FLAIR Hyperintense Lesions Research Articles

SURG-08. A PHASE III RANDOMIZED TRIAL OF GROSS TOTAL RESECTION VERSUS POSSIBLE RESECTION OF FLAIR HYPERINTENSITY LESION ON MR IMAGE FOR NEWLY DIAGNOSED SUPRATENTORIAL GLIOBLASTOMA (JCOG2209)

Abstract BACKGROUND Complete resection of contrast-enhanced lesion (gross total resection: GTR) without severe neurological deficits has been generally accepted as the goal of surgery. However, it remains unclear if additional resection of surrounding FLAIR hyper-intense lesion combined with GTR (FLAIRectomy) has survival advantage of primary glioblastoma patients. Purpose: Multicenter, open-label, randomized phase III trial was commenced to confirm the superiority of FLAIRectomy to GTR alone followed by radiotherapy with concomitant and adjuvant temozolomide in terms of overall survival (OS) for primary glioblastoma IDH-wildtype patients. This trial investigates not only survival but also postoperative neurological and neurocognitive deficits in detail. METHODS We assumed a 2-year OS of 50% in the GTR arm and expected a 15% improvement in the FLAIRectomy arm. A total of 130 patients is required with a one-sided alpha of 5%, power of 70%, and will be accrued from 49 Japanese institutions in 4 years and follow-up will last 2.5 years. Patients 18-75 years of age will be registered and randomly assigned to each arm with 1:1 allocation. The primary endpoint is OS, and the secondary endpoints are progression-free survival, frequency of adverse events, proportion of Karnofsky performance status preservation, proportion of National Institutes of Health stroke scale preservation, proportion of mini-mental state examination preservation and proportion of health-related quality of life preservation. The Japan Clinical Oncology Group Protocol Review Committee approved this study protocol in May 2023. Ethics approval was granted by the National Cancer Center Hospital Certified Review Board. Patient enrollment began in July 2023. DISCUSSION If FLAIRectomy is superior to GTR alone, aggressive surgery will become a standard surgical treatment for glioblastoma with resectable contrast-enhanced lesion. Trial registration: Registry number: jRCT1031230245. Date of registration: 19/July/2023. Date of first participant enrollment: 28/July/2023. Funding for this study is provided by National Cancer Center Research and Development Fund (2023-J-03).

SURG-29. EXTENDED RESECTION IMPROVES PATIENT SURVIVAL OUTCOMES AFTER GROSS-TOTAL RESECTION OF GLIOBLASTOMA

Abstract In recent years, the effectiveness of extended resection (ER) beyond the contrast-enhanced (CE) lesion has been reported as a surgical strategy for glioblastoma. This study was to determine whether ER of FLAIR-hyperintense area beyond the CE tumor affected the overall survival (OS) and progression-free survival (PFS) of patients with glioblastoma after gross-total resection (GTR). From October 2019 to March 2022, a total of 62 patients who underwent resection of glioblastoma (GBM) at Kobe University Hospital were retrospectively examined. Volumetric measurements of the CE area and surrounding FLAIR-hyperintense area were performed, and clinical variables related with OS and PFS were analyzed. In total, 33 patients with GBM who underwent GTR of the CE tumor met the inclusion criteria. The mean volume of CE tumor and FLAIR hyperintensity area before resection was 36.5 cm3 and 77.2 cm3 respectively. The mean excision rate of FLAIR-hyperintense area was 18.2%. Removal rates of 20% or more in the FLAIR-hyperintense region significantly increased OS and PFS (p=0.040 and 0.026, respectively). In multivariate analysis, age ≥ 65 years (HR 2.73; 95% CI 1.09–7.22; p=0.032) were associated with shorter OS, but ER ≥ 20% (HR 0.41; 95% CI 0.17–0.99; p=0.049) and MGMT promotor methylation (HR 0.36; 95% CI 0.15–0.85; p=0.020) were associated with longer OS. ER of FLAIR-hyperintense lesions of 20% or more in glioblastoma contributed to prolonging patient survival. It is necessary to validate the effect of ER in prospective clinical trials.

