Gadolinium Enhancements Research Articles

Differences of spinal cord gadolinium enhancement features of neuromyelitis optica spectrum disorder and long-segment degenerative cervical myelopathy.

Neuromyelitis optica spectrum disorder (NMOSD) and long-segment degenerative cervical myelopathy (DCM) may have a similar appearance on MRI. This study aimed to identify the differences in spinal cord gadolinium enhancement features between NMOSD and long-segment DCM. Spinal cord gadolinium enhancement of 27 NMOSD patients and 30 long-segment DCM patients were retrospectively analyzed. Enhancements were evaluated for their number, length, location on the sagittal images, distribution on the axial images, and form on the sagittal images. The Wilcoxon rank sum test was performed to compare numerical variables. The Pearson chi-squared test was performed to compare categorical variables. The median number of enhanced lesions (p < 0.05), the median length of the enhancements (p < 0.05), and the location of enhancement on sagittal images (p < 0.05) of NMOSD patients and long-segment DCM patients showed significant differences. The axial distribution of enhancements did not show a significant difference between NMOSD and long-segment DCM patients (p = 0.115). On the sagittal images, linear and ring-formed enhancements were observed in 10 (27.0%) and 17 (63.0%) NMOSD patients, respectively. The enhancements in long-segment DCM patients had a transverse band or pancake-like appearance in 15 (50%) patients and an irregular flake-like appearance with a longitudinally oriented long axis in 15 patients (50%). By analyzing the number, length, location, and form of the gadolinium enhancements, NMOSD and long-segment DCM could be well-differentiated.

Characteristics and Prognosis of a Contemporary Cohort with Myocardial Infarction with Non-Obstructed Coronary Arteries (MINOCA) Presenting Different Patterns of Late Gadolinium Enhancements in Cardiac Magnetic Resonance Imaging.

To analyze the characteristics and prognosis of a contemporary cohort of patients with myocardial infarction with non-obstructed coronaries (MINOCA) were referred for cardiac magnetic resonance (CMR) imaging, focusing on late gadolinium enhancement (LGE) patterns. We retrospectively examined and prospectively followed up with 135 patients (49 ± 21 years old, 48% female) undergoing CMR imaging due to a MINOCA diagnosis from 2014 to 2016. We grouped and analyzed the sample according to ischemic (focal or transmural) and non-ischemic LGE patterns. The primary outcome was cardiac-related death; the secondary outcome was a composite of cardiac-related rehospitalizations, the new occurrence of acute myocardial infarction (AMI), heart failure (HF), or arrhythmias. CMR exams were performed after a median of 28 days from the acute event. One-third of the ischemic MINOCA were first managed as myocarditis, while CMR helped to adopt a different therapy regimen in 22% of patients (30/135). After a median follow-up of 2.3 years, more cardiac-related deaths occurred in the ischemic than non-ischemic group (2 vs. 1, p = 0.36), but it was not statistically significant. The ischemic group also experienced more cardiac-related-rehospitalizations (42%, p < 0.001). In a multivariable Cox regression model, dyslipidemia, reduced left ventricular ejection fraction, ST-elevation at the hospitalization, and the LGE transmural pattern were the independent predictors of cardiac-related rehospitalizations. In a contemporary cohort of MINOCA patients who underwent CMR, ischemic and non-ischemic patterns had distinct features and outcomes. Among the MINOCA patients, CMR can identify patients at higher risk who require more aggressive therapeutic approached and strict follow-up.

Assessment of cardiac involvement in patients recovered from corona virus disease-19 (COVID-19) infection using magnetic resonance imaging

