A rare presentation of POEMS syndrome on magnetic resonance neurography: a case series.
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SUBMITTER: Su X
PROVIDER: S-EPMC9244921 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Peripheral neuropathies account for the most frequent disorders seen by neurologists, and causes are manifold. The traditional diagnostic gold-standard consists of clinical neurologic examinations supplemented by nerve conduction studies. Due to well-known limitations of standard diagnostics and atypical clinical presentations, establishing the correct diagnosis can be challenging but is critical for appropriate therapies. Magnetic resonance neurography (MRN) is a relatively novel technique that was developed for the high-resolution imaging of the peripheral nervous system. In focal neuropathies, whether traumatic or due to nerve entrapment, MRN has improved the diagnostic accuracy by directly visualizing underlying nerve lesions and providing information on the exact lesion localization, extension, and spatial distribution, thereby assisting surgical planning. Notably, the differentiation between distally located, complete cross-sectional nerve lesions, and more proximally located lesions involving only certain fascicles within a nerve can hold difficulties that MRN can overcome, when basic technical requirements to achieve sufficient spatial resolution are implemented. Typical MRN-specific pitfalls are essential to understand in order to prevent overdiagnosing neuropathies. Heavily T2-weighted sequences with fat saturation are the most established sequences for MRN. Newer techniques, such as T2-relaxometry, magnetization transfer contrast imaging, and diffusion tensor imaging, allow the quantification of nerve lesions and have become increasingly important, especially when evaluating diffuse, non-focal neuropathies. Innovative studies in hereditary, metabolic or inflammatory polyneuropathies, and motor neuron diseases have contributed to a better understanding of the underlying pathomechanism. New imaging biomarkers might be used for an earlier diagnosis and monitoring of structural nerve injury under causative treatments in the future.
| S-EPMC8804110 | biostudies-literature
Project description:ObjectiveTo compare the magnetic resonance imaging (MRI) features of deep infiltrating endometriosis (DIE) lesions before and after pregnancy.DesignRetrospective study.SettingA single French university tertiary referral hospital.PatientsTwenty-one women without a prior history of surgery for endometriosis with a radiological diagnosis by MRI with two sets of examinations performed before and after pregnancy.InterventionsThe volumes of the lesions were compared using the same protocol before and after pregnancy based on MRI (1.5 T) examinations by a single experienced radiologist who is a referring practitioner for image-based diagnosis of endometriosis.Main outcome measure(s)The DIE lesion volume.Measurements and main resultsBetween October 2012 and December 2016, a total of 21 patients (67 lesions) were included and compared before and after pregnancy. The mean time interval between the MRI before pregnancy and delivery was 19.6 ± 8.5 months (median: 17.6, IQR 13.5-25.2 months). The mean time interval between delivery and the MRI after pregnancy was 11.0 ± 6.4 months (median: 8.3, IQR 6-15.2 months). The mean overall DIE lesion volume by MRI was significantly higher before pregnancy compared to after pregnancy (2,552 ± 3,315 mm3 vs. 1,708 ± 3,266 mm3, respectively, p < 0.01). The mean volume by MRI of the largest lesion of each patient was significantly higher before pregnancy compared to after pregnancy (4,728 ± 4,776 mm3 vs. 3165 ± 5299 mm3; p < 0.01).ConclusionOur data indicate a favorable impact of pregnancy on DIE lesion volumes as measured by MRI.
| S-EPMC6777797 | biostudies-literature
Project description:Axenfeld-Rieger syndrome (ARS) is an extremely rare autosomal dominant disorder characterized by ocular, craniofacial, dental and periumbilical abnormalities. We present a case of a 10-year-old boy. Its awareness among oral surgeons is essential for timely diagnosis and subsequent prevention of ophthalmic and systemic complications as craniofacial and dental features constitute the early recognizable symptoms of this syndrome. Systematic ophthalmic surgeries aid in relieving vision abnormalities, while symptomatic dental treatment should be provided for masticatory and esthetic rehabilitation.
| S-EPMC7410934 | biostudies-literature
Project description:The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site ( https://www.scmr.org ) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.
