Influence of elevated temperature on the species and mobility of chromium in ferrous sulfate-amended contaminated soil.

Ferrous sulfate (FeSO4) combined with acid pretreatment is usually employed to remediate contaminated soils containing Cr(VI). However, the long-term efficiency of this stabilization method is important for its sustainability. In this study, a gradient temperature-elevating exposure test was employed to investigate the stability of Cr in FeSO4-remediated soil when exposed to elevated temperatures (40 °C, 120 °C, and 500 °C), possibly caused by hot weather and/or wildfires. The results of chemical extraction and X-ray absorption near edge structure spectroscopy (XANES) showed that the Cr(VI) in contaminated soil was successfully transformed to Cr(III) after stabilization, resulting in the dramatic decrease of water-leachable Cr(VI). The stabilization efficiency was further improved under 40 °C treatment after 30 days. Subsequently, the 120 °C treatment (7 days) had relatively little effect on the Cr speciation and mobility in soils. However, even one day of 500 °C calcination resulted in the deterioration of stabilization efficiency, and the water-leachable Cr(VI) re-increased and became higher than the Chinese environmental standards (total Cr 15 mg/L, Cr(VI) 5 mg/L) for the classification of hazardous solid wastes. XANES results reflected that heating at 500 °C facilitate the formation of Cr2O3, which was mainly caused by thermal decomposition and dehydration of Cr(OH)3 in the soil. Besides, the transformation of Cr species resulted in the enhanced association of Cr with the most stable residual fraction (88.3%-91.6%) in soil. Based on chemical extraction results, it was suggested that the oxidation of Cr(III) to Cr(VI) contributed to the re-increased mobility of Cr(VI) in soil. However, the XANES results showed that almost no significant re-oxidization of Cr(III) to Cr(VI) happened after heating at 500 °C, which was probably caused by XANES linear combination fits (LCF) uncertainties. Moreover, the changes in soil properties, including a rise in pH to a slightly alkaline range and/or the decomposition of organic matter, possibly contributed to the enhanced mobility of Cr(VI) in soil. This study contributes to clarifying the mobility and transformation of Cr in contaminated soils and provides a support for the sustainable management of remediated soils.

High-level production of poly-γ-glutamic acid by a newly isolated Bacillus sp. YJY-8 and potential use in increasing the production of tomato.

Strain YJY-8, a new γ-polyglutamic acid producer, was separated from fermented soybean paste samples. The strain was identified as a genus of Bacillus by morphological and 16S rDNA sequence analysis and was named Bacillus sp. YJY-8. The optimal medium composition and cultural conditions were studied using a single-factor experiment and a response surface experiment. The optimized medium consisted of monosodium glutamate 70 g/L, glucose 54.3 g/L, glycerol 31.8 g/L, ammonium sulfate 11.1 g/L, yeast extract 3.2 g/L, tryptone 1.5 g/L, L-glutamic acid 6.8 g/L, MgSO4 7H2O 0.5 g/L, FeCl3 6H2O 0.02 g/L, KH2PO4 0.9 g/L, CaCl2 0.03 g/L, MnSO4 H2O 0.3 g/L, ammonium molybdate 0.02 g/L, pH 7.0. The optimal cultivation conditions were 35 °C and pH 7.0. Under the optimized conditions, after 48 hr of cultivation, the highest shaking flask fermentation level of γ-PGA reached 65.2 ± 0.36 g/L. In addition, through fed-batch fermentation in 30 L fermenters, the fermentation level of γ-PGA reached its highest level at 88.42 g/L and productivity was 1.23 g/(L hr) after 72 hr. Then, the effect of γ-PGA on tomato yield was investigated. At the seedling stage, the plant height and stem diameter of γ-PGA treated plants increased by 5.69 and 15.735% after spraying γ-PGA for 19 days. During the flowering and fruiting period, the stem diameter of the γ-PGA treatment group increased by 6.74%, with a maximum increase of 11.65%. The number of fruit branches increased by 0.56-16.29% and the number of fruit sets increased by 1.01-28.47%. At the fruit maturation stage, the yield of tomatoes increased by 10.51, 14.27, and 5.83%.

[Researchers' Knowledge About the Ethical Norms of National Natural Science Foundation of China Funded Medical Research:Current Status and Suggestions].

