OpenMed/multicare-articles huggingface dataset:
The undiscovered syndrome: Macdonald Critchley's case of semantic dementia
Authors: Pirada Witoonpanich, Sebastian J Crutch, Jason D Warren, Martin N Rossor
Journal: Neurocase (2015)
Keywords: macdonald critchley, historical, nosology, semantic dementia
MeSH terms: Frontotemporal Dementia / history, Case Reports, Historical Article, Research Support, Non-U.S. Gov't, Female, Frontotemporal Dementia / diagnosis, Frontotemporal Dementia / physiopathology, History, 20th Century, Humans, Middle Aged

Semantic dementia is a unique clinicopathological syndrome in the frontotemporal lobar degeneration spectrum. It is characterized by progressive and relatively selective impairment of semantic memory, associated with asymmetric antero-inferior temporal lobe atrophy. Although the syndrome became widely recognized only in the 1980s, descriptions of cases with typical features of semantic dementia have been on record for over a century. Here, we draw attention to a well documented historical case of a patient with features that would have fulfilled current consensus criteria for semantic dementia, as reconstructed from the notes made by her neurologist, Macdonald Critchley, in 1938. This case raises a number of issues concerning the nosology of the semantic dementia syndrome and the potential value of archived case material.
Acute myocardial infarction caused by filgrastim: a case report
Authors: Cemil Bilir, Hüseyin Engin, Yasemin Bakkal Temi, Bilal Toka, Turgut Karabağ
Journal: Case Rep Oncol Med (2012)

Common uses of the granulocyte-colony stimulating factors in the clinical practice raise the concern about side effects of these agents. We presented a case report about an acute myocardial infarction with non-ST segment elevation during filgrastim administration. A 73-year-old man had squamous cell carcinoma of larynx with lung metastasis treated with the chemotherapy. Second day after the filgrastim, patient had a chest discomfort. An ECG was performed and showed an ST segment depression and negative T waves on inferior derivations. A coronary angiography had showed a critical lesion in right coronary arteria. This is the first study thats revealed that G-CSF can cause acute myocardial infarction in cancer patients without history of cardiac disease. Patients with chest discomfort and pain who are on treatment with G-CSF or GM-CSF must alert the physicians for acute coronary events.
Metastatic cerebellar tumor of papillary thyroid carcinoma mimicking cerebellar hemangioblastoma
Authors: Makoto Ideguchi, Takafumi Nishizaki, Norio Ikeda, Shigeki Nakano, Tomomi Okamura, Natsumi Fujii, Tokuhiro Kimura, Eiji Ikeda
Journal: Springerplus (2016)
Keywords: cerebellar hemangioblastoma, challenging diagnosis, metastatic cerebellar tumor, papillary thyroid carcinoma, solid tumor
MeSH terms: Case Reports

