The role of lipid peroxidation in neurological disorders

There has been much evidence demonstrating the involvement of oxidative stress in the pathology of neurological disorders. Moreover, the vulnerability of the central nervous system to reactive oxygen species mediated injury is well established since neurons consume large amounts of oxygen, the brain has many areas containing high iron content, and neuronal mitochondria generate large amounts of hydrogen peroxide. Furthermore, neuronal membranes are rich in polyunsaturated fatty acids, which are particularly susceptible to oxidative stress. Recently, the biological roles of products produced by lipid peroxidation have received much attention, not only for their pathological mechanisms associated with neurological disorders, but also for their practical clinical applications as biomarkers. Here, we discuss the production mechanisms of reactive oxygen species in some neurological disorders, including Alzheimer’s disease, Down syndrome, Parkinson’s disease, and stroke. We also describe lipid peroxidation biomarkers for evaluating oxidative stress.     

Episodic headache with spontaneous hypothermia reveal Shapiro’s syndrome variant with effectiveness of clonidine therapy

BackgroundEpisodic headache with spontaneous hypothermia constitute an uncommon association and is not well recognized in the International Classification of Headache Disorders (ICHD-3). Spontaneous periodic hypothermia, also called Shapiro’s syndrome, is a rare disease characterized by hypothermia attacks associated or not with hyperhidrosis without any triggering factor.Case presentationWe report a rare case of Shapiro’s syndrome variantrevealed by episodes of headache with spontaneous hypothermia witheffectiveness of clonidine therapy in a 76-year-old Parkinson’s disease woman.ConclusionsIn the literature, apart from Shapiro’s syndrome, headache withhypothermia seem to occur very rarely. In our case,these symptoms may be considered as a very rare non-motor fluctuation ofParkinson’s disease.     

Postoperative diarrhea in Crohn’s disease: Pathogenesis,diagnosis, and therapy

Diarrhea is a frequent symptom in postoperative patients with Crohn’s diseases (CD), and several different mechanisms likely account for postoperative diarrhea in CD. A targeted strategy based on a comprehensive understanding of postoperative diarrhea is helpful for better postoperative recovery.     

Clinical profiles of SS-ILD compared with SS-NILD in a Chinese population: a retrospective analysis of 735 patients

BackgroundInterstitial lung disease (ILD) is a serious complication in patients with Sjögren’s syndrome (SS). Most studies on primary SS (pSS) with ILD are limited in sample size, and studies on secondary SS (sSS) with ILD are rare. This study aimed to elucidate both primary and secondary SS-associated ILD (SS-ILD) based on a large cohort.MethodsThe medical records of hospitalized patients diagnosed with SS at the Second Xiangya Hospital of Central South University from January 2010 to May 2020 were retrospectively reviewed. Clinical manifestations, medical history, biological results and imaging data were collected.ResultsOf the 735 SS patients enrolled in this study, 563 (76.6%) were diagnosed with pSS, 172 (23.4%) were diagnosed with sSS. Additionally, 316 (43.0%) were diagnosed with SS-ILD. No significant difference was found between the pSS and sSS groups concerning the incidence of ILD (p = .718). Factors associated with SS-ILD were older age (p < .001), male sex (p = .032), female sex at menopause (p = .002), Raynaud’s phenomenon (p < .001), low levels of albumin (p = .010) and respiratory symptoms (p < .001). The SS-ILD group showed higher counts of platelets (p < .001). The three most frequent high-resolution CT (HRCT) findings of SS-ILD were irregular linear opacities (42.7%), grid shadows (30.7%) and pleural thickening (28.5%). NSIP (56.3%) was the most frequent HRCT pattern. Compared with pSS patients with ILD (pSS-ILD) patients, sSS patients with ILD (sSS-ILD) patients had a higher incidence of proteinuria (p < .001) and hypercreatinaemia (p = .013), a higher level of erythrocyte sedimentation rate (ESR) (p = .003), low levels of complement 3 (C3) (p = .013), lymphocytes (p = .009) and leukocytes (p = .024), and worse DLCO (%Pred) (p = .035).ConclusionsILD is a common pulmonary involvement in both pSS patients and sSS patients. Older age, male sex, female sex at menopause, Raynaud’s phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. NSIP is important HRCT feature of SS-ILD. sSS-ILD patients showed worse laboratory results and pulmonary function.

