Hyperintense Signal Research Articles

Relationship between hyperintensity on MRI-T1WI and hemorrhagic transformation after cerebral infarction and its influencing factors

This study aims to investigate the correlation between hyperintensity on Magnetic Resonance Imaging-T1 weighted imaging (MRI-T1WI) and post-infarction hemorrhagic transformation (HT) after cerebral infarction (CI) and analyze the influencing factors. This retrospective study comprised 115 patients diagnosed with cerebral infarction at our hospital. Their clinical data were collected, and they were then divided into a hyperintensity and a non-hyperintensity group based on their T1WI image characteristics. Comparative analysis was performed and the diagnostic value of T1WI hyperintensity for HT and influencing factors were assessed. Lesions in the 115 cerebral infarction patients were distributed as follows: 52 in the cerebral cortex, 37 in the basal ganglia, 14 in the cerebellum, 7 in the thalamus, and 5 in the subcortex. Hyperintensity on T1WI was observed in 4 cases before treatment, which increased to 27 cases after treatment, including 16 affecting the cerebral cortex. These hyperintense signals manifested as spotty, patchy or linear patterns along the gyri. In the basal ganglia, 10 cases exhibited spotty or patchy signals, surrounded by an annular hypointense shadow. Additionally, 3 cases involved the cerebellum, 1 the thalamus, and 1 the subcortex, all with spotty or patchy hyperintensities. HT occurred in 17 out of 115 patients (14.78%) one month after treatment. The diagnostic performance of T1WI hyperintensity for HT showed sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of 94.12%, 84.69%, 86.09%, 51.61% and 98.81%, respectively, with a Kappa value of 0.588. Multivariate logistic regression analysis revealed that age, atherosclerosis, and infarct size were significant risk factors for T1WI hyperintensity in cerebral infarction (p < 0.05). Hyperintensity on T1WI in cerebral infarction primarily correlates with HT and could be a valuable diagnostic marker for HT, with age, atherosclerosis and infarct size identified as potential influencing factors.

Excessive Exercise by Gym Enthusiast Leading to Rhabdomyolysis: A Case Report

Rhabdomyolysis is a rare condition characterised by muscle oedema and inflammation. Radiological imaging, particularly Magnetic Resonance Imaging (MRI), plays a crucial role in the diagnosis and management of patients with muscle injuries. Hereby, the authors present a case report of a 21-year-old male who recently joined a gym a month ago. After a recent upper body workout, he began experiencing vague pain around the posterior aspect of the entire right arm, radiating towards the shoulder and elbow. An MRI was performed to evaluate the musculature of the right arm and forearm. T2-weighted axial images revealed hyperintense signals within the long head of the triceps muscle. Short Tau Inversion Recovery (STIR) coronal and STIR axial sequences showed increased signal intensity in the short field of view sequences, consistent with the presence of subcutaneous inflammation. The patient was managed conservatively with anti-inflammatory medications. A follow-up scan after one month showed complete resolution of muscle oedema. The ability of MRI to accurately assess the degree and severity of muscle injury allows for appropriate treatment planning and monitoring of disease progression. In cases of suspected muscle injury, prompt radiological evaluation can facilitate early diagnosis and intervention, ultimately improving patient outcomes.

Diabetic Striatopathy in Pediatric Patient: A Case Report on a Reversible and Acquired Movement Disorder

Purpose: To present a rare case of diabetic striatopathy (DS) in a child with type 1 diabetes mellitus (DM) with reversible unilateral hemichorea-hemiballismus. Case Report: We report a case of an adolescent girl with uncontrolled type 1 DM who presented with hemichorea-hemiballismus of the right extremities. Cranial magnetic resonance imaging (MRI) showed hyperintense signals on bilateral basal ganglia. There was improvement in hemichorea- hemiballismus as blood glucose levels normalized. Conclusion: A high index of suspicion is required to make a diagnosis of DS in children presenting with abnormal movement disorder and characteristic neuroimaging findings in a background of uncontrolled DM.

Creutzfeldt-Jakob disease presenting as psychiatric disorder: case presentation and systematic review.

