scholarly journals Paraneoplastic encephalomyeloradiculits with multiple autoantibodies against ITPR-1, GFAP and MOG: case report and literature review

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Anna Cirkel ◽  
Klaus-Peter Wandinger ◽  
Claudia Ditz ◽  
Jan Leppert ◽  
Lars Hanker ◽  
...  
Keyword(s):  
Literature Review ◽  
Ovarian Teratoma ◽  
Caucasian Woman ◽  
Surgical Removal ◽  
Extensive Literature ◽  
Borderline Tumor ◽  
Case Presentation ◽  
Small Series ◽  
Neuronal Autoantibodies ◽  
Mog Antibodies

Abstract Background Recently, antibodies against the alpha isoform of the glial-fibrillary-acidic-protein (GFAPα) were identified in a small series of patients with encephalomyelitis. Coexisting autoantibodies (NMDA receptor, GAD65 antibodies) have been described in a few of these patients. We describe a patient with rapidly progressive encephalomyeloradiculitis and a combination of anti-ITPR1, anti-GFAP and anti-MOG antibodies. Case presentation and literature review A 44-year old caucasian woman with a flu-like prodrome presented with meningism, progressive cerebellar signs and autonomic symptoms, areflexia, quadriplegia and respiratory insufficiency. MRI showed diffuse bilateral T2w-hyperintense brain lesions in the cortex, white matter, the corpus callosum as well as a longitudinal lesion of the medulla oblongata and the entire spinal cord. Anti-ITPR1, anti-GFAP and anti-MOG antibodies were detected in cerebrospinal fluid along with lymphocytic pleocytosis. Borderline tumor of the ovary was diagnosed. Thus, the disease of the patient was deemed to be paraneoplastic. The patient was treated by surgical removal of tumor, steroids, immunoglobulins, plasma exchange and rituximab. Four months after presentation, the patient was still tetraplegic, reacted with mimic expressions to pain or touch and could phonate solitary vowels. An extensive literature research was performed. Conclusion Our case and the literature review illustrate that multiple glial and neuronal autoantibodies can co-occur, that points to a paraneoplastic etiology, above all ovarian teratoma or thymoma. Clinical manifestation can be a mixture of typically associated syndromes, e.g. ataxia associated with anti-ITPR1 antibodies, encephalomyelitis with anti-GFAPα antibodies and longitudinal extensive myelitis with anti-MOG antibodies.

scholarly journals Synovial sarcoma of the hand-wrist: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Serenella Serinelli ◽  
Lorenzo Gitto ◽  
Daniel J. Zaccarini
Keyword(s):  
Literature Review ◽  
Synovial Sarcoma ◽  
Malignant Tumors ◽  
Surgical Excision ◽  
Caucasian Woman ◽  
Review Of The Literature ◽  
Case Presentation ◽  
Synovial Sarcomas ◽  
Uncommon Case

Abstract Background Synovial sarcomas are infrequent malignant tumors occurring mostly in adolescents and young adults. The occurrence of synovial sarcoma in the hand-wrist area is rare (4 to 8.5% of all synovial sarcomas in different studies). Case presentation This report documents an uncommon case of synovial sarcoma occurring in the hand-wrist of a 69-year-old Caucasian woman. She was subsequently treated with surgical excision and radiotherapy without recurrence after follow up. Conclusions This paper aims to characterize the demographic, pathologic, and clinical features with a literature review. The present literature review confirms that hand-wrist synovial sarcomas are more frequent among males and subjects 10 to 40 years old. Most cases in this location are usually not larger than 5 cm in size. The five-year survival rate is higher than that reported in a previous review on hand synovial sarcomas, and this suggests an improved survival in recent decades.

scholarly journals Case report: meningitis as a presenting feature of anti-NMDA receptor encephalitis

