scholarly journals Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships

2021 ◽  
Vol 12 ◽  
Author(s):  
Yacov Balash ◽  
Amos D. Korczyn ◽  
Nadejda Khmelev ◽  
Anda Eilam ◽  
Meital Adi ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Clinical Course ◽  
Case Reports ◽  
Brain Diseases ◽  
Rare Presentation ◽  
Laboratory Features ◽  
Uncommon Disease ◽  
Median Lifespan ◽  
Jakob Disease ◽  
Τ Protein

Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG), and laboratory features of patients with this very rare phenomenon. Here, we review the clinical characteristics, onset features, and clinical course variants of stroke-like CJD in 23 such patients. The median age of the patients was 71 years (range: 56–84 years); 12 were women. In 20 patients, CJD was sporadic. Thirteen patients developed apoplexy-like onset of symptoms, whereas the others had prodromal non-specific complaints. Most often the patients manifested with pyramidal signs (n = 13), ataxia (n = 9), and aphasia (n = 8). On MRI DWI sequence, all subjects had abnormal hyperintensities in various parts of the cerebral cortex, striatum, or thalamus, while EEG detected periodic triphasic waves only in 11. CSF 14-3-3 protein and total τ-protein were abnormal in 17 of 23 cases. All patients died, median lifespan being 2 months (range: 19 days−14 months). In conclusion, a complex of clinical, radiological, and laboratory manifestations of stroke-like onset of CJD is outlined. The clinical relationships between CJD and stroke are considered, in an attempt to highlight this rare presentation of an uncommon disease.

scholarly journals Immune Thrombocytopenic Purpura With Unusual Presentations – Reports of Two Cases

2020 ◽  
Vol 38 (4) ◽  
pp. 218-222
Author(s):  
Tasmina Chowdhury ◽  
Abdul Basit Ibne Momen ◽  
Hironmoy Barman ◽  
Mohammad Tariqul Ahsan Khan ◽  
Kohinoor Begum ◽  
...  
Keyword(s):  
Immune Thrombocytopenic Purpura ◽  
Ovarian Hyperstimulation Syndrome ◽  
Intracranial Haemorrhage ◽  
Hemorrhagic Stroke ◽  
Clinical Course ◽  
Case Reports ◽  
Alveolar Haemorrhage ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated ◽  
Uncommon Disease

Immune thrombocytopenic purpura (ITP) is an immune mediated bleeding disorder, usually has a relatively benign clinical course. Deep seated bleeding like intracranial haemorrhage or haemoperitonium or massive haemorrhage requiring transfusion or other intervention are rare in ITP, unless platelet count are extremely low or other complicating conditions coexist. Here are two case reports of ITP presenting in uncommon and devastating manners. The 1st  one is of a 21- yearold married nulliparaous lady with ITP complicating her undiagnosed ovarian hyperstimulation syndrome leading to haemoperitonium (ruptured ovarian cyst), post operative alveolar haemorrhage resulting in ARDS and later on DVT of right leg on her 9th  POD. She was managed by multi discipline team. A new consequence of her disease one after another was striking and made her management more challenging. Ultimately the lady recovered and was discharged with advice which was not less than a miracle. The 2nd  case is of a 50- year- old elderly lady who had a hemorrhagic stroke as a presenting feature of ITP. Though ITP is not an uncommon disease but in these cases its presentation, consequences and severity was unusual and making its management very much challenging. J Bangladesh Coll Phys Surg 2020; 38(4): 218-222  

Clinical Characteristics in Neonates with Alloimmune Neutropenia: Significance of the Detection of Antineutrophil Antibodies

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4647-4647
Author(s):  
Yoko Mizoguchi ◽  
Kazuhiro Nakamura ◽  
Shuhei Karakawa ◽  
Takashi Sato ◽  
Rie Onodera ◽  
...  
Keyword(s):  
Japanese Population ◽  
Clinical Characteristics ◽  
Spontaneous Recovery ◽  
Clinical Course ◽  
Case Reports ◽  
High Strength ◽  
Long Duration ◽  
Control Serum ◽  
The Mean