KEY MESSAGE

• Older age, male sex, female sex at menopause, Raynaud’s phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD.
• SS-ILD patients show higher counts of platelets and less purpura.
• sSS-ILD patients have worse laboratory results and pulmonary function.

     

Association between cognitive function and olfactory identification ability in community-dwelling older individuals

[Purpose] We investigated whether olfactory identification ability may be useful for early detection of cognitive decline. [Participants and Methods] The study included 55 community-dwelling older individuals without a history of mild cognitive impairment or dementia, who were capable of living independently. Cognitive function was evaluated using the Japanese versions of the Mini-Mental State Examination and the Montreal Cognitive Assessment tools. The olfactory identification ability was evaluated using the Odor Stick Identification Test for the Japanese. We also investigated the association between olfactory identification ability and cognitive function. [Results] Based on the Japanese version of the Mini-Mental State Examination, all participants were categorized into the noncognitive decline group, and based on the Japanese version of the Montreal Cognitive Assessment tool, 21 participants were categorized into the cognitive decline group. With regard to olfactory discrimination ability, we observed a significant difference between participants with and without cognitive decline based on the Japanese version of the Montreal Cognitive Assessment scores. Furthermore, we observed a significant positive correlation between the Japanese version of the the Montreal Cognitive Assessment scores and the Odor Stick Identification Test for the Japanese scores, although no significant correlation was observed between the Japanese version of the Mini-Mental State Examination and the Odor Stick Identification Test for the Japanese scores. [Conclusion] Olfactory identification ability may be useful to detect early-stage cognitive decline in community-dwelling older individuals.     

Not the final diagnosis: from Addison’s disease to POEMS syndrome: a case report and literature review

We report the case of a 47-year-old male patient with pigmentation of the head, face and hands, who was initially diagnosed as having primary adrenal insufficiency (Addison’s disease). Laboratory testing, imaging and physical examination revealed subclinical hypothyroidism, high circulating prolactin and oestradiol concentrations, gynaecomastia, lymphadenopathy, splenomegaly and weakness of both lower limbs. These findings led us to consider whether a single or multiple diseases were present in this patient. Indeed, Addison’s disease can represent one aspect of a wider systemic disease. Therefore, we performed further examinations, and found high serum M protein (5.1%) and vascular endothelial growth factor [1005.30 pg/mL (normal range 0 to 142 pg/mL)] concentrations. As a consequence, we diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. Consequently, when a single disease cannot fully explain the multiple symptoms and signs of one patient, clinicians should consider the possibility of the presence of a wider syndrome and undertake more detailed diagnostic testing.     

Sever’s Disease of the Pediatric Population: Clinical,Pathologic, and Therapeutic Considerations

Sever’s disease is an underreported prevalent pediatric condition that causes heel pain in children worldwide. It is often described as an overuse injury that can present with either unilateral or bilateral heel pain. Even though the exact mechanism of injury is unknown, it is often thought it involves repetitive stress and pressure on the calcaneal growth plate. Diagnosing Sever’s disease mainly relies on a thorough clinical investigation and physical examination, with a positive squeeze test usually sufficient to establish diagnosis. Nevertheless, radiographic imaging can help exclude other differential diagnoses. Therapeutic options of Sever’s disease are mostly conservative, and these include rest, physical therapy, kinesiotherapy, and orthoses. Educating parents and coaches on the symptomatology and presentation of Sever’s disease is pivotal for the establishment of efficient preventive interventions and earlier diagnoses. This study presents a case of a pediatric patient with Sever’s disease and offers medical insight into the diagnostic, clinical, pathologic, and therapeutic characteristics of this condition, in light of the current existing literature.     

