ObjectiveTo investigate immediate changes in hyolaryngeal movement and swallowing function after a cycle of neuromuscular electrical stimulation (NMES) on both submental and throat regions and submental placement alone in patients with dysphagia.MethodsFifteen patients with dysphagia were recruited. First, videofluoroscopic swallowing study (VFSS) was performed before NMES. All patients thereafter received a cycle of NMES by 2 methods of electrode placement: 1) both submental and throat regions and 2) submental placement alone concomitant with VFSS. The Penetration-Aspiration Score (PAS) and the NIH-Swallowing Safety Scale (NIH-SSS) were measured for swallowing function.ResultsDuring swallowing, hyolaryngeal descent significantly occurred by NMES on both submental and throat regions, and anterior displacement of hyolaryngeal complex was significant on submental placement alone. NMES on submental placement alone did not change the PAS and NIH-SSS. However, NMES on both submental and throat regions significantly reduced the NIH-SSS, although it did not change the PAS. Patients with no brainstem lesion and with dysphagia duration of <3 months showed significantly improved the NIH-SSS.ConclusionImmediate hyolaryngeal movement was paradoxically depressed after NMES on both submental and throat regions with significant reductions in the NIH-SSS but not the PAS, suggesting improvement in pharyngeal peristalsis and cricopharyngeal functions at the esophageal entry rather than decreased aspiration and penetration. The results also suggested that patients with dysphagia should be carefully screened when determining motor-level NMES.

Background: We investigated the clinical course of patients with prolonged disorders of consciousness (PDoC), predictors of emergence from PDoC (EDoC), and the temporal dynamics of six neurobehavior domains based on the JFK Coma Recovery Scale-Revised (CRS-R) during the recovery. Methods: A total of 50 traumatic and non-traumatic patients with PDoC were enrolled between October 2014 and February 2017. A retrospective analysis of the clinical findings and neurobehavioral signs was conducted using standardized methodology such as CRS-R. The findings were used to investigate the incidence and predictors of EDoC and determine the cumulative pattern of neurobehavioral recovery at 6 months, 1 year, and 2 years post-injury. Results: The results showed that 46% of the subjects emerged from PDoC after 200 median days (64-1197 days) of injury onset. The significant predictors of EDoC included minimally conscious state (MCS) (vs. vegetative state), higher auditory, communication, arousal, total CRS-R scores, shorter lag time post-injury, and the absence of intra-axial lesions. In terms of cumulative recovery of motor and communication signs in patients who emerged from PDoC, 39 and 32% showed EDoC at 6 months post-injury, and 88 and 93% exhibited EDoC at 2 years post-injury, respectively. Conclusions: Nearly half of the patients with PDoC recovered consciousness during inpatient rehabilitation. MCS, shorter lag time, the absence of intra-axial lesions, higher auditory, communication, arousal, and total CRS-R scores were important predictors for EDoC. Motor scores in the early stage of recovery and communication scores after prolonged intervals contributed to the higher levels of cumulative EDoC.

Olfactory bulb (OB) plays an important role in protecting against harmful substances via the secretion of antioxidant and detoxifying enzymes. Environmental enrichment (EE) is a common rehabilitation method and known to have beneficial effects in the central nervous system. However, the effects of EE in the OB still remain unclear. At 6 weeks of age, CD-1® (ICR) mice were assigned to standard cages or EE cages. After 2 months, we performed proteomic analysis. Forty-four up-regulated proteins were identified in EE mice compared to the control mice. Gene Ontology analysis and Kyoto Encyclopedia of Genes and Genomes Pathway demonstrated that the upregulated proteins were mainly involved in metabolic pathways against xenobiotics. Among those upregulated proteins, 9 proteins, which participate in phase I or II of the xenobiotic metabolizing process and are known to be responsible for ROS detoxification, were validated by qRT-PCR. To explore the effect of ROS detoxification mediated by EE, glutathione activity was measured by an ELISA assay. The ratio of reduced glutathione to oxidized glutathione was significantly increased in EE mice. Based on a linear regression analysis, GSTM2 and UGT2A1 were found to be the most influential genes in ROS detoxification. For further analysis of neuroprotection, the level of iNOS and the ratio of Bax to Bcl-2 were significantly decreased in EE mice. While TUNEL+ cells were significantly decreased, Ki67+ cells were significantly increased in EE mice, implicating that EE creates an optimal state for xenobiotic metabolism and antioxidant activity. Taken together, our results suggested that EE protects olfactory layers via the upregulation of glutathione-related antioxidant and xenobiotic metabolizing enzymes, eventually lowering ROS-mediated inflammation and apoptosis and increasing neurogenesis. This study may provide an opportunity for a better understanding of the beneficial effects of EE in the OB.