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy caused by misfolded human prion proteins (PrP)s. Due to variability in presentation, the diagnosis may be missed in lieu of various psychiatric disorders. Our study reports on a prototypical case and psychiatric mimic for CJD, and the workup used to establish the correct diagnosis. A 54-year-old male with a past medical history of traumatic brain injury and major depressive disorder presented with chest pain. During the hospital stay, he was found to be increasingly aggressive, and behaved out of character. Further review of clinical history revealed that the patient was diagnosed with cognitive impairment and depression one year prior. The patient was agitated, poorly redirectable, and had unstable gait on neurological examination. Magnetic resonance imaging (MRI) of the brain demonstrated restricted diffusion (DWI) along the parietooccipital and temporal regions (L > R) and in the subcortical structures, including the basal ganglia and thalami, with accompanying subtle fluid attenuation inversion recovery (FLAIR) hyperintense signal abnormality in these regions, deemed as artifactual at the time. Repeat MRI brain two months later demonstrated progression of the DWI signal with ADC correlate and FLAIR findings. Cerebrospinal fluid 14-3-3 and RT-QuIC samples were positive. Upon passing a few months later, brain autopsy and Western Blot confirmed the CJD diagnosis. Literature review was conducted on PubMed to identify CJD cases initially diagnosed as psychiatric disorder. Search terms included "CJD" or "Creutzfeldt-Jakob disease" with three common psychiatric diagnoses, "Depression," "Psychosis," and "Mania." Positive EEG, MRI, PET, and CSF (including protein 14-3-3 and tau) findings for CJD were found in 66.7, 81.1, 50, and 72.7% of cases, respectively. Overall, CJD can present as a psychiatric mimic. In suspicious cases, EEG, imaging, and CSF studies should be promptly utilized to arrive at the correct diagnosis. Repeated MRI imaging is often required to help in the diagnostic process. Brain biopsy should be considered in selected cases.

Tendinous Signal Alterations on MRI in the Asymptomatic Elbow: A Retrospective Cross-Sectional Study.

It is clinically relevant to prevent overtreatment of tendinopathy diagnosed solely on imaging. Therefore, the prevalence of presumable asymptomatic signal changes in the common flexor origin, biceps insertion, brachialis insertion, and triceps insertion were assessed. Two hundred and five magnetic resonance imaging (MRI) exams of the elbow with coronal and axial fat-saturated fluid-sensitive sequences between January 1, 2018 and July 31, 2022 were retrospectively identified in our center.Two radiology residents reviewed the exams independently. The elbow tendons were given a score from 0 to 4. Score 0: no signal abnormality; score 1: increased T2-weighted signal around the tendon; score 2: increased T2-weighted signal compared to muscle within the tendon; score 3: partial tear; and score 4: complete tear. The common flexor tendon showed signal alterations in 8% of patients; nine patients had an increased signal around the tendon, and eight patients had an increased signal within the tendon. Three patients (1.5%) had an altered signal intensity in the biceps tendon. All triceps tendons showed a linear hyperintense signal, suggesting that it is physiological. There were no partial or complete tears. No signal abnormalities were noted in the brachialis tendon among all patients. The prevalence of presumable asymptomatic signal alterations seen in the common flexor origin on MRI is not negligible; therefore, clinical correlation is advised to prevent overtreatment of tendinopathy in these cases. No partial or complete tears were seen.

Machine Learning Algorithm: Texture Analysis in CNO and Application in Distinguishing CNO and Bone Marrow Growth-Related Changes on Whole-Body MRI.

