Mild Encephalitis/Encephalopathy with a Reversible Isolated Splenial Lesion (MERS) in Adult Patients: A Small Case Series

2020 ◽  
Vol 83 (3) ◽  
pp. 279-286
Author(s):  
Zhen Zhang ◽  
Jing Guo ◽  
Xiaobin Li ◽  
Chuanling Li ◽  
Xiaolin Ma ◽  
...  

Background: Mild encephalitis/encephalopathy with a reversible isolated splenial lesion (MERS) is a rare and complex clinico-radiological condition that aggregates various diseases. From a geographical point of view, most cases have been reported in Asia, especially in Japan. However, a very limited number of studies have properly evaluated Chinese patients diagnosed with MERS. Methods: Putative encephalitis/encephalopathy patients affected by a reversible isolated SCC lesion, as detected by magnetic resonance imaging (MRI), were retrospectively evaluated. Specifically, we reviewed extensive patient data, including prodromal and neurologic symptoms, neurologic examinations, MRI and electroencephalography (EEG) findings, laboratory results, treatments, and prognosis. Results: Six clinically mild encephalitis/encephalopathy patients, affected by a reversible isolated SCC lesion, were identified in a cohort of 22 patients. Six patients presented fever symptoms before the onset of neurological manifestations. Initial MRI data exhibited isolated SCC lesions with isointense or slightly hypointense on T1WI, hyperintense on T2WI, Flair, and DWI, as well as decreased ADC values, without contrast enhancement. EEG data indicated abnormalities (3/4), as well as elevated serum inflammatory markers (5/6) and hyponatremia (4/6). Cell content and protein yields were normal or slightly increased in the CSF of 6 patients, excluding one episode of significant increase in cell number. Patients were treated with corticosteroids, antivirals, and antibiotics, resulting in their full clinical recovery. SCC lesions mostly disappeared in all patients. Conclusion: In our study, the clinical features identified in all affected patients were mostly identical, which consisted of relatively mild CNS manifestations with a promising prognostic status.

2019 ◽  
Vol 82 (4-6) ◽  
pp. 86-92
Author(s):  
Wen Xu ◽  
Yan Zhou ◽  
Yan Jiang ◽  
Li Zhang ◽  
Qiqiang Tang

Background: To study clinical and magnetic resonance imaging (MRI) features of reversible splenial lesion syndrome (RESLES) in adult patients. Methods: A retrospective analysis was performed using clinical, cerebrospinal fluid (CSF), laboratory results, and neuroimaging data obtained from 6 adult RESLES patients. Results: All 6 patients (3 male cases, 3 female cases) were determined to be acute or subacute onset, most of them associated with infection or fever. All initial MRI data exhibited splenium of corpus callosum lesions with hypointensity on T1WI, hyperintensity on T2WI, diffusion-weighted imaging (DWI) and Flair, without significant gadolinium enhancement. Five patients were treated with glucocorticoids and showed significant improvement in 1–15 days, with the lesion having disappeared or weakened, and one case was lost of follow-up. The cell number and protein amount in CSF were determined to be at normal levels, or slightly increased in 3 patients with thyroid dysfunction. Conclusion: The etiology of adult RESLES was observed to be complex and diverse, primarily related to infection, fever, and thyroid dysfunction. DWI was found to be more sensitive in these lesions, and CSF cytology was observed to be either normal or mildly abnormal. A majority of patients were found to be sensitive to glucocorticoid, and have a good prognosis with lesions that disappeared rapidly.


2019 ◽  
Vol 12 (3) ◽  
pp. 220-228 ◽  
Author(s):  
Laura Evangelista ◽  
Lea Cuppari ◽  
Luisa Bellu ◽  
Daniele Bertin ◽  
Mario Caccese ◽  
...  