Objective To understand the current status of Chinese medical researchers' knowledge regarding the ethical norms of the research involving humans or laboratory animals,and provide reference for further improving the ethics review norms. Methods The questionnaire method was employed to survey the applicants for the 2019 projects supported by the Department of Medical Sciences,National Natural Science Foundation of China (NSFC) about their knowledge of ethical requirements.Furthermore,the ethical supervision of the NSFC and affiliations at the project application and implementation stages was analyzed. Results The survey showed that 29.9% medical researchers were familiar with NSFC's ethical requirements for research involving human or laboratory animals.During the project application stage,59.0% affiliations adopted the simplified review method.Regarding the ethical supervison,95.5% medical researchers believed that the affiliations should fulfill the ethical supervision obligations and take relevant measures during the project implementation period.In addition,55.0% medical researchers fully agreed to discuss with the review experts about the ethical issues involved in the project. Conclusions The NSFC should establish rules and regulations to improve institutional management responsibilities and institutionalize the training about research ethics to comprehensively strengthening the training.Taking the management of research project ethics as a starting point,the NSFC should form a multi-party linkage between project funding and management and establish an accountability mechanism for ethics management.Furthermore,the NSFC should double the endeavors at the review of ethical issues during expert review and process management and attach importance to the research,judgment,and prevention of ethical risks.

Optimization of fermentation conditions for production of neutral metalloprotease by Bacillus subtilis SCK6 and its application in goatskin-dehairing.

In this study, a high protease-producing strain was screened by spread plate method and identified by molecular biology and morphological identification. It was identified as Bacillus sp. LCB14. A neutral protease gene was cloned and heterologous expressed by B. subtilis SCK6. Then, the recombinant protease was used to dehair the goat skins. The fermentation conditions of neutral protease production by B. subtilis SCK6 were optimized. The single factor experiments, Plackett-Burma experiment, and response surface method were conducted to determine fermentation medium and culture conditions. The optimized medium contained corn meal 49 g/L, soluble starch 28 g/L, soybean meal 17 g/L, corn steep liquor powder 8 g/L, yeast extract 10 g/L, Na2HPO4 2.3 g/L, KH2PO4 1.9 g/L, MgSO4 0.5 g/L, MnCl2 0.1 g/L and ZnSO4 0.05 g/L. The optimized culture conditions were 35 °C and pH 7.0. Under the optimum conditions, the recombinant strain reached 33467.28 U/mL after 72 hr ferment. Moreover, by fed batch in 30 L fermenters, neutral protease production reached 39,440.78 U/mL and shortened fermentation time from 72 hr to 46 hr. Finally, the crude enzyme was utilized to replace sodium sulfide for dehairing of goatskins. The enzymatic dehaired pelts were white, smooth, and soft; the grain side of enzymatic dehaired pelts were clear; there was no obvious damage to the grain side of enzymatic dehaired pelts by visual observation and tactile test. Furthermore, there were no hair roots, hair follicles and other glands in enzymatic dehaired belts, and the collagen fibers of enzymatic dehaired belt were dispersed well by histological analysis.

Grab-AD: Generalizability and reproducibility of altered brain activity and diagnostic classification in Alzheimer's Disease.

Alzheimer's disease (AD) is associated with disruptions in brain activity and networks. However, there is substantial inconsistency among studies that have investigated functional brain alterations in AD; such contradictions have hindered efforts to elucidate the core disease mechanisms. In this study, we aim to comprehensively characterize AD-associated functional brain alterations using one of the world's largest resting-state functional MRI (fMRI) biobank for the disorder. The biobank includes fMRI data from six neuroimaging centers, with a total of 252 AD patients, 221 mild cognitive impairment (MCI) patients and 215 healthy comparison individuals. Meta-analytic techniques were used to unveil reliable differences in brain function among the three groups. Relative to the healthy comparison group, AD was associated with significantly reduced functional connectivity and local activity in the default-mode network, basal ganglia and cingulate gyrus, along with increased connectivity or local activity in the prefrontal lobe and hippocampus (p < .05, Bonferroni corrected). Moreover, these functional alterations were significantly correlated with the degree of cognitive impairment (AD and MCI groups) and amyloid-ß burden. Machine learning models were trained to recognize key fMRI features to predict individual diagnostic status and clinical score. Leave-one-site-out cross-validation established that diagnostic status (mean area under the receiver operating characteristic curve: 0.85) and clinical score (mean correlation coefficient between predicted and actual Mini-Mental State Examination scores: 0.56, p < .0001) could be predicted with high accuracy. Collectively, our findings highlight the potential for a reproducible and generalizable functional brain imaging biomarker to aid the early diagnosis of AD and track its progression.

