OpenMed/multicare-articles huggingface dataset:
Experiential seizures related to the hippocampal-parahippocampal spatial representation system
Authors: Eline Revdal, Vibeke Arntsen, Thanh Pierre Doan, Marte Kvello-Alme, Kjell Arne Kvistad, Geir Bråthen, Eylert Brodtkorb
Journal: Epilepsy Behav Rep (2020)
Keywords: experiential seizures, focal aware seizures, hippocampal-parahippocampal system, parahippocampal cortex, visual hallucinations
MeSH terms: Case Reports

Ictal visual hallucinations may have occipital as well as temporal lobe origin. We report a patient with clustering of focal aware seizures with visual hallucinations. Ictal EEG findings and seizure semiology with alternating contralateral elementary visual phenomena and non-lateralizing experiential hallucinations (visual scenes, memory flashbacks, spatial distortion) corresponded to a lesion in the posterior part of the right parahippocampal gyrus. This area is part of the hippocampal-parahippocampal system for mapping allocentric space. Within this system, the parahippocampal cortex encodes information about visual environmental scenes in concert with functionally defined neurons relevant for episodic memory and spatial cognitive processes (place, grid, border and head direction cells, as well as neurons tracking the passage of time). These functions are tightly linked to visual exploration. We suggest that the hippocampal-parahippocampal spatial navigation system is a crucial part of the networks responsible for the semiology of experiential seizures with complex visual hallucinations and elements of recall.
Papillary thyroid carcinoma with nodular fasciitis-like stroma: a rare variant
Authors: Mayur Parkhi, Debajyoti Chatterjee, Anand Bardia, Anwin Joseph Kanaval, Ashwani Sood, Uma Nahar Saikia, Naval Bansal
Journal: Autops Case Rep (2021)
Keywords: beta catenin, fasciitis, myofibroblasts, proto-oncogene proteins b-raf, thyroid cancer, papillary
MeSH terms: Case Reports

Papillary thyroid carcinoma with desmoid-type fibromatosis (PTC-DTF) or nodular fasciitis-like stroma (PTC-NFS) is a rare morphological variant of PTC with a favorable prognosis. There is a paucity of molecular data regarding this entity. We present the case of a 20-year-old female who presented with a palpable mass over the anterior aspect of the neck for the past 3-4 months, which was diagnosed as PTC-NFS. Ultrasonogram of the neck revealed a bulky left lobe of thyroid that contained a well-defined heterogenous lesion measuring around 24 × 26 × 36 mm with involvement of the adjacent isthmus. She underwent a total thyroidectomy with central compartment lymph node dissection. Histological examination revealed a biphasic tumor with epithelial and stromal components resembling nodular fasciitis. Two dissected lymph nodes showed metastasis of the epithelial component only. On immunohistochemistry, BRAF mutant protein expression was evident in the epithelial component only, while β-catenin was negative in both the components. The histopathological diagnosis of papillary thyroid carcinoma with nodular fasciitis-like stroma was offered. Sanger sequencing revealed a BRAFV600E (c.1799T>A, Val600Glu) mutation. Post-operatively, no residual tumor was detected on ultrasound and radioiodine scans. The patient was doing well at follow-up of 9 months. PTC-NFS/DTF is a histological variant of PTC with a favorable prognosis. Our index case was associated with the BRAF mutation, which was restricted to the epithelial component. Thorough sampling of the excised specimen is essential in order not to miss the epithelial component, which, in most reported cases (including ours) appears to be small.
Case Report: Cerebral Revascularization in a Child With Mucopolysaccharidosis Type I
Authors: Nathan Grant, J Michael Taylor, Zach Plummer, Kasiani Myers, Thomas Burrow, Lori Luchtman-Jones, Anna Byars, Adrienne Hammill, Katie Wusick, Edward Smith, James Leach, Sudhakar Vadivelu
Journal: Front Pediatr (2021)
Keywords: cerebral arteriopathy, cerebral revascularization, mucopolysaccharidosis i, pial synangiosis, stroke, ventriculomegaly
MeSH terms: Case Reports