Purpose: The aims of the present study were to: 1- critically assess the utility of L-3,4- dihydroxy-6-18Ffluoro-phenyl-alanine (18F-DOPA) and O-(2-18F-fluoroethyl)-L-tyrosine (18F-FET) Positron Emission Tomography (PET)/Computed Tomography (CT) in patients with high grade glioma (HGG) and 2- describe the results of 18F-DOPA and 18F-FET PET/CT in a case series of patients with recurrent HGG. Methods: We searched for studies using the following databases: PubMed, Web of Science and Scopus. The search terms were: glioma OR brain neoplasm and DOPA OR DOPA PET OR DOPA PET/CT and FET OR FET PET OR FET PET/CT. From a mono-institutional database, we retrospectively analyzed the 18F-DOPA and 18F-FET PET/CT of 29 patients (age: 56 ± 12 years) with suspicious for recurrent HGG. All patients underwent 18F-DOPA or 18F-FET PET/CT for a multidisciplinary decision. The final definition of recurrence was made by magnetic resonance imaging (MRI) and/or multidisciplinary decision, mainly based on the clinical data. Results: Fifty-one articles were found, of which 49 were discarded, therefore 2 studies were finally selected. In both the studies, 18F-DOPA and 18F-FET as exchangeable in clinical practice particularly for HGG patients. From our institutional experience, in 29 patients, we found that sensitivity, specificity and accuracy of 18F-DOPA PET/CT in HGG were 100% (95% confidence interval- 95%CI - 81-100%), 63% (95%CI: 39-82%) and 62% (95%CI: 39-81%), respectively. 18F-FET PET/CT was true positive in 4 and true negative in 4 patients. Sensitivity, specificity and accuracy for 18F-FET PET/CT in HGG were 100%. Conclusion: 18F-DOPA and 18F-FET PET/CT have a similar diagnostic accuracy in patients with recurrent HGG. However, 18F-DOPA PET/CT could be affected by inflammation conditions (false positive) that can alter the final results. Large comparative trials are warranted in order to better understand the utility of 18F-DOPA or 18F-FET PET/CT in patients with HGG.


Author(s):  
Renate B. Schnabel ◽  
Stephan Camen ◽  
Fabian Knebel ◽  
Andreas Hagendorff ◽  
Udo Bavendiek ◽  
...  

AbstractThis expert opinion paper on cardiac imaging after acute ischemic stroke or transient ischemic attack (TIA) includes a statement of the “Heart and Brain” consortium of the German Cardiac Society and the German Stroke Society. The Stroke Unit-Commission of the German Stroke Society and the German Atrial Fibrillation NETwork (AFNET) endorsed this paper. Cardiac imaging is a key component of etiological work-up after stroke. Enhanced echocardiographic tools, constantly improving cardiac computer tomography (CT) as well as cardiac magnetic resonance imaging (MRI) offer comprehensive non- or less-invasive cardiac evaluation at the expense of increased costs and/or radiation exposure. Certain imaging findings usually lead to a change in medical secondary stroke prevention or may influence medical treatment. However, there is no proof from a randomized controlled trial (RCT) that the choice of the imaging method influences the prognosis of stroke patients. Summarizing present knowledge, the German Heart and Brain consortium proposes an interdisciplinary, staged standard diagnostic scheme for the detection of risk factors of cardio-embolic stroke. This expert opinion paper aims to give practical advice to physicians who are involved in stroke care. In line with the nature of an expert opinion paper, labeling of classes of recommendations is not provided, since many statements are based on expert opinion, reported case series, and clinical experience.


2021 ◽  
Vol 10 (15) ◽  
pp. 3439
Author(s):  
Irene Motta ◽  
Juri Giannotta ◽  
Marta Ferraresi ◽  
Kordelia Barbullushi ◽  
Nicoletta Revelli ◽  
...  

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.


Medicina ◽  
2021 ◽  
Vol 57 (1) ◽  
pp. 60
Author(s):  
Daniele De Santis ◽  
Federico Gelpi ◽  
Giuseppe Verlato ◽  
Umberto Luciano ◽  
Lorena Torroni ◽  
...  