The purpose of this study is to analyze the texture characteristics of chronic non-bacterial osteomyelitis (CNO) bone lesions, identified as areas of altered signal intensity on short tau inversion recovery (STIR) sequences, and to distinguish them from bone marrow growth-related changes through Machine Learning (ML) and Deep Learning (DL) analysis. We included a group of 66 patients with confirmed diagnosis of CNO and a group of 28 patients with suspected extra-skeletal systemic disease. All examinations were performed on a 1.5 T MRI scanner. Using the opensource 3D Slicer software version 4.10.2, the ROIs on CNO lesions and on the red bone marrow were sampled. Texture analysis (TA) was carried out using Pyradiomics. We applied an optimization search grid algorithm on nine classic ML classifiers and a Deep Learning (DL) Neural Network (NN). The model's performance was evaluated using Accuracy (ACC), AUC-ROC curves, F1-score, Positive Predictive Value (PPV), Mean Absolute Error (MAE) and Root-Mean-Square Error (RMSE). Furthermore, we used Shapley additive explanations to gain insight into the behavior of the prediction model. Most predictive characteristics were selected by Boruta algorithm for each combination of ROI sequences for the characterization and classification of the two types of signal hyperintensity. The overall best classification result was obtained by the NN with ACC = 0.91, AUC = 0.93 with 95% CI 0.91-0.94, F1-score = 0.94 and PPV = 93.8%. Between classic ML methods, ensemble learners showed high model performance; specifically, the best-performing classifier was the Stack (ST) with ACC = 0.85, AUC = 0.81 with 95% CI 0.8-0.84, F1-score = 0.9, PPV = 90%. Our results show the potential of ML methods in discerning edema-like lesions, in particular by distinguishing CNO lesions from hematopoietic bone marrow changes in a pediatric population. The Neural Network showed the overall best results, while a Stacking classifier, based on Gradient Boosting and Random Forest as principal estimators and Logistic Regressor as final estimator, achieved the best results between the other ML methods.

A Case Study on Cardiovascular Embolism and Its Impact on Facial Recognition

This case study presents an 84-year-old man patient with cardio-genic cerebral embolism in the right middle artery territory, who showed difficulty in recognizing faces and often mistook unfamiliar people for someone he knew. He was fully conscious, with normal visual acuity, and no hemianopia, neglect, and deafness. Results from a neuropsychological examination indicated visual agnosia for prosopagnosia. An MRI showed hyperintense signals from the frontal to temporal lobes on the right side. Initially suspected to be delusional misidentification syndrome, the patient’s case differed from Fregoli syndrome as he identified unfamiliar people as persons close to him. This case highlights the importance of careful diagnosis and assessment in patients with visual agnosia for prosopagnosia, which can have significant impact on daily life and functioning.

Detection of pulmonary thrombembolism and postmortem clotting on postmortem magnetic resonance imaging

The purpose of this study was to develop a feasible imaging protocol superior to postmortem computed tomography (PMCT) and to establish diagnostic parameters for diagnosing pulmonary thromboembolism (PE) on postmortem magnetic resonance imaging (PMMR).The study collective of 113 subjects was prospectively investigated by PMMR for the presence of PE and / or postmortem clotting (cruor). PE was detected in 20 cases; the remaining 93 cases were investigated for the morphology of cruor. Age grading was performed by PMMR, autopsy and histology. The postmortem sedimentation effect was used for the applied imaging protocol on PMMR (supine and prone position). Visual distension of the pulmonary arteries in PE was seen in all cases, but not in the controls. Re-positioning of the corpse from supine in prone position proved to be beneficial in 90 %. Postmortem motion artifacts are firstly described in 20.4 %. Hyperacute PE (grade 1) presented with a homogenous and hypointense signal on T2w images, acute PE (grade 2) with slightly heterogeneous, but still homogenous hypointense signal, subacute PE (grade 3) was with heterogeneous and slightly hyperintense signal and chronic PE (grade 4) with predominately homogenous with scarce portions of heterogeneous but hyperintense signal. PMMR allowed for the detection of PE and for in situ depiction of combined age grading.

Delayed Gadolinium Leakage in Ocular Structures on Brain MR Imaging: Prevalence and Associated Factors.

