OpenMed/multicare-articles huggingface dataset:
Pancreatic Myeloid Sarcoma Causing Obstructive Jaundice: A Case Report and Literature Review
Authors: Laura E Lavette, Angela G Niehaus, Clancy J Clark, Jason D Conway, Girish Mishra, Darius A Jahann
Journal: Case Rep Gastrointest Med (2024)
MeSH terms: Case Reports

Myeloid sarcoma (MS) is an extramedullary manifestation of acute myeloid leukemia (AML) and commonly occurs in sites such as the lymph nodes, skin, soft tissues, and bone. It more rarely manifests in the pancreas, with less than 20 cases reported in the literature since 1987. Despite its rarity, MS should be considered in the differential diagnosis of a soft tissue mass causing obstructive jaundice, especially if the patient has a known hematologic disease. Isolated cases of pancreatic MS have been known to progress to AML; therefore, it is crucial to differentiate MS from more common diagnoses, such as pancreatic cancer or pancreatitis. This is a case of a 70-year-old male with symptomatic obstructive jaundice secondary to pancreatic MS, ultimately requiring endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) for diagnosis and management. Also included is a comprehensive review of previous case reports with similar clinical presentations, management, and treatment of pancreatic MS.
Targeting Weakness With a Combination of Isotonic Exercises in Dermatomyositis With Polyneuropathy: A Case Report
Authors: Vaishnavi R Waghe, Anam R Sasun, Raghuveer Raghumahanti
Journal: Cureus (2024)
Keywords: dermatomyositis, physiotherapy, polyneuropathy, proprioceptive neuromuscular facilitation, weakness
MeSH terms: Case Reports

Dermatomyositis, an autoimmune inflammatory myositis commonly linked to polymyositis, is marked by inflammatory and degenerative transformations impacting muscles, skin, limb girdles, the neck, and the pharynx. These changes result in symmetrical weakness and diverse levels of muscle atrophy. Uncommonly, the condition may impact the esophagus, lungs, and heart. While dermatomyositis is believed to involve genetic, immunological, and environmental factors, its precise etiology remains elusive. Typically, the classical presentation involves a symmetrical proximal myopathy alongside dermatological manifestations such as a purplish-red rash affecting the face, arms, hands, legs, and other areas. Additional symptoms may include dysphagia, myalgia, fever, and weight loss. The primary objectives of managing dermatomyositis are to address muscular weakness, skin manifestations, and any underlying health concerns. Integral to this management is the utilization of physical therapy and rehabilitation interventions. This study introduces a 23-year-old female patient with a noteworthy medical history covering a duration of two months. The patient reported a chief complaint of persistent thigh pain and a concurrent complaint concerning bilateral weakness in upper and lower extremities. Furthermore, the patient faced the additional challenge of difficulty swallowing. Intriguingly, the patient's clinical presentation was marked not only by the aforementioned symptoms but also by the development of a distinctive facial rash. This facial rash was accompanied by symptoms of stiffness in both small and large joints and a reduction in the range of affected joints. The physiotherapeutic assessment revealed quadriparesis of bilateral upper and lower limbs. The rehabilitation programme for the patient was planned by targeting proprioceptors to increase dynamic trunk balance in patients with DM. The Proprioceptive Neuromuscular Facilitation (PNF) technique employs diagonal movement patterns, thereby proving instrumental in enhancing the patient's daily activities. This methodology serves to optimize the individual's capacity to execute routine daily tasks, promoting independence in their daily life. An investigation like the Nerve Conduction Velocity (NCV) report shows the absence of motor excitation, suggesting motor axonal neuropathy. This approach, comprising isometric, concentric, and eccentric contraction exercises, demonstrated efficacy in mitigating muscular weakness, enhancing motor function, and alleviating the diverse symptoms associated with this condition.
A case report: Null-cell cardiac lymphoma in an English bulldog
Authors: Liza S Köster, Kim Newkirk, Philip Krawec
Journal: Front Vet Sci (2024)
Keywords: cardiac tumor, case report, dog, echocardiogram, null-cell lymphoma, pericardial effusion, right heart failure
MeSH terms: Case Reports