Abstract Neonate alloimmune neutropenia (NAIN) is caused by the transplacental transfer of maternal alloantibodies directed against antigens on the infant’s neutrophils. To date, there are scant studies about its clinical characteristics and characterization of antineutrophil antibodies though some case reports are found. In this study we analyzed 11 cases with NAIN from January 2005 to December 2007. The diagnosis of NAIN was confirmed by the transient neutropenia less than 500/μl of absolute neutrophil count (ANC), the detection of maternal antineutrophil antibody, the incompatibility of neutrophil antigens between parents, and their mothers without autoimmune diseases. Antineutrophil antibodies were detected by granulocyte indirect immunofluorescence test using flow cytometry. To quantify the strength of the antibodies, the ratio of the mean fluorescence channel of each sample to that of control serum was expressed as relative fluorescence intensity according to the method reported previously (Blood99: 3468, 2002). The median age at diagnosis in NAIN patients was 8 days after birth ranged from 0 to 30 days. The average of ANC at the presentation was 170/μl ranged from 0 to 500/μl. All antineutrophil antibodies detected in sera of both neonates and their mothers were against HNA-1 antigens. The alloantibody against HNA-1a was found in 2 cases, that against HNA-1b was in 6 cases, and that against FcγR IIIb was found in 3 cases. The fact that the frequencies of homozygote of HNA-1a and HNA-1b in Japanese population were approximately 50% and 12%, respectively may reflect the frequency of alloantibody specificity in NAIN. During the neutropenic period, 7 cases with NAIN showed mild to moderate infections associated with neutropenia, such as pyrexia and pyodermia. In contrast, 4 of 11 cases with NAIN did not have any infectious episodes in their clinical course. In all patients, the spontaneous recovery of neutropenia with the disappearance of alloantibody was observed within 6 months (median 85 days ranged from 3 weeks to 6 months). The duration until spontaneous resolution of neutropenia was dependent on the strength of alloantibody found in sera of the mothers and neonates. Two patients with significantly high strength of alloantibodies had the relatively long duration to restore the neutropenia(4 months and 6 months). In conclusion, the specificity of antineutrophil antibodies in patients with alloimmune neutropenia is dependent on the frequencies of neutrophil antigens in Japanese population. The quantification of alloantibodies in neonates and their mothers may be useful in considering the clinical course of neutropenia in neonates.

scholarly journals Erythema Multiforme and COVID-19: What Do We Know?

Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 828
Author(s):  
Luigi Bennardo ◽  
Steven Paul Nisticò ◽  
Stefano Dastoli ◽  
Eugenio Provenzano ◽  
Maddalena Napolitano ◽  
...  
Keyword(s):  
Erythema Multiforme ◽  
Clinical Characteristics ◽  
Web Of Science ◽  
Clinical Course ◽  
Case Reports ◽  
Case Series ◽  
Drug Induced ◽  
Cutaneous Eruption ◽  
Isi Web Of Science ◽  
Juvenile Type

(1) Background: Erythema multiforme (EM) is an acute cutaneous eruption often associated with infections and more rarely with drugs. This review aimed to evaluate the association between erythema multiforme and coronavirus disease 2019 (COVID-19). (2) Methods: A systematic search of PubMed/MEDLINE, Scimago Scopus, and ISI/Web of Science was performed. Original articles, case series, or case reports were evaluated and selected. (3) Results: Fourteen articles were selected, describing a total of 70 patients. EM is a cutaneous eruption rarely occurring in COVID-19 and is, in most cases, associated with a hypersensitivity reaction to the virus. In these cases, EM seems to affect patients younger than 30 years or older than 55 years. Infrequently, some drugs used in the management of COVID-19 may induce EM, especially hydroxychloroquine. The three groups of patients seem to have different clinical characteristics and courses. (4) Conclusions: From these data, it is possible to preliminarily propose that EM or EM-like eruptions linked to COVID-19 might be divided into three types: the virus-related juvenile type (affecting patients <30-year-old), the virus-related older type (affecting patients >55 years), and the drug-induced type. The occurrence of a skin rash does not seem to be related to the severity and clinical course of COVID-19.

