A total of 40 patients with SBMA were analyzed in this study. The inter-rater reliability testing showed good agreement for the pharyngeal residue (ICC = 0.84) and videofluoroscopic dysphagia scale (ICC = 0.75). The vallecular residue and pyriform sinus residue after swallowing 9 mL yogurt were significantly reduced (26.8 ± 22.6 to 14.6 ± 14.5, p < 0.001, 14.9 ± 16.9 to 7.6 ± 9.9, p < 0.001, respectively). The swallowing subscore of amyotrophic lateral sclerosis functional rating scale-revised improved after androgen suppression therapy (3.3 ± 0.5 to 3.5 ± 0.6, p = 0.041). CONCLUSIONS: Leuprorelin significantly reduced the pharyngeal residue in patients with SBMA after 1 year of treatment without any serious adverse events and longitudinal studies are needed to confirm these results.

Dysfunction of anterior insula in the non- affected hemisphere in patients with post- stroke depression: A resting-state fMRI study.

BACKGROUND: Post-stroke depression (PSD) is a consequential neuropsychiatric sequela that occurs after stroke. However, the pathophysiology of PSD are not well understood yet. OBJECTIVE: To explore alterations in functional connectivity (FC) between anterior insula and fronto-cortical and other subcortical regions in the non-affected hemisphere in patients with PSD compared to without PSD and healthy control. METHODS: Resting-state FC was estimated between the anterior insula and cortical and subcortical brain regions in the non-affected hemisphere in 13 patients with PSD, 12 patients without PSD, and 13 healthy controls. The severity of depressive mood was measured by the Beck Depression Inventory (BDI)-II. RESULTS: Patients with PSD showed significant differences in FC scores between the anterior insula and the superior frontal, middle frontal, and orbitofrontal gyrus in the non-affected hemisphere than healthy control or patients without PSD (P< 0.05). In post-hoc, patients with PSD showed higher FC scores between the anterior insula and the superior frontal region than patients without PSD (P< 0.05). Furthermore, alterations in FC of the superior frontal, middle frontal, and orbitofrontal gyrus were positively correlated with depression severity, as measured with the BDI-II (P< 0.001).

Effect of Nusinersen in a late onset spinal muscular atrophy patient for 14 months: A case report.

RATIONALE: Spinal muscular atrophy (SMA) is a genetic disorder caused by genetic defect of SMN1 gene. SMA was an untreatable disease until 2016, when Nusinersen an antisense oligonucleotide therapy was approved for treatment. We report the effect of Nusinersen in a late onset SMA for 14 months. PATIENT CONCERNS: A 13-year-old boy who was diagnosed as SMA with progressive proximal limb weakness was treated with intrathecal injection of Nusinersen. DIAGNOSIS: The patient had progressive proximal limb weakness after 2 years of age. The patient had elevated creatine kinase level and shoed neurogenic changes in the needle electromyography study. After genetic analysis, homozygous deletion in Exon 7 and 8 of SMN1 protein was found and he was diagnosed as late onset SMA. INTERVENTIONS: Intrathecal Nusinersen was administered per protocol. OUTCOMES: After 14 months of treatment, the patient showed significant clinical improvement in the revised Hammersmith functional rating scale and 6-minute walk test. LESSONS: Although there is limited data on the effect of Nusinersen in late onset SMA patients, our case adds on the effectiveness even in late onset SMA. More studies are needed to consolidate the effects and adverse events of Nusinersen in late onset SMA.

Remote Assessment of Post-Stroke Elbow Function Using Internet-Based Telerobotics: A Proof-of-Concept Study.

Purpose: Upper limb hemiparesis is the most common impairment in stroke survivors, and adequate assessment is crucial for setting the rehabilitation strategy and monitoring the effect of treatment. However, adequate timely assessments are difficult due to the limited accessibility to clinics for stroke survivors. We designed this study to investigate whether teleassessments for motor impairments of the spastic elbow (i.e., passive range of motion (PROM), muscle strength, and spasticity) are feasible in stroke survivors. Methods: To implement a telerobotic system for remote assessment with physical interaction, we constructed a system with a master robot interacting with a doctor (assessor) and a slave robot interacting with the elbow of a subject with stroke. The master robot is operated by the doctor, where the torque and the speed are transferred to the slave robot via the Internet, and the reaction of the patient's elbow to the slave robot's movement is measured with a torque sensor, then finally transferred back to the master robot. An intercontinental remote assessment, which is considered one of the worst possible scenarios, was used as a clinical test to strictly check the feasibility. For the clinical tests, the examiner for the teleassessment was located at a lab in the National Institutes of Health (NIH, Bethesda, MD, USA) while the stroke patients were located at Seoul National University Bundang Hospital (Bundang, Kyeonggido, South Korea). Results: In total, 12 stroke patients' elbows (age range, 28-74; M:F = 6:6) were tested. For the PROM, the absolute difference between two assessments (in-person vs. remote) was 5.98 ± 3.51° on average (range, 0-11.2). The agreements for the strength and the spasticity of elbow flexor between in-person and remote assessments were substantial (k = 0.643) and fair (k = 0.308), respectively. No adverse events were observed during or immediately after the telerobotic assessment. Conclusions: Internet-based telerobotic remote assessment for motor impairment of spastic elbow in stroke using our system is feasible even in the worst setting, with too long of a distance and a delayed communication network.