This case report describes a novel example of an extranodal null-type lymphoma in the myocardium of a middle-aged English bulldog who presented with signs of right heart failure. An echocardiogram found, in addition to the pericardial effusion, thickened right and left ventricular free walls and the interventricular septum. The right ventricular free wall myocardium had multinodular lesions, suspicious for infiltrative disease. The owner elected humane euthanasia, and permission for necropsy was obtained. Multifocal left and right ventricular nodules and an incidental aortic root mass were detected, the latter of which was later confirmed as a chemodectoma. Microscopically, the myocardial nodules were sheets of round cells consistent with a high-grade lymphoma. Neoplastic cells were not immunoreactive to CD3 (T-cell) or CD20 and CD79a (B-cell), Mum-1 (plasma cell), CD117 (mast cell), or CD18 (histiocyte). These findings are consistent with a high-grade, null-cell-type lymphoma.
A Rare Presentation of Wegener's Granulomatosis With Perinuclear Antineutrophil Cytoplasmic Antibody Positivity
Authors: Dhammika P Rathnayake, Kvc Janaka, Hassan Hussain, Sudeshan Senanayake, Wds Lakindi
Journal: Cureus (2024)
Keywords: autoimmune vasculitis, granulomatosis with polyangiitis (gpa), p-anca, vasculitis, wegeners granulomatosis
MeSH terms: Case Reports

Wegener's granulomatosis (WG), also termed granulomatosis with polyangiitis, is a vasculitic condition with numerous systemic manifestations. It is mainly a pauci-immune vasculitis which involves small and medium-sized vessels. Herein, we report a case of a 72-year-old female with a background history of type 2 diabetes mellitus and hypertension. She presented to the hospital with recurrent nasal congestion, headache, and intermittent facial pain for two months' duration. At the initial presentation, she was diagnosed with pansinusitis, for which she was treated with antibiotics and was discharged. Two weeks later, she presented with progressively worsening bilateral lower limb numbness and weakness for one week's duration. With serial investigations, she was diagnosed with WG and was treated with intravenous methylprednisolone along with cyclophosphamide, after which she made a significant improvement in her clinical status.
Streptococcus suis meningitis in China: a case report
Authors: Tiantian Liu, Hengfang Liu, Yanjie Jia
Journal: Front Public Health (2024)
Keywords: streptococcus suis, case report, hearing loss, meningitis, metagenomic next-generation sequencing
MeSH terms: Meningitis, Bacterial / diagnosis, Meningitis, Bacterial / drug therapy, Streptococcal Infections / drug therapy, Streptococcus suis / isolation & purification, Case Reports, Anti-Bacterial Agents / therapeutic use, China, Hearing Loss, Sudden / drug therapy, Hearing Loss, Sudden / etiology, Humans, Male, Middle Aged, Sepsis / drug therapy, Anti-Bacterial Agents

Introduction:

Streptococcus suis is one of the porcine pathogens that have recently emerged as a pathogen capable of causing zoonoses in some humans. Patients infected with S. suis can present with sepsis, meningitis, or arthritis. Compared to common pathogens, such as Meningococcus, Streptococcus pneumoniae, and Haemophilus influenzae, S. suis infections in humans have been reported only rarely.
Methods:
This case report described a 57-year-old man who presented with impaired consciousness and fever following several days of backache. He was a butcher who worked in an abattoir and had wounded his hands 2 weeks prior. The patient was dependent on alcohol for almost 40 years. S. suis was detected in the cerebrospinal fluid by metagenomic next-generation sequencing. Although he received adequate meropenem and low-dose steroid therapy, the patient suffered from bilateral sudden deafness after 5 days of the infection. The final diagnosis was S. suis meningitis and sepsis.
Results:
The patient survived with hearing loss in both ears and dizziness at the 60-day follow-up.
Discussion:
We reported a case of S. suis infection manifested as purulent meningitis and sepsis. Based on literature published worldwide, human S. suis meningitis shows an acute onset and rapid progression in the nervous system. Similar to bacterial meningitis, effective antibiotics, and low-dose steroids play important roles in the treatment of human S. suis meningitis.
Optic nerve and chiasm hemangioblastomas in von Hippel-Lindau disease: report of 12 cases and review of the literature
Authors: Evelynn Vergauwen, Jan-Helge Klingler, Marie T Krüger, Christine Steiert, Robert Kuijpers, Steffen Rosahl, Anne-Marie Vanbinst, Corina Emilia Andreescu, Sven Gläsker
Journal: Front Oncol (2024)
Keywords: vhl, eye, hemangioblastoma, optic chiasm, optic hemangioblastoma, optic nerve, review, von hippel-lindau