THYROID TYPE CARCINOMA ARISING IN STRUMA OVARII. SIX CLINICAL CASES AND REVIEW

2018 ◽  
Vol 64 (6) ◽  
pp. 708-715
Author(s):  
Natalya Severskaya ◽  
Andrey Rodichev ◽  
Aleksey Ilin ◽  
Dmitriy Semin ◽  
Pavel Isaev ◽  
...  
Keyword(s):  
Radioiodine Therapy ◽  
Clinical Course ◽  
Case Reports ◽  
Thyroid Tissue ◽  
Follicular Carcinoma ◽  
Complete Response ◽  
Pelvic Surgery ◽  
Ovarian Teratoma ◽  
Struma Ovarii

Struma ovarii is a rare variant of the mature ovarian teratoma composed of more than 50% thyroid tissue. Thyroid type carcinoma can occur in 5% of struma ovarii. Given the rarity of this pathology, as well as the different clinical course, approaches to the treatment of this disease are controversial. The proposed approaches to treatment vary from ovarian resection to total hysterectomy with bilateral salpingo-oophorectomy and adjuvant therapy. We present here 6 case reports of thyroid type carcinoma in struma ovarii and outcome of patients treated in our clinic. All patients had pelvic surgery of different extent, followed by thyroidectomy and radioiodine therapy. The incidence of metastasis is 67% (4/6), 2 - intraperitoneal metas-tases, 2 - bone metastases. Among patients with metastases, 2 have reached a complete response, one with a good response continues treatment, one had progression. The follow-up period is 1 to 15 years (median 4 years). One patient with follicular carcinoma died of progression 8 years after diagnosis. The remaining patients are alive.

scholarly journals Pericardial effusion and cardiac tamponade requiring pericardial window in an otherwise healthy 30-year-old patient with COVID-19: a case report

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Christina Walker ◽  
Vincent Peyko ◽  
Charles Farrell ◽  
Jeanine Awad-Spirtos ◽  
Matthew Adamo ◽  
...  
Keyword(s):  
Case Report ◽  
Pericardial Effusion ◽  
Cardiac Tamponade ◽  
Case Reports ◽  
Healthy Woman ◽  
Signs And Symptoms ◽  
Rare Presentation ◽  
Pericardial Window ◽  
Acute Pericarditis ◽  
Novel Coronavirus

Abstract Background This case report demonstrates pericardial effusion, acute pericarditis, and cardiac tamponade in an otherwise healthy woman who had a positive test result for coronavirus disease 2019. Few case reports have been documented on patients with this presentation, and it is important to share novel presentations of the disease as they are discovered. Case presentation A Caucasian patient with coronavirus disease 2019 returned to the emergency department of our hospital 2 days after her initial visit with worsening chest pain and shortness of breath. Imaging revealed new pericardial effusion since the previous visit. The patient became hypotensive, was taken for pericardial window for cardiac tamponade with a drain placed, and was treated for acute pericarditis. Conclusion Much is still unknown about the implications of coronavirus disease 2019. With the novel coronavirus disease 2019 pandemic, research is still in process, and we are slowly learning about new signs and symptoms of the disease. This case report documents a lesser-known presentation of a patient with coronavirus disease 2019 and will help to further understanding of a rare presentation.

Malignant thyroid teratoma: an integrated analysis of case series and case reports

2021 ◽  
Author(s):  
Huy Gia Vuong ◽  
Truong P.x. Nguyen ◽  
Hanh T.t. Ngo ◽  
Lewis Hassell ◽  
Kennichi Kakudo
Keyword(s):  
Clinical Course ◽  
Case Reports ◽  
Case Series ◽  
Seer Program ◽  
Integrated Analysis ◽  
Continuous Variables ◽  
Chi Square ◽  
Large Tumor ◽  
Exact Test