Abstract Background Cardiac sequelae after recovery from COVID-19 infection has not been well-established. Recent studies have used cardiac magnetic resonance (CMR) imaging to assess myocardial involvement or ongoing myocarditis in patients with prior COVID-19 infection. Objectives The primary objective of this study was to identify the CMR imaging findings in adult COVID-19 recovered patients. Methods This was a single-center retrospective observational cohort study of adult COVID-19 recovered patients who underwent cardiac magnetic resonance imaging. Patient demographics, CMR findings, blood marker results, and treatment received were obtained. Results Of the 77 included patients, 42 (54.55%) were male, and the median age was 45 years. Only 6 patients had completely normal scans, making the prevalence of an abnormal CMR 92.21% (95% CI 83.5%–96.5%). All those with an abnormality demonstrated late gadolinium enhancement (LGE), and of these, 64 (90%) had preserved left ventricular ejection fraction (LVEF). Thirty-four patients (44%) had evidence of myocardial edema. Among patients with myocardial edema and LGE, the median numbers of involved segments were 4 (range 1–16) and 7 (range 1–15), respectively. Myocardial edema was most frequently found in the mid inferolateral segment (53%), followed by the basal inferior septum, basal anterior septum, and basal inferolateral segments (each with 41%). Meanwhile, late gadolinium enhancements were most commonly located in the basal inferior septum (75%), mid inferior septum (80%), and basal anterior septum (73%). Conclusion Cardiac involvement, particularly edema and late gadolinium enhancement, affecting multiple myocardial segments were observed in a considerable number of patients recovered from COVID-19. Funding Acknowledgement Type of funding sources: None.

Abstract TP185: Cardiac Gadolinium Enhancement And Cerebral Small Vessel Disease

Background: Accumulation of cerebral small vessel disease (CSVD) leads to various well described detrimental clinical consequences. Similar to CSVD, the development of coronary microvascular disease (CMD) yields end-organ dysfunction, namely myocardial ischemia and chamber dilation. We aimed to investigate the relationship between cardiac gadolinium enhancements (CGE), a marker for CMD, on CSVD burden on brain MRI. Methods: We performed a retrospective cross-sectional analysis of adults between January 2014 and December 2019 who completed a cardiac and brain MRI within one year of each other. The ordinal total small vessel disease score (tSVD) was used to quantify CSVD with two cohorts being created: tSVD 0 to 1 (low CSVD) and tSVD 2 to 4 (high CSVD). Cardiac MRI data was obtained under the guidance of an experienced cardiologist with advanced cardiac imaging training. Chi-squared or Fisher’s, Wilcoxon rank-sum analyses were performed for binary and ordinal variables, respectively. Adjusted multivariate logistic regression models were used to calculate odds ratios. Statistical significance was defined as &lt; 0.05. Results: A total of 259 individuals were included with 140 and 119 in the low and high CSVD groups, respectively. Those with low CSVD were significantly lower in age, predominately female and had lower rates of hypertension, hyperlipidemia, diabetes mellitus and congestive heart failure. CGE was more common in those with higher burdens of CSVD (23.2% vs 47.2%, p&lt;0.001). CGE increased the odds of being within the high CSVD group despite adjustment for significant clinical and demographic factors (OR 2.1, 95% CI 1.1 - 4.0). Discussion: In those with CMD as identified by CGE, there was a 2 fold increase in being within the high burden of CSVD cohort likely as a result of similar intrinsic pathological processes. Further studies are needed to determine whether CGE predates development of CSVD, its association with development of acute cerebrovascular diseases and its influence on specific subtypes of CSVD.

Prognostic Value of Late Enhanced Cardiac Magnetic Resonance Imaging Derived Texture Features in Dilated Cardiomyopathy Patients With Severely Reduced Ejection Fractions.

Background: Late enhanced cardiac magnetic resonance (CMR) images of the left ventricular myocardium contain an enormous amount of information that could provide prognostic value beyond that of late gadolinium enhancements (LGEs). With computational postprocessing and analysis, the heterogeneities and variations of myocardial signal intensities can be interpreted and measured as texture features. This study aimed to evaluate the value of texture features extracted from late enhanced CMR images of the myocardium to predict adverse outcomes in patients with dilated cardiomyopathy (DCM) and severe systolic dysfunction.Methods: This single-center study retrospectively enrolled patients with DCM with severely reduced left ventricular ejection fractions (LVEFs < 35%). Texture features were extracted from enhanced late scanning images, and the presence and extent of LGEs were also measured. Patients were followed-up for clinical endpoints composed of all-cause deaths and cardiac transplantation. Cox proportional hazard regression and Kaplan–Meier analyses were used to evaluate the prognostic value of texture features and conventional CMR parameters with event-free survival.Results: A total of 114 patients (37 women, median age 47.5 years old) with severely impaired systolic function (median LVEF, 14.0%) were followed-up for a median of 504.5 days. Twenty-nine patients experienced endpoint events, 12 died, and 17 underwent cardiac transplantations. Three texture features from a gray-level co-occurrence matrix (GLCM) (GLCM_contrast, GLCM_difference average, and GLCM_difference entropy) showed good prognostic value for adverse events when analyzed using univariable Cox hazard ratio regression (p = 0.007, p = 0.011, and p = 0.007, retrospectively). When each of the three features was analyzed using a multivariable Cox regression model that included the clinical parameter (systolic blood pressure) and LGE extent, they were found to be independently associated with adverse outcomes.Conclusion: Texture features related LGE heterogeneities and variations (GLCM_contrast, GLCM_difference average, and GLCM_difference entropy) are novel markers for risk stratification toward adverse events in DCM patients with severe systolic dysfunction.