Alterations in power spectral density in motor- and pain-related networks on neuropathic pain after spinal cord injury.

BACKGROUND: The mechanisms by which mobility function and neuropathic pain are mutually influenced by supraspinal plasticity in motor- and pain-related brain networks following spinal cord injury (SCI) remains poorly understood. OBJECTIVE: To determine cortical and subcortical resting-state network alterations using power spectral density (PSD) analysis and investigate the relationships between these intrinsic alterations and mobility function and neuropathic pain following SCI. METHODS: A total of 41 patients with incomplete SCI and 33 healthy controls were included. The degree of mobility and balance function and severity of neuropathic pain and depressive mood were evaluated. The resting-state functional magnetic resonance imaging data of low-frequency fluctuations were analyzed based on PSD. Differences in PSD values between patients with SCI and controls were assessed using the two-sample t-test (false discovery rate-corrected P < 0.05). The relationship between PSD values and mobility function and pain intensity was assessed using Pearson's correlation coefficient adjusted for the severity of depressive mood. RESULTS: Compared with healthy controls, lower PSD values in supplementary motor and medial prefrontal areas (the anterior cingulate cortex, ventral medial prefrontal cortex, and superior orbito-prefrontal cortex) were associated with greater pain severity and poorer postural balance and mobility (P < 0.05) in patients with SCI, whereas higher PSD values in the primary motor cortex, premotor cortex, thalamus, and periaqueductal gray were associated with greater pain severity and poorer postural balance and mobility (P < 0.05). CONCLUSIONS: Cortical and subcortical plastic alterations in intrinsic motor- and pain-related networks were observed in patients with SCI and were simultaneously associated with neuropathic pain intensity and degree of mobility function.

Prediction of Motor Recovery in Patients with Basal Ganglia Hemorrhage Using Diffusion Tensor Imaging.

Predicting prognosis in patients with basal ganglia hemorrhage is difficult. This study aimed to investigate the usefulness of diffusion tensor imaging in predicting motor outcome after basal ganglia hemorrhage. A total of 12 patients with putaminal hemorrhage were included in the study (aged 50 ± 12 years), 8 patients were male (aged 46 ± 11 years) and 4 were female (aged 59 ± 9 years). We performed diffusion tensor imaging and measured clinical outcome at baseline (pre) and 3 weeks (post1), 3 months (post2), and 6 months (post3) after the initial treatment. In the affected side of the brain, the mean fractional anisotropy (FA) value on pons was significantly higher in the good outcome group than that in the poor outcome group at pre (p = 0.004) and post3 (p = 0.025). Pearson correlation analysis showed that mean FA value at pre significantly correlated with the sum of the Brunnstrom motor recovery stage scores at post3 (R = 0.8, p = 0.002). Change in the FA ratio on diffusion tractography can predict motor recovery after hemorrhagic stroke.

Interhemispheric Functional Connectivity in the Primary Motor Cortex Assessed by Resting-State Functional Magnetic Resonance Imaging Aids Long-Term Recovery Prediction among Subacute Stroke Patients with Severe Hand Weakness.

This study aimed to evaluate the usefulness of interhemispheric functional connectivity (FC) as a predictor of motor recovery in severe hand impairment and to determine the cutoff FC level as a clinically useful parameter. Patients with stroke (n = 22; age, 59.9 ± 13.7 years) who presented with unilateral severe upper-limb paresis and were confirmed to elicit no motor-evoked potential responses were selected. FC was measured using resting-state functional magnetic resonance imaging (rsfMRI) scans at 1 month from stroke onset. The good recovery group showed a higher FC value than the poor recovery group (p = 0.034). In contrast, there was no statistical difference in FC value between the good recovery and healthy control groups (p = 0.182). Additionally, the healthy control group showed a higher FC value than that shown by the poor recovery group (p = 0.0002). Good and poor recovery were determined based on Brunnstrom stage of upper-limb function at 6 months as the standard, and receiver operating characteristic curve indicated that a cutoff score of 0.013 had the greatest prognostic ability. In conclusion, interhemispheric FC measurement using rsfMRI scans may provide useful clinical information for predicting hand motor recovery during stroke rehabilitation.