The effects of visual cues from optical stimulation devices on gait disturbance in patients with Parkinson’s disease

[Purpose] The purpose of this study was to identify the optimal visual cues for gait disturbance in patients with Parkinson’s disease based on the luminous duration and the individual patient preferences for a wearable visual cue device. [Participants and Methods] Twenty-four patients with Parkinson’s disease walked while wearing only a visual cue device in the control condition. They then walked while the device was set to two stimulus conditions: the luminous duration at 10% and 50% of the individual gait cycle. After walking under the two stimulus conditions, the patients were asked for their preferred visual cue condition. The walking results were compared between the two stimulus conditions and the control condition. Gait parameters were compared among the three conditions. The comparisons with preference, non-preference, and control conditions were also made for the same gait parameter. [Results] When compared to the control condition, walking with visual cues in the stimulus conditions reduced stride duration and increased cadence. The preference and non-preference conditions had shorter stride durations than the control condition. Furthermore, the preference condition also resulted in a faster gait speed than the non-preference condition. [Conclusion] This study suggests that a wearable visual cue device with the patient’s preferred luminous duration may help manage gait disturbance in patients with Parkinson’s disease.     

Olfactory identification ability in patients with mild cognitive impairment and Alzheimer’s disease

[Purpose] To examine the olfactory identification abilities and specify the difficult-to-identify odors in community-dwelling individuals with mild cognitive impairment (MCI) and Alzheimer’s disease (AD). [Participants and Methods] We included, 12 and 17 patients with MCI (MCI group) and AD (AD group), respectively, and 30 community-dwelling older adults with no history of MCI or a dementia diagnosis (control group). Scores on the Japanese odor stick identification test (OSIT-J), an olfactory identification ability test, were compared among the three groups with intergroup differences examined accordingly. Next, we performed intergroup comparisons of the ratios of correct responses for each odor, and the difficult-to-identify odors were examined. [Results] OSIT-J scores of the MCI and AD groups were significantly lower than those of the control group. There were no intergroup differences in the correct identification of pungent odors. No patients in the AD group could identify the odor of cooking gas. The ability to identify food-related odors was reduced in the MCI and AD groups. [Conclusion] Patients with MCI and AD had reduced olfactory identification abilities in comparison to community-dwelling older adults without cognitive decline. These findings suggest the importance of olfactory evaluation before providing patients with dementia with therapeutic interventions associated with olfactory stimuli.     

Pediatric case of Crohn’s disease with preceding vulvitis granulomatosa

Crohn’s disease (CD) of the vulva is a rare and under‐recognized condition. Since vulvar lesions may precede the diagnosis of digestive CD in approximately 25% of all cases, the coexistence or future onset of CD should be considered regardless of the gastrointestinal symptoms, even for pediatric patients.     

Effect of intramuscular lidocaine injection with physical therapy on camptocormia in patients with Parkinson’s disease who had previously had deep brain stimulation

[Purpose] We aimed to evaluate the effects of an intervention consisting of intramuscular lidocaine injection in combination with physical therapy on the standing posture, balance ability, and walking ability in patients with Parkinson’s disease who had camptocormia after deep brain stimulation. [Participants and Methods] The participants were nine patients with Parkinson’s disease who had previously undergone deep brain stimulation. The intervention comprised a lidocaine injection into the abdominal external oblique muscles for five days in combination with physical therapy, including body weight-supported treadmill training for two weeks. Before and after the intervention, the total and upper camptocormia angles were used to assess the standing posture; the Berg balance scale was used to assess the balancing ability; and maximum walking speed and stride length were used to assess the walking ability. [Results] The total and upper camptocormia angles, and Berg balance scale improved significantly more after the intervention than before. Before and after the intervention, there was no significant difference in maximum walking speed, but the stride length was significantly greater after the intervention than before. [Conclusion] The intervention was effective in alleviating camptocormia and improving the balance and walking abilities of patients with Parkinson’s disease with camptocormia after deep brain stimulation.     