FLAIR-Hyperintense Lesions in Anti-MOG-Associated Encephalitis with Seizures: A Case Report

Introduction: Myelin oligodendrocytic glycoprotein antibody-associated disease (MOGAD) is an immune-mediated demyelinating disorder of the central nervous system. We described, a case of encephalitis associated with anti-MOG antibodies with hyperintense lesions on FLAIR and seizures (FLAMES). Case Report: We report a case of a 5-year-old girl, with no significant personal or family history, who entered the emergency room with paroxysmal events and anarthria, in a febrile context. On neurological examination she presented right sided facial paresis and pyramidal signs on the right. Lumbar puncture (LP), showed pleocytosis, hyperproteinorrhaquia and the absence of oligoclonal bands. Electroencephalogram revealed encephalopathy and slow paroxysmal left frontal activity. The brain MRI detected hyperintense lesions, however, with an area of left frontal cortico-subcortical hyperintense signal on T2/FLAIR and diffusion restriction. Anti-MOG antibody was detected. These findings pointed to the diagnosis of FLAMES. She completed 7 days of methylprednisolone (30mg/kg/day), with a good response. On the day of discharge, a repeat EEG was conducted, demonstrating significant improvement compared to the initial one, with slightly slow wake and sleep patterns for her age, and no evidence of epileptic activity. She was assessed approximately four months post-hospitalisation and was found to be asymptomatic, with no focal neurological deficits. A further EEG was performed, which showed a structurally age-appropriate wake and sleep pattern with no abnormal graphoelements. Conclusion: We highlight a rare clinical-radiological entity, which is still poorly described, particularly in the paediatric population, known as FLAMES, with a favourable response to steroids. Early recognition and timely diagnosis of his condition improve clinical and therapeutic management and minimize neurological damage. Immediate initiation of appropriate treatment is important for achieving a favorable outcome.

FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES) Presenting with Psychosis and Refractory Seizures

FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES) Presenting with Psychosis and Refractory Seizures

First Episode Psychosis and Status Epilepticus as Presenting Symptom of FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES)

First Episode Psychosis and Status Epilepticus as Presenting Symptom of FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES)

Clinical characteristics and multimodal imaging can help diagnosing and treating mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy.

Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a recently described, histopathologically and molecularly defined (SLC35A2-mutated) type of cortical malformation. Although increasingly recognized, the diagnosis of MOGHE remains a challenge. We present the characteristics of the first six patients diagnosed in Bulgaria, with the aim to facilitate identification, proper presurgical evaluation, and surgical treatment approach in this disease. Revision of histopathological specimens of 202 patients operated on for drug-resistant focal epilepsy identified four cases with MOGHE. Another two were suggested, based on clinical characteristics and subsequently, were histologically confirmed. Sanger SLC35A2 sequencing on paraffin-embedded or fresh-frozen brain tissue was performed. Analysis of seizure types, neuropsychological profiles, electroencephalographic (EEG), imaging features and epilepsy surgery outcomes was done. Three out of the six cases (50%) harbored pathogenic SLC35A2 mutations. One patient had a heterozygous somatic variant with uncertain significance. Clinical characteristics included epilepsy onset in infancy (in 100% under 3 years of age), multiple seizure types, and moderate or severe intellectual/developmental delay. Epileptic spasms with hypsarrhythmia on EEG were the initial seizure type in five patients. The subsequent seizure types resembled those in Lennox-Gastaut syndrome. The majority of the patients (n = 4) presented prominent and persisting autistic features. Magnetic resonance imaging (MRI) showed multilobar (n = 6) and bilateral (n = 3) lesions, affecting the frontal lobes (n = 5; bilaterally in three) and characterized by increased signal on T2/fluid-attenuated inversion recovery (FLAIR). Voxel-based morphometric MRI post-processing and positron emission tomography helped determining the localization and extent of the lesions and presumed epileptogenic zones. After surgery, four patients (66.7%) were seizure-free ≥2 years. Interestingly, all seizure-free patients carried somatic SLC35A2-alterations. Epileptic spasms, early prominent neuropsychological disturbances, MRI-T2/FLAIR hyperintense lesions with cortico-subcortical blurring, frequently multilobar and especially frontal, can preoperatively help to suspect MOGHE. Epilepsy surgery is still the only successful treatment option in MOGHE.