Gadolinium leakage in ocular structures (GLOS) is characterized by hyperintense signal in the chambers of the eye on FLAIR and has been reported in association with blood-ocular barrier breakdown in patients with ischemic strokes. The underlying mechanism of GLOS remains poorly understood; however, some studies suggest it may be part of a physiologic excretion pathway of gadolinium. This study aimed to determine the prevalence of GLOS in an unselected patient population. A retrospective analysis was conducted on 439 patients who underwent brain MR imaging within 7 days of receiving a gadolinium-based contrast agent injection for a prior MR imaging study. Clinical, imaging, and laboratory data were collected. Descriptive and logistic regression analyses were performed. GLOS was observed in 26 of 439 patients (6%). The occurrence of GLOS varied with time, with 3 (12%), 14 (54%), 8 (31%), and 1 (4%) patient showing GLOS within 24, 25-72, 73-120, and >120 hours after gadolinium-based contrast agent injection, respectively. Patients with GLOS were older (median age: 72 versus 55 years, P = .001) and had higher median serum creatinine levels (73 versus 64 µmol/L, P = .005) and a lower median estimated glomerular filtration rate (84 versus 101 mL/min/1.73 m2, P < .001). A shorter median time interval between gadolinium-based contrast agent injection and the index brain MR imaging was observed in the group positive for GLOS (62 versus 91 hours, P = .003). Multivariable regression analysis identified the estimated glomerular filtration rate (OR = 0.970; 95% CI, 0.049-0.992; P = .008) and time interval since gadolinium-based contrast agent injection (OR = 0.987; 95% CI, 0.977-0.997; P = .012) as independent factors associated with GLOS. GLOS was observed in only a small percentage of patients receiving gadolinium-based contrast agent within 7 days before brain MR imaging. This phenomenon was noted in patients with normal findings on brain MR imaging and those with various CNS pathologies, and it was associated with lower estimated glomerular filtration rates and shorter time intervals after gadolinium-based contrast agent injection. While GLOS may be a physiologic gadolinium-based contrast agent excretion pathway, the presence of ocular disease was not formally evaluated in the included population. Awareness of GLOS is nonetheless useful for appropriate radiologic interpretation.

Experimental replacement of various bladder volumes with allogeneic tissue-engineered constructions

The results of experimental replacement of the bladder wall up to subtotal using multicomponent tissue-engineered structures are presented. Purpose. Development and experimental use of a tissue-engineered structure for replacing various volumes of the bladder wall. Material and methods. The original poly-L,L-lactide matrix is reinforced with silk fibroin. Mesenchymal cells were introduced into the constructs. 6 intact animals underwent filling cystometry. The maximum cystometric capacity was 11.2±0.97 ml. In these same 6 animals, the anesthetic capacity of the bladder was measured, which was 23.83±0.71 ml. 36 animals underwent reconstruction of the bladder using a prepared tissue-engineered construct after resection of the corresponding volume of the organ. Groups of 9 animals received bladder volumes of 5, 10, 15 and 20 ml. The observation period was 3 months. Results: According to computed tomography of the abdominal and pelvic organs (native study and with intravesical administration of a radiocontrast agent), 4, 8, 12 weeks after surgery, a bladder of physiological capacity is determined in all study groups, the implanted structure is visualized as a hyperintense signal in area of the apex of the bladder. no leakage of contrast agent is detected. Filling cystometry in 2 animals that underwent replacement of 20 ml of bladder volume (subtotal replacement) after 12 weeks showed that the capacity of the formed reservoir correlates with preoperative parameters. Macroscopically, the anastomosis zone is consistent in all groups of animals, the tissue-engineered structure is determined at the implantation site, lysis of the structure is noted by 12 weeks of observation with the preservation of small residual fragments at the implantation site. Conclusion. The experimental use of the developed tissue-engineered multicomponent structure turned out to be effective for replacing defects of the bladder wall of various volumes up to subtotal reconstruction. Further study of technologies for the use of tissue-engineered allogeneic constructs can significantly improve the results of treatment of urological pathologies for which obtaining autologous material is not possible.

Magnetic resonance imaging shows spinal curvature in Chinook salmon (Oncorhynchus tshawytscha) is associated with chronic inflammation of peri-vertebral soft tissues.

Chinook salmon (Oncorhynchus tshawytscha) farmed in New Zealand are known to develop abnormal spinal curvature late in seawater production. Its cause is presently unknown, but there is evidence to suggest a neuromuscular pathology. Using magnetic resonance imaging (MRI), we evaluated the relationship between soft tissue pathology and spinal curvature in farmed Chinook salmon. Regions of interest (ROIs) presenting as pathologic MRI signal hyper-intensity were identified from scans of 24 harvest-sized individuals: 13 with radiographically-detectable spinal curvature and 11 without. ROIs were excised from individuals using anatomical landmarks as reference points and histologically analysed. Pathologic MRI signal was observed more frequently in individuals with radiographic curvature (92%, n = 12) than those without (18%, n = 2), was localized to the peri-vertebral connective tissues and musculature, and presented as three forms: inflammation, fibrosis, or both. These pathologies are consistent with a chronic inflammatory process, such as that observed during recovery from a soft tissue injury, and suggest spinal curvature in farmed Chinook salmon may be associated with damage to and/or compromised integrity of the peri-vertebral soft tissues. Future research to ascertain the contributing factors is required.