Introduction:
Well-differentiated papillary thyroid carcinoma generally (PTC) have a favorable prognosis. This metastasis is rare in the central nervous system. Brain metastasis has a relatively poor prognosis. We present a rare case of cerebellar metastasis, one that mimics a solid type cerebellar hemangioblastoma and because of which it was very hard to reach accurate preoperative diagnosis. Accurate diagnosis was challenging because of the similar imaging and histopathological findings for these two tumors.
Case description:
A brain lesion was detected by routine medical checkup of the brain with MRI in a 49-year-old woman 2 years after thyroidectomy for well-differentiated PTC. Gadolinium-enhanced MRI showed a homogeneous prominently enhanced lesion with surrounding enhanced dilated vessels in the left cerebellar hemisphere. Digital subtraction angiography showed a strongly stained lesion fed by the peripheral branch of the left posterior inferior cerebellar artery with drainage into the inferior vermian vein, revealing arteriovenous shunting. The most like likely preoperative diagnosis was felt to be that of a solid cerebellar hemangioblastoma. Gross total resection of the tumor was achieved by bilateral suboccipital craniotomy, and intraoperative pathological analysis suggested hemangioblastoma. Histopathological findings showed proliferation of vacuolated sheeted tumor cells with clear and eosinophilic cytoplasm and numerous thin-walled microvessels, consistent with hemangioblastoma. However, the final diagnosis was brain metastasis of the follicular variant of PTC due to a partial thyroid follicle-like pattern including eosinophilic fluid pathologically and positive TTF-1 immunostaining.
Discussion and evaluation:
Since presented rare case of cerebellar metastasis of PTC was very similar to solid type cerebellar hemangioblastoma on imaging and histopathological findings, accurate diagnosis was challenging. Moreover, it is extremely rare for a cerebellar metastasis to occur as an initial distant metastasis of PTC, and hemangioblastoma is the most common primary cerebellar neoplasm in adults. This epidemiological data was also one of the reason of difficulty to reach preoperative accurate diagnosis.
Conclusions:
To the best of our knowledge, there are no other reports of challenging diagnosis case of these two tumors in the literature. Brain metastasis of a well-differentiated PTC could be a relatively poor prognostic factor, and accurate diagnosis and suitable surgical therapy or radiotherapy are needed.
Case report: L5 tomita En bloc spondylectomy for oligometastatic liposarcoma with post adjuvant stereotactic ablative radiotherapy
Authors: Priyanshu Saha, Mohsen Raza, Angelo Fragkakis, Bisola Ajayi, Timothy Bishop, Jason Bernard, Aisha Miah, Shane H Zaidi, Mohamed Abdelhamid, Pawan Minhas, Darren F Lui
Journal: Front Surg (2023)
Keywords: carbon fibre (cf), case report, dual stage tomita en bloc spondylectomy, oncological spine surgery, sarcoma, spinal oligometastatic disease, spine surgery, stereotactic ablative radiotherapy (sabr)
MeSH terms: Case Reports

Introduction:
Tomita En-bloc spondylectomy of L5 is one of the most challenging techniques in radical oncological spine surgery. A 42-year-old female was referred with lower back pain and L5 radiculopathy with a background of right shoulder liposarcoma excision. CT-PET confirmed a solitary L5 oligometastasis. MRI showed thecal sac indentation hence wasn't suitable for Stereotactic Ablative Radiotherapy (SABR) alone. The seeding nature of sarcoma prevents the indication of separation surgery hence excisional surgery is considered for radical curative treatment. This case report demonstrates dual-staged modified TES including the utilisation of novel techniques to allow for maximum radical oncological control in the era of SABR and lesser invasive surgery.
Methods:
First-stage: Carbonfibre pedicle screws planned from L2 to S2AI-Pelvis, aligned, to her patient-specific rods. Radiofrequency ablation of L5 pedicles prior to osteotomy was performed to prevent sarcoma cell seeding. Microscope-assisted thecal sac tumour separation and L5 nerve root dissection was performed. Novel surgical navigation of the ultrasonic bone-cutter assisted inferior L4 and superior S1 endplate osteotomies. Second-stage: Vascular-assisted retroperitoneal approach at L4-S1 was undertaken protecting the great vessels. Completion of osteotomies at L4 and S1 to En-bloc L5: (L4 inferior endplate, L4/5 disc, L5 body, L5/S1 disc and S1 superior endplate). Anterior reconstruction used an expandable PEEK cage obviating the need for a third posterior stage. Reinforced with a patient-specific carbon plate L4-S1 promontory.
Results:
Patient rehabilitated well and was discharged after 42 days. Cyberknife of 30Gy in 5 fractions was delivered two months post-op. Despite left foot drop, she's walking independently 9 months post-op.
Conclusion:
These are challenging cases require a truly multi-disciplinary team approach. We share this technique for a dual stage TES and metal-free construct with post adjuvant SABR to achieve maximum local control in spinal oligometastatic disease. This case promotes our modified TES technique in the era of SABR and separation surgery in carefully selected cases.
Arterial Embolization and Methylene Blue Injection into the Aberrant Artery in Two Infants with Intralobar Sequestration
Authors: Anna Ayako Accarain, Marc Laureys, Luc Joyeux, Nasroola Damry, Henri Steyaert, Helena Reusens
Journal: European J Pediatr Surg Rep (2022)
Keywords: vats, hybrid management, intralobar sequestration
MeSH terms: Case Reports