Introduction:
Optic nerve and chiasm hemangioblastomas are rare tumors, occurring sporadically or in the context of von Hippel-Lindau (VHL) disease. They have only been portrayed in isolated case reports and small cohorts. Their natural history and therapeutic strategies are only scarcely described. To better characterize these rare tumors, we retrospectively analyzed an optic nerve and chiasm hemangioblastoma series of 12 VHL patients. By combining our own experience to a review of all known cases in literature, we intended to create treatment recommendations for optic nerve and chiasm hemangioblastomas in VHL patients.
Methods:
We reviewed two electronic databases in the hospitals of our senior authors, searching for VHL patients with optic nerve or chiasm hemangioblastomas. Clinical data were summarized. Tumor size and growth rate were measured on contrast enhanced MRI. Comparable data were collected by literature review of all available cases in VHL patients (Pubmed, Trip, Google and Google Scholar).
Results:
Of 269 VHL patients, 12 had optic nerve or chiasm hemangioblastomas. In 10 of 12 patients, tumors were diagnosed upon annual ophthalmoscopic/MRI screening. Of 8 patients who were asymptomatic at diagnosis, 7 showed absent or very slow annual progression, without developing significant vision impairment. One patient developed moderate vision impairment. Two symptomatic patients suffered from rapid tumor growth and progressive vision impairment. Both underwent late-stage surgery, resulting in incomplete resection and progressive vision impairment. One patient presented with acute vision field loss. A watchful-waiting approach was adopted because the hemangioblastoma was ineligible for vision-sparing surgery. One patient developed progressive vision impairment after watchful waiting. In the literature we found 45 patient cases with 48 hemangioblastomas.
Discussion:
When optic nerve and chiasm hemangioblastomas are diagnosed, we suggest annual MRI follow-up as long as patients do not develop vision impairment. If tumors grow fast, threaten the contralateral eye, or if patients develop progressive vision deficiency; surgical resection must be considered because neurological impairment is irreversible, and resection of large tumors carries a higher risk of further visual decline.
Development of Root Caries Associated With the Use of Sugar-Free Nicotine Lozenges: A Long-Term Case Report
Authors: Coral Ehrhardt, Kauko K Mäkinen, Charles M Cobb
Journal: Case Rep Dent (2024)
Keywords: dental caries, lozenge, maltodextrin, mannitol, root caries, sugar alcohols, tobacco cessation aids
MeSH terms: Case Reports

The authors present a case concerning an adult male patient who developed multiple sites of root caries adjacent to the area where he habitually held a sugar-free nicotine lozenge that contained mannitol and maltodextrin. The root caries occurred despite the patient's excellent oral hygiene, exemplary dietary habits, and clinically normal salivary flow. Between 1999 and 2008, he had only required two restorations to repair carious lesions. This patient had a 20+-year habit of using smokeless tobacco before switching to a cessation aid nicotine lozenge in May of 2008. A full-mouth series of radiographs taken in November 2009 revealed carious lesions on virtually every posterior tooth. The nicotine lozenge's principal ingredients were mannitol (75.7%) and maltodextrin. According to the United States' current Food and Drug Administration (FDA) guidelines, manufacturers can advertise these lozenges as sugar-free. Thus, it is assumed by the public that these types of products are incapable of "causing a cavity." However, this case report presents evidence suggesting that frequent use of sugar-free nicotine lozenges may be associated with dental caries.
Prospective assessment of probe-based confocal laser endomicroscopy under direct cholangioscopic visualization for biliary strictures that could not be definitively diagnosed using endoscopic retrograde cholangiopancreatography (with video)
Authors: Yuki Tanisaka, Shomei Ryozawa, Masafumi Mizuide, Akashi Fujita, Ryuhei Jinushi, Ryuichi Watanabe, Ryo Sato, Tomoaki Tashima, Yumi Mashimo
Journal: DEN Open (2024)
Keywords: biliary stricture, biliary tract neoplasms, cholangioscopy, confocal laser microscopy, endoscopic retrograde cholangiopancreatography
MeSH terms: Case Reports

The definitive diagnosis of patients with indeterminate biliary strictures remains challenging. Probe-based confocal laser endomicroscopy (pCLE) provides real-time histological assessment of bile duct tissues. Since no previous studies have evaluated the efficacy of pCLE under direct cholangioscopic visualization for biliary strictures that cannot be definitively diagnosed through endoscopic retrograde cholangiopancreatography using fluoroscopy, we prospectively assessed the feasibility and safety of this procedure in three cases. pCLE findings were obtained in three cases, providing accurate diagnoses. Additionally, no adverse event was reported. pCLE under direct cholangioscopic visualization for indeterminate biliary strictures might be feasible and safe, even though these strictures were not previously diagnosed using endoscopic retrograde cholangiopancreatography. Further studies with more cases are warranted to clarify the effectiveness of pCLE under direct cholangioscopic visualization.
A novel SSR4 variant associated with congenital disorder of glycosylation: a case report and related analysis
Authors: Wenqiang Sun, Xinyun Jin, Xueping Zhu
Journal: Front Genet (2024)
Keywords: ssr4 gene, x-linked disease, congenital disorders of glycosylation, developmental delay, feeding difficulty
MeSH terms: Case Reports