Case of Myelin Oligodentrocyte Glycoprotein Antibody-associated Disease (MOGAD) with FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES) in a Pediatric Patient (P9-14.012)

Case of Myelin Oligodentrocyte Glycoprotein Antibody-associated Disease (MOGAD) with FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES) in a Pediatric Patient (P9-14.012)

Clinical and Imaging Findings in Children with Myelin Oligodendrocyte Glycoprotein Antibody Associated Disease (MOGAD): From Presentation to Relapse.

Myelin oligodendrocyte glycoprotein-antibody associated disease (MOGAD) is an increasingly recognized cause of demyelinating disease in children. The purpose of this study is to characterize the CNS imaging manifestations of pediatric MOGAD and identify clinical and imaging variables associated with relapse. We retrospectively identified children with serum antibody-positive MOGAD evaluated at our institution between 1997 and 2020. Clinical and demographic data were collected. MRIs of the brain, orbit, and spine at presentation and relapse were reviewed for location and pattern of abnormality. Among 61 cases (34 girls), mean age at presentation was 7 years (IQR 4-11). At presentation, there was imaging involvement of the brain in 78.6% (44/56), optic pathway in 55.4% (31/56), and spine in 19.6% (11/56). Brain involvement was commonly in the frontal (70.5%, 31/44) and subcortical (75%, 33/44) white matter, with involvement of the thalamus and pons in 47.7% each (21/44). Optic neuritis (ON) was commonly bilateral (80.6%, 25/31) involving intraorbital segments (77.4%, 24/31). Spinal cord lesions were typically cervical (72.7%, 8/11) and multifocal (72.7%, 8/11).The imaging patterns were age-dependent; children ≤9 years more commonly demonstrated ADEM-like imaging pattern at presentation (39.4%, 13/33) and first relapse (8/23, 34.8%), while children >9 years more commonly had ON at presentation (34.8%, 8/23, P = .001) and FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures at first relapse (5/18, 27.8%, P = .008). We describe the CNS imaging findings in pediatric MOGAD. The imaging pattern is age-dependent at presentation and first relapse. Younger age at presentation is associated with longer time to relapse.

FLAIR-Hyperintense Lesions in Anti-MOG-Associated Encephalitis With Seizures (FLAMES).

FLAIR-Hyperintense Lesions in Anti-MOG-Associated Encephalitis With Seizures (FLAMES).

CNSC-36. INDOLENT INTRAVASCULAR LARGE B-CELL LYMPHOMA (IVLBCL) ORIGINALLY PRESENTING AS A STROKE MIMIC

Abstract BACKGROUND IVBCL is a rare subtype of extranodal diffuse large B-cell lymphoma, characterized by the growth of neoplastic cells in blood vessels with no other apparent extravascular mass, making diagnosis a challenge. It usually has an aggressive and fast-paced course; however, there is limited data regarding incidence due to its rarity. CASE A 73 year-old woman presented with progressive gait worsening a year prior to presentation to our institution. Her symptoms then developed to include left-sided hemiparesis with associated headaches, warranting a local emergency department visit. Brain magnetic resonance imaging (MRI) without contrast showed a Flair hyperintense lesion in the right MCA territory with restricted diffusion, assumed to be an ischemic stroke, and she was started on stroke prevention measures. The patient continued to decline, becoming wheelchair-bound and developing short-term memory loss, chronic headaches, and pseudobulbar affect. Repeat brain MRI without contrast five months later showed progression of the Flair hyperintensity to the left hemisphere with restricted diffusion. Brain MRI with contrast followed, revealing subtle enhancement. Vessel imaging was unremarkable, and cerebrospinal fluid (CSF) analysis was normal with negative cytology and flow cytometry, yet elevated beta-2-microglobulin at 3.25 mcg/mL. A year after symptoms onset, the patient presented to our Neuro-Oncology clinic. Repeat Brain MRI with contrast and spectroscopy showed progression on the left-sided lesion with persistent restriction diffusion and enhancement, prompting a biopsy. Pathology confirmed large B cell lymphoma. PET-CT body scan showed no evidence of systematic disease. The patient was treated with Rituximab, Methotrexate, Vincristine, and Procarbazine (RMVP) with complete response demonstrated by complete resolution of the diffusion restriction and symptoms improvement. CONCLUSIONS Although IVLBCL is known for being a fast progressive disease, we present a case of indolent course delaying diagnosis. Unexplained progressive neurological symptoms with restricted diffusion on MRI should prompt consideration for this diagnosis.