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Maria Stavrou ◽  
Jing Ming Yeo ◽  
Alexander David Slater ◽  
Oliver Koch ◽  
Sarosh Irani ◽  
...  
Keyword(s):  
Case Report ◽  
Nmda Receptor ◽  
Rapid Development ◽  
Clonic Seizure ◽  
Ovarian Teratoma ◽  
Caucasian Woman ◽  
Case Presentation ◽  
Nmda Receptor Encephalitis ◽  
Autonomic Instability ◽  
Lymphocytic Meningitis

Abstract Background Meningitis is a very rare atypical presenting feature of anti-NMDA receptor encephalitis. In our case report, we describe an unusual clinical presentation of anti-NMDA receptor encephalitis with a biphasic pattern of meningitis followed by encephalitis and discuss potential mechanisms underlying this presentation. We aim to widen the differential diagnosis to be considered in a patient presenting with clinical meningitis and pyrexia. Case presentation This is a case of a 33-year old Caucasian woman who initially presented with a lymphocytic meningitis attributed to a viral infection. She subsequently developed fluctuating consciousness, agitation, visual hallucinations, dyskinetic movements, a generalized tonic-clonic seizure, and autonomic instability. Investigations revealed a diagnosis of anti-NMDA receptor encephalitis secondary to a previously unidentified ovarian teratoma. She made an excellent recovery with immunotherapy and removal of the teratoma. Conclusion Clinicians should consider autoimmune encephalitides in individuals with meningitis, particularly where extensive investigations fail to identify a causative pathogen and there is rapid development of an encephalitic phenotype.

scholarly journals Successful pregnancy after operation in an infertile woman caused by luteinizing hormone-secreting pituitary adenoma: case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yi Zhang ◽  
Cheng Chen ◽  
Min Lin ◽  
Kan Deng ◽  
Huijuan Zhu ◽  
...  
Keyword(s):  
Case Report ◽  
Pituitary Adenoma ◽  
Literature Review ◽  
Pituitary Adenomas ◽  
Sella Turcica ◽  
Infertile Woman ◽  
Mass Treatment ◽  
Successful Pregnancy ◽  
Case Presentation ◽  
Transsphenoidal Resection

Abstract Background Functional gonadotroph adenomas (FGAs) are rare adenomas that most commonly secrete FSH. However, solitary LH-secreting pituitary adenomas are unusual. Case presentation A 30-year-old woman with elevated LH and normal FSH presented with inability to conceive. An MRI revealed an enlarged sella turcica and an intrasellar mass. Treatment with transsphenoidal resection led to normalization of LH and estradiol, as well as successful pregnancy. And we reviewed 6 cases of LH-secreting pituitary adenomas from 1981 to 2020. Conclusions Our case is unique because of the LH-secreting pituitary adenoma without FSH hypersecretion. This case indicates that pituitary adenoma should be considered when other diseases causing infertility have been excluded.

scholarly journals Cerebral fat embolization with paroxysmal sympathetic hyperactivity syndrome and septic shock at high altitude: a case report and literature review

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Min Li ◽  
Gang Zhu ◽  
Hao Guo ◽  
Shun Nan Ge ◽  
Guo Dong Gao ◽  
...  
Keyword(s):  
Septic Shock ◽  
Differential Diagnosis ◽  
Case Report ◽  
Literature Review ◽  
High Altitude ◽  
Fat Embolism ◽  
Neurological Rehabilitation ◽  
Case Presentation ◽  
Sympathetic Hyperactivity ◽  
Paroxysmal Sympathetic Hyperactivity

AbstractBackgroundCerebral fat embolism (CFE) syndrome at high altitude was rare complicated with paroxysmal sympathetic hyperactivity (PSH) syndrome and septic shock. It is a challenge to differential diagnosis and treatment at high altitude.Case presentationThis case presents a CFE with PSH and septic shock of a 23-year-old man occurred at high altitude of 3800 m above sea level, transferred by airplane successfully and cured in the department of neurosurgery, Xi’an Tangdu Hospital.ConclusionsIt is key that CFE with PSH can be rapid diagnosed and treatment bundles of septic shock should be initiated as soon as possible. Early neurological rehabilitation played an important role for good outcome.