A comparative study of microbial community and functions of type 2 diabetes mellitus patients with obesity and healthy people.

The gut microbiota is crucial in the pathogenesis of type 2 diabetes mellitus (T2DM). However, the metabolism of T2DM patients is not well-understood. We aimed to identify the differences on composition and function of gut microbiota between T2DM patients with obesity and healthy people. In this study, 6 T2DM patients with obesity and 6 healthy volunteers were recruited, and metagenomic approach and bioinformatics analysis methods were used to understand the composition of the gut microbiota and the metabolic network. We found a decrease in the abundance of Firmicutes, Oribacterium, and Paenibacillus; this may be attributed to a possible mechanism and biological basis of T2DM; moreover, we identified three critical bacterial taxa, Bacteroides plebeius, Phascolarctobacterium sp. CAG207, and the order Acidaminococcales that can potentially be used for T2DM treatment. We also revealed the composition of the microbiota through functional annotation based on multiple databases and found that carbohydrate metabolism contributed greatly to the pathogenesis of T2DM. This study helps in elucidating the different metabolic roles of microbes in T2DM patients with obesity.

Family refusal of emergency medical treatment in China: An investigation from legal, empirical and ethical perspectives.

This paper is an analysis of the limits of family authority to refuse life saving treatment for a family member (in the Chinese medical context). Family consent has long been praised and practiced in many non-Western cultural settings such as China and Japan. In contrast, the controversy of family refusal remains less examined despite its prevalence in low-income and middle-income countries. In this paper, we investigate family refusal in medical emergencies through a combination of legal, empirical and ethical approaches, which is highly relevant to the ongoing discussion about the place of informed consent in non-Western cultures. We first provide an overview of the Chinese legislation concerning informed consent to show the significance of family values in the context of medical decision-making and demonstrate the lack of legal support to override family refusal. Next, we present the findings of a vignette question that investigated how 11,771 medical professionals and 2,944 patients in China responded to the family refusal of emergency treatment for an unconscious patient. In our analysis of these results, we employ ethical reasoning to question the legitimacy of family refusal of life-sustaining emergency treatment for temporarily incompetent patients. Last, we examine some practical obstacles encountered by medical professionals wishing to override family refusal to give context to the discussion.

A novel CSF-1R mutation in a family with hereditary diffuse leukoencephalopathy with axonal spheroids misdiagnosed as hydrocephalus.

Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) is a rare autosomal dominant disease caused by mutations in the colony stimulating factor 1 receptor (CSF1R) gene that often results in cognitive impairment, psychiatric disorders, motor dysfunction and seizure. We report familial cases of a novel CSF1R mutation causing HDLS similar to hydrocephalus. The patients initially presented with a gait disturbance and then developed progressive cognitive decline, urinary incontinence, epileptic seizures and became bedridden as the disease progressed. A brain magnetic resonance imaging (MRI) scan revealed striking ventricular enlargement and diffuse brain atrophy with frontotemporal predominance, which was later accompanied by white matter changes. Genetic testing in this family showed a novel c.2552T>C (p.L851P) mutation in exon 19 of the CSF1R gene. However, three gene carriers in the family remained clinically asymptomatic. Because of its heterogeneous clinical phenotypes, HDLS patients are often misdiagnosed with other diseases. This is the first genetically proven HDLS case resembling hydrocephalus, and the clinical symptoms of HDLS may be related to the specific genetic mutation.

Welfare, wellness, and job satisfaction of Chinese physicians: A national survey of public tertiary hospitals in China.