Bronchopulmonary sequestration is a rare congenital lung dysplasia. An intralobar sequestration (ILS) is a nonfunctional mass within the lung parenchyma without bronchial communication and with aberrant systemic arterial blood supply. Surgical resection or close observation can be proposed in the management of asymptomatic and low-risk ILS, but there is a lack of consensus. Endovascular embolization before thoracoscopic resection of ILS has been described to limit perioperative bleeding. Another technique previously reported is the injection of methylene blue in the feeding artery to macroscopically mark the sequestration from the healthy lung. In that way, a nonanatomical resection can be performed instead of a lobectomy without the risk of leaving abnormal lung tissue in place. We describe the first two cases of these two techniques combined: a 3-year-old girl with an ILS in the right lower lobe with an artery originating from the abdominal aorta, and a 14-month-old girl with an ILS in the right lower lobe with an artery coming from the celiac trunk. The combination of embolization and injection of methylene blue in the aberrant artery leads to a clear macroscopic demarcation of the blue-colored ILS from the healthy lung parenchyma and allowed safe nonanatomical resection of the ILS without risk of bleeding or compromising normal lung tissue.
Schwannoma causing resorption of zygomatic arch
Authors: Ajaz A Shah, Suhail Latoo, Irshad Ahmad, Altaf H Malik, Amrit Pal Singh, Shahid Hassan
Journal: J Oral Maxillofac Pathol (2011)
Keywords: bone, erosion, mandible, neurilemmoma, schwannoma
MeSH terms: Case Reports

Schwannoma (also known as neurilemmoma, peripheral glioma and peripheral nerve sheath tumor) is a common, histologically distinctive, benign, usually encapsulated, peripheral nerve tumor of Schwann cell origin. Schwannomas can appear anywhere in the body, but are more frequently reported in the head and neck with an incidence of 25-48% in maxillofacial region. Resorption of bones due to schwannoma is rarely noticed in maxillofacial region. We hereby present a case report of schwannoma in a 35-year-old female, causing resorption of zygomatic arch along with review of literature.
Self-paced (asynchronous) BCI control of a wheelchair in virtual environments: a case study with a tetraplegic
Authors: Robert Leeb, Doron Friedman, Gernot R Müller-Putz, Reinhold Scherer, Mel Slater, Gert Pfurtscheller
Journal: Comput Intell Neurosci (2007)

The aim of the present study was to demonstrate for the first time that brain waves can be used by a tetraplegic to control movements of his wheelchair in virtual reality (VR). In this case study, the spinal cord injured (SCI) subject was able to generate bursts of beta oscillations in the electroencephalogram (EEG) by imagination of movements of his paralyzed feet. These beta oscillations were used for a self-paced (asynchronous) brain-computer interface (BCI) control based on a single bipolar EEG recording. The subject was placed inside a virtual street populated with avatars. The task was to "go" from avatar to avatar towards the end of the street, but to stop at each avatar and talk to them. In average, the participant was able to successfully perform this asynchronous experiment with a performance of 90%, single runs up to 100%.
A cutaneous metastasis of unresectable rectal adenocarcinoma: A case report and literature review
Authors: Riyadh Hakami, Mohammed N Alali, Turki Alshammari, Sulaiman AlShammari, Zyad Alyahya, Mohammed Ayesh, Khaled AlSaad, Alaa Abduljabbar
Journal: Int J Surg Case Rep (2020)
Keywords: colorectal cutaneous metastases, cutaneous cancer, cutaneous nodule, groin skin metastasis, rectal adenocarcinoma
MeSH terms: Case Reports