White matter hyperintensities in behavioral variant frontotemporal dementia and semantic variant of primary progressive aphasia

AbstractBackgroundWhite matter hyperintensities (WMHs) are imaging abnormalities of white matter noted by hyperintense signals on fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI). When present among patients with CVRF (hypertension, diabetes, hypercholesteremia and smoking), WMHs are interpreted as cerebral vascular changes. However, WMHs in older individuals can be a feature of late onset leukodystrophies or varied neurodegenerative diseases such as Alzheimer disease (AD), Parkinson’s disease, and Frontotemporal Lobar Degeneration (FTLD). Additionally, WMHs can be present before symptom onset in neurodegenerative disorders and have been linked to the presence of some mutations, including progranulin (GRN). A clearer understanding of the underling etiology of WMHs in bvFTD and svPPA may improve management and treatment considerations.MethodsIn this cross‐sectional study, we included all participants meeting research diagnostic criteria for bvFTD (n = 152) and svPPA (n = 49) from ongoing studies at the UCSF Memory and Aging Center between the dates of September 2008 and December 2021 in comparison to a group of healthy controls enrolled at the same center with similar imaging parameters (n = 152, 1:1 matching for age and sex). All participants underwent a 3T FLAIR MRI and automated quantification of WMHs was performed. Linear regression analyses were performed to determine if WMHs were more frequent in participants with bvFTD or svPPA in comparison to controls and to explore associations with CVRF.ResultsAfter adjusting for age, sex, apolipoprotein E4 (APO‐E 4) status, and intracranial volume (ICV), both groups demonstrated a higher burden compared to controls (bvFTD (p = 0.012, R2 = 0.146) and svPPA (p = 0.008, R2 = 0.329). Modeling cardiovascular risk factors (CVRFs) as none or at least one, we did not find an association between CVRFs and WMH volume in models adjusting for ICV, ApoE4, and age among those with svPPA and bvFTD combined (p = 0.450).ConclusionIndividuals with bvFTD and svPPA appear to face a greater burden of WMHs compared to age‐ and sex‐matched healthy controls. WMHs in bvFTD and svPPA do not appear to be related CVRF.

Comparison of Imaging Characteristics of Gangliogliomas between Child/Adolescent Group and Adult Group.

Ganglioglioma is a rare, slowly proliferating mixed glioneuronal tumor, with the highest incidence observed in children and young adults, but it can also occur in adults. This study aimed to compare the imaging characteristics of ganglioglioma in children/adolescents and adults to facilitate radiographic diagnosis. In this retrospective study, a total of 32 patients were included and divided into two groups: the child/adolescent group (age < 18 years, n=19) and the adult group (age ≥ 18 years, n=13). Various variables were analyzed, including maximum diameter, location, periphery, border, calcification, unenhanced CT attenuation, T1WI, T2WI/FLAIR, and DWI signal intensity, enhancement pattern, degree of enhancement, homogeneity of enhancement, solid/cystic component, peri-tumoral edema, intra-tumoral septa, peri-tumoral capsule, and intra-tumoral hemorrhage. Most gangliogliomas were situated in the peripheral regions, particularly in the temporal lobe. The majority exhibited hypointense/isointense signals on T1WI and hyperintense signals on T2WI/FLAIR and DWI, with predominantly heterogeneous nodular enhancement. Peri-tumoral edema was significantly less frequent in the child/adolescent group, while marked enhancement was significantly more common in the adult group. There was no significant difference in maximum diameter between the child/adolescent group and the adult group. Peri-tumoral edema was significantly less prevalent in the child/adolescent group, whereas marked enhancement was significantly more frequent in the adult group. To ensure accurate results, a larger case series should be conducted to validate our findings.