Mucopolysaccharidosis (MPS) type I is a rare lysosomal storage disorder caused by an accumulation of glycosaminoglycans (GAGs) resulting in multisystem disease. Neurological morbidity includes hydrocephalus, spinal cord compression, and cognitive decline. While many neurological symptoms have been described, stroke is not a widely-recognized manifestation of MPS I. Accordingly, patients with MPS I are not routinely evaluated for stroke, and there are no guidelines for managing stroke in patients with this disease. We report the case of a child diagnosed with MPS I who presented with overt stroke and repeated neurological symptoms with imaging findings for severe ventriculomegaly, infarction, and bilateral terminal carotid artery stenosis. Direct intracranial pressure evaluation proved negative for hydrocephalus. The patient was subsequently treated with cerebral revascularization and at a 3-year follow-up, the patient reported no further neurological events or new ischemia on cerebral imaging. Cerebral arteriopathy in patients with MPS I may be associated with GAG accumulation within the cerebrovascular system and may predispose patients to recurrent strokes. However, further studies are required to elucidate the etiology of cerebrovascular arteriopathy in the setting of MPS I. Although the natural history of steno-occlusive arteriopathy in patients with MPS I remains unclear, our findings suggest that cerebral revascularization is a safe treatment option that may mitigate the risk of future strokes and should be strongly considered within the overall management guidelines for patients with MPS I.
Surgical Approach to the Cavernous Sinus for a Trigeminal Schwannoma Resection: Technical Note and Case Report
Authors: Riccardo Caruso, Alessandro Pesce, Venceslao Wierzbicki, Luigi Marrocco, Emanuele Piccione
Journal: Case Rep Surg (2016)

We report a rare case of schwannoma of the lateral wall of the cavernous sinus, an exceedingly rare lesion affecting this anatomical district, and discuss salient aspects of the surgical approach to the cavernous sinus, which are traditionally considered technically challenging due to the high risk of postoperative morbidity and mortality related to the presence of the cranial nerves and internal carotid artery.
The Liver That Cured Christmas: Case Report of Orthotopic Liver Transplant in a Patient with Hemophilia B
Authors: Jesse E Harris, Jonathan L Balk, Constance M Mobley, Kirk Heyne
Journal: Case Rep Transplant (2020)
MeSH terms: Case Reports

Herein, we discuss a case of a 39-year-old male with hemophilia B, who developed end-stage liver disease secondary to nonalcoholic steatohepatitis, that underwent orthotopic liver transplantation (OLT) as a curative means for his liver disease and coagulation disorder. Existing case reports have demonstrated favorable outcomes in patients outside of the United States who received continuous infusions of recombinant factor IX replacement in the perioperative setting after liver transplant. Given limitations in the stability of the recombinant factor IX products in the United States, a dosing strategy was comprised of once daily bolus dosing to achieve satisfactory factor IX levels. Within 48 hours of initial surgery, the patient had sustained factor IX levels above 70% of normal and the patient required no further dosing of factor IX products to date. This strategy helped facilitate less frequent dosing as well as achieved targeted factor levels while synthetic function of the transplanted liver recovered.
The impact of 1.5-T intraoperative magnetic resonance imaging in pediatric tumor surgery: Safety, utility, and challenges
Authors: Victoria Becerra, José Hinojosa, Santiago Candela, Diego Culebras, Mariana Alamar, Georgina Armero, Gastón Echaniz, David Artés, Josep Munuera, Jordi Muchart
Journal: Front Oncol (2023)
Keywords: intraoperative magnetic resonance imaging, neurooncological surgery, oncology, pediatric brain tumors, residual tumor

Objective:
In this study, we present our experience with 1.5-T high-field intraoperative magnetic resonance imaging (ioMRI) for different neuro-oncological procedures in a pediatric population, and we discuss the safety, utility, and challenges of this intraoperative imaging technology.
Methods:
A pediatric consecutive-case series of neuro-oncological surgeries performed between February 2020 and May 2022 was analyzed from a prospective ioMRI registry. Patients were divided into four groups according to the surgical procedure: intracranial tumors (group 1), intraspinal tumors (group 2), stereotactic biopsy for unresectable tumors (group 3), and catheter placement for cystic tumors (group 4). The goal of surgery, the volume of residual tumor, preoperative and discharge neurological status, and postoperative complications related to ioMRI were evaluated.
Results:
A total of 146 procedures with ioMRI were performed during this period. Of these, 62 were oncology surgeries: 45 in group 1, two in group 2, 10 in group 3, and five in group 4. The mean age of our patients was 8.91 years, with the youngest being 12 months. ioMRI identified residual tumors and prompted further resection in 14% of the cases. The mean time for intraoperative image processing was 54 ± 6 min. There were no intra- or postoperative security incidents related to the use of ioMRI. The reoperation rate in the early postoperative period was 0%.
Conclusion:
ioMRI in pediatric neuro-oncology surgery is a safe and reliable tool. Its routine use maximized the extent of tumor resection and did not result in increased neurological deficits or complications in our series. The main limitations included the need for strict safety protocols in a highly complex surgical environment as well as the inherent limitations on certain patient positions with available MR-compatible headrests.
Case Report: A Case of Severe Clinical Deterioration in a Patient With Multiple Sclerosis
Authors: Katharina Breitkopf, Aykut Aytulun, Moritz Förster, Bastian Kraus, Bernd Turowski, Doreen Huppert, Norbert Goebels, Harald Hefter, Orhan Aktas, Imke Metz, Wolfgang Brück, Guido Reifenberger, Hans-Peter Hartung, Philipp Albrecht
Journal: Front Neurol (2020)
Keywords: demyelinating disease, immunocompromised multiple sclerosis patient, multiple sclerosis, multiple sclerosis rebound, progressive brain lesions, tumefactive multiple sclerosis