Background:
Colorectal cancer is ranked third among the most commonly diagnosed malignancies and fourth among the leading causes of cancer death in the world. However, only a few case reports are found in the literature regarding skin metastasis originating from rectal cancer, which usually shows widespread disease and poor prognosis. Approximately, 0.8% of the patients will have skin lesion as the first indication of a silent internal malignancy, which is rare.
Case report:
We report a complicated case of a 45-year-old male patient who referred to our highly specialized governmental hospital for diversion loop colostomy as well as biopsies of rectal and inguinal skin areas followed by palliative radiation therapy to the pelvis. Histopathological exam of rectal biopsies revealed moderately differentiated rectal adenocarcinoma, while the skin of the right inguinal area showed metastatic cutaneous rectal adenocarcinoma. Unfortunately, palliative radiation therapy was not started as the patient passed away secondary to respiratory failure which ended by cardiopulmonary arrest.
Conclusion:
A patient who is having new or evolving skin lesions with an oncology history should be well investigated as cutaneous metastasis is a strong possibility.
Ophthalmological and electrophysiological findings in monozygotic twin sisters with phosphomannomutase 2 deficiency (PMM2-CDG) over a period of 37 years
Authors: Ines Van Hees, Jaak Jaeken, Wouter Meersseman, Ingele Casteels
Journal: GMS Ophthalmol Cases (2019)
MeSH terms: Case Reports

Aims: To describe the evolution of ophthalmological and electrophysiological findings in monozygotic twin sisters with phosphomannomutase 2 deficiency (PMM2-CDG). Methods: A clinical ophthalmological examination with visual acuity measurement, fundoscopy and flash electroretinogram (fERG) was performed at the age of 4, 18 and 41 years. Results: Ophthalmic examination in both girls at the age of 4 years showed an alternating convergent squint and a saccadic pursuit, with visual acuity of 6/9 in both eyes (Ffooks symbols test). Fundoscopy revealed a normal aspect of the optic discs, narrowed blood vessels and a mild irregular pigmentation in the peripheral retina. Flash ERG in one girl showed a recognisable a, b1 and b2 wave, but with a reduction of the amplitude to less than 40% of the normal amplitude. In the other twin girl, the amplitude was more reduced, but a small b1 wave for the white flash was still noticeable. At the age of 18 years, vision had remained stable. Fundus examination revealed a pink aspect of the optic discs, with moderately narrowing of the vasculature and bone spicules in the mid peripheral retina. fERG showed obvious progression with a completely extinguished trace bilaterally. At the age of 41 years, vision had slightly diminished to 6/12 in both women. Fundoscopy and electroretinogram did not show any changes. Conclusions: Despite obvious deterioration of the fERG between the age of 4 and 18 years, the central vision showed only a minor decrease between the age of 18 and 41 years with still a good functional visual acuity.
Celiac artery thrombosis and superior mesenteric artery stenoses with essential thrombocythemia: a case report
Authors: Hasan Attila Keskin, Fahri Yetisir, Huseyin Bayram, Mehmet Selahattin Bayraktaroglu, Erdal Simsek, Mehmet Kilic, Salih Fehmi Katircioglu
Journal: Case Rep Med (2012)