Ancient to modern secular changes in the cranial/cephalic index in Korea: historical brachycephalization and recent debrachycephalization.

We investigated changes in the cranial/cephalic index of the Korean population in millennia, centuries, and recent decades. Secular changes of Korean's cephalic index in history were studied using the data of archaeology literature and our measurement data of different adult skull sets for the fifteenth-nineteenth century Joseon people, the Korean War victims (1950-1953), and the Korean skeletons collected by medical schools in the 1960s. A change in head shape during the last century was also estimated by the analysis on Korean cephalometric datasets of Korean Research Institute of Standards and Science. In brief, over the past 2000 years, the crania of Korean people have steadily changed from mesocephalic to brachycephalic, mainly due to the cranial length shortening. Brachycephalization accelerated at the beginning of the twentieth century and continued until the early twenty-first century, largely caused by increased cephalic breadth. We also note that debrachycephalization began in birth cohorts around 1965 for males and around 1970 for females. Taken together, we figure out that the head shape of Korean people has been gradually shortened over millennia and then has undergone dramatic shortening in the last century. In recent decades, however, the changing pattern has reversed to debrachycephalization, for which we discussed about the possible causes in the present report.

Association between clinical risk factors and severity of dysphagia after extubation based on a videofluoroscopic swallowing study.

BACKGROUND/AIMS: This study aimed to evaluate the correlation between clinical risk factors of post-extubation dysphagia (PED) and the severity of impaired pharyngeal swallowing function assessed via videofluoroscopic swallowing studies (VFSSs). METHODS: This study was a retrospective review of medical records. Of 116 patients who were admitted to the intensive care unit and underwent VFSS, 32 who had non-neurologic disorders and experienced prolonged intubation (for more than 48 hours) were diagnosed with PED. The severity of PED was evaluated by using a functional dysphagia scale (FDS) and a penetration aspiration scale (PAS), on the basis of VFSS. RESULTS: The Simplified Acute Physiology Score 3 and total FDS score were positively correlated (r = 0.40, p = 0.02). Intubation duration was positively correlated with total PAS and FDS scores (r = 0.62, p < 0.001; r = 0.65, p < 0.001, respectively). The amounts of residue in the valleculae (RV) and pyriform sinuses (RP) were associated with intubation duration (r = 0.58, p < 0.001; r = 0.57, p < 0.001, respectively). Multivariate regression analysis revealed that intubation duration was significantly associated with the total FDS score, RV and RP subscales of the FDS, and total PAS score. CONCLUSION: The severity of impaired swallowing function, particularly the amount of residue in the pharyngeal recesses assessed via VFSS, was strongly associated with both severity of medical illness and intubation duration. Intubation duration could be a prognostic factor for assessing impaired swallowing function on the basis of VFSS.

Clinical Experience of Nusinersen in a Broad Spectrum of Spinal Muscular Atrophy: A Retrospective Study.

BACKGROUND: Nusinersen has recently been approved and more widely used as first-line treatment of spinal muscular atrophy (SMA). This study aimed to evaluate the real-world experience of nusinersen use for patients with a broad spectrum of SMA. METHODS: We reviewed consecutive patients with SMA treated with nusinersen from April 2018 to April 2020. Data collected included clinical and diagnostic characteristics, molecular genetics, functional motor outcomes, and adverse events. RESULTS: Seven patients including four with SMA type 1 and three with SMA type 2 were treated with nusinersen. The median disease duration at the time of the first dose and the median follow-up duration were 37 months (range: 0.5-254 months) and 6.1 months (range: 2.1-22.1 months), respectively. Of the 41 lumbar punctures (LPs), seven fluoroscopy-guided LPs were successfully performed for two patients without sedation. All patients showed improvement in motor function even though the current tools for motor assessment seemed unable to detect subtle subjective improvement. All patients maintained a stable respiratory status. No patient has experienced a severe adverse event or discontinued treatment so far. CONCLUSION: Although the number of patients in this study was small, our results suggest that nusinersen is effective even in patients with a later stage of the disease. Additional long-term prospective studies with more number of patients having a broad spectrum of diseases are needed to identify meaningful improvement in the motor function and quality of life after nusinersen treatment.

Corrigendum: Remote Assessment of Post-Stroke Elbow Function Using Internet-Based Telerobotics: A Proof-of-Concept Study.

[This corrects the article DOI: 10.3389/fneur.2020.583101.].