Little national data are available on Chinese physicians' welfare, wellness, and job satisfaction. We conducted a self-administered smartphone-based national survey in early 2016 of 17 945 physicians from 136 tertiary hospitals across 31 provinces in China. In addition to collecting the physicians' basic information, we also measured 5 domains (the ethical and working environments, welfare, wellness, and job satisfaction). Half of the physicians reported a hospital-based annual income of less than RMB 72 000 ($10 300), and 60.31% of them did not think that the current medical pricing system reflects physicians' value. More than half (58.64%) of them did not have or did not know about medical malpractice insurance. These physicians worked long hours (an average of 10 h) and slept short hours (average 6 h). Only 35.78% of them thought that they were in good health, and 51.03% were in good mental health. Approximately, a quarter of them had helped to pay medical bills for patients who could not afford care, and 1 in 7 has been penalised for seeing patients who generated bad debts. Only 33.42% of them thought that their occupation receives social recognition and respect, and 70.98% would not encourage their children to pursue a medical career. The top 3 factors that may influence physician job satisfaction as chosen by the physicians were as follows: (1) the income distribution policy (45.92%), (2) working environment safety (25.86%), and (3) public trust and respect for their job (16.10%). In conclusion, we found that Chinese physicians bear heavy physical, mental, and financial stress, and many of them lack confidence that they receive trust and respect from society.

A case of rapid-onset dystonia-parkinsonism accompanied by pyramidal tract impairment.

BACKGROUND: Rapid-onset dystonia-parkinsonism (RDP) is a rare autosomal dominant disorder that is caused by mutations in the ATP1A3 gene and is characterized by an acute onset of asymmetric dystonia and parkinsonism. To date, fewer than 75 RDP cases have been reported worldwide. Clinical signs of pyramidal tract involvement have been reported in several RDP cases, and none of them included the Babinski sign. CASE PRESENTATION: We report a 24-year-old Chinese female with RDP who exhibited a strikingly asymmetric, predominantly dystonic movement disorder with a rostrocaudal gradient of involvement and parkinsonism. Physical examiniations revealed hyperactive reflexes, bilateral ankle clonus and positive Babinski sign in the right. DTI showed reduced white matter integrity of the corticospinal tract in the frontal lobe and subpontine plane. Genetic testing revealed a missense mutation of the ATP1A3-gene (E277K) in the patient. CONCLUSION: We suggest that pyramidal tract impairment could be involved in rapid-onset dystonia-parkinsonism and the pyramidal tract impairment in RDP needs to be differentiated from HSP.

Novel SPG 11 Mutations in Hereditary Spastic Paraplegia With Thin Corpus Callosum in a Chinese Family.

BACKGROUND: Hereditary spastic paraplegia (HSP) is a neurodegenerative disease that is characterized by progressive weakness and spasticity of the lower extremities; HSP can present as complicated forms with additional neurological signs. More than 70 disease loci have been described with different modes of inheritance. METHODS: In this study, nine subjects from a Chinese family that included two individuals affected by HSP were examined through detailed clinical evaluations, physical examinations, and genetic tests. Targeted exome capture technology was used to identify gene mutations. RESULTS: Two novel compound heterozygous mutations in the SPG 11 gene were identified, c.4001_4002insATAAC and c.4057C>G. The c.4001_4002insATAAC mutation leads to a reading frame shift during transcription, resulting in premature termination of the protein product. The missense mutation c.4057C>G (p.H1353D) is located in a highly conserved domain and is predicted to be a damaging substitution. CONCLUSIONS: Based on the results described here, we propose that these novel compound heterozygous mutations in SPG 11 are the genetic cause of autosomal recessive HSP in this Chinese family.

Metabolic Syndrome, Its Components, and Diabetes on 5-Year Risk of Recurrent Stroke among Mild-to-Moderate Ischemic Stroke Survivors: A Multiclinic Registry Study.