353 SARS-CoV-2 pulmonary vasculitis and Takotsubo myocarditis syndrome. Hypoxia, endothelial dysfunction, and inflammation are the trigger?

353 SARS-CoV-2 pulmonary vasculitis and Takotsubo myocarditis syndrome. Hypoxia, endothelial dysfunction, and inflammation are the trigger?

Association of QRS-T angle and Late Gadolinium Enhancement in patients with a Clinical Suspicion of Myocarditis.

Objective: To investigate the association of a wide QRS-T angle on the surface ECG and late gadolinium enhancement on contrast-enhanced cardiovascular magnetic (CMR) imaging in patients with clinically suspected myocarditis.Background: Diagnosis and risk stratification in patients with suspected myocarditis is particularly challenging due to a great spectrum of clinical presentations. Late gadolinium enhancement (LGE) visualizes myocardial necrosis and fibrosis in patients with biopsy-proven myocarditis. The presence or absence of late gadolinium enhancements in these patients is prognostically meaningful. The QRS-T angle from the surface ECG, on the other hand, may serve as a simple and easily available risk marker in suspected myocarditis.Methods: We enrolled 97 consecutive patients that were referred to CMR imaging for a clinical suspicion of myocarditis. All patients obtained a standardized digital 12-lead ECG for the calculation of the QRS-T angle and underwent contrast-enhanced CMR imaging. Patients were divided into two groups according to the absence or presence of LGE on CMR.Results: 78 of 97 patients with suspected myocarditis had LGE on CMR. Patients with LGE had wider QRS-T angles as compared to the patient group without LGE (53.95-47.5 vs. 26.2-21.2; p<0.001). The sensivity, specificity, negative predictive value and positive predictive value for a QRS-T angle above 90 degrees for LGE positive myocarditis were 16.5%, 100%, 24.7%, and 100%, respectively.Conclusion: A wide QRS-T angle of 90 degrees or more is linked to myocardial fibrosis or necrosis (late gadolinium enhancement) in patients with suspected myocarditis.

The role of right ventricular late gadolinium enhancement scoring by cardiac magnetic resonance imaging in cardiac sarcoidosis