Thrombosis of the celiac artery trunk is a rare cause of acute abdominal pain. Thrombosis of the celiac artery carries a high mortality and morbidity when the diagnoses and treatment are delayed. It is frequently associated with other cardiovascular events. The most common etiology is atherosclerosis. 20-30% of cases may have symptoms of chronic mesenteric ischemia. Main goal of the treatment is to reestablish the diminished or stopped mesenteric blood flow and to avoid end-organ ischemia. Essential thrombocythemia is a chronic myeloproliferative disorder characterized by marked increase in thrombocyte number and clinical presentation may be with thrombotic episodes, hemorrhage, or both. To our knowledge this is the first report of celiac artery thrombosis and superior mesenteric artery stenoses in a patient with essential thrombocythemia. The patient was managed successfully with surgical treatment.
Unusual case of lower back pain-piriformis myositis: a case report and literature review
Authors: Ahmed Elhagar, Ibrahim Kamar, Mohamed Faisal Hassan Elsheikh, Anant Mahapatra, Tarig Fadlallah Altahir Ahmed, Yogesh Acharya, Khalid Khan
Journal: Pan Afr Med J (2019)
Keywords: piriformis pyomyositis, case study, low back pain, review literature
MeSH terms: Low Back Pain / etiology, Muscle, Skeletal / pathology, Pyomyositis / diagnosis, Case Reports, Review, Adult, Anti-Bacterial Agents / therapeutic use, Humans, Magnetic Resonance Imaging, Male, Muscle, Skeletal / microbiology, Pyomyositis / complications, Pyomyositis / drug therapy, Tomography, X-Ray Computed, Anti-Bacterial Agents

We present a case of a 37-year-old male security officer with fever, severe low back pain radiating to left lower leg and diminished mobility for 1 week. His Lumbar spine X-ray was unremarkable, but his inflammatory markers including CRP, ESR and Neutrophils were high. CT scan with contrast showed rim enhancing fluid collection within the left obturator foramen with inflammatory change in the mesorectal fat. Confirmatory MRI scans depicted inflammatory change in the left piriformis muscle and a localized collection without any abnormality in the spine. Urgent CT guided aspiration was performed and the sample sent for microbiological analysis. Intravenous antibiotics commenced and continued for two weeks with complete resolution.
Retrobulbar Optic Neuritis Post Typhoid fever: Atypical Case Report
Authors: Aparajita Chaudhary, Kriti Bhatt, Shalini Verma, Apurva Bagla, Vijay Kumar Maurya
Journal: Rom J Ophthalmol (2023)
Keywords: retrobulbar optic neuritis, typhoid fever
MeSH terms: Optic Neuritis / diagnosis, Optic Neuritis / drug therapy, Optic Neuritis / etiology, Typhoid Fever / complications, Typhoid Fever / diagnosis, Typhoid Fever / drug therapy, Case Reports, Adult, Child, Humans, Male, Optic Nerve / pathology, Vision Disorders, Visual Acuity

Purpose: Post typhoid autoimmune-mediated simultaneous retrobulbar optic neuritis (RBN) involving both eyes is a rare complication requiring early diagnosis and prompt treatment. Case presentation: We present a case of bilateral RBN in a six-year-old male who came to our department with a chief complaint of sudden onset painless profound loss of vision in both eyes, after an episode of high-grade fever 2 weeks earlier. Perception of light was doubtful in right eye (RE) and vision was hand movement in left eye (LE). On ocular examination, anterior segment and fundoscopy of both eye were normal. Blood investigation was normal except for raised ESR. CT of brain and orbit was normal. MRI of brain and orbit revealed bilateral thickening and restriction of optic nerve suggestive of ON. He was initiated with intravenous methyl-prednisolone for three consecutive days after which tapering doses of oral corticosteroid was given. Results: A rapid and marked improvement in Uncorrected Visual Acuity (UCVA) was observed with UCVA improving to 6/ 12 RE and 6/ 9 LE post 1 month. The pupillary reaction also became normal in both eyes. Moreover, there was a significant reduction in the Widal titre of the patient post 2 weeks of treatment. Discussion: Paediatric ON has rare and unique characteristics, which differentiates it from adult ON. No clinical trials have been performed for paediatric ON, so current clinical practice follows the evidence drawn from the Optic Neuritis Treatment Trial (ONTT). Conclusion: Paediatric ON is uncommon. Despite having clinically severe bilateral vision loss, retrobulbar optic neuritis in children post typhoid fever has excellent response to steroid therapy if early diagnosed and treated. Abbreviations: RBN = Retrobulbar Optic Neuritis, MRI = Magnetic Resonance Imaging, CT = Computerized Tomography, UCVA = Uncorrected Visual Acuity, RE = Right eye, LE = Left eye, ON = Optic neuritis, ONTT = Optic Neuritis Treatment Trial.
Multidisciplinary management of CDH1 germinal mutation and prophylactic management hereditary lobular breast cancer: A case report
Authors: Sara Mirandola, Francesca Pellini, Eleonora Granuzzo, Maya Lorenzi, Beatrice Accordini, Maurizio Ulgelmo, Alessandra Invento, Davide Lombardi, Marina Caldana, Giovanni Paolo Pollini
Journal: Int J Surg Case Rep (2019)
Keywords: cdh1, case report, lobular breast cancer, prophylactic bilateral mastectomy