NIMG-89. CNS-ISOLATED RICHTER’S TRANSFORMATION: THE FALLIBILITY OF NEURO-IMAGING AND THE CRITICAL ROLE OF BRAIN BIOPSY IN A CHALLENGING DIAGNOSIS

Abstract INTRODUCTION An 81-year-old male was admitted for acute encephalopathy and failure to thrive. Workup revealed a lymphocyte-predominant leukocytosis, prominent axillary and mediastinal lymphadenopathy on CT, and multiple supra-tentorial FLAIR-hyperintense lesions that were homogenously contrast-enhancing. Axillary lymph node biopsy demonstrated small lymphocytic lymphoma (SLL), a variant of CLL. Despite concern for CNS malignancy, brain biopsy was initially deferred since the patient’s brain lesions were attributed to SLL metastasis. However on hospital day 15, he developed partial seizures, prompting stereotactic biopsy which revealed DLBCL. DATA/DISCUSSION Our case highlights the pitfalls of relying solely upon neuro-imaging to diagnose Richter’s transformation (RT) isolated to the CNS. First, MRI does not differentiate CNS metastasis of systemic lymphoma from CNS RT. A review of 35 biopsy or LP-proven cases of CNS-isolated RT showed imaging characteristics consistent with primary and metastatic CNS lymphoma. When parenchymal lesions are present, all are T2/FLAIR hyper-intense; 60% are unifocal (40% multifocal); and 95% homogenously enhance. Second, CNS-isolated RT is at least 3 times rarer than CNS metastases of CLL. Among patients with CLL, ~1% will eventually exhibit CNS metastasis but < 0.3% will develop CNS-isolated RT. In patients with known CLL and no evidence of high-grade transformation, it is reasonable to attribute brain lesions to CNS metastasis of CLL as opposed to RT, absent a definitive tissue diagnosis. CONCLUSION These data emphasize the importance of biopsy in the workup of CNS lymphoma, especially since treatment differs based on histopathological subtype. Given its chronic indolent course, SLL is typically managed expectantly, whereas the aggressive nature of DLBCL warrants more intensive chemotherapy. Our case also shows that in patients with suspicious CNS lesions, presence of systemic malignancy does not eliminate the need for brain biopsy, and that clinical deterioration should prompt biopsy if not already performed.

Undetected pseudoprogressions in the CeTeG/NOA-09 trial: hints from postprogression survival and MRI analyses

PurposeIn the randomized CeTeG/NOA-09 trial, lomustine/temozolomide (CCNU/TMZ) was superior to TMZ therapy regarding overall survival (OS) in MGMT promotor-methylated glioblastoma. Progression-free survival (PFS) and pseudoprogression rates (about 10%) were similar in both arms. Further evaluating this discrepancy, we analyzed patterns of postprogression survival (PPS) and MRI features at first progression according to modified RANO criteria (mRANO).MethodsWe classified the patients of the CeTeG/NOA-09 trial according to long vs. short PPS employing a cut-off of 18 months and compared baseline characteristics and survival times. In patients with available MRIs and confirmed progression, the increase in T1-enhancing, FLAIR hyperintense lesion volume and the change in ADC mean value of contrast-enhancing tumor upon progression were determined.ResultsPatients with long PPS in the CCNU/TMZ arm had a particularly short PFS (5.6 months). PFS in this subgroup was shorter than in the long PPS subgroup of the TMZ arm (11.1 months, p = 0.01). At mRANO-defined progression, patients of the CCNU/TMZ long PPS subgroup had a significantly higher increase of mean ADC values (p = 0.015) and a tendency to a stronger volumetric increase in T1-enhancement (p = 0.22) as compared to long PPS patients of the TMZ arm.ConclusionThe combination of survival and MRI analyses identified a subgroup of CCNU/TMZ-treated patients with features that sets them apart from other patients in the trial: short first PFS despite long PPS and significant increase in mean ADC values upon mRANO-defined progression. The observed pattern is compatible with the features commonly observed in pseudoprogression suggesting mRANO-undetected pseudoprogressions in the CCNU/TMZ arm of CeTeG/NOA-09.