Abstract Background The right ventricular (RV) function is a significant predictor of the prognosis and exercises tolerance in patients with left ventricular dysfunction. The previous study reported that RV late gadolinium enhancements (LGE) as assessed by cardiac magnetic resonance (CMR) imaging were present in 16% of pulmonary sarcoidosis. Although RV LGE is associated with a poor prognosis, a semi-quantitative assessment of RV LGE was not been established so far. Moreover, the frequency of RV dysfunction (RVD) in cardiac sarcoidosis (CS) and predictors of RVD are unclear. Purpose Our aim of this study is to establish RV LGE semi-quantitative scores and to clarify the rate and predictors of RVD in CS. Methods Forty-four consecutive patients with CS who underwent CMR imaging (median age, 63 [IQR 54–71] years old; 10 males) were studied. They were diagnosed as definitive based on the Heart Rhythm Society expert consensus statement. Two patients were excluded due to no long axial view. Thus, a total of 42 patients were included in this study. RV LGE was semi-quantitatively evaluated using the original score developed by ours. RV was classified into RV outflow tract (OT), basal, middle and apex. RVOT and apex are assigned 1 segment for each, and basal and middle are assigned 4 segments include anterior, septal, inferior and lateral (Figure1). In total, 10 segments were evaluated. RV LGE scores were analysed independently by the two operators. The RV ejection fraction was assessed in a long axial view without an RVOT (20 phases, 12 slices). We divided the cohort into the non-RVD group (RVEF ≥45%) and the RVD group (RVEF &amp;lt;45%) and compared the RV scores between the two groups. Results The RVD was present in 31% of the entire cohort. The presence of RV LGE was significantly higher in the RVD group than the non-RVD group (83% vs. 43%, p=0.037). RV LGE score in the RVD group was significantly higher than that in the non-RVD group (2.83±1.95 vs. 0.83±1.21, p=0.001). The septal lesions in basal and middle levels accounted for 54% in all segments. RVEF showed a significant inverse correlation with RV LGE score (r=−0.57, p&amp;lt;0.0001). In the ROC curve for RVD, the RV LGE score of more than 2 and equal could discriminate RVD from non-RVD with AUC of 0.813 (Sensitivity = 0.77, Specificity = 0.76, p=0.003). Conclusion RVD was observed in 31% of patients with definitive CS. The septal lesions were the most frequent in the RV LGE site, which suggests that the longitudinal abnormal motion of RV is a significant predictor of the RVD in patients with CS. RV LGE score is a valuable method to assess RVD. RV LGE score Funding Acknowledgement Type of funding source: None

A Systematic Review of COVID-19 and Myocarditis

The COVID-19 infection which emerged in December 2019, is caused by the virus SARS-CoV-2. Infection with this virus can lead to severe respiratory illness, however, myocarditis has also been reported. The purpose of this study is to identify the clinical features of myocarditis in COVID-19 patients. A systematic review was conducted to investigate characteristics of myocarditis in patients infected with COVID-19 using the search term "Coronavirus" or "COVID" and "myocarditis," "heart," or "retrospective." Case reports and retrospective studies were gathered by searching Medline/Pubmed, Google Scholar, CINAHL, Cochrane CENTRAL, and Web of Science databases. 11 articles were selected for review. COVID-19 myocarditis affected patients over the age of 50 and incidences among both genders were equally reported. Patients presented with dyspnea, cough, fever with hypotension and chest pain. Laboratory tests revealed leukocytosis with increased C-reactive protein, while arterial blood gas analysis demonstrated respiratory acidosis. All cardiac markers were elevated. Radiographic imaging of the chest showed bilateral ground glass opacities or bilateral infiltrates, while cardiac magnetic resonance imaging produced late gadolinium enhancements. Electrocardiography demonstrated ST-segment elevation or inverted T waves, while echocardiography revealed reduced left ventricular ejection fraction with cardiomegaly or increased wall thickness. Management with corticosteroids was favored in most cases, followed by antiviral medication. The majority of studies reported either recovery or no further clinical deterioration. Current available data on COVID-19 myocarditis is limited. Further research is needed to advance our understanding of COVID-19 myocarditis.

En mann i 60-årene med gangvansker, dysartri og lett kognitiv svikt

BACKGROUND Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010). CASE PRESENTATION We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general condition. He had experienced dizziness and unstable gait for a year, and had been periodically confused, especially in the previous month. MR imaging showed characteristic punctuate and curvilinear gadolinium enhancements in the pons. Our patient was diagnosed with CLIPPERS and was given corticosteroid treatment, initially methylprednisolone intravenously and then prednisone orally. Other differential diagnoses, such as CNS lymphoma, high-grade glioma, CNS vasculitis, neurosarcoidosis, demyelinating disease, Bickerstaff brainstem encephalitis, and acute disseminated encephalomyelitis were ruled out. The patient's condition improved dramatically after corticosteroid treatment. INTERPRETATION In 2017, the diagnostic criteria for CLIPPERS were published. Based on these criteria we were able to diagnose this patient with possible CLIPPERS, consistent with clinical symptoms, MRI findings, absence of better explanations for the condition, and clinical and radiological improvement after treatment with corticosteroids. An unequivocal diagnosis of CLIPPERS can only be established by characteristic pathological findings.

Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern.