Malignant thyroid teratoma (MTT) is a very rare thyroid malignancy. These neoplasms have been reported only in case reports and small-sized case series so far. In this study, we searched for MTTs in the Surveillance, Epidemiology, and End Result (SEER) program during 1975-2016. Subsequently, we incorporated the SEER data with published MTT cases in the literature to analyze the characteristics and prognostic factors of MTTs. Integrated data were analyzed using Chi-square or Fisher’s exact test for categorical covariates, and t-test or Mann-Whitney test for continuous variables. We included 28 studies with 36 MTT cases and found additional 8 cases from the SEER program for final analyses. Our results showed that MTT is typically seen in adult females. These neoplasms were associated with an aggressive clinical course with high rates of extrathyroidal extension (80%) and nodal involvement (62%). During follow-up, the development of recurrence and metastases were common (42% and 46%, respectively), and one-third of patients died at the last follow-up. Large tumor size (p = 0.022) and the presence of metastases during follow-up (p = 0.008) were associated with a higher mortality rate. In conclusion, our study demonstrated the characteristic features of MTT patients and outlined some parameters associated with a negative outcome which could help clinicians better predict the clinical course of these neoplasms.

Myositis-associated Interstitial Lung Disease: Predictors of Failure of Conventional Treatment and Response to Tacrolimus in a US Cohort

2017 ◽  
Vol 44 (11) ◽  
pp. 1612-1618 ◽  
Author(s):  
Niharika Sharma ◽  
Michael S. Putman ◽  
Rekha Vij ◽  
Mary E. Strek ◽  
Anisha Dua
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Characteristics ◽  
Conventional Treatment ◽  
Case Reports ◽  
Response To Therapy ◽  
Disease Modifying Antirheumatic Drugs ◽  
Antirheumatic Drugs ◽  
Small Series ◽  
The Mean

Objective.Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases.Methods.A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids.Results.Thirty-one of 54 patients (57%) responded to conventional treatment based on the predefined variables of improvement in myositis and/or ILD. Patients with polymyositis (PM)-ILD were more likely to respond to conventional treatment than those with dermatomyositis (DM)-ILD (67% vs 35%, p = 0.013). Twenty-three patients failed conventional treatment, 18 of whom subsequently received adjunctive tacrolimus. Ninety-four percent had improvements in ILD and 72% showed improvement in both myositis and ILD. The mean doses of prednisone decreased from baseline by 65% at 3–6 months (p = 0.002) and 81% at 1 year (p < 0.001).Conclusion.Patients with PM-ILD were more likely to respond to conventional treatment than patients with DM-ILD, but clinical characteristics and serology did not otherwise predict response to therapy. A majority of patients with MA-ILD refractory to conventional therapy improved while receiving tacrolimus and were able to decrease their dose of both glucocorticoids and other disease-modifying antirheumatic drugs.

scholarly journals The Importance of Rapid Consideration of Creutzfeldt- Jakob Disease in the Differential Diagnosis of Progressive Neurodegenerative Disease: A Case Report

2016 ◽  
Vol 4 (2) ◽  
pp. 72-75
Author(s):  
Arthur Joseph ◽  
Jacob Core ◽  
Daniel Solano ◽  
Marquand Patton ◽  
Shaun Smart
Keyword(s):  
Differential Diagnosis ◽  
Status Epilepticus ◽  
High Signal ◽  
Convulsive Status Epilepticus ◽  
Rare Presentation ◽  
Progressive Encephalopathy ◽  
Jakob Disease ◽  
Low Incidence ◽  
Patient’S History ◽  
Sporadic Cjd

Background: Creutzfeldt-Jakob disease (CJD) is a prion disease characterized by misfolded proteins that lead to neurodegeneration and inevitable death. Classic sporadic CJD presents primarily with cognitive symptoms and ataxia without visual impairment at the onset of the illness. Seizure activity is a rare presentation of patients with sporadic CJD. Case: We present a rare case of rapidly progressive encephalopathy in a 57-year-old female who presented to the emergency department with bizarre behavior and vision deterioration. Imaging was unrevealing, and infectious and organic causes were ruled out. Electroencephalogram showed evidence of encephalopathy and non-convulsive status epilepticus. Magnetic resonance imaging conducted later displayed high signal intensity in centrum ovale. The patient’s history, results from diagnostic analyses, and clinical presentation suggested the diagnosis of CJD (sporadic type). Conclusion: Due to the low incidence and varying clinical presentations, it is difficult to include CJD in a differential diagnosis without specific analytic measures. However, for the benefit of the patient and healthcare resources, CJD needs to be quickly considered when rapid neurological decline or non-convulsive status epilepticus is not suggestive of another entity

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