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Introduction: This cohort study aimed to investigate the factors associated with noninvasive positive pressure ventilation adherence and assess the long-term effects of noninvasive positive pressure ventilation adherence in patients with amyotrophic lateral sclerosis (ALS). Methods: The medical records of patients with ALS admitted to a tertiary hospital for noninvasive positive pressure ventilation initiation were retrospectively reviewed. Pulmonary function parameters, variables of blood gas analysis, the site of symptom onset, the time from onset and diagnosis to noninvasive positive pressure ventilation application, ALS Functional Rating Scale-Revised, neurophysiological index, and the length of hospital stay were evaluated. The adherence to noninvasive positive pressure ventilation was defined as the use of noninvasive positive pressure ventilation for ≥ 2 h/day or ≥ 4 h/day. The correlations between noninvasive positive pressure ventilation adherence or length of hospital stay and other clinical parameters were analyzed. Results: Fifty-one patients with ALS were included in the study. The time from onset and diagnosis to NIPPV application was reduced by 16 months in the adherent group than that in the non-adherent group; however, the parameters of blood gas analysis and pulmonary function tests did not differ significantly between the groups. Furthermore, the neurophysiological index of the abductor digiti minimi muscle was higher by 4.05 in the adherent group than that in the non-adherent group. The adherence to noninvasive positive pressure ventilation prolonged tracheostomy-free survival compared to that of non-adherence. Desaturation events, lower forced vital capacity, last pCO2, bicarbonate, and base excess, and higher differences in pCO2, were associated with an increase in the length of hospital stay. Conclusions: Noninvasive positive pressure ventilation application shortly after symptom onset and ALS diagnosis in patients with CO2 retention and reduced forced vital capacity can be considered for successful adherence. Adherence to noninvasive positive pressure ventilation may result in reduced tracheostomy conversion rates and prolonged tracheostomy-free survival. [ABSTRACT FROM AUTHOR]

Background: Tracheostomy invasive ventilation (TIV) is applied to a subset of amyotrophic lateral sclerosis (ALS) patients; however, its frequency and impact on prognosis vary across countries. Methods: We conducted a nationwide retrospective cohort study using Korean National Health Insurance claims data. All patients diagnosed with sporadic ALS from 2012 to 2017 were included, with the observation period until 2020. The survival time between the TIV and non-TIV groups was compared using propensity score matching analysis, and prognostic factors were assessed within the TIV group. Results: This study included 3484 ALS patients (mean [standard deviation] age, 62.4 [11.9] years, 60.4% male), among whom 1230 (35.3%) underwent TIV. After 1:1 propensity score matching, the survival duration between the two groups was not significantly different (28 vs. 25 months, p = 0.057). Cox regression indicated that older age (hazard ratios [HRs] for each decade compared to <40 years: 3.89, 3.83, 5.30, 6.78, and 8.40 [ ≥ 80 years]; p < 0.005 for all) and lower income (HR, 1.28; 95% confidence interval [CI], 1.09–1.52; p = 0.003) negatively impacted survival, while gastrostomy (HR, 0.57; 95% CI, 0.50–0.66; p < 0.001) and supportive care services (HR, 0.43; 95% CI, 0.32–0.59; p < 0.001) were associated with prolonged survival. Conclusions: TIV was administered to more than one-third of Korean ALS patients without significant survival prolongation. Older age, lower income, lack of gastrostomy, and insufficient supportive care were independent poor prognostic factors for survival, underscoring the importance of comprehensive management for ALS patients. [ABSTRACT FROM AUTHOR]

Botrytis cinerea is a necrotrophic model fungal plant pathogen that causes grey mould, a devastating disease responsible for large losses in the agriculture sector. As important targets of fungicides, membrane proteins are hot spots in the research and development of fungicide products. We previously found that membrane protein Bcest may be closely related to the pathogenicity of Botrytis cinerea. Herein, we further explored its function. We generated and characterised ΔBcest deletion mutants of B. cinerea and constructed complemented strains. The ΔBcest deletion mutants exhibited reduced conidia germination and germ tube elongation. The functional activity of ΔBcest deletion mutants was investigated by reduced necrotic colonisation of B. cinerea on grapevine fruits and leaves. Targeted deletion of Bcest also blocked several phenotypic defects in aspects of mycelial growth, conidiation and virulence. All phenotypic defects were restored by targeted-gene complementation. The role of Bcest in pathogenicity was also supported by reverse-transcriptase real-time quantitative PCR results indicating that melanin synthesis gene Bcpks13 and virulence factor Bccdc14 were significantly downregulated in the early infection stage of the ΔBcest strain. Taken together, these results suggest that Bcest plays important roles in the regulation of various cellular processes in B. cinerea. [ABSTRACT FROM AUTHOR]