SURG-20. USEFULNESS OF PHOTODYNAMIC THERAPY (PDT) WITH TALAPORFIN SODIUM FOR GLIOBLASTOMA

Abstract BACKGROUND Photodynamic therapy (PDT) with talaporfin sodium uses the toxicity of singlet oxygen generated by a reaction between a photosensitizer in tumor cells and light with a specific wavelength. PDT was introduced in our hospital in April 2017. We retrospectively analyzed the glioblastoma (GBM) patients treated with PDT and compared with the patients who did not receive PDT. MATERIAL AND METHODS From April 2015 to March 2023, total 145 patients with GBM were treated in our hospital. Among them, patients of recurrence, IDH mutant, biopsy and no chemo/radiotherapy were excluded. The patients’ characteristics, adverse event, changes of the MR image, recurrence pattern, outcome of the patients were analyzed. In addition, survival time of the patients between the PDT group and the non-PDT group was compared. RESULTS A total of 80 GBM patients were analyzed. Forty-four patients received PDT. 4 patients had adverse event; cyst formation, venous infarction, brain swelling, and delirium. In most of the patients, linear high signals of 5-7 mm on DWI were detected at the surface of the resected cavity and matched the PDT-irradiated area from 1 day after PDT. All hyperintense signal on DWI disappeared in about 30 days without any neurological deterioration. Sixty-seven patients had recurrence (PDT group; 33 cases, non-PDT group; 34 cases). Recurrence pattern of distant/dissemination were significantly increased in the PDT group compared with the non-PDT group. The PDT group tended to have longer survival in both OS (p = 0.08) and PFS(p = 0.07) than the non-PDT group. In multivariate analysis for OS, age and MGMT promotor methylation were significant prognostic factors, but not PDT. CONCLUSION PDT was safety and useful for GBM. However, the PDT group had significantly more distant/disseminated recurrences than the non-PDT group. We need to analyze more cases to clarify efficacy and recurrence patterns.

CTNI-63. A PHASE IIA CLINICAL TRIAL FOR COMBINATION OF NAVIGATION-GUIDE FOCUSED ULTRASOUND AND BEVACIZUMAB FOR PATIENTS WITH RECURRENT GLIOBLASTOMA

Abstract INTRODUCTION Bevacizumab (BEV), a vascular normalizing therapy that prolongs progression free survival (PFS) for patients with recurrent glioblastoma (rGBM). Focused ultrasound combines with microbubbles (MB-FUS), an emergent technology for targeted blood-brain barrier opening (BBBO), is under investigation for its combinatorial effect with current standard of care for GBM treatment. This study aims to evaluate the safety and efficacy of BEV + MB-FUS for treating patients with rGBM. METHODS This is a phase IIa, open-label, single-arm clinical trial (NCT04446416). Eligible patients received BEV (10 mg/kg), then MB-FUS with a MB dosage of 0.1 ml/kg and neuronavigation-guided FUS system (NaviFUS-001, NaviFUS, Taiwan). The MB-FUS is planned to be an add-on modality to the bi-weekly BEV therapy for up to 18 sessions (FUSBEV, 34 weeks). RESULTS Six patients underwent total 156 FUS-BEVs (26 times per patients). Three patients (50%) completed the trial protocol, 2 of another 3 patients had disease progression during the treatments, the remaining one patient was in stable disease. The mean progression free survival was 7.5 months. Among the 156 sessions of MB-FUS, there were 3 (1.9%) transient focal scalp pain related to heating. An evaluation of the MB-FUS penetrated regions revealed a significant normalization effect of T2 signal hyperintensity at beam-concentrated areas. CONCLUSIONS The PFS-6 was 67% in this study. RESULTS have demonstrated the safety, feasibility, and a potential to decrease edema by the mechanism of enhance bevacizumab delivery to the treated site. The preliminary results are encouraging. More data are needed to proof the efficacy of BEV-FUS on rGBM patients.

Abstract 17755: Post-Cardiac Injury Syndrome From Robotic Atrial Septal Defect Closure: A Case of Recurrent Pericarditis