Beneficial effect of transient desflurane inhalation on relieving inflammation and reducing signaling induced by MPTP in mice

ObjectiveTo determine if the beneficial effects of transient desflurane application mitigates inflammation and decrease associated signaling induced by 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine (MPTP) in mice.MethodsMice were induced to develop Parkinson’s disease (PD) by intraperitoneal injection with MPTP for 20 consecutive days, and validated mice were randomly allocated to four groups. Collected samples from euthanized mice were designated for the following analyses: 1) immunohistochemical staining for positive dopaminergic neurons in the substantia nigra and striatum, 2) immunofluorescence staining for ionized calcium binding adaptor molecule-1 (Iba1) and glial fibrillary acid protein (GFAP), and 3) western blotting for p38, p-p38, toll-like receptor 4, and tumor necrosis factor (TNF)-α.ResultsThe inhalation of desflurane for 1 hour ameliorated locomotory dysfunctions of PD mice by recovering the loss of Iba1- and GFAP-positive dopaminergic neurons, deactivating microglial cells and astrocytes, and decreasing the amounts of inflammatory cytokines (TNF-α).ConclusionsThese findings suggest that transient desflurane inhalation may provide some benefits for PD through ameliorating inflammation and enhancing locomotor activity.     

Evaluation of analytic and clinical performance of two immunoassays for detecting thyroid‐stimulating receptor antibody in the diagnosis of Graves’ disease

ObjectiveTo evaluate the analytical and clinical performance of two immunoassays for diagnosis of Graves’ disease (GD), the Immulite thyroid‐stimulating immunoglobulin (TSI), and Elecsys Anti‐TSH receptor (TSHR) assay.MethodsPrecision and analytical measurement range were assessed using pooled samples of patients. The comparison between the two methods was evaluated using 579 clinical samples, and receiver operating characteristic (ROC) curves were drawn using the final diagnosis as reference. Clinical sensitivity and specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) were calculated for the two tests.ResultsThe repeatability and intermediate imprecision coefficient of variation (CV%) of the TSI assay were 3.8% and 4.1% at 0.95 IU/L, and 3.5% and3.6% at 19.5 IU/L, respectively. The assays were linear over a range 0.27–38.5 IU/L. There was a high correlation between the quantitative results of the two methods (correlation coefficient r = 0.930). The cut‐off value obtained by ROC analysis for TSI assay was 0.7 IU/L with sensitivity of 93.7% and specificity of 85.1%. An overall qualitative agreement of 91.5% between two methods was observed. Among 44 patients with discordant qualitative results, the TSI assay provided more satisfactory results consistent with clinical diagnoses.ConclusionThe TSI assay showed excellent analytical performance and provided a high PPV for GD.     

Wilkie’s syndrome as a cause of anxiety-depressive disorder: A case report and review of literature

BACKGROUNDSuperior mesenteric artery syndrome is a disease with a complex diagnosis, and it is associated with complications that make it even harder to identify. Currently, a frequent association with psychiatric disorders has been noted. Despite numerous case reports and case series, the variability of the disease has not allowed the development of protocols regarding diagnosis and management.CASE SUMMARYA 33-year-old woman presented with abdominal pain, nausea, and bile vomiting over the last 15 mo, associated with a 15-kg weight loss over the last three months. After the onset of the symptoms, the patient was diagnosed with anxiety-depressive disorder and treated appropriately. Standard examinations excluded an organic cause, and the cause of the symptoms was considered psychogenic. The persistence of symptoms, even under treatment, prompted a computer tomography angiography examination of the abdomen and pelvis. The examination identified emergence at a sharp angle of 13.7° of the superior mesenteric artery, with a reduced distance between the artery and the anterior wall of the aorta up to a maximum of 8 mm. A diagnosis of aortomesenteric clamp was established. Surgical treatment by laparoscopic duodenojejunostomy was performed. Postoperative evolution was marked by a patent anastomosis at 1 mo, with a 10-kg weight gain and improvement of the associated anxiety.CONCLUSIONThis case report underlines two major aspects. One aspect refers to the predisposition of patients with superior mesenteric artery syndrome to develop psychiatric disorders, with an excellent outcome when proper treatment is administered. The second aspect underlines the key role of a multidisciplinary approach and follow-up.     