Introduction:
Germline CDH1 mutations, classically associated with hereditary diffuse gastric cancer (HDGC), also imply an increased lifetime risk of developing lobular breast cancer (LBC) in a highly penetrant autosomal dominant manner.
Presentation of case:
We report a 44-year-old woman CDH1 mutation carrier with a strong family history of cancer, who previously had prophylactic total gastrectomy. We registered normal findings at the breast and axilla assessment. Mammography, ultrasonography and breast MRI scans were negative for cancer. In our Institute a bilateral prophylactic mastectomy followed by breast reconstruction was performed. Foci of atypical lobular hyperplasia(ALH) and lobular carcinoma in situ (LCIS) were histologically shown.
Discussion:
The current consensus guidelines for women with pathogenic CDH1 mutations recommend annual mammography, ultrasound, breast MRI scans and clinical breast examination starting at the age of 35. Due to the well-documented aggressive behavior of this particular type of cancer, bilateral mastectomy and reconstruction would be more beneficial for this kind of high-risk patients.
Conclusion:
Conflicting evidences and lacking data about the benefits in terms of overall survival, disease-free survival and the long-term outcomes related to prophylactic bilateral mastectomy for CDH1 mutation carriers restrict the instruction for this type of procedure to selected cases, which should always be managed by a multidisciplinary team.
Gastrointestinal Autonomic Nerve Tumor of the Colon: A Rare Cause of Persistent Abdominal Pain in a Child
Authors: Riccardo Guanà, Elisabetta Teruzzi, Salvatore Garofalo, Isabella Morra, Riccardo Lemini, Jürgen Schleef
Journal: APSP J Case Rep (2016)
Keywords: child, gastrointestinal autonomic nerve tumor, gastrointestinal stromal tumor, pain abdomen
MeSH terms: Case Reports

Gastrointestinal autonomic nerve tumor (GANT) is extremely rare and considered a variant of gastrointestinal stromal tumors (GISTs). GANT originates from the intestinal autonomic nervous system mostly of small intestine or the stomach. We report a colonic GANT diagnosed in a 5-year-old child who presented with abdominal pain and fever for a long period. Colonic resection and end to end anastomosis proved curative without the need of chemo-radiotherapy. Given the rarity of the tumor, the patient is on our long term follow-up.
Laparoscopic retrieval of a peritoneal mouse
Authors: Dara O Kavanagh, Diarmaid Moran, Robert Flynn, Paul C Neary
Journal: Case Rep Med (2010)
MeSH terms: Case Reports

A 67-year-old Caucasian male was referred by the urology service with a history of incomplete bowel emptying. He complained of tenesmus. MRI scan suggested a leiomyoma lying anterior to the rectum. He underwent examination under anaesthesia and attempted endorectal ultrasound and biopsy. However, the lesion seemed to migrate cranially and was impalpable. At laparoscopy, a mobile, unattached, 5.5 × 5 × 3.5, cream-coloured 'egg was retrieved from the retrovesical space. Histology confirmed a hyalinised fibrocollagenous lesion lined with mesothelium. A comprehensive review of the literature is presented.