Case Presentation: A 23-year-old female rugby player presented for recurrent chest pain. She has a history of atrial septal defect (ASD) repaired with robotic ASD closure. She developed acute pericarditis postoperatively and was treated with naproxen and colchicine inpatient. Her subsequent recurrences occurred with playing rugby and were treated with ibuprofen, colchicine, and prednisone. Electrocardiogram showed sinus bradycardia at 59 beats per minute but without ST segment changes. Cardiac magnetic resonance (CMR) imaging showed mild-to-moderate circumferential pericardial late gadolinium enhancement (LGE) without signal hyperintensity on T2 STIR imaging. She was diagnosed with chronic recurrent pericarditis secondary to post-cardiac injury syndrome (PCIS) and was started on rilonacept. On follow-up after 1 year, the patient remained symptom-free. She gradually increased her activity level to walking 3 miles per day and weaned down to rilonacept monotherapy. Repeat CMR showed mild pericardial LGE that was improved from before. Discussion: PCIS is a known cause of pericarditis due to local and systemic inflammatory responses to cardiothoracic surgery. Robotic ASD closure is a minimally invasive endoscopic procedure that has a favorable safety profile; PCIS is not a previously documented complication. Treatment of pericarditis secondary to PCIS includes NSAIDs plus 3 months of colchicine for acute pericarditis, NSAIDs plus 6 months of colchicine for a first recurrence, with corticosteroids or interleukin-1 inhibitors added for further recurrences. Incomplete treatment of acute pericarditis, as in this patient's case, is a risk factor for recurrent pericarditis. The risk of recurrences with physical activity is debated and current guidelines recommend restriction from physical activity until there is no evidence of active disease, though the data is not robust. In clinical practice, a gradual return-to-play program is frequently utilized.

Effectiveness of Low Dose D-Penicillamine Therapy in Neurologic Wilson Disease - A Prospective Observational Study

Background: Wilson disease is an inherited disorder of copper metabolism that mostly manifests as hepatic and neurologic symptoms. Chelation therapy specially penicillamine is given as first line treatment in children with symptomatic Neurologic Wilson disease. Objective: Objective of the study was to assess the safety &amp; the clinical outcome of treatment with low dose penicillamine in Neurologic Wilson disease. Methods: A longitudinal observational study was conducted at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, a tertiary care Premier Postgraduate Medical Institution of Bangladesh. Thirty-nine (39) patients of Neurologic Wilson disease who fulfill the inclusion and exclusion criteria were evaluated at In-patient Department of Paediatric Neurology, during the period of January 2015 to December 2019. All study children were treated with low dose penicillamine (Cap Artamin 250 mg) rather with conventional dose penicillamine (Cap Artamin 500 mg or 20 mg/kg/day). Subsequent follow up examination was performed at 2 weeks, 1, 3 and 6 months. Follow up was done by global assessment scoring (GAS) and slit lamp examination to see the clinical improvement after treatment with low dose penicillamine therapy. Results: Total number of studied cases were 39. Mean age was 10.2 ± 3.1 year and male to female ratio was 2:1. Most of the patients (66.67%) were arrived from rural area and 20.51% children had history of consanguineous mating parents. Common presenting features were progressive deterioration of school performance (89.74%), gait disturbance (92.31%), dysarthria (92.31%) and dystonia (82.06%) of our studied children. Ophthalmological manifestations like KF ring (100%) found in all patients. Neuroimaging showed bilateral basal ganglia involvement in (63.63%) children followed by hyperintense signal changes (18.18%) and ventricular dilatation in (18.18%) of cases. Majority of the children (74.36%) were improved with low penicillamine therapy clinically and KF ring disappeared in (5.12%) cases after drug therapy on follow up. Commonest side effects were worsening of neurological symptoms in (25.64%) and rash &amp; thrombocytopenia in (5.1%) cases after penicillamine therapy. Conclusion: About three-fourth children of studied Neurologic Wilson disease showed gradual improvement with low-dose penicillamine therapy. Moreover, one-fourth of cases experienced neurological deterioration, which was lower than previously used high dose penicillamine therapy. Therefore, low dose penicillamine may be beneficial as an initial therapy for Wilson disease with neurological manifestations.

Adenoid cystic carcinoma of head and neck: Summary and review of imaging findings

PurposeCurrent reports of adenoid cystic carcinoma of the head and neck (ACC) are all case reports, and there is no basilar summary of its imaging findings. This study aims to summarise ACC's computed tomography (CT) and magnetic resonance imaging (MRI) findings to improve radiologists' knowledge of this disease. MethodsWe collected clinical and imaging data of patients with ACC during the last decade, and two radiologists retrospectively analysed the imaging characteristics. ResultsOf the 16 patients included, six were able to self-perceive bulkiness, and 11 had regional pain. Tumour morphology was regular in six cases, with clear borders in 11 cases, invasion of the surrounding bony mass in 12 cases, and invasion of peripheral nerves in 15 cases. CT mostly shows an irregular soft-tissue density mass with mild-to-moderate enhancement after contrast medium administration. On MRI, the ACC showed isointense or hypointense signals on T1-weighted images (T1WI) and hyperintense or slightly hyperintense signals on T2-weighted images (T2WI). All signals were markedly enhanced after gadolinium enhancement. ConclusionsACC often has an irregular morphology, sometimes with a cystic component, enhancement on enhancement scans, easy destruction of adjacent bone, and invasion of peripheral nerves. The diagnosis should be considered when these features are encountered in clinical practice.