Predictors of necessity for endoscopic balloon dilatation in patients with Crohn’s disease-related small bowel stenosis

Background and AimIn patients with Crohn’s disease (CD) and small bowel stenosis, endoscopic balloon dilation (EBD) is considered to be useful in improving stenotic symptoms and avoiding surgery. However, it carries risks such as bleeding and perforation. The aim of this study was to identify the indications for endoscopic intervention in patients with CD and small bowel stenosis.MethodsFrom November 2007 to March 2020, 143 CD patients with small bowel stenosis were enrolled in this study. We identified the factors associated with not requiring endoscopic intervention during long-term follow-up of these patients.ResultsForty of the 143 patients had abdominal symptoms of stenosis and had undergone EBD, whereas the remaining 103 were asymptomatic and had not undergone endoscopic intervention. During long-term follow-up, 95 of those 103 patients never required endoscopic or surgical intervention. Multivariate logistic regression analysis revealed that not consuming an elemental diet (OR 3.18, 95% CI 1.48–6.82; p < .01) and ileocecal valve (ICV) stenosis (OR 0.30, 95% CI 0.11–0.83; p = .02) were independently associated with not requiring EBD. The cumulative emergency hospitalisation-free rate also tended to be higher in patients not consuming an elemental diet or with ICV stenosis.ConclusionsTwo factors, namely not consuming an elemental diet and ICV stenosis, predict a long-term intervention-free prognosis in CD patients with small bowel stenosis.

Key messages

• When an endoscopically impassable small bowel stenosis is found in a CD patient, long-term follow-up without endoscopic intervention may be possible if the patient is asymptomatic, is not using an elemental diet, and the stenosis is ICV.

     

Diagnostic and surgical challenges of progressive neck and upper back painless masses in Madelung’s disease: A case report and review of literature

BACKGROUNDMadelung’s disease (MD) is a chronic alcoholism-associated metabolic syndrome characterized by symmetrical subcutaneous deposition of adipose tissue in the head, neck, shoulders, back, trunk, and nerve roots of the upper and lower limbs. It is relatively rare in Asian individuals and is prone to misdiagnosis. Herein, we report a case of a patient with MD who had undergone surgical management at our hospital, and we discuss the pathogenesis, diagnosis, and treatment of MD.CASE SUMMARYWe report a case of MD in a 65-year-old man of Han descent. The patient had multiple, painless progressive masses for more than five years in the neck and more than 30 years in the upper back. Because of neck mobility limitations and progressive cosmetic deformities caused by the masses, he was admitted to our hospital. He drank approximately 500 mL of liquor per day and smoked heavily for more than 30 years. Contrast-enhanced computed tomography of the neck and chest documented abundant unencapsulated, subcutaneous fatty deposits. We prepared a staged operation plan. The patient was diagnosed with MD; he was advised to abstain from alcohol and was followed up regularly. After a 3-month follow-up, no recurrence of fat accumulation was found in the surgical areas.CONCLUSIONThis report presents a case of surgical treatment for MD to improve clinicians'' understanding of the disease.     

A case of post COVID‐19 multisystem inflammatory syndrome and Bell's palsy in a young adult

The clinician should put MIS‐A at the top of differential diagnoses of a patient with febrile illness and multiple organ dysfunction during the early post‐COVID‐19 period. Also, facial nerve palsy might follow COVID‐19, related to the autoimmune phenomenon.     