Background and purpose: Spinal cord infarction (SCI) is difficult to diagnosis using MRI findings. We aimed to suggest the optimal timing of MRI studies for diagnosing SCI. Materials and methods: This retrospective study was approved by our institutional review board. The requirement for informed consent was waived. MRI scans of SCI patients diagnosed between January 2015 and August 2019 were enrolled in the SCI group and subdivided according to the interval between symptom onset and time of MRI scan (A, within 6 h; B, 6–12 hours; C, 12–24 hours; D, 24–72 hours; E, 3–7 days). Three radiologists analyzed the T2WI scans and evaluated the confidence level of diagnosing SCI using a five-point Likert scale: 1, certainly not; 2, probably not; 3, equivocal; 4, probably yes; 5, certainly yes. Scores of 4 and 5 were defined as "T2WI-positive SCI" and scores of 1–3 were defined as "T2WI-negative SCI". Results: The SCI group included 58 MRI scans of 34 patients (mean age, 60.6 ± 14.0 years; 18 women). The T2WI positivity rate was 72.4% (42/58). In contrast to the other subgroups, subgroup A included fewer cases of T2WI-positive SCI (1/4, 25%) than T2WI-negative SCI. A confidence score of 5 was the most common in subgroup D (4/27, 14.8%). Among the 12 patients who underwent MRI studies more than twice, confidence scores increased with time. Conclusion: In patients with suspected SCI showing equivocal initial MRI findings, follow-up MRI studies are helpful, especially when performed between 24 and 72 hours after symptom onset. [ABSTRACT FROM AUTHOR]

To purchase or authenticate to the full-text of this article, please visit this link: http://dx.doi.org/10.1111/j.1552-6569.2007.00153.x Byline: Yoon-Ho Hong (1), Jung-Joon Sung (1), Sung-Min Kim (1), Kyung-Seok Park (1), Kwang-Woo Lee (1), Kee-Hyun Chang (1), In Chan Song (1) Keywords: Amyotrophic lateral sclerosis; diffusion tensor magnetic resonance imaging; diffusion tensor tractography; pyramidal tract Abstract: ABSTRACT BACKGROUND AND PURPOSE We attempted to measure DTI parameters of the brainstem pyramidal tract using two approaches, ie, simple ROI and tract-specific analyses. Results obtained for healthy subjects and ALS patients were compared. METHODS DTI was performed using a single shot SE-EPI with 25 noncollinear diffusion gradient directions (b= 1000 second/mm.sup.2) and with no diffusion gradient on a 3.0-T MR system in 10 ALS patients and in 8 age- and sex-matched normal controls. To delineate the brainstem pyramidal tract, tractography was performed using two ROIs, ie, a seed ROI at the cerebral peduncle (ROI-1) and a target ROI at the lower pons (ROI-2). ROI-1 was subsequently restricted to voxels that contained streamlines in the tract reconstruction, thus creating a sub-ROI. RESULTS Mean fractional anisotropy (FA) and mean diffusivity values were highly reproducible by tract specific analysis, whereas simple ROI analysis yielded larger variabilities between operators. FA values were significantly lower in ALS patients than in normal controls in the tractography-derived sub-ROI (P= .01), but not in the seed or target ROIs. CONCLUSIONS These results suggest, compared with simple ROI analysis, that tract-specific analysis using DTI fiber-tracking is more reliable and sensitive for detecting upper motor neuron pathology in ALS. Author Affiliation: (1)Department of Neurology, Seoul National University Boramae Hospital, Seoul, South Korea (Y-HH); Department of Neurology, Seoul National University, Seoul, South Korea (J-JS, S-MK); Department of Neurology, Seoul National University Bundang Hospital, Seoul, South Korea (K-SP); Seoul National University Hospital, Seoul National University, College of Medicine-Neurology, Seoul, South Korea (K-WL); Seoul National University Hospital, Seoul National University, College of Medicine-Radiology, Seoul, South Korea (K-HC); Department of Radiology, Seoul National University Hospital, Seoul, South Korea (ICS) Article History: Acceptance: Received December 15, 2006, and in revised form January 25, 2007. Accepted for publication March 5, 2007. Article note: Correspondence: Address correspondence to In Chan Song, PhD, Department of Radiology, Seoul National University Hospital, Yongon-Dong 28, Chongno-Gu, Seoul 110-744, South Korea. E-mail: icsong@radcom.snu.ac.kr.