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Interestingly, degeneration of BDNF-dependent sensory neurons requires BAX and appears to rely on reactive oxygen species (ROS) generation rather than caspases to induce degeneration. These results highlight the complexity and divergence of mechanisms regulating developmental sensory neuron death.

To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability.

We performed database review for patients seen between 1997 and 2020 at the Emory ALS Center in Atlanta, Georgia. Patients with ALS were included for analyses if race was self-reported as Black or White and symptom onset was prior to January 1, 2017. Variables examined include race, age at onset, diagnostic delay, site of onset, median income,

mutation status, feeding tube and tracheostomy status, vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale-revised(ALSFRS-R) score, and survival time.

A total of 2,363 patient records were queried, and 1,298 were included in analysis; 203 self-identified as Black and 1,095 as White. Black patients had younger age at symptom onset, lower frequency of

mutations, lower median income, longer diagnostic delay, and lower baseline ALSFRS-R and vital capacity compared to White patients. Bler baseline ventilatory and functional status at first clinic visit compared to White patients, which could be suggestive of barriers to tertiary care. Further studies are needed to identify the underlying causes of ALS racial differences.

To test the hypothesis that immunotherapy prevents long-term disability in relapsing-remitting multiple sclerosis (MS), we modeled disability outcomes in 14,717 patients.

We studied patients from MSBase followed for ≥1 year, with ≥3 visits, ≥1 visit per year, and exposed to MS therapy, and a subset of patients with ≥15-year follow-up. Marginal structural models were used to compare the cumulative hazards of 12-month confirmed increase and decrease in disability, Expanded Disability Status Scale (EDSS) step 6, and the incidence of relapses between treated and untreated periods. Marginal structural models were continuously readjusted for patient age, sex, pregnancy, date, disease course, time from first symptom, prior relapse history, disability, and MRI activity.

A total of 14,717 patients were studied. During the treated periods, patients were less likely to experience relapses (hazard ratio 0.60, 95% confidence interval [CI] 0.43-0.82,

= 0.0016), worsening of disability (0.56, 0.38-0.82,

= 0.0026), and progress to EDSS step 6 (0.33, 0.19-0.59,

= 0.00019). Among 1,085 patients with ≥15-year follow-up, the treated patients were less likely to experience relapses (0.59, 0.50-0.70,

= 10

) and worsening of disability (0.81, 0.67-0.99,

= 0.043).

Continued treatment with MS immunotherapies reduces disability accrual by 19%-44% (95% CI 1%-62%), the risk of need of a walking aid by 67% (95% CI 41%-81%), and the frequency of relapses by 40-41% (95% CI 18%-57%) over 15 years. This study provides evidence that disease-modifying therapies are effective in improving disability outcomes in relapsing-remitting MS over the long term.

This study provides Class IV evidence that, for patients with relapsing-remitting MS, long-term exposure to immunotherapy prevents neurologic disability.