SDPS-08 OSSEOUS METASTASES IN H3G34-MUTANT DIFFUSE HEMISPHERIC GLIOMAS

Abstract Subset of patients with H3G34 gliomas may present with extra-CNS metastasis further complicating both diagnosis and treatment. We describe a case of a 19-year-old female presenting initially with focal motor seizure in right leg along with leg pain. MRI of the leg showed an ill-defined right distal femur expansile osteolytic lesion with central fluid signal, thick rim enhancement, endosteal scalloping, cortical invasion, and smooth enhancing periosteal reaction/soft tissue extension associated with surrounding enhancing marrow signal abnormality worrisome for lesion extension. Contrast brain MRI exhibited multiple frontal and partial lobe T2 FLAIR hyperintense lesions. The largest lesion demonstrated contrast enhancement, internal foci of restricted diffusion, and a focus of intratumoral hemorrhage. A whole-body PET/CT showed intensely FDG-avid lytic lesion in the right distal femur, with FDG activity along the biopsy tract. Hypermetabolic lytic metastases to the right femoral head and mid-sacral body were also observed. A craniotomy with brain lesion biopsy two months later indicated preliminary histopathology of malignant small round blue cell neoplasm. A biopsy from the distal right femur was non-diagnostic of malignancy but a sacral bone biopsy reported malignant small blue cell neoplasm, consistent with involvement by the patient's known cranial neoplasm. Final pathological diagnosis including molecular diagnostics was consistent with WHO grade 4 IDH-wildtype H3G34 mutant diffuse hemispheric glioma with osseous metastases. Patient was treated with chemoradiotherapy with temozolomide (TMZ). Following the sixth cycle of TMZ, MRI brain imaging was stable. Apart from holocephalic headaches, the patient had no significant medication side effects and was seizure-free. A second whole-body PET scan showed improvement and interval decrease in FDG uptake of the sacrum and right femoral head. Given the stress that patients with H3G34 can experience and the poor prognosis, it is imperative to expand our knowledge and ascertain accurate screening and diagnostic methodologies.

Central nervous system manifestations of LRBA deficiency: case report of two siblings and literature review

BackgroundLPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency disease (PID) characterized by a regulatory T cell defect resulting in immune dysregulation and autoimmunity. We present two siblings born to consanguineous parents of North African descent with LRBA deficiency and central nervous system (CNS) manifestations. As no concise overview of these manifestations is available in literature, we compared our patient’s presentation with a reviewed synthesis of the available literature.Case presentationsThe younger brother presented with enteropathy at age 1.5 years, and subsequently developed Evans syndrome and diabetes mellitus. These autoimmune manifestations led to the genetic diagnosis of LRBA deficiency through whole exome sequencing with PID gene panel. At 11 years old, he had two tonic–clonic seizures. Brain MRI showed multiple FLAIR-hyperintense lesions and a T2-hyperintense lesion of the cervical medulla. His sister presented with immune cytopenia at age 9 years, and developed diffuse lymphadenopathy and interstitial lung disease. Genetic testing confirmed the same mutation as her brother. At age 13 years, a brain MRI showed multiple T2-FLAIR-hyperintense lesions. She received an allogeneic hematopoietic stem cell transplantation (allo-HSCT) 3 months later. Follow-up MRI showed regression of these lesions.ConclusionsNeurological disease is documented in up to 25% of patients with LRBA deficiency. Manifestations range from cerebral granulomas to acute disseminating encephalomyelitis, but detailed descriptions of neurological and imaging phenotypes are lacking. LRBA deficiency amongst other PIDs should be part of the differential diagnosis in patients with inflammatory brain lesions. We strongly advocate for a more detailed description of CNS manifestations in patients with LRBA deficiency, when possible with MR imaging. This will aid clinical decision concerning both anti-infectious and anti-inflammatory therapy and in considering the indication for allo-HSCT.