Introduction:
Congenital disorders of glycosylation (CDG) refer to monogenetic diseases characterized by defective glycosylation of proteins or lipids causing multi-organ disorders. Here, we investigate the clinical features and genetic variants of SSR4-CDG and conduct a preliminary investigation of its pathogenesis.
Methods:
We retrospectively report the clinical data of a male infant with early life respiratory distress, congenital diaphragmatic eventration, cosmetic deformities, and moderate growth retardation. Peripheral blood was collected from the case and parents, genomic DNA was extracted and whole-exome sequencing was performed. The mRNA expression of SSR4 gene was quantified by Real-time Quantitative PCR. RNA sequencing analysis was subsequently performed on the case and a healthy child.
Results:
Whole-exome sequencing of the case and his parents' genomic DNA identified a hemizygous c.80_96del in SSR4, combined with the case's clinical features, the diagnosis of CDG was finally considered. In this case, the expression of SSR4 was downregulated. The case were present with 1,078 genes downregulated and 536 genes upregulated. SSR4 gene expression was significantly downregulated in the case. Meanwhile, gene set enrichment analysis (GSEA) revealed that SSR4-CDG may affect hemostasis, coagulation, catabolism, erythrocyte development and homeostatic regulation, and muscle contraction and regulation, etc. Improvement of growth retardation in case after high calorie formula feeding and rehabilitation training.
Conclusion:
Our study expanded the SSR4-CDG variant spectrum and clinical phenotype and analyzed pathways potentially affected by SSR4-CDG, which may provide further insights into the function of SSR4 and help clinicians better understand this disorder.
Dynamic Surgical Restoration of Mid and Lower Facial Paralysis: A Single-Greek-Centre Experience
Authors: Foteini Neamonitou, Maria Kotrotsiou, Spyros Stavrianos
Journal: Cureus (2024)
Keywords: facial nerve, facial palsy, facial paralysis, myoplasty, reconstruction

Background Facial palsy detrimentally impacts an individual's quality of life due to its effects on function and appearance. There are several reconstructive surgical techniques available that aim to restore facial symmetry. Techniques such as direct neurorrhaphy, nerve grafts, dual reanimation, and reinnervation have the potential to enable varying motor functions, including the re-establishment of a dynamic smile. This study aimed to assess the outcomes of facial palsy reconstructive surgeries undertaken at a tertiary care centre for facial nerve reconstruction in Athens. Methods This study consisted of a comprehensive case series showcasing the outcomes of facial palsy reconstructive surgeries on 29 patients at our Tertiary General Oncological Anti-Cancer Hospital of Athens 'Agios Savvas'. The surgical procedures from October 2004 to December 2023 included reinnervation, nerve grafting, free muscle transfer, and myoplasties following our recommended algorithm. We categorized the patients into two groups: Group A and Group B based on the timing of the reconstruction: delayed or immediate. The House-Brackmann grading scale evaluated the degree and improvement of facial paralysis. Results In Group A, two of the seven patients exhibited activation of the mimetic musculature immediately postsurgery, while the remaining five experienced enhanced facial nerve function in the subsequent months. Adverse outcomes were temporalis dysfunction in one case and tongue atrophy in another. Conversely, in Group B, 21 of 22 patients demonstrated facial activation immediately postsurgery. Only one patient from this group did not show any facial nerve function postoperatively. Two of the 22 patients in Group B encountered complications: one with trismus and another with temporalis dysfunction. All patients were observed for a minimum of 12 months postsurgery. Conclusion With the exception of one patient, all participants showed improved postoperative results, which were satisfactory when weighed against the observed morbidity rate. While our case analysis did not reveal any clear indication of one particular technique being superior, the selection of methods should be based on several factors, and this algorithm could serve as a useful aid in that regard. A comprehensive and standardized clinical assessment of facial palsy, both before and after surgery, is crucial to establish a consensus and plan individualized therapy.
Posterior reversible encephalopathy syndrome post stem cell transplantation in sickle cell disease: case series and literature review
Authors: Hussain A BinAmir, Ali AlAhmari, AlWaleed AlQahtani, Gamal Mohamed, Fawaz Alotaibi, Mohamed AlShamrani, Ali AlSaeed, Suwaidi AlGhanmi, Alaa Heji, Abdulrahman Alreshaid, Ammar AlKawi, Adel AlHazzani, Mohamed AlZawahmah, Ashfaq Shuaib, Fahad Al-Ajlan, Fahad AlMohareb
Journal: Front Med (Lausanne) (2024)
Keywords: bi-hemispheric infarctions, cerebral microbleeds, posterior reversible encephalopathy syndrome, sickle cell disease, stem cell transplantation