OBJECTIVES: The pieces of evidence regarding whether metabolic syndrome (MetS) is a better predictor than its individual components, especially diabetes, for recurrent stroke are limited. This study aimed to examine these associations. METHODS: A total of 1087 ischemic stroke patients were recruited consecutively from 2003 to 2004. They were followed up until the end of 2008. Baseline clinical and laboratory characteristics and new stroke event during follow-up were recorded. MetS was defined by the definition issued by the Chinese Medical Association/Chinese Diabetes Society. RESULTS: One hundred forty-three new stroke cases were recorded. After adjusting for baseline age, gender, education, marriage status, subtype stroke, length of index stroke to baseline assessment, history of cardiac diseases, smoking status, drinking status, clinics, aspirin treatment, and fibrinogen by Cox regression models, the risk of recurrent stroke was 43% higher in MetS patients than in non-MetS patients (hazard ratio [HR] = 1.43, 95% confidence interval [CI]: 1.01-2.01). The strength of this association is weaker than MetS individual components such as elevated glycemia (adjusted HR = 1.78, 95% CI: 1.26-2.52), elevated blood pressure (adjusted HR = 1.91, 95% CI: 1.11-3.30), or low high-density lipoprotein cholesterol (adjusted HR = 1.57, 95% CI: 1.08-2.51). Compared with the group with neither MetS nor diabetes, the adjusted risk of recurrent stroke was highest in the group with diabetes (HR = 2.77, 95% CI: 1.66-4.63), followed by those with both MetS and diabetes (HR = 1.91, 95% CI: 1.25-2.94). The risk of recurrent stroke in patients with MetS in the absence of diabetes was similar to those with neither. CONCLUSION: MetS is not superior to its individual components in predicting future recurrent stroke in patients who experience mild-to-moderate ischemic stroke.

Silver-catalyzed C(sp2)-H functionalization/C-O cyclization reaction at room temperature.

Silver-catalyzed C(sp(2))-H functionalization/C-O cyclization has been developed. The scalable reaction proceeds at room temperature in an open flask. The present method exhibits good functional-group compatibility because of the mild reaction conditions. Using a AgNO3 catalyst and a (NH4)2S2O8 oxidant in CH2Cl2/H2O solvent, various lactones are obtained in good to excellent yields. A kinetic isotope effect (KIE) study indicates that the reaction may occur via a radical process.

Thinner changes of the retinal nerve fiber layer in patients with mild cognitive impairment and Alzheimer's disease.

BACKGROUND: Alzheimer's disease (AD) is the most common form of dementia and patients often have visual disorders. Mild cognitive impairment (MCI) is characterized by a memory deficit when compared with those of a similar age and education level which could indicate an earlier onset of AD. The aim of this study is to measure the changes of the retinal nerve fiber layer (RNFL) thickness of AD and MCI patients in comparison with the normal age controls. METHODS: The RNFL thickness was assessed using optical coherence tomography (OCT) in patients with MCI, AD (mild, moderate and severe) and the age matched controls. RESULTS: The thickness of RNFL in the superior quadrant and total mean values are gradually and significantly decreased from MCI to severe AD when compared to that in the controls. There is also a significant reduction of the retinal nerve fiber layer in the inferior quadrant in severe AD patients. CONCLUSIONS: Our data indicate that the retinal nerve fiber layer degeneration is paralleled with dementia progression. Owing to its non-invasive and cost effective nature, monitoring RNFL thickness may have a value in assessing disease progression and the efficacy of any treatments.

Impaired long distance functional connectivity and weighted network architecture in Alzheimer's disease.

Alzheimer's disease (AD) is increasingly recognized as a disconnection syndrome, which leads to cognitive impairment due to the disruption of functional activity across large networks or systems of interconnected brain regions. We explored abnormal functional magnetic resonance imaging (fMRI) resting-state dynamics, functional connectivity, and weighted functional networks, in a sample of patients with severe AD (N = 18) and age-matched healthy volunteers (N = 21). We found that patients had reduced amplitude and regional homogeneity of low-frequency fMRI oscillations, and reduced the strength of functional connectivity, in several regions previously described as components of the default mode network, for example, medial posterior parietal cortex and dorsal medial prefrontal cortex. In patients with severe AD, functional connectivity was particularly attenuated between regions that were separated by a greater physical distance; and loss of long distance connectivity was associated with less efficient global and nodal network topology. This profile of functional abnormality in severe AD was consistent with the results of a comparable analysis of data on 2 additional groups of patients with mild AD (N = 17) and amnestic mild cognitive impairment (MCI; N = 18). A greater degree of cognitive impairment, measured by the mini-mental state examination across all patient groups, was correlated with greater attenuation of functional connectivity, particularly over long connection distances, for example, between anterior and posterior components of the default mode network, and greater reduction of global and nodal network efficiency. These results indicate that neurodegenerative disruption of fMRI oscillations and connectivity in AD affects long-distance connections to hub nodes, with the consequent loss of network efficiency. This profile was evident also to a lesser degree in the patients with less severe cognitive impairment, indicating that the potential of resting-state fMRI measures as biomarkers or predictors of disease progression in AD.