| S-EPMC8015162 | biostudies-literature
Project description:BackgroundCardiac magnetic resonance (CMR) has a unique role in evaluating pericardial disease, permitting non-invasive tissue analysis, and haemodynamic assessment.Case summaryIn Case 1 of recurrent pericarditis, CMR confirmed reactivation of inflammation with late gadolinium enhancement and native T1/T2 mapping techniques, prompting therapeutic changes. In constrictive pericarditis, CMR is the only modality capable of differentiating a subacute potentially reversible form (Case 2), from a chronic, burnt out irreversible phase characterized by constrictive physiology (Case 3).DiscussionCardiac magnetic resonance is an effective tool to tailor individual therapy, particularly in cases of recurrent and constrictive pericarditis. Late gadolinium enhancement provides diagnostic and prognostic information, and multiparametric mapping has emerged as a promising tool with incremental diagnostic value.
| S-EPMC8728722 | biostudies-literature
Project description:OBJECTIVE:To study the impact of brachial plexus MR neurography (MRN) in the diagnostic thinking and therapeutic management of patients with suspected plexopathy. METHODS:MRN examinations of adult brachial plexuses over a period of 18 months were reviewed. Relevant data collection included-patient demographics, clinical history, pre-imaging diagnostic impression, pre-imaging treatment plan, post-imaging diagnosis, post-imaging treatment plan, surgical notes and electrodiagnostic (ED) results. Impact of imaging on the pre-imaging clinical diagnosis and therapeutic management were classified as no change, mild change or substantial change. RESULTS:Final sample included 121 studies. The common aetiologies included inflammatory in 31 (25.6%) of 121 patients, trauma in 29 (23.9%) of 121 patients and neoplastic in 26 (21.5%) of 121 patients. ED tests were performed in 47 (38.8%) of 121 patients and these showed concordance with MRN findings in 31 (66.0%) of 47 patients. Following MRN, there was change in the pre-imaging clinical impression for 91 (75.2%) of 121 subjects, with a mild change in diagnosis in 57 (47.1%) of 121 patients and a substantial change in 34 (28.0%) of 121 patients. 19 (15.7%) of 121 patients proceeded to therapies that would not have been performed in the same manner without the information obtained from MRN. CONCLUSION:MRN of the brachial plexus significantly impacts clinical decision-making and should be routinely performed in suspected brachial plexopathy. Advances in knowledge: MRN significantly impacts the diagnostic thinking and therapeutic management of patients with suspected brachial plexopathy. MRN not only provides concordant information to ED tests in majority of cases, but also supplements with additional diagnostic data in patients who are ED negative.
| S-EPMC5124848 | biostudies-other
Project description:Background and purposeChronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon demyelinating disorder. Although treatable, it is difficult to diagnose. The purpose of this study was to evaluate the diagnostic performance and abnormalities of plexus via quantitative multisequence magnetic resonance neurography (MRN) for CIDP.MethodsBrachial and lumbosacral (LS) plexus of 37 CIDP patients and 37 age- and gender-matched controls were examined by using multisequence MRN. Nerve diameter, nerve-to-muscle T2 signal intensity ratio (nT2), contrast-enhanced ratio (CR), fractional anisotropy (FA), and apparent diffusion coefficient (ADC) were determined in both plexus, and tractographies were performed. The disease histories and the Inflammatory Rasch-built Overall Disability Scale (I-RODS) questionnaire scores were documented before MRI scans.ResultsThe sizes of nerve roots were larger in CIDP (p < 0.01). CR, nT2, and ADC were significantly higher, while FA was lower in CIDP than in controls (p < 0.01). FA had the highest sensitivity (0.809) and area under the curve (AUC) (0.925), while the highest specificity was 0.961 for CR in single parameters. The combination of FA and CR has the highest sensitivity, specificity, accuracy, and AUC in the LS plexus. CR only had a weak correlation with nT2 (p < 0.05). ADC and diameter had a positive correlation with nT2, and the diameter and nT2 had a negative correlation with FA in CIDP (p < 0.05). FA had a negative correlation with the duration in the CIDP (r's = -0.404, p < 0.05). There was no significant correlation between the I-RODS scores and MR multiparameters (p < 0.05).ConclusionMultisequence MRN possesses a high diagnostic performance in the LS plexus. Sampling perfection with application-optimized contrasts using different flip angle evolutions (SPACE) combined with DTI and contrast enhancement serves as a recommended composite protocol for CIDP.