Introduction:
Posterior reversible encephalopathy syndrome (PRES) is a serious neurological syndrome that may develop following immunosuppressive therapy for stem cell transplantation (SCT). We report 8 patients with sickle cell disease (SCD) who developed PRES, which is likely to be related to immunosuppression.
Methods:
This is retrospective cohort analysis of the SCD registry at the King Faisal Specialist Hospital and Research Center (KFSHRC) in Riyadh, Saudi Arabia. Inclusion criteria included all adults SCD patients who underwent SCT from 2011 until 2022. We explored all cases of PRES in patients with SCT. PRES was diagnosed with MRI imaging showing reversible vasogenic cerebral edema associated with neurological symptoms including severe headache, seizures, encephalopathy, delirium, and visual disturbances.
Results:
During ten years follow-up (2011-2022) we found 8 patients with PRES (age range between 14 to 37 years at diagnosis) PRES occurred 8 to 124 days following SCT in 7 cases and one patient developed PRES 8 months prior to SCT. All patients were on immunosuppressive medications, including tacrolimus, cyclosporine, sirolimus and or mycophenolate mofetil. Headache, seizures, visual hallucinations, confusion, and drowsiness were the most common presenting symptoms. MRI showed abnormalities in the occipital, parietal and frontal lobes in most cases. Recovery was complete in all patients and no recurrences were noted. Two patients had graft versus host disease (GVHD). We compared risk factors for PRES among the 8 cases and 136 SCT in SCD patients who did not develop PRES. There was a significant association between PRES and imaging abnormalities, including previous bi-hemispheric infarctions (p = 0.001), and cerebral microbleeds (CBMs). PRES was strongly associated with presence (p = 0.006), size (p = 0.016) and number (p = 0.005) of CMBs.
Conclusion:
PRES can develop days to weeks following SCT in patients with SCD, and is associated with immunosuppressive therapy, previous bi-hemispheric infarctions and CMB. Prompt recognition and intervention leads to good recovery.
Resection of the tumor in the trigone of the lateral ventricle via the contralateral posterior interhemispheric transfalcine transprecuneus approach with multi-modern neurosurgery technology: a case report
Authors: Yunfei Song, Zhen Wang, Jun Zhang, Xiaopeng Cui, Zhuolin Wu, Zilin Zhao, Yan Chen, Suqin Zhang, Xiaowei Zhu, Zhitao Wang, Huijie Zhang, Chao Gao, Shuyuan Yang, Yan Zhao, Xinyu Yang
Journal: Front Surg (2024)
Keywords: contralateral, interhemispheric approach, lateral ventricle, microsurgical resection, surgical technique, trigone, tumor
MeSH terms: Case Reports

Choroid plexus papilloma (CPP) is a rare benign intracranial tumor origin that predominantly manifests in the lateral ventricle in children, accounting for 0.3%-0.6% of all primary intracranial tumors. It is extremely rare to have the CPP in the trigone of the lateral ventricle through the contralateral posterior interhemispheric transfalcine transprecuneus approach (PITTA). Herein, we report this rare case. A 7-year-old girl presented with headache. Magnetic resonance imaging of the brain showed periatrial lesions, and histopathological examination confirmed CPP (WHO grade I). The contralateral PITTA is a safe, effective, reasonable, and appropriate for some lesions in the trigone of the lateral ventricle. It provides a wider surgical angle (especially for the lateral extension) and reduces the risk of disturbance of the optic radiation compared with the conventional approaches. The use of multiple modern neurosurgical techniques, including interventional embolization, intraoperative navigation, microscope, and electrophysiological monitoring, make the procedure much easier and more accurate, and the neuroendoscope adds to the visualization of the microscope and can reduce surgical complications.