We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement. In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms). A good functional outcome was defined as a modified Rankin scale ⩽2 at last follow-up, regardless of the occurrence of relapse. Among the 102 patients in the cohort, 26 (25%) had isolated small-vessel PACNS, whereas the 76 others demonstrated large/medium-vessel involvement. Patients with isolated small-vessel PACNS had more seizures (P < 0.0001), cognitive (P = 0.02) or consciousness impairment (P = 0.03) and more dyskinesias (P = 0.002) but less focal deficits (P = 0.0002) than other PACNS patients. They also had more abnormal cerebrospinal fluid analysis (P = 0.008) and gadolinium enhancements on MRI (P = 0.001) but less frequent acute ischaemic lesions (P < 0.0001) than patients with large/medium-vessel involvement. Treatments and modified Rankin scale at last follow-up did not differ between groups. Thirty-two (31%) patients relapsed; 14 (54%) with isolated small-vessel PACNS vs 18 (24%) with large/medium-vessel involvement (P = 0.004). Eight patients died, with no difference between the groups (P = 0.97). In our cohort, adult patients with isolated small-vessel PACNS presented some distinct disease features and relapsed more often than other PACNS patients who had large/medium-vessel involvement. Functional outcomes and mortality did not differ.

CLIPPERS with diffuse white matter and longitudinally extensive spinal cord involvement

Zhang et al.1 reported confluent lesions in fluid-attenuated inversion recovery (FLAIR) involving the centrum semiovale matching with subcortical punctate and curvilinear gadolinium enhancements (PCGE). PCGE in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) invariably involve the brainstem and could spread rostrally up to the juxtacortical areas and caudally up to the conus. FLAIR lesions present in the corresponding areas of PCGE are usually faint and indicate a blood–brain barrier disruption. In our case series, we also described confluent lesions …

Punctate and curvilinear gadolinium enhancing lesions in the brain: a practical approach.

Cerebral punctate and curvilinear gadolinium enhancements (PCGE) correspond to opacification of small vessel lumen or its perivascular areas in case of blood-brain barrier (BBB) disruption. We will discuss the possible causes of intra-parenchymal central nervous system PCGE. Our review is based on French database including patients presenting with central nervous system PCGE and literature search using PubMed database with the following keywords: punctate enhancement, linear enhancement, and curvilinear enhancement. Disorders which displayed linear leptomeningeal or periventricular enhancements without intra-parenchymal PCGE are excluded of this review. Among our 39 patients with PCGE, 16 different diagnoses were established. After combining our PCGE causes with those described in the literature, we propose a practical approach. Besides physiologic post-contrast enhancement of small vessels, three pathologic conditions may exhibit PCGE: (1) small collateral artery network seen in Moyamoya syndrome, (2) small veins congestions related to developmental or acquired venous outflow disturbance, and (3) disorders causing small vessels BBB disruption indicated by T2 and FLAIR hyperintensities in the corresponding areas of PCGE. Disruption of the BBB could be caused by a direct injury of the endothelial cell, as in posterior reversible encephalopathy syndrome, Susac syndrome, and radiochemotherapy-induced injuries, or by an angiocentric cellular infiltrate, as in inflammatory disorders, demyelinating diseases, host immune responses fighting against infections, prelymphoma states, lymphoma, and in CLIPPERS. PCGE may conceal several causes, including physiological and pathological conditions. Nevertheless, a practical approach could improve its management and limit the indications of brain biopsy to very specific situations.

Abstract 19548: Comparison of a Novel Four Dimensional CT Myocardial Strain Analyzing Technique With MRI, TTE, and Endomyocardial Biopsy