Concatenation of molecular docking and molecular simulation of BACE-1, γ-secretase targeted ligands: in pursuit of Alzheimer’s treatment

IntroductionAlzheimer''s disease (AD), the most predominant cause of dementia, has evolved tremendously with an escalating frequency, mainly affecting the elderly population. An effective means of delaying, preventing, or treating AD is yet to be achieved. The failure rate of dementia drug trials has been relatively higher than in other disease-related clinical trials. Hence, multi-targeted therapeutic approaches are gaining attention in pharmacological developments.AimsAs an extension of our earlier reports, we have performed docking and molecular dynamic (MD) simulation studies for the same 13 potential ligands against beta-site APP cleaving enzyme 1 (BACE-1) and γ-secretase as a therapeutic target for AD. The In-silico screening of these ligands as potential inhibitors of BACE-1 and γ-secretase was performed using AutoDock enabled PyRx v-0.8. The protein-ligand interactions were analyzed in Discovery Studio 2020 (BIOVIA). The stability of the most promising ligand against BACE-1 and γ-secretase was evaluated by MD simulation using Desmond-2018 (Schrodinger, LLC, NY, USA).ResultsThe computational screening revealed that the docking energy values for each of the ligands against both the target enzymes were in the range of −7.0 to −10.1 kcal/mol. Among the 13 ligands, 8 (55E, 6Z2, 6Z5, BRW, F1B, GVP, IQ6, and X37) showed binding energies of ≤−8 kcal/mol against BACE-1 and γ-secretase. For the selected enzyme targets, BACE-1 and γ-secretase, 6Z5 displayed the lowest binding energy of −10.1 and −9.8 kcal/mol, respectively. The MD simulation study confirmed the stability of BACE-6Z5 and γ-secretase-6Z5 complexes and highlighted the formation of a stable complex between 6Z5 and target enzymes.ConclusionThe virtual screening, molecular docking, and molecular dynamics simulation studies revealed the potential of these multi-enzyme targeted ligands. Among the studied ligands, 6Z5 seems to have the best binding potential and forms a stable complex with BACE-1 and γ-secretase. We recommend the synthesis of 6Z5 for future in-vitro and in-vivo studies.     

Anesthesia and perioperative management for giant adrenal Ewing’s sarcoma with inferior vena cava and right atrium tumor thrombus: A case report

BACKGROUNDEwing’s sarcoma of the adrenal gland with inferior vena cava (IVC) and right atrium thrombus is extremely rare. Here, we report a case of giant adrenal Ewing’s sarcoma with IVC and right atrium tumor thrombus and summarize the anesthesia and perioperative management.CASE SUMMARYA young female was admitted to the Department of Urology with intermittent pain under the right costal arch for four months. Enhanced abdominal computed tomography revealed a large retroperitoneal mass (22 cm in diameter), which may have originated from the right adrenal gland and was closely related to the liver. Transthoracic echocardiography showed a strong echogenic filling measuring 70 mm extended from the IVC into the right atrium and ventricle. After preoperative preparation with cardiopulmonary bypass, sufficient blood products, transesophageal echocardiography and multiple monitoring, tumor and thrombus resection by IVC exploration and right atriotomy were successfully performed by a multidisciplinary team. Intraoperative hemodynamic stability was the major concern of anesthesiologists and the status of tumor thrombus and pulmonary embolism were monitored continuously. During transfer of the patient to the intensive care unit (ICU), cardiac arrest occurred without external stimulus. Cardiopulmonary resuscitation was performed immediately and cardiac function was restored after 1 min. In the ICU, extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT) were provided to maintain cardiac, liver and kidney function. Histopathologic examination confirmed the diagnosis of Ewing’s sarcoma. After postoperative treatments and rehabilitation, the patient was discharged from the urology ward.CONCLUSIONAn adrenal Ewing’s sarcoma with IVC and right atrium thrombus is extremely rare, and its anesthesia and perioperative management have not been reported. Thus, this report provides significant insights in the perioperative management of patients with adrenal Ewing’s sarcoma and IVC tumor thrombus. Intraoperative circulation fluctuations and sudden cardiovascular events are the major challenges during surgery. In addition, postoperative treatments including ECMO and CRRT provide essential support in critically ill patients. Moreover, this case report also highlights the importance of multidisciplinary cooperation during treatment of the disease.