| S-EPMC8346234 | biostudies-literature
Project description:BackgroundDiagnosis of constrictive pericarditis requires demonstration of interventricular interdependence which can prove difficult even with invasive haemodynamics. Its treatment requires invasive surgical procedures prior to which diagnostic certainty is necessary. Cardiac magnetic resonance imaging (MRI) is an underutilized tool for identification of this pathology.Case summaryWe present two cases of heart failure due to interventricular interdependence with inconclusive invasive haemodynamic. Prior to recommending invasive surgical treatment, confirmation of the diagnosis was required. This was achieved using cardiac MRI leading to pericardiectomy followed by clinical improvement.DiscussionThese cases demonstrate the clinical utility, sensitivity, and specificity of cardiac MRI for ventricular interdependence.
| S-EPMC8286845 | biostudies-literature
Project description:PurposeInflammatory pseudotumors of the liver (IPTL) are not exceptional benign lesions with various etiologies, histology, and imaging appearances. The incomplete knowledge of this pathology and the wide polymorphism sometimes resembling malignancy often induce long and expensive diagnostic flow, biopsy and occasionally unnecessary surgery. We propose a systematic revision of MRI literature data (2000-2021) with some narrative inserts and 10 new complete MRI cases, with the aim of organizing the data about IPTL and identifying some typical features able to improve its diagnosis from imaging.MethodsWe performed a systematic revision of literature from 2000 to 2021 to obtain MRI features, epidemiological, and clinical data of IPTL. The basic online search algorithm on the PubMed database was "(pseudotumor) AND (liver) AND (imaging)." Quality assessment was performed using both scales by Moola for case report studies and by Munn for cross-sectional studies reporting prevalence data. A case-based retrospective study by collecting patients diagnosed with IPTL from three different university hospitals from 2015 to 2021 was done as well. Only cases with MR examinations complete with T1/T2/contrast-enhanced T1/Diffusion-Weighted (W) images and pathology-proven IPTL were selected.ResultsAfter screening/selection 38 articles were included for a total of 114 patients. In our experience we selected 10 cases for a total of 16 IPTLs; 8 out of 10 patients underwent at least 1 MRI follow-up. Some reproducible and rather typical imaging findings for IPTL were found. The targetoid aspect of IPTL is very frequent in our experience (75% on T1W, 44% on T2W, 81% on contrast-enhanced T1W (at least one phase), 100% on Diffusion-W images) but is also recurrent in the literature (6% on T1W, 31% on T2W, 51% on CE-T1W (at least one phase), 18% on Diffusion-W images, and 67% on hepatobiliary phase). In our experience, Apparent Diffusion Coefficient map values were always equal to or higher than those of the surrounding parenchyma, and at MRI follow-up, nodule/s disappeared at first/second control, in six patients, while in the remaining 2, lesions persisted with tendency to dehydration.ConclusionA targetoid-like aspect of a focal liver lesion must raise diagnostic suspicion, especially if IgG4-positive plasma is detected. MRI follow-up mainly shows the disappearance of the lesion or its reduction with dehydration.
| S-EPMC9300573 | biostudies-literature
Project description:BackgroundIn the year 1724, Hermann Boerhaave reported a case of a Dutch admiral who died due to spontaneous rupture of the esophagus following vomiting. The aim of this retrospective study is to analyze the therapeutic modality, morbidity and mortaliy of a group of patients with spontaneous esophageal rupture treated in our hospital.MethodsTen patients were treated from March 1990 to August 2016. Seven patients were males and three were females. The age range was, 52-72 years, with an average of 66.2 years. In four patients, the diagnosis and posterior treatment were performed within 24 h (Group I) and the remaining six patients after 24 h (Group II).ResultsThe mean hospital stay was 36.6 days (range 17-62 days). The mortality rate was 50%, which was due to septic shock and the morbidity of patients who survived was 40% due to pneumonia in one case and fistula in another.DiscussionsThis condition has a high mortality rate with a lethality that depends on the time between recognition of symptoms and proper surgical treatment.
| S-EPMC6651841 | biostudies-literature