Background and Objective: Guided bone regeneration allows new bone formation in anatomical sites showing defects preventing implant rehabilitation. Material and Methods: The present case series reported the outcomes of five patients treated with customized titanium meshes manufactured with a digital workflow for achieving bone regeneration at future implant sites. A significant gain in both width and thickness was achieved for all patients. Results: From a radiographic point of view (CBTC), satisfactory results were reached both in horizontal and vertical defects. An average horizontal gain of 3.6 ± 0.8 mm and a vertical gain of 5.2 ± 1.1 mm. Conclusions: The findings from this study suggest that customized titanium meshes represent a valid method to pursue guided bone regeneration in horizontal, vertical or combined defects. Particular attention must be paid by the surgeon in the packaging of the flap according to a correct method called the “poncho” technique in order to reduce the most frequent complication that is the exposure of the mesh even if a partial exposure of one mesh does not compromise the final outcome of both the reconstruction and the healing of the implants.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Ya-Bing Wang ◽  
Ou Wang ◽  
Min Nie ◽  
Yan Jiang ◽  
Mei Li ◽  
...  

Abstract Background Autoimmune polyendocrine syndrome type 1 (APS1) is a hereditary disease caused by mutations in the AIRE gene with both endocrine and non-endocrine organ involvement. The existing data from China are limited, and this study aims to describe the phenotypes and genetic characterization in Chinese APS1 patients. In this single-center, retrospective, observational study, comprehensive endocrine and extra-endocrine manifestations were collected, and genetic analysis in AIRE was conducted in patients with APS1 between the years of 1984 and 2018 at Peking Union Medical College Hospital. Results In total, 13 patients from 12 unrelated families were enrolled, seven of whom were female, with hypoparathyroidism, chronic mucocutaneous candidiasis, and Addison’s disease being the most frequently observed manifestations. Up to 84.7% presented with two or three of the above-mentioned manifestations, and nearly 4.9 ± 1.8 components presented in patients aged 21.2 ± 7.9 years old. Several less common phenotypes, such as myeloproliferative disease, pure red cell aplasia, renal tubular acidosis, asplenia, autoimmune hepatitis, and ankylosing spondylitis, were also observed in patients. Altogether, seven different AIRE mutations were found in six patients, four of which (K161fs, G208V, A246fs, and L308F) had not been previously reported in patients with APS1. Conclusion We have provided a comprehensive profile of Chinese patients with APS1, with less commonly observed features being observed in addition to more regularly seen manifestations. Additionally, different AIRE mutations that were observed have expanded the genetic spectrum, which will help with future understanding of the molecular pathogenesis of APS1.


1928 ◽  
Vol 48 (5) ◽  
pp. 659-665 ◽  
Author(s):  
Frederick S. Hammett ◽  
Vilma L. Wallace

Our study of the effect of the lead ion on the development of the chick embryo has brought out the following facts: 1. Gross growth is retarded. 2. Somite growth is retarded to a degree greater than that exhibited by body length and width. 3. The head and optic anlagen are regions of particular sensitivity. Their differential development is markedly inhibited. From the purely biological point of view these results are in line with the findings of Child (10) and his school as to the sensitivity of the head end of rapidly growing organisms to harmful influences, and with those of Stockard (11) as to the peculiar sensitivity of the optic anlagen. It is almost too well known to need repetition that the head region and the somites of embryos are specific areas of intense growth by increase in cell number. Therefore, turning from the general to the particular, the differential retardation of these regions which is caused by lead, is evidence justifying the conclusion that it is areas of rapid growth by cell proliferation which are selectively inhibited by this metallic ion.