Local label learning (LLL) for subcortical structure segmentation: application to hippocampus segmentation.

Automatic and reliable segmentation of subcortical structures is an important but difficult task in quantitative brain image analysis. Multi-atlas based segmentation methods have attracted great interest due to their promising performance. Under the multi-atlas based segmentation framework, using deformation fields generated for registering atlas images onto a target image to be segmented, labels of the atlases are first propagated to the target image space and then fused to get the target image segmentation based on a label fusion strategy. While many label fusion strategies have been developed, most of these methods adopt predefined weighting models that are not necessarily optimal. In this study, we propose a novel local label learning strategy to estimate the target image's segmentation label using statistical machine learning techniques. In particular, we use a L1-regularized support vector machine (SVM) with a k nearest neighbor (kNN) based training sample selection strategy to learn a classifier for each of the target image voxel from its neighboring voxels in the atlases based on both image intensity and texture features. Our method has produced segmentation results consistently better than state-of-the-art label fusion methods in validation experiments on hippocampal segmentation of over 100 MR images obtained from publicly available and in-house datasets. Volumetric analysis has also demonstrated the capability of our method in detecting hippocampal volume changes due to Alzheimer's disease.

Abnormal salience network in normal aging and in amnestic mild cognitive impairment and Alzheimer's disease.

The salience network (SN) serves to identify salient stimuli and to switch between the central executive network (CEN) and the default-mode network (DMN), both of which are impaired in Alzheimer's disease (AD)/amnestic mild cognitive impairment (aMCI). We hypothesized that both the structural and functional organization of the SN and functional interactions between the SN and CEN/DMN are altered in normal aging and in AD/aMCI. Gray matter volume (GMV) and resting-state functional connectivity (FC) were analyzed from healthy younger (HYC) to older controls (HOC) and from HOC to aMCI and AD patients. All the SN components showed significant differences in the GMV, intranetwork FC, and internetwork FC between the HYC and HOC. Most of the SN components showed differences in the GMV between the HOC and AD and between the aMCI and AD. Compared with the HOC, AD patients exhibited significant differences in intra- and internetwork FCs of the SN, whereas aMCI patients demonstrated differences in internetwork FC of the SN. Most of the GMVs and internetwork FCs of the SN and part of the intranetwork FC of the SN were correlated with cognitive differences in older subjects. Our findings suggested that structural and functional impairments of the SN may occur as early as in normal aging and that functional disconnection between the SN and CEN/ DMN may also be associated with both normal aging and disease progression.

Convergent Neuroimaging and Molecular Signatures in Mild Cognitive Impairment and Alzheimer's Disease: A Data-Driven Meta-Analysis with N = 3,118.

The current study aimed to evaluate the susceptibility to regional brain atrophy and its biological mechanism in Alzheimer's disease (AD). We conducted data-driven meta-analyses to combine 3,118 structural magnetic resonance images from three datasets to obtain robust atrophy patterns. Then we introduced a set of radiogenomic analyses to investigate the biological basis of the atrophy patterns in AD. Our results showed that the hippocampus and amygdala exhibit the most severe atrophy, followed by the temporal, frontal, and occipital lobes in mild cognitive impairment (MCI) and AD. The extent of atrophy in MCI was less severe than that in AD. A series of biological processes related to the glutamate signaling pathway, cellular stress response, and synapse structure and function were investigated through gene set enrichment analysis. Our study contributes to understanding the manifestations of atrophy and a deeper understanding of the pathophysiological processes that contribute to atrophy, providing new insight for further clinical research on AD.

Posterior reversible encephalopathy syndrome in a leber hereditary optic neuropathy patient with mitochondrial DNA 11778G>A point mutation.

Leber hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disorder that primarily affects the optic nerve. We report a case of reduced visual acuity secondary to optic atrophy in a 13-year-old boy. Transient seizures developed subsequently. Serial magnetic resonance imaging of the brain showed posterior reversible encephalopathy syndrome. Ragged red fibers were not detected on skeletal muscle biopsy. A 11778G>A mitochondrial DNA point mutation was identified in the lymphocytes isolated from peripheral blood. His younger brother was a carrier with the same mutation. The presentation of this case is unusual documenting LHON in association with PRES.