Introduction: Myocardial strain is a new indicator of myocardial characteristics and function, mainly evaluated by transthoracic echocardiography (TTE) or magnetic resonance imaging (MRI) techniques that rely on dynamic two dimensional images. Hypothesis: We hypothesized that utilizing the full capability of four dimensional (4D) volumetric 320 slice CT image data with retrospective ECG gating we could provide additional information on myocardial morphology, characteristics and function on Novel 4D CT myocardial stain analysis (developed with PhyZioDynamics, Zio). Method: We retrospectively analyzed ten subjects with various rare and un- typical myocardial diseases diagnosed by 320 slice CT using retrospective ECG gating and dose modulation. Some diagnoses were confirmed using MRI, TTE or endomyocardial biopsy (EMB). 4D CT myocardial strain analysis confirmed their diagnosis (see fig). Areas with maximum strain are colored red and decreasing strain, yellow, green, blue, and finally no color (=no strain). Results: Case 1 had a large, idiopathic fat deposit in the left ventricle (LV) that could be observed using enhanced CT with a low CT attenuation area. On 4D CT strain analysis, the fat deposit showed no strain. Case 2 had cardiac amyloidosis confirmed by EMB and Case 3 had type 2 hypertrophic cardiomyopathy (HCM). MRI showed late gadolinium enhancements (LGE) in both cases. At corresponding sites, no strain was observed by 4D CT. Other cases analyzed were dilated cardiomyopathy with focal LGE on MRI, subendocardial myocardial infarction with enlarged LV myocardium, etc. Conclusion: This is the first report worldwide of a 4D CT myocardial strain analyzing technique that utilizes full volumetric data. Our new technique uses ordinary cardiac CT data acquired with retrospective ECG gating, to provide detailed additional information on myocardial characteristics and function in addition to LV ejection fraction volume data and information on coronary arteries.

Primary neurolymphomatosis diagnosis and treatment: A retrospective study

BackgroundTo discuss the therapeutic approach for primary neurolymphomatosis. MethodsWe report all primary neurolymphomatosis cases referred to our institution, with descriptions of clinical, radiological, electrophysiological, histological features and long-term follow-up. We treated all patients with a combination of high-dose methotrexate and alkylating agents. ResultsFive patients were diagnosed with histologically confirmed primary neurolymphomatosis. The majority of them presented with painful asymmetric sensory–motor neuropathy. Magnetic resonance imaging was abnormal in 4 of 5 patients, as shown with gadolinium enhancements. Electroneuromyography revealed denervation in all 4 cases with contributive examinations. All our patients received a chemotherapy combination of high-dose methotrexate and alkylating agent. Median progression-free survival was 8months (2 complete responses and 2 partial responses), and overall survival was 24months. ConclusionsPrimary neurolymphomatosis is rare and polymorphic; it represents a difficult diagnosis of neuropathy. In our cohort, treatment with a chemotherapy combination with high-dose methotrexate showed encouraging results.

Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system.

To describe characteristics and outcomes of a multicenter cohort of patients diagnosed as having primary angiitis of the central nervous system (PACNS). In 2010, we initiated a cohort study of adults diagnosed as having PACNS ≤15 years ago and with followup of >6 months (unless they died earlier of biopsy-proven PACNS). Its first analysis was planned at 2 years. Multidisciplinary investigators verified that appropriate investigations were done and excluded patients with possible alternative diagnoses. We analyzed patient demographics and symptoms, laboratory, radiographic, and histologic findings, and treatments. Studied outcomes included treatment response(s), relapse, death, and disability. We included 52 patients (30 males; median age at diagnosis 43.5 years [range 18-79 years]) in whom PACNS was diagnosed between 1996 and 2012. Nineteen (61%) of 31 patients who had undergone brain biopsy had histologic vasculitis (biopsy-proven PACNS), while the other 12 patients had normal or noncontributive biopsy samples. An additional 21 patients had signs suggestive of PACNS on conventional cerebral angiography. All but 1 patient received corticosteroids, and 44 patients received cyclophosphamide (CYC). After a median followup of 35 months (range 2-148 months) postdiagnosis (1 patient with biopsy-proven PACNS died 2 months after diagnosis), 32 patients responded to treatment with improved modified Rankin scale scores, 4 patients (8%) did not respond, 14 patients (27%) had relapse of their disease at least once, and 3 patients (6%) died (1 patient after a relapse). Relapse was more common in patients with than in those without meningeal gadolinium enhancements on magnetic resonance imaging (MRI) (8 of 10 [80%] versus 6 of 32 [19%]; P = 0.001) and more common in patients with than in those without seizures at diagnosis (8 of 17 [47%] versus 6 of 35 [17%]; P = 0.04). In this cohort of patients with PACNS, most patients received corticosteroids and CYC, and mortality was low. Patients with seizures at diagnosis or meningeal enhancements on MRI may be prone to relapse and require a different treatment strategy.

Ingested Type I Interferon-State of the Art as Treatment for Autoimmunity Part 2.

