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Continuum (Minneapolis, Minn.) Aug 2019This article offers clinicians a strategic approach for making sense of a symptom complex that contains myoclonus. The article presents an evaluation strategy that... (Review)
Review
PURPOSE OF REVIEW
This article offers clinicians a strategic approach for making sense of a symptom complex that contains myoclonus. The article presents an evaluation strategy that highly leverages the two major classification schemes of myoclonus. The goal of this article is to link evaluation strategy with diagnosis and treatment of myoclonus.
RECENT FINDINGS
The growth of medical literature has helped better define myoclonus etiologies. Physiologic study of myoclonus types and etiologies with electrophysiologic testing has provided greater clarity to the pathophysiology of the myoclonus in various diseases. Although studies have been limited, the role of newer treatment agents and methods has made progress.
SUMMARY
Myoclonus has hundreds of different etiologies. Classification is necessary to evaluate myoclonus efficiently and pragmatically. The classification of myoclonus etiology, which is grouped by different clinical presentations, helps determine the etiology and treatment of the myoclonus. The classification of myoclonus physiology using electrophysiologic test results helps determine the pathophysiology of the myoclonus and can be used to strategize symptomatic treatment approaches. Both basic ancillary testing (including EEG and imaging) and more comprehensive testing may be necessary. Treatment of the underlying etiology is the ideal approach. However, if such treatment is not possible or is delayed, symptomatic treatment guided by the myoclonus physiology should be considered. More controlled study of myoclonus treatment is needed. Further research on myoclonus generation mechanisms should shed light on future treatment possibilities.
Topics: Adult; Aged; Anticonvulsants; Carbamazepine; Electroencephalography; Female; Humans; Male; Middle Aged; Myoclonus; Young Adult
PubMed: 31356293
DOI: 10.1212/CON.0000000000000750 -
Journal of Cardiothoracic and Vascular... Aug 2019Transcranial Doppler is a bedside procedure that measures linear cerebral blood flow velocity (CBFV) and the pulsatility index through the intracranial circulation.... (Review)
Review
Transcranial Doppler is a bedside procedure that measures linear cerebral blood flow velocity (CBFV) and the pulsatility index through the intracranial circulation. Transcranial color-coded duplex Doppler (TCCD) provides both CBFV and B-mode functions. In this review they are both referred to as brain ultrasound TCCD. Brain ultrasound can be applied in various environments, including out-of-hospital, emergency room, surgery, intensive care, and ward settings. The most common neurologic disease processes evaluated with TCCD are subarachnoid hemorrhage, traumatic brain injury, and ischemic and hemorrhagic stroke. However, TCCD also is used outside the neuroenvironment for diseases such as sickle cell anemia or for cerebral hemodynamic assessment during the cardiovascular perioperative period. In these applications, TCCD can be used for the detection of cerebral vessel occlusion, estimation of cerebrovascular reactivity, right-to-left cardiac shunts, noninvasive estimation of cerebral perfusion and intracranial pressure, optic nerve sheath diameter, midline shift, hydrocephalus, and the presence of foreign objects. Finally, TCCD has a high accuracy in confirming total cerebral circulatory arrest and has been used as an ancillary test to support clinical diagnosis of brain death. Other indications for TCCD include assessment of collateral blood flow and embolization during carotid endarterectomy, assessment of patterns and extent of collateral circulation in severe stenosis or occlusion, assessment of patent foramen ovale/paradoxical embolism, assessment of arteriovenous malformations and studying their supply arteries and flow patterns, assessment of noncardiac right-to-left shunts, assessment of severe stenosis in the arteries of the circle of Willis, and assessment of vertebral artery dissection.
Topics: Blood Flow Velocity; Cerebrovascular Circulation; Cerebrovascular Disorders; Humans; Intraoperative Neurophysiological Monitoring; Optic Nerve; Ultrasonography, Doppler, Transcranial
PubMed: 31279352
DOI: 10.1053/j.jvca.2019.03.040 -
Surgical Pathology Clinics Jun 2021PRAME (PReferentially expressed Antigen in MElanoma) is a melanoma-associated antigen expressed in cutaneous and ocular melanomas and some other malignant neoplasms,... (Review)
Review
PRAME (PReferentially expressed Antigen in MElanoma) is a melanoma-associated antigen expressed in cutaneous and ocular melanomas and some other malignant neoplasms, while its expression in normal tissue and benign tumors is limited. Detection of PRAME protein expression by immunohistochemistry in a cohort of 400 melanocytic tumors showed diffuse nuclear immunoreactivity for PRAME in most metastatic and primary melanomas. In contrast, most nevi were negative for PRAME or showed nondiffuse immunoreactivity. The difference in the extent of immunoreactivity for PRAME in unambiguous melanocytic tumors prompted the study of PRAME as an ancillary tool for evaluating melanocytic lesions in more challenging scenarios.