The electrical conductivities of MgO single crystals doped with 0.375, 0.75, 1, and 5% NiO were measured from 900 to 1200°under oxygen partial pressures, P02, of 10-1 to 10-7 atm. As P02 is increased from 10-7 atm, conductivities increase up to a maximum occurring in the region between 10-4 and 10-3 atm and then decrease as P02 is further increased higher than 10-3 atm. The point of maximum conductivity can be explained as the point at which there is a change in the current carrier type. It is suggested that the current carriers are O2at P02 greater than that corresponding to maximum conductivity and Mg2+ at lower To2's•

Background Ataxia-telangiectasia is a rare autosomal recessive, neurodegenerative disorder caused by alterations in the ATM gene. The majority of ATM pathogenic variants are frameshift or nonsense variants which are predicted to truncate the whole ATM protein. Herein, we report on an ataxia telangiectasia child with atypical phenotype who was identified as compound heterozygous for two ATM variants involving a previously described pathogenic single nucleotide variation (SNV) and a novel copy number variation (CNV). Case presentation A 6-year-old boy presented with delayed development and oculomotor apraxia. Brain magnetic resonance imaging showed interval development of mild atrophy in the cerebellum. Serum alpha fetoprotein level was in normal range. Next-generation sequencing and single-nucleotide polymorphism array tests were performed. Next-generation sequencing revealed a heterozygous nonsense pathogenic variant in ATM, c.742C > T (p.Arg248Ter) inherited from the father. Single-nucleotide polymorphism array revealed a compound heterozygous CNV, arr[GRCh37] 11q22.3(10851766–108183226) × 1, 31460 bp (exons 24–40 deletion of ATM) inherited from the mother, which was validated by reverse transcription-polymerase chain reaction analysis (RT-PCR). We demonstrated that this variant (NM_000051.4:c.3403_6006del) generated a product of in-frame deletion of exon 24–40 of ATM (p.Ser1135_Gln2002del). Conclusions The compound heterozygosity for ATM variants involving a previously described pathogenic SNV and a novel CNV may be associated with the atypical clinical manifestations. This clinical report extends the genetic and phenotypic spectrum of ATM pathogenic variants in atypical ataxia-telangiectasia, thus making implementation of advanced analysis beyond the routine next-generation sequencing an important consideration in diagnosis and rehabilitation services for children with ataxia-telangiectasia.

Background Bone loss after stroke escalates the risk of fractures, mainly in the hip, leading to further disability in individuals with stroke. We aimed to investigate the skeletal effect of bone mineral density (BMD) based on the duration of onset of stroke, compare the BMD of the paretic and non-paretic sides, and elucidate the relationship between BMD and disability variables. Methods The 31 male hemiplegic stroke patients between 20 and 70 years of age with cerebral infarction or hemorrhage were considered in this study. Subacute and chronic cases included 13 and 18 patients with lag time from the onset of 1 to 6 months and beyond 6 months, respectively. BMD in the lumbar, paretic, and non-paretic hip as well as the disability variables were analyzed retrospectively. Results The subacute group showed a significant reduction in the femoral neck BMD on the paretic side compared to that on the non-paretic side based on T-scores ( P =0.013). Bone loss was significantly correlated with lower limb muscle strength and overall physical impairment ( P <0.05). The chronic group demonstrated significant reduction in femur neck and total femur BMD on the paretic side compared to that on the non-paretic side based on T-scores ( P =0.002 and P <0.001, respectively). T-scores of BMD in the chronic phase were not significantly associated with the clinical parameters. Conclusions Early screening of bilateral hip BMD in the early stages after stroke, monitoring, and timely implementation of prevention strategies are important to minimize subsequent bone loss and prevent possible complications in patients who experience stroke.

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Acquired brain injury (ABI) is a leading cause of serious long-term disability resulting in substantial economic costs for post-ABI care. This study was conducted to estimate the socioeconomic burden of persons with ABI in Korea. We used a prevalence-based approach and societal perspective to estimate the direct medical, non-medical costs and indirect costs of ABI, including stroke, traumatic brain injury (TBI), and non-traumatic ABI (anoxia, brain tumor, encephalitis, meningitis, hydrocephalus, and other brain disorders) from 2015 to 2017. The study population included patients with ABI over 20 years of age and analyzed according to insurance types encompassing National Health Insurance and automobile insurance. The socioeconomic burden of ABI was 4.67, 5.18, and 5.73 trillion KRW (approximately 4,162, 4,612, and 5,106 million USD) from 2015 to 2017 and around 0.3% of Korea's GDP annually. Estimating by disease, the socioeconomic cost was 72.4% for stroke, 18.6% for TBI, and 9.0% for non-traumatic ABI. Calculated by cost component, medical costs and non-medical costs showed a slight increase every year. Through this study, establishment of rehabilitation systems maximizing the health and quality of life for injured persons remain the key public health strategy for ABI to reduce socioeconomic burden and financial policies to support patients should be needed.