Tumefactive multiple sclerosis (MS) is a rare variant of MS that may lead to a rapidly progressive clinical deterioration requiring a multidisciplinary diagnostic workup. Our report describes the diagnostic and therapeutic approach of a rare and extremely severe course of MS. A 51-year-old man with an 8-year history of relapsing-remitting MS (RRMS) was admitted with a subacute progressive left lower limb weakness and deterioration of walking ability. After extensive investigations including repeated MRI, microbiological, serological, cerebrospinal fluid (CSF) studies, and finally brain biopsy, the diagnosis of a tumefactive MS lesion was confirmed. Despite repeated intravenous (IV) steroids as well as plasma exchanges and IV foscarnet and ganciclovir owing to low copy numbers of human herpesvirus 6 (HHV-6) DNA in polymerase chain reaction (PCR) analysis, the patient did not recover. The clinical presentation of tumefactive MS is rare and variable. Brain biopsy for histopathological workup should be considered in immunocompromised patients with rapidly progressive clinical deterioration with brain lesions of uncertain cause.
Bilateral dacryoadenitis in adult-onset Still's disease: A case report
Authors: Qing Huang, Weimin He
Journal: Open Life Sci (2022)
Keywords: adult-onset still’s disease, dacryoadenitis, lacrimal gland
MeSH terms: Case Reports

We present an unusual case of bilateral dacryoadenitis in a middle-aged patient with adult-onset Still's disease (AOSD). We reviewed relevant clinical studies addressing the association between lacrimal lesions and AOSD. A 50-year-old Chinese woman with a 4 year history of recurrent fever and rashes was admitted to the hospital. She had also developed nodules on both eyelids 10 months before admission. After undergoing resection of the left lacrimal gland, the patient received steroids and immunosuppressive therapy. The patient showed good postoperative recovery during the 20 month follow-up. In this case, the pathological examination conducted after orbital surgery helped clinicians differentiate between dacryoadenitis and other orbital lesions. In a review of the literature, dacryoadenitis occurred after the onset of AOSD, and all cases showed non-granulomatous chronic inflammation by histopathology, which indicated that the lacrimal gland may be an inflammatory target and is affected by systemic inflammation in AOSD.
Primary pericardial angiosarcoma: case report and review of treatment options
Authors: Udit Yadav, Ankit Mangla
Journal: Ecancermedicalscience (2020)
Keywords: angiosarcoma, cardiac tumour
MeSH terms: Case Reports

A primary cardiac angiosarcoma is a rare type of soft-tissue sarcoma with a high mortality rate. This report describes a young woman who presented with chest pain and worsening shortness of breath over the course of a year. She was diagnosed with and treated for latent tuberculosis and autoimmune pericarditis over the last year, however, her condition kept worsening. Further workup revealed a large pericardial and right atrial mass associated with multiple lung nodules. The biopsy from the lung mass showed angiosarcoma, and she was diagnosed with primary metastatic angiosarcoma of the pericardium. She was treated with doxorubicin and Ifosfamide (AIM-75 regimen), which led to a partial response. However, soon after completion of six cycles, the tumour progressed rapidly, leading to cardio-respiratory failure. In this report, we will discuss the clinical challenges and treatment options (surgical and medical) that are available for treating patients with angiosarcoma of the heart.
Pedicle anterolateral thigh flap reconstruction after pelvic tumor resection: a case report
Authors: Robert M Whitfield, David King, Peter Rossi, Michael Loffredo
Journal: Plast Surg Int (2010)
MeSH terms: Case Reports