Topics: Antigens, Neoplasm; Humans; Immunohistochemistry; Melanoma; Skin Neoplasms
PubMed: 34023098
DOI: 10.1016/j.path.2021.01.001 -
RoFo : Fortschritte Auf Dem Gebiete Der... Jun 2023The Liver Imaging Reporting and Data System (LI-RADS v2018) standardizes the interpretation and reporting of MDCT and MRI examinations in patients at risk for... (Review)
Review
PURPOSE
The Liver Imaging Reporting and Data System (LI-RADS v2018) standardizes the interpretation and reporting of MDCT and MRI examinations in patients at risk for hepatocellular carcinoma (HCC).
MATERIALS AND METHODS
For focal liver lesions (called "observations") it assigns categories (LR-1 to 5, LR-M, LR-TIV, LR-TR), which reflect the probability of benignity or malignancy (HCC or other non-HCC malignancies) of the respective observation. The categories assigned are based on major and ancillary image features, which have been developed by the American College of Radiology (ACR), revised several times (now v2018), and validated in many studies. The value of ancillary features to modify LI-RADS categories assigned to observations based on major features is shown.
RESULTS
This review summarizes the relevant CT and MRI features and presents a step-by-step approach for readers not familiar with LI-RADS on how to use the system. Relevant imaging features and the value of different modalities (contrast-enhanced CT, MRI with extracellular gadolinium chelates or liver-specific contrast agents) is explained.
CONCLUSION
The widespread adoption of LI-RADS for CT/MRI reporting in high-risk patients would help to reduce inter-reader variability. It could improve communication between radiologists, oncologists, hepatologists, pathologists, and liver surgeons, and lead to better patient management.
KEY POINTS
· LI-RADS has been developed and revised to address the need for improved diagnosis and standardized categorization of findings in chronic liver disease.. · CT/MRI LI-RADS consists of major criteria and ancillary features to classify observations.. · LI-RADS terminology helps to clarify the communication of liver observations between radiologists and referring physicians..
CITATION FORMAT
· Schima W, Kopf H, Eisenhuber E. LI-RADS made Easy. Fortschr Röntgenstr 2023; 195: 486 - 494.
Topics: Humans; Carcinoma, Hepatocellular; Liver Neoplasms; Retrospective Studies; Magnetic Resonance Imaging; Contrast Media; Sensitivity and Specificity
PubMed: 36724803
DOI: 10.1055/a-1990-5924 -
The American Journal of Dermatopathology Nov 2023Preferentially expressed antigen in melanoma (PRAME) is a tumor-associated antigen first identified in a melanoma patient and found to be expressed in most melanomas as...
Preferentially expressed antigen in melanoma (PRAME) is a tumor-associated antigen first identified in a melanoma patient and found to be expressed in most melanomas as well as in variable levels in other malignant neoplasms of epithelial, mesenchymal, or hematolymphoid lineage. Detection of PRAME expression in formalin-fixed paraffin-embedded tissue is possible by immunohistochemistry (IHC) with commercially available monoclonal antibodies. In situ and invasive melanoma frequently show a diffuse pattern of nuclear PRAME immunoreactivity which contrasts with the infrequent and typically nondiffuse staining seen in nevi. In many challenging melanocytic tumors, results of PRAME IHC and other ancillary tests correlate well, but not always: The tests are not interchangeable. Most metastatic melanomas are positive for PRAME, whereas nodal nevi are not. Numerous studies on PRAME IHC have become available in the past few years with results supporting the value of PRAME IHC as an ancillary tool in the evaluation of melanocytic lesions and providing insights into limitations in sensitivity and specificity as well as possible pitfalls that need to be kept in mind by practicing pathologists.