2018 ◽  
Vol 6 (12) ◽  
pp. 232596711881104 ◽  
Author(s):  
Desmond J. Bokor ◽  
Sumit Raniga ◽  
Petra L. Graham

Background: The axillary nerve is at risk during repair of a humeral avulsion of the glenohumeral ligament (HAGL). Purpose: To measure the distance between the axillary nerve and the free edge of a HAGL lesion on preoperative magnetic resonance imaging (MRI) and compare these findings to the actual intraoperative distance measured during open HAGL repair. Study Design: Case series; Level of evidence, 4. Methods: A total of 25 patients with anterior instability were diagnosed as having a HAGL lesion on MRI and proceeded to open repair. The proximity of the axillary nerve to the free edge of the HAGL lesion was measured intraoperatively at the 6-o’clock position relative to the glenoid face. Preoperative MRI was then used to measure the distance between the axillary nerve and the free edge of the HAGL lesion at the same position. Distances were compared using paired t tests and Bland-Altman analyses. Results: The axillary nerve lay, on average, 5.60 ± 2.51 mm from the free edge of the HAGL lesion at the 6-o’clock position on preoperative MRI, while the mean actual intraoperative distance during open HAGL repair was 4.84 ± 2.56 mm, although this difference was not significant ( P = .154). In 52% (13/25) of patients, the actual intraoperative distance of the axillary nerve to the free edge of the HAGL lesion was overestimated by preoperative MRI. In 36% (9/25), this overestimation of distance was greater than 2 mm. Conclusion: The observed overestimations, although not significant in this study, suggest a smaller safety margin than might be expected and hence a substantially higher risk for potential damage. We recommend that shoulder surgeons exercise caution in placing capsular sutures in the lateral edge when contemplating arthroscopic repair of HAGL lesions, as the proximity of the nerve to the free edge of the HAGL tear is small enough to be injured by arthroscopic suture-passing instruments.


2018 ◽  
Vol 33 (11) ◽  
pp. 713-717 ◽  
Author(s):  
Afnan AlGhamdi ◽  
Muhammad Talal Alrifai ◽  
Abdullah I. Al Hammad ◽  
Fuad Al Mutairi ◽  
Abdulrahman Alswaid ◽  
...  

Propionic acidemia is an inborn error of metabolism that is inherited in an autosomal recessive manner. It is characterized by a deficient propionyl-CoA carboxylase due to mutations in either of its beta or alpha subunits. In the literature, there is a clear association between propionic acidemia and epilepsy. In this cohort, we retrospectively reviewed the data of 14 propionic acidemia patients in Saudi Arabia and compared the findings to those of former studies. Six of the 14 (43%) patients developed epileptic seizure, mainly focal seizures. All patients were responsive to conventional antiepileptic drugs as their seizures are controlled. The predominant electroencephalographic (EEG) findings were diffuse slowing in 43% and multifocal epileptiform discharges in 14% of the patients. In 1 patient, burst suppression pattern was detected, a pattern never before reported in patients with propionic acidemia. Brain magnetic resonance imaging (MRI) findings mainly consisted of signal changes of the basal ganglia (36%), generalized brain atrophy (43%), and delayed myelination (43%).The most common genotype in our series is the homozygous missense mutation in the PCCA gene (c.425G>A; p. Gly142Asp). However, there is no clear genotype–seizure correlation. We conclude that seizure is not an uncommon finding in patients with propionic acidemia and not difficult to control. Additional studies are needed to further elaborate on genotype–seizure correlation.


1993 ◽  
Vol 35 (4) ◽  
pp. 361-365 ◽  
Author(s):  
Cláudio L. Rossi ◽  
Emilia E. H. Takahashi ◽  
Cláudia D. Partel ◽  
Lívia G.V.L. Teodoro ◽  
Luiz J. da Silva

Total serum IgE, and Strongyloides - specific IgG and IgA antibodies were studied in 27 patients with parasitologically proven strongyloidiasis. Clinical manifestations in this case series were investigated by a restrospective study of the patient's records. Total serum IgE levels were elevated (greater than 250 IU/ml) in 59% of the patients (mean concentration = 1364 IU/ml). Parasite - specific IgG and IgA antibodies were detected by ELISA in the serum of 23 (85.2%) and 21 (77.8%) patients, respectively. Elevated serum IgE and clinical manifestations were not useful indexes of the presence of strongyloidiasis. On the other hand, our results support the view that serologic tests, particularly ELISA for detecting Strongyloides - specific IgG antibodies, can be usefully exploited for diagnostic purposes in strongyloidiasis.


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