scholarly journals Unprovoked internal jugular vein thrombosis: a case report and literature review

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Loïc Payrard ◽  
Léa Iten ◽  
Jacques Donzé ◽  
Gregor John
Keyword(s):  
Literature Review ◽  
Internal Jugular Vein ◽  
Lower Limb ◽  
Jugular Vein ◽  
Scientific Data ◽  
Jugular Vein Thrombosis ◽  
Case Presentation ◽  
Vein Thrombosis ◽  
Arm Pain ◽  
Internal Jugular Vein Thrombosis

Abstract Background Managing thrombosis in rare sites is challenging. Existing studies and guidelines provide detailed explanations on how to overcome lower-limb thromboses and pulmonary embolisms, but few studies have examined thrombosis in rare sites. Lack of data makes clinical practice heterogeneous. Recommendations for diagnosing, treating, and following-up internal jugular vein thrombosis are not clearly defined and mostly based on adapted guidelines for lower-limb thrombosis. Case presentation A 52-year-old Caucasian woman came to the Emergency Department with chest, neck, and left arm pain. Computed tomography imagery showed a left internal jugular vein thrombosis. An extensive workup revealed a heterozygous factor V Leiden gene. Therapy was initiated with intravenous unfractionated heparin, then switched to oral acenocoumarol, which resolved the symptoms. Based on this case presentation and a literature review, we summarize the causes, treatment options, and prognosis of unprovoked internal jugular vein thrombosis. Conclusions Managing internal jugular vein thrombosis lacks scientific data from large randomized clinical trials, partly because such thromboses are rare. Our literature review suggested that clinical treatments for internal jugular vein thrombosis often followed recommendations for treating lower-limb thrombosis. Future specific studies are required to guide clinicians on the modalities of diagnosis, screening for thrombophilia or oncologic disease, treatment duration, and follow-up.

Fetal pulmonary choristoma: report of first case and literature review

2020 ◽  
Vol 9 (1) ◽  
Author(s):  
Anupam Nanda ◽  
Rajinder Nanda ◽  
Seema Thakur ◽  
Tej Prakash Gupta ◽  
Sudhir Jain ◽  
...  
Keyword(s):  
Literature Review ◽  
Exome Sequencing ◽  
Posterior Fossa ◽  
Spinal Column ◽  
Fetal Autopsy ◽  
Case Presentation ◽  
Antenatal Ultrasound ◽  
Clinical Exome Sequencing ◽  
First Case ◽  
Pathologic Findings

AbstractObjectivesLung tissue choristoma is a very rare disorder where mature lung tissues develop in the site not normal to the lung.Case presentationWe hereby report a first case of fetal pulmonary choristoma in a 23–24 weeks fetus where antenatal ultrasound showed a mass in posterior fossa with severe ventriculomegaly. The mass extended inferiorly in cervical spinal column and thereafter extended in the skin over the back of fetus. Fetal autopsy confirmed these findings. Pathologic findings showed mature lung tissues with bronchi, bronchioles, and alveoli. Clinical exome sequencing showed normal results.ConclusionsWe describe the antenatal ultrasound, fetal autopsy and pathologic findings of an intracranial and cutaneous pulmonary choristoma.

scholarly journals Small intestine duplication cyst with recurrent hematochezia: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhicheng Zhang ◽  
Xiaowei Huang ◽  
Qian Chen ◽  
Demin Li ◽  
Qi Zhou ◽  
...  
Keyword(s):  
Small Intestine ◽  
Case Report ◽  
Literature Review ◽  
Ct Scan ◽  
Duplication Cyst ◽  
Abdominal Ct ◽  
Case Presentation ◽  
Abdominal Ct Scan ◽  
Capsule Endoscopes