Simple quantitative planimetric measurement of nigrosome-1 for clinical settings

IntroductionLoss of MRI hyperintense signal in nigrosome-1 (assessed with susceptibility-weighted imaging) is a biomarker for Parkinson's disease (PD). Current clinical practice involves subjectively rating the appearance of nigrosome-1 which is challenging. The study aimed to test and compare a simple method for quantifying nigrosome-1 with the current subjective rating method. MethodsTwo experienced neuroradiologists measured area of hyperintense signal in nigrosome-1 (quantitative method) and rated nigrosome-1 appearance (as normal, attenuated, or absent; subjective method) in 42 patients encompassing the full spectrum of nigrosome-1 integrity (21 patients aged 55.5 ± 20.9 years with Essential tremor (ET) and a subset of 21 patients aged 69.6 ± 8.6 years with PD). Neuroradiologists were blinded to each other's measurements, clinical notes, and patient group. ResultsBoth methods yielded a significant difference between the groups (PD vs ET; p < 0.001). Pooled (across sides) area of nigrosome-1 hyperintense signal was significantly smaller in the PD group (median = 2.1 mm2, range = 0–15.8 mm2) than ET group (median = 8.3 mm2, range = 0–15.7 mm2; p < 0.001). Inter-rater reliability was high to very high for both methods (subjective: weighted kappa = 0.640, p < 0.001; quantitative: W = 0.733, p = 0.004). Our primary hypothesis that area of nigrosome-1 hyperintense signal exhibits higher inter-rater reliability than subjective rating of nigrosome-1 appearance was not supported. ConclusionThe simple quantitative method, used with subjectively rated nigrosome-1 appearance, may improve confidence in longitudinal clinical reporting, when nigrosome-1 is attenuated. However, further work on the incremental diagnostic value of planimetry and bias, repeatability and reproducibility are needed before it can be recommended in clinical practice.

Residual diffusion-weighted imaging hyperintense signal in primary central nervous system lymphoma can predict early recurrence.

The treatment response of primary central nervous system lymphomas (PCNSLs) is mainly evaluated using postcontrast T1-weighted imaging (T1WI). Because poorly enhanced lesions may contain residual tumors, the combination of evaluation methods will potentially improve the accuracy of determining treatment effectiveness. In this study, we evaluated the usefulness of diffusion-weighted imaging (DWI) in predicting recurrence among patients with PCNSL who achieved complete response (CR)/unconfirmed CR (CRu). Fifty-four patients newly diagnosed with PCNSL who were treated at our institution and achieved CR/CRu at the end of treatment were included in this study. The patients were divided into two groups according to the presence or absence of residual DWI hyperintense signal at the tumor site at the end of treatment. Kaplan-Meier analysis was performed to analyze the median overall survival (OS) and progression-free survival (PFS). The mean age of the 54 patients was 66.4 ± 13.3 years. The induction therapies were HD-MTX in 20 patients, R-MPV in 29 patients, and other chemotherapies in five patients. Radiotherapy was performed in 35 patients, high-dose cytarabine therapy in 14 patients, and autologous hematopoietic stem cell transplantation in one patient, and of the 54 patients, 10 had no consolidation therapy. The residual DWI hyperintense signal sign was observed in 18 patients. The R-MPV regimen was statistically associated with a lower rate of residual DWI hyperintense signal (p = 0.0453). The median PFS was statistically shorter in the residual DWI hyperintense signal group than in the non-residual DWI hyperintense signal group (14.0 months vs. 85.1 months) (p < 0.0001, log-rank test). A residual DWI hyperintense signal at the end of treatment was statistically associated with shorter PFS. Among patients who achieved CR/CRu evaluated based on postcontrast T1WI, DWI could be a valuable additional sequence to predict the early recurrence of PCNSL.