Rejected

Porphyrias constitute a group of rare genetic diseases due to various, mostly autosomal dominant mutations, causing enzymatic deficiency in heme biosynthesis. As a result, neurotoxic porphyrin precursors and light-sensitive porphyrins accumulate, while dysfunction in their targets determines the disease symptoms. Variegate porphyria (VP), one of the acute hepatic porphyrias, is caused by a protoporphyrinogen oxidase (PPOX) mutation. During acute attacks, among other factors, triggered by drugs, stressors, or fasting, an increase in urinary and fecal porphobilinogen (PBG), aminolevulinic acid (ALA), and porphyrins occurs, damaging the autonomous, peripheral, or central nervous system. The disease remains often latent or displays minimal symptoms usually overlooked, exposing undiagnosed patients to potentially serious complications in the presence of the aforementioned triggers. This 46-year-old woman presented, some days after a bariatric surgery, with severe flaccid tetraparesis and neuropathic pain, initially misdiagnosed as a functional neurological disorder. The severe axonal sensorimotor polyneuropathy led to further investigations, disclosing high urinary porphobilinogen, ALA, and porphyrin levels due to a new PPOX mutation. Retrospectively, it appeared that the patient had had typical VP symptoms (abdominal pain, fragile skin, and dark urine episodes) for years prior to the surgery. Treated with carbohydrate load, neurorehabilitation, and analgesics, she slowly recovered to full mobility, with partial autonomy in her daily life activities, although fatigue and severe pain persisted, preventing her from returning to work. This case documents gastric bypass surgery as a trigger of severe VP invalidating neurological symptoms and illustrates how the delayed diagnosis and post-interventional complications could have been prevented by screening for porphyria cardinal symptoms prior to the intervention. Likewise, this cost-effective screening should be performed before any treatment influencing the diet, which would dramatically improve the porphyria diagnosis rate and outcome.

A healthy 23-year-old female developed generalized pruritus over a year that began on her feet and gradually progressed to involve more than 50% of her entire body surface area. Punch skin biopsies were inconclusive, whereas a two-view chest x-ray was suspicious for lymphadenopathy. A chest computed tomography scan with contrast identified an anterior mediastinal mass which was biopsied and diagnosed as a nodular sclerosis type of Hodgkin's lymphoma. Subsequently, appropriate therapy was initiated resulting in complete resolution of the patient's chronic itch. This case underscores the clinical significance of a comprehensive systemic evaluation in chronic pruritus of unclear etiology.

This study aimed to investigate portable polysomnography (PSG)-based 'sleep' and pre-diagnosis of obstructive sleep apnoea (OSA) in acute temporomandibular disorder (TMD) and patients with chronic TMD.

In rare cases, chronic subdural hematoma can be a complication following cranioplasty implantation. Therefore, it can develop spontaneously or after a trauma in the underlying site of a duroplasty and represent, if compression of the brain structures, a life-threatening condition. In case of a patient with cranioplasty in polyetheretherketone (PEEK), performing a burr hole on prosthesis can represent, although unusual, an effective and safe technique for evacuation of the chronic subdural hematoma, avoiding the need to remove the prosthesis itself. Nevertheless, a rare and insidious prosthesis infection can occur, even after years.

Smell and taste disturbances are among the most frequent neurological symptoms in patients with COVID-19. A concomitant impairment of the trigeminal nerve has been suggested in subjects with olfactory dysfunction, although it has not been confirmed with objective measurement techniques. In this study, we explored the trigeminal function and its correlations with clinical features in COVID-19 patients with impaired smell perception using electrophysiological testing.

Chronic low back pain (CLBP) is an important cause for reduced daily physical activity (PA) and loss of quality of life, especially in women. In Suriname, a middle-income country in South America, the relationship between PA and CLBP is still unknown.

Intravenous patient-controlled analgesia (IV-PCA) has been widely used; however, regimen criteria have not yet been established. In China, the most often used opioid is sufentanil, for which repeated doses are a concern, and empirical flurbiprofen axetil (FBP) as an adjuvant. We hypothesized that hydromorphone would be a better choice and also evaluated the effectiveness of FBP as an adjuvant. This historical cohort study was conducted in two tertiary hospitals in China and included 12,674 patients using hydromorphone or sufentanil for IV-PCA between April 1, 2017, and January 30, 2021. The primary outcome was analgesic insufficiency at static (AIS). The secondary outcomes included analgesic insufficiency with movement (AIM) and common opioid-related adverse effects such as postoperative nausea and vomiting (PONV) and dizziness. Sufentanil, but not the sufentanil-FBP combination, was associated with higher risks of AIS and AIM compared to those for hydromorphone (OR 1.64 [1.23, 2.19], < 0.001 and OR 1.42 [1.16, 1.73], < 0.001). Hydromorphone combined with FBP also decreased the risk of both AIS and AIM compared to those for pure hydromorphone (OR 0.74 [0.61, 0.90], = 0.003 and OR 0.80 [0.71, 0.91], < 0.001). However, the risk of PONV was higher in patients aged ≤35 years using FBP (hydromorphone-FBP vs. hydromorphone and sufentanil-FBP vs. hydromorphone, OR 1.69 [1.22, 2.33], = 0.001 and 1.79 [1.12, 2.86], = 0.015). Hydromorphone was superior to sufentanil for IV-PCA in postoperative analgesia. Adding FBP may improve the analgesic effects of both hydromorphone and sufentanil but was associated with an increased risk of PONV in patients <35 years of age.

This study is aimed at investigating the correlation between lumbar spinal stenosis (LSS) severity, ligamentum flavum hypertrophy, and the upregulation of inflammatory markers.

Gout is one of the most common inflammatory arthritis, where identification of MSU crystals in synovial fluid is a widely used diagnostic measure. Ultrasonography has a great sensitivity in detecting signs of MSU deposits, such as tophi and double contour (DC), as mentioned in the latest gout criteria, allowing early clinical diagnosis and therapy.