Abstract Background Small intestine duplication cysts (SIDCs) are rare congenital anatomical abnormalities of the digestive tract and a rare cause of hematochezia. Case presentation We describe an adult female presented with recurrent hematochezia. The routine gastric endoscope and colonic endoscope showed no positive findings. Abdominal CT scan indicated intussusception due to the "doughnut" sign, but the patient had no typical symptoms. Two subsequent capsule endoscopes revealed a protruding lesion with bleeding in the distal ileum. Surgical resection was performed and revealed a case of SIDC measuring 6 * 2 cm located inside the ileum cavity. The patient remained symptom-free throughout a 7-year follow-up period. Conclusion SIDCs located inside the enteric cavity can easily be misdiagnosed as intussusception by routine radiologic examinations.

scholarly journals The concept of “Four Walls, Two Poles” in the lesions of the thalamus and ganglion regions: case report and literature review

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Haiyang Yang ◽  
Gang Bai ◽  
Yongli Zhang ◽  
Guolong Chen ◽  
Lei Duan ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Literature Review ◽  
Surgical Resection ◽  
Three Dimensional ◽  
Surgical Techniques ◽  
Surgical Approaches ◽  
Surgical Results ◽  
Case Presentation ◽  
Operation Process ◽  
Thalamic Glioma

Abstract Background There are few articles about the surgical techniques of thalamic glioma and the lesions in the basal ganglia area. According to three existing cases and the literature review (Twelve articles were summarized which mainly described the surgical techniques), we discuss the surgical characteristics of lesions of the thalamus and basal ganglia area and summarize the relevant surgical skills. Case presentation Of the three cases, two were thalamic gliomas and one was brain abscess in basal ganglia. According to the three-dimensional concept of the “Four Walls, Two Poles”, lesions of the thalamus and basal ganglia were surgically removed, and the operative effect was analysed by relevant surgical techniques. Surgical resection of the lesions of the thalamus and basal ganglia area according to the three-dimensional concept of the “Four Walls, Two Poles” has achieved good surgical results. Relevant surgical techniques, such as the use of retractors, the use of aspirators, the choice of surgical approaches, and the haemostasis strategy, also played an important role in the operation process. Conclusions In the presented three cases the three-dimensional concept of the “Four Walls, Two Poles” allowed for safe surgical resection of lesions of the thalamus and basal ganglia.

scholarly journals Delayed hypersensitivity reaction to orodispersible budesonide in a case with eosinophilic esophagitis

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Caroline Michèle Andrist ◽  
Lukas Jörg ◽  
Thomas Greuter ◽  
Anna Gschwend ◽  
Alex Straumann ◽  
...  
Keyword(s):  
Hypersensitivity Reaction ◽  
Eosinophilic Esophagitis ◽  
Systemic Reaction ◽  
Chronic Inflammatory Disease ◽  
Delayed Hypersensitivity ◽  
Caucasian Woman ◽  
Case Presentation ◽  
Commercial Test ◽  
Delayed Hypersensitivity Reaction ◽  
Subsequent Intake

Abstract Background Eosinophilic esophagitis (EoE) is a chronic inflammatory disease that has been known since the early 1990s. Swallowed topical corticosteroids (STC) belong to the therapeutic cornerstones. We describe a delayed hypersensitivity reaction to Jorveza®, a newly developed orodispersible budesonide tablet licensed for the treatment of eosinophilic esophagitis. Case presentation A 32-year-old Caucasian woman with EoE was newly treated with Jorveza®. Hours after the first intake, she felt a “strange pruritus” in the throat. This sensation worsened with each subsequent intake. On day 4 she developed oral mucosal symptoms (paresthesia of the tongue, sore and an itchy throat). Intraoral, throat and facial swellings, but no systemic reaction were observed. Patch testing using two commercial test series as well as the orodispersible budesonide tablet revealed a strong sensitization, proving a T cell mediated allergy to budesonide. Conclusions Orodispersible budesonide is increasingly prescribed for the treatment of eosinophilic esophagitis. The development of oropharyngeal symptoms after initiating should alert the treating physician to the possibility of a hypersensitivity reaction.

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