We have proposed a unifying hypothesis of the etiopathogenesis of autoimmunity that defines autoimmunity as a type I interferon (IFN) immunodeficiency syndrome. We have examined toxicity and potential efficacy in two phase I (type 1 diabetes [T1D], multiple sclerosis [MS]) and phase II clinical trials in T1D and MS. In a phase I open label trial in T1D, ingested IFN-alpha preserved residual beta-cell function in recent onset patients. In a second phase I trial in MS, there was a significant decrease in peripheral blood mononuclear cell IL-2 and IFN-gamma production after ingesting IFN-alpha. In a phase II randomized, placebo-controlled, double-blind trial in MS, 10,000 IU ingested IFN-alpha significantly decreased gadolinium enhancements compared to the placebo group at month 5. TNF-alpha and IFN-gamma cytokine secretion in the 10,000 IU group at month 5 showed a significant decrease that corresponded with the effect of ingested IFN-alpha on decreasing gadolinium enhancements. In a phase II randomized, placebo-controlled, double-blind trial in T1D, patients in the 5,000 unit hrIFN-alpha treatment group maintained more beta-cell function one year after study enrollment compared to individuals in the placebo group. Ingested IFN-alpha was not toxic in these clinical trials. These studies suggest that ingested IFN-alpha may have a potential role in the treatment of autoimmunity.

SYPHILITIC MYELITIS: RARE, NONSPECIFIC, BUT TREATABLE

Syphilis outbreaks have re-emerged throughout the world, particularly among homosexual men.1 Syphilitic myelitis is a rare manifestation of syphilis and a rare cause of myelopathic syndromes in general. We report a patient with complete clinical and radiologic recovery following treatment. As the clinical presentation was nonspecific, only serologic testing revealed the diagnosis. ### Case report. A 46-year-old man was admitted with a 7-day history of progressive genital and sacral numbness, pain in the groin, without motor or autonomic dysfunction. Examination documented hypoalgesia and thermhypesthesia, normal sphincter tone, and pyramidal signs with hyperreflexia of the legs. Spinal MRI revealed swelling and high signal intensity of the central portion of the spinal cord parenchyma below T6 on T2-weighted images and two focal gadolinium enhancements (figure). Brain MRI was normal. CSF examination showed 113 mononuclear cells/μL, 0.72 g/L protein, and normal glucose and lactate levels. Antinuclear antibodies, antineutrophil cytoplasmic antibodies, and rheumatic factor were negative. The Treponema pallidum hemagglutination test and the Venereal Disease Research Laboratory (VDRL) test were positive with 1:81,920 (<1:80) and 1:64 (<1:2). A positive intrathecal Treponema pallidum antibody (iTPA) index confirmed neurosyphilis. Active HIV, herpes, human T-cell lymphotrophic virus, Mycoplasma pneumoniae , Schistosoma , or …

Editorial: Challenges in developing new multiple sclerosis therapies

Editorial: Challenges in developing new multiple sclerosis therapies

Segmentation of gadolinium‐enhanced lesions on MRI in multiple sclerosis

To develop and implement a method for identification and quantification of gadolinium (Gd) enhancements with minimal human intervention. Dual fast spin echo (FSE), fluid attenuation inversion recovery (FLAIR), and pre- and postcontrast T1-weighted spin echo were acquired on 22 subjects. The enhancements were identified on the postcontrast T1-weighted images based on morphological operations. A single threshold based on the ratio of the difference of postcontrast and precontrast T1 images with that of precontrast T1 images is applied to the reconstructed images to reduce the false classifications. False classification of enhancements arising from enhancing vasculature and structures such as the choroid plexus that lack a blood-brain barrier were reduced by assuming that the true enhancements are always associated with hyperintense lesions on T2-weighted images (T2 lesions). The enhanced lesions were further delineated based on fuzzy connectivity. The segmented Gd enhancements were evaluated quantitatively with manually identified enhancements based on similarity measures. The average similarity index (SI) of 0.76 suggests excellent performance of the proposed methodology. The Bland-Altman plot shows a close agreement between the results obtained manually and those based on the proposed methodology. The proposed algorithm identifies and quantifies Gd enhancements accurately with minimal human intervention.