A 47-year-old female with a locally advanced urologic malignancy previously managed with resection, diversion, and postoperative radiation therapy presented for management of her recurrent cancer that had eroded through the soft tissues of the left inner thigh and vulva. On all staging studies the tumor involved the left common femoral artery, and vein, both above and below the inguinal ligament. The difficulty with such tumors is the availability of tissue to reconstruct the defect. The patient had a history of deep venous thrombosis in the femoral venous system. A local flap was the most logical type of reconstruction. The patient had a right lower quadrant ureterostomy with a large parastomal hernia which further limited the local flap options. An anterolateral thigh flap from the opposite thigh was used to reconstruct the soft tissue deficit in this patient. This resurfaced the defect and provided coverage for the vascular reconstruction.
Autoimmune Limbic Encephalitis in a Patient with Acute Encephalopathy and Hyponatremia
Authors: K A Agarwal, M Albertolle, M Tiru
Journal: Case Rep Med (2019)
MeSH terms: Case Reports

Acute encephalopathy is a common clinical presentation for hospital admissions. Autoimmune encephalitis is a rare cause of encephalopathy which has increasingly been recognized over the last decade. The detection of various neuronal antibodies has helped diagnose these syndromes, but they have limited availability, mostly in the developed countries. We present a case of a middle-aged female presenting with memory impairment, gait disturbances, and hyponatremia. A clinical diagnosis of autoimmune limbic encephalitis was made based on faciobrachial dystonic seizures, SIADH, and MRI changes 10 days prior to autoantibody titer returned. Prompt treatment with steroids and intravenous immunoglobulin was started with improvement in her neurological symptoms. This case highlights the importance of considering autoimmune encephalitis syndromes in the differential diagnosis of patients with classical neurological presentations and prompt diagnosis and immunotherapy to improve neurological outcomes.
Endoscopic Drainage of Giant Pancreatic Pseudocysts Using Both Lumen-Apposing Metal Stent and Plastic Stent: A Report of Two Cases and Review of the Current Literature
Authors: Hussam I A Alzeerelhouseini, Muawiyah Elqadi, Mohammad N Elqadi, Sadi A Abukhalaf, Hazem A Ashhab
Journal: Case Rep Gastrointest Med (2021)
MeSH terms: Case Reports

Introduction:
A pancreatic pseudocyst (PP) with major diameter equal to 10 cm or more is called a giant pseudocyst. The ideal management for giant PPs is controversial. Endoscopic drainage is an alternative nonsurgical approach for PP management. Only a few cases of giant PPs were reported to be managed by endoscopic drainage. Case Presentation. We reported two cases of giant PPs following an episode of acute pancreatitis. Both were resolved following endoscopic cystogastrostomy using metallic and double-pigtail stents with excellent outcomes. There was no history of recurrence or complications on follow-up. In addition, we extensively reviewed all available literature studies of giant pancreatic pseudocyst presentation, management, and complications. We summarized all reported cases and presented them in a comprehensive table.
Conclusion:
The endoscopic cystogastrostomy approach is cost saving, can avoid surgical complications, and offers an early hospital discharge.
Endodontic Treatment of Bilateral Maxillary First Premolars with Three Roots Using CBCT: A Case Report
Authors: Sujatha Gopal, Gijo John, K Pavan Kumar, Swarna Latha, Suma Latha, Sowmya Kallepalli
Journal: Case Rep Dent (2014)

One of the determining factors for the success of endodontic therapy is understanding the morphological anatomy of the tooth structure and its variants in relation to its template anatomy. The internal anatomy of maxillary first premolars is particularly complex due to their variation in number of roots and canal configuration. However, the bilateral presence of three roots in a maxillary first premolar is of rare occurrence. This case report describes the unusual anatomy bilaterally detected in maxillary first premolars using Cone-Beam Computed Tomography.
Arthroscopic Osteosynthesis for the Treatment of Coronoid Process Fractures: A Case Series
Authors: Noriaki Shimada, Katsuhiko ShirakiIt, Kazuo Saita
Journal: Case Rep Orthop (2018)
MeSH terms: Case Reports