Topics: Humans; Antigens, Neoplasm; Biomarkers, Tumor; Immunohistochemistry; Melanoma; Nevus; Skin Neoplasms; Transcription Factors; Melanoma, Cutaneous Malignant
PubMed: 37856737
DOI: 10.1097/DAD.0000000000002440 -
American Journal of Clinical Pathology Feb 2020The objective of this study was to review and illustrate the sometimes diagnostically challenging features of cardiac sarcoidosis. We emphasize variable phenotypes... (Review)
Review
OBJECTIVES
The objective of this study was to review and illustrate the sometimes diagnostically challenging features of cardiac sarcoidosis. We emphasize variable phenotypes presented at explant and biopsy evaluation and review literature regarding ancillary clinical and pathologic studies to enhance diagnostic accuracy.
METHODS
A literature review was performed and two cardiac sarcoidosis cases were illustrated.
RESULTS
Our cases and literature review demonstrate the pathologic spectrum of cardiac sarcoidosis. Irregular left ventricular free wall involvement is most common, followed by the interventricular septum and right ventricle. Although granulomas are often composed of tight epithelioid macrophage aggregates, early granulomas comprise loosely associated macrophages with lymphocyte predominance. Chronic disease leads to fibrosis and end-stage heart failure. Sampling errors and variable histology cause low endomyocardial biopsy sensitivity.
CONCLUSIONS
Current guidelines use clinical, radiologic, and immunohistologic criteria for diagnosing cardiac sarcoidosis. Knowledge of these guidelines will assist pathologists in making accurate diagnosis of this disease.
Topics: Cardiomyopathies; Granuloma; Humans; Macrophages; Myocardium; Sarcoidosis
PubMed: 31769474
DOI: 10.1093/ajcp/aqz169 -
Surgical Pathology Clinics Sep 2023Hepatic inflammatory pseudotumor (IPT) describes a mass lesion composed of fibroblasts or myofibroblasts with a dense inflammatory infiltrate comprising lymphocyte,... (Review)
Review
Hepatic inflammatory pseudotumor (IPT) describes a mass lesion composed of fibroblasts or myofibroblasts with a dense inflammatory infiltrate comprising lymphocyte, plasma cells, and histiocytes. These lesions are presumed to be an exuberant response to an infectious organism, although in most cases the causative agent is unknown. In specific circumstances, pathologists should consider ancillary techniques to exclude specific infections, such as mycobacteria, Candida, or syphilis. IgG4-related disease may cause a plasma-cell rich IPT. Finally, true neoplasms can mimic IPTs and must be excluded with appropriate ancillary studies, including inflammatory myofibroblastic tumor, follicular dendritic cell tumor, inflammatory angiomyolipoma, Hodgkin lymphoma, and inflammatory hepatocellular carcinoma.
Topics: Humans; Granuloma, Plasma Cell; Carcinoma, Hepatocellular; Hodgkin Disease; Liver Neoplasms
PubMed: 37536889
DOI: 10.1016/j.path.2023.04.010 -
Mediastinum (Hong Kong, China) 2020Primary mediastinal fibroblastic sarcomas constitute a rare, heterogeneous group of neoplasms, mainly including solitary fibrous tumor (SFT) (benign and malignant), low... (Review)
Review
Primary mediastinal fibroblastic sarcomas constitute a rare, heterogeneous group of neoplasms, mainly including solitary fibrous tumor (SFT) (benign and malignant), low grade fibromyxoid sarcoma (LGFMS), adult fibrosarcoma (FS), myxofibrosarcoma, sclerosing epithelioid FS, etc. Although morphologically diverse, they frequently have similar clinical and radiological features. Overlapping of histological features among these neoplasms can make it challenging for pathologists to come to an accurate diagnosis. In addition, other mesenchymal neoplasms and spindle cell neoplasms of the epithelial cell origin can occur in the mediastinum. Immunostaining and molecular testing are important ancillary studies to confirm or rule out primary mediastinal fibroblastic neoplasms. SFT and LGFMS occur more often than adult FS in the mediastinum and both have reliable immunostaining markers STAT6 and MUC4, respectively, and unique molecular changes. The incidence of adult FS has decreased dramatically due to recognition of morphologically and genetically distinctive subtypes of fibroblastic sarcoma and better understanding of mesenchymal and non-mesenchymal mimickers. Adult FS is extremely rare and a diagnosis of exclusion. Adult FS can be rendered only after careful histological examination and thorough ancillary studies have ruled out all its mimickers. This article is focused on reviewing clinicopathological features, immunostaining, molecular changes, prognosis and differential diagnosis of SFT, LGFMS, and adult FS. Correct diagnosis is crucial for oncologists to make appropriate clinical management plans.
PubMed: 35118294
DOI: 10.21037/med-20-44