This study provides Class IV evidence that, for patients with relapsing-remitting MS, long-term exposure to immunotherapy prevents neurologic disability.Cardiac disease after mediastinal radiotherapy can result in progressive valvular thickening and dystrophic calcification with ensuing leaflet restriction and dysfunction. This can ultimately manifest as valvular stenosis and/or regurgitation. Kynurenic acid We report a case of a 61-year-old woman with symptomatic severe aortic stenosis and severe mitral stenosis due to severe dystrophic calcification postmediastinal radiotherapy for lymphoma. She was deemed surgically inoperable due to dense, continuous calcification throughout the leaflets and annuli of both valves, aortomitral continuity, proximal coronary arteries and proximal porcelain aorta. She underwent simultaneous transcatheter aortic valve replacement and transcatheter mitral valve replacement with an excellent technical and clinical result at 7-month follow-up. We also describe the central role of multimodality three-dimensional transoesophageal echocardiography and multidetector cardiac CT imaging in assessing the severity of valve disease, characterising the nature of cardiac calcification and guiding decisions on surgical operability and transcatheter intervention.Ossifying fibroma (OF) is a rare, benign, fibro-osseous lesion of the jawbone characterised by replacement of the normal bone with fibrous tissue. The fibrous tissue shows varying amount of calcified structures resembling bone and/or cementum. The central variant of OF is rare, and shows predilection for mandible among the jawbone. Although it is classified as fibro-osseous lesion, it clinically behaves as a benign tumour and can grow to large size, causing bony swelling and facial asymmetry. This paper reports a case of large central OF of mandible in a 40-year-old male patient. The lesion was treated by segmental resection of mandible. Reconstruction of the surgical defect was done using avascular fibula bone graft. link2 Role of three-dimensional printing of jaw and its benefits in surgical planning and reconstruction are also highlighted.We present a previously healthy man in his 30s who presented with typical viral prodrome symptoms and worsening abdominal pain. He was found to have portal vein thrombosis, with extensive hypercoagulability workup performed. It was determined that the aetiology of thrombus was secondary to acute cytomegalovirus infection. The patient was started on anticoagulation therapy, with later clot resolution demonstrated on abdominal Doppler ultrasound and abdominal CT scan. Given the atypical presentation of this common virus, we performed a literature review of cytomegalovirus-associated portal vein thrombosis in healthy individuals; we found that most patients present with non-specific symptoms of fever and abdominal pain in the setting of a viral prodrome. This case and literature review suggest physicians must consider cytomegalovirus-associated portal vein thrombosis as a potential diagnosis when patients present with abdominal pain and viral symptoms. The literature highlights the need for a consensus on anticoagulation and antiviral therapy.Bilateral adrenal haemorrhage is a rare and often fatal condition that most commonly occurs under conditions of severe physiological stress. We describe a 33-year-old male patient with ulcerative colitis who presented with acute worsening epigastric pain, vomiting and raised inflammatory markers. Initial differentials included gastritis and peptic ulceration. Gastroscopy revealed no abnormalities. By day 3, he had developed sepsis with a sequential organ failure assessment score of 2 as well as coagulopathy. A subsequent CT scan diagnosed bilateral adrenal haemorrhage. A short Synacthen Test confirmed adrenal insufficiency and he was treated with replacement steroids and antibiotics for a possible urinary tract infection or pyelonephritis and he recovered well. Several days later he developed fever, dyspnoea and a productive cough. Subsequently, he became hypotensive (Blood Pressure (BP) 95/65 mm Hg) and unresponsive with a Glasgow Coma Scale of 7 and was hyponatraemic and hyperkalaemic. He was intubated and transferred to a tertiary hospital for intensive care unit management where investigations confirmed the patient to be influenza A positive.A 71-year-old man presented with a 3-month history of low back pain radiating to his right hip and thigh associated with lower limb weakness and constitutional symptoms. Imaging confirmed a lumbosacral spondylodiscitis at L2-3 and L5-S1 as well as a right-side psoas abscess which was treated with urgent CT-guided drainage and intravenous antibiotics. His admission was complicated by a number of issues, including the development of osteomyelitis with vertebral body destruction at multiple sites, epidural abscess formation and deep vein thrombosis. Additionally, the patient developed severe sepsis which necessitated admission to the intensive care unit. The patient's clinical condition improved gradually with intravenous antibiotics until he was well enough for transfer to a rehabilitation centre, where he underwent regular occupational and physical therapy. Repeat imaging showed overall resolution of the aforementioned pathologies and is currently being followed up by the spinal surgeons on an outpatient basis.An 85-year-old malnourished woman presented with symptoms of small bowel obstruction of uncertain aetiology. She had presented numerous times over the previous 2 years with symptoms of left groin and thigh pain, vomiting and abdominal distension. A CT of her abdomen and pelvis ultimately revealed a left-sided pelvic hernia, between the obturator internus and pectineal muscles. This was consistent with an obturator hernia. Diagnostic laparoscopy confirmed an obturator hernia of Richter type, incarcerated within the left obturator canal. Reduction revealed a hernia sac containing viable small bowel. A primary repair was performed using a double-layer suture technique to both close and plug the hernia defect. The patient rapidly recovered following hernia repair, with resolution of all previous long-standing symptoms. This case exemplifies the typical presentation of an obturator hernia and the diagnostic challenge it poses to clinicians.Perineal hernia with bowel gangrene is uncommon but known complication of laparoscopic extralevator abdominoperineal excision (ELAPE). We present a rare case of closed loop small bowel obstruction with bowel gangrene secondary to an incarcerated perineal hernia that developed 7 years after an ELAPE. Intraoperatively, we found a definitive transition point due to adhesions in pelvis and a closed loop obstruction of the distal small bowel at different site with gangrenous intestine. She was managed successfully surgically with adhesiolysis and fixation of defect with biological mesh. Prevalence of perineal hernias will rise in future because of the increasing cases of ELAPE, in which no repair of pelvic floor is performed. The need of follow-up of these operations and more reporting of such cases are important in increasing awareness of these complications. Patients should be made aware of such complications and should seek urgent medical care.Neurological manifestations of hypothyroidism include peripheral neuropathy and pituitary hyperplasia. However, these associations are rarely encountered during pregnancy. We report a case of a known hypothyroid with very high thyroid stimulating hormone (TSH) values (512 μIU/mL) in the second trimester. At 24 weeks she developed facial palsy and pituitary hyperplasia which responded to a combination of steroids and thyroxine. She had caesarean delivery at 35 weeks and 3 days gestation in view of pre-eclampsia with severe features and was discharged on oral antihypertensives and thyroxine. link3 On follow-up at 5 months, TSH normalised and pituitary hyperplasia showed a greater than 50% reduction in size. To our knowledge, this is the first reported case of facial palsy and pituitary hyperplasia associated with hypothyroidism during pregnancy.