The treatment strategy and surgical indication for coronoid process fractures are not clear. Many methods of surgery have been described. We report on the use of arthroscopic osteosynthesis for this type of fracture. This method is minimally invasive and effective for confirming the reduction, and it is advantageous for postoperative pain and early recovery after surgery.
Pancreatic neuroendocrine tumor, lymphoma, and squamous cell carcinoma of hypopharynx; A case report of three primary cancers in one patient
Authors: Vorapatu Tangsirapat, Kitti Wongta, Kobkool Chakrapan Na Ayudhya, Vichack Chakrapan Na Ayudhya, Paiboon Sookpotarom
Journal: Int J Surg Case Rep (2019)
Keywords: case report, hypopharynx, lymphoma, neuroendocrine tumor, pancreas, squamous cell carcinoma

Background:
Pancreatic neuroendocrine tumors (PNETs) are rarely found and the majority of PNETs are nonfunctioning. At present, a patient with pancreatic neuroendocrine tumor (PNET), lymphoma, and squamous cell carcinoma (SCC) occurring simultaneously has not been reported. We presented all these three primary cancers in a same patient.
Case presentation:
A 51-year-old male presented with a right testicular mass for four months. He underwent right orchidectomy, and the patient was diagnosed malignant diffuse large B-cell lymphoma. Consequently, CHOP regimen and intrathecal methotrexate were given as treatment. During the metastasis work up, a hypodense lesion at the tail of pancreas was found. Distal pancreatectomy and splenectomy were completed, and the pathological report revealed PNET. Eighteen months later, the patient developed a symptom of odynophagia, direct laryngoscopy was performed and a mass at the left arytenoid was found. The biopsy was done and confirmed the diagnosis of SCC. The patient went through concurrent chemo-radiation with a complete response. Currently the patient remains in a complete remission of all three cancers.
Discussion:
As the patient had non-functioning PNET and was symptom-free, the diagnosis would have not been discovered without the metastasis workup of lymphoma. Although non-functioning PNET less than 2 cm can be safely observed, the tumor was 4.3 × 1.3 cm and adenocarcinoma was suspected, so the patient underwent the surgery.
Conclusion:
Although many cases with multiple primary cancers have been published, a case of patient with PNET, lymphoma, and SCC occurring as primary cancers has not been yet reported.
Case Report: Exome sequencing reveals recurrent RETSAT mutations and a loss-of-function POLDIP2 mutation in a rare undifferentiated tongue sarcoma
Authors: Jason Y K Chan, Peony Hiu Yan Poon, Yong Zhang, Cherrie W K Ng, Wen Ying Piao, Meng Ma, Kevin Y Yip, Amy B W Chan, Vivian Wai Yan Lui
Journal: F1000Res (2018)
Keywords: head and neck, poldip2 and retsat mutations, sarcoma of the tongue, low mutational burden

Soft tissue sarcoma of the tongue represents a very rare head and neck cancer with connective tissue features, and the genetics underlying this rare cancer are largely unknown. There are less than 20 cases reported in the literature thus far. Here, we reported the first whole-exome characterization (>×200 depth) of an undifferentiated sarcoma of the tongue in a 31-year-old male. Even with a very good sequencing depth, only 19 nonsynonymous mutations were found, indicating a relatively low mutation rate of this rare cancer (lower than that of human papillomavirus (HPV)-positive head and neck cancer). Yet, among the few genes that are somatically mutated in this HPV-negative undifferentiated tongue sarcoma, a noticeable deleterious frameshift mutation (with a very high allele frequency of >93%) of a gene for DNA replication and repair, namely POLDIP2 (DNA polymerase delta interacting protein 2), and two recurrent mutations of the adipogenesis and adipocyte differentiation gene RETSAT (retinol saturase), were identified. Thus, somatic events likely affecting adipogenesis and differentiation, as well as potential stem mutations to POLDIP2, may be implicated in the formation of this rare cancer. This identified somatic whole-exome sequencing profile appears to be distinct from that of other reported adult sarcomas from The Cancer Genome Atlas, suggesting a potential unique genetic profile for this rare sarcoma of the tongue. Interestingly, this low somatic mutation rate is unexpectedly found to be accompanied by multiple tumor protein p53 and NOTCH1 germline mutations of the patient's blood DNA. This may explain the very early age of onset of head and neck cancer, with likely hereditary predisposition. Our findings are, to our knowledge, the first to reveal a unique genetic profile of this very rare undifferentiated sarcoma of the tongue.