FLAIR-hyperintense lesions in anti-MOG-associated Encephalitis with Seizures (FLAMES) mimicking new daily persistent headache. Updating the diagnostic paradigm with recommendations for timely diagnosis and management. (P13-5.024)

<h3>Objective:</h3> We present a case report of a patient with abrupt right fronto-temporal headache and neck pain that persisted for 13 days, followed by first-time generalized tonic-clonic seizures, weakness, neglect, and encephalopathy. She was ultimately diagnosed with FLAIR-hyperintense lesions in anti-MOG-associated Encephalitis with Seizures (FLAMES), which resolved with IV methylprednisolone. <h3>Background:</h3> FLAIR-hyperintense lesions in anti-MOG-associated Encephalitis with Seizures <b>(</b>FLAMES) was first described in 2017 as a distinct clinical and radiological syndrome from other anti-MOG-associated syndromes. MOG is a protein on the surface of oligodendrocytes. It helps to modulate myelin immune interactions, and its location on the outermost part of the myelin sheath serves as an immunogenic agent. Main presenting symptoms are seizures (85%) and headaches (70%). <h3>Design/Methods:</h3> History, physical exam, review of literature <h3>Results:</h3> We discuss a case of a 25-year-old previously healthy Asian woman, not on home medications, who experienced abrupt onset of right fronto-temporal headache and neck pain, which persisted for 13 days. She saw her primary care doctor four days prior to admission who recommended continuing ibuprofen. Brain imaging was not obtained at that time. She subsequently developed generalized tonic-clonic seizures with acute left sided weakness, neglect, and encephalopathy, which prompted presentation to the hospital. Video EEG showed right sided lateralized rhythmic delta activity (LRDA), as well as diffuse slowing. Lumbar puncture was performed: OP of 22 mm H20, W108 (35% PMNs, 55% lymphocyte), R 20, P 63.4, G 58.7, CSF culture was negative. CSF MOG antibody was positive at 1:100 and serum MOG antibody was positive at 1:320. Antivirals and antibiotics were stopped when infectious workup returned negative, and she began a five-day course of IV methylprednisolone with dramatic improvement. <h3>Conclusions:</h3> FLAIR-hyperintense lesions in anti-MOG-associated Encephalitis with Seizures (FLAMES) should be included in the differential diagnosis of new, sudden-onset, severe, and persistent headache. Recommendations include early imaging and treatment with corticosteroids. <b>Disclosure:</b> Dr. Michaelson has nothing to disclose. Dr. Langan has nothing to disclose. Dr. Kaunzner has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Kaunzner has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Medlearning.

Epilepsia partialis continua and unilateral cortical-subcortical FLAIR-hyperintense lesion in Rasmussen's encephalitis: Is it diagnostic?

Rasmussen encephalitis (RE) is a focal encephalitis, characterized by epilepsia partialis continua (EPC) with or without seizures and progressive unilateral deficits. Imaging characteristics of RE have been rarely described in detail in relation to EPC. So, the study aimed to explore if any relationship exists between the imaging characteristics and the presence or evolution of EPC in patients with RE. This retrospective study included 11 patients with RE fulfilling the European consensus statement on RE followed between 2015 and 2020. The mean age for onset of seizures was 12 years (range 2.5-24 years). Seven patients had limb EPCs, two had face EPCs, face, and limb EPC in one, and lingual EPC in one patient. The first MRI was done within 1 day to 1 month of the onset of seizures. It was normal in two patients and showed only cortical atrophy, focal or hemispheric in four patients, caudate atrophy in two, and cortical or subcortical hyperintensity (HI) in six patients. Follow-up MRI, within 3 weeks to 6 months of the onset of EPC (mean 1.6 months) showed paramedian frontal HI with limb EPC in six patients. Insular HI in four patients; two had facial EPCs while lingual EPC and limb EPC with facial EPC was observed in one patient each. Fluid-attenuated inversion recovery (FLAIR) HI and focal cortical atrophy on MRI is the most common finding in the early course of RE. T2 and FLAIR hyperintensity in the paramedian frontal or insular cortex may antedate the onset of EPC or may occur simultaneously with EPC.

FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures overlaying anti-N-methyl-D-aspartate receptor encephalitis: a case report and literature review

FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) has been identified increasingly frequently in recent years. However, this rare MOG antibody disease may coexist with anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARe), in an overlap syndrome with unknown clinical features and prognosis. We report a new case of this overlap syndrome and present a systematic review of similar cases in the literature to provide information on the clinical presentation, MRI features, EGG abnormalities, treatment, and prognosis of patients with this rare syndrome. A total of 12 patients were analyzed in the study. The most common clinical manifestations of FLAMES overlaid with anti-NMDARe were epilepsy (12/12), headache (11/12), and fever (10/12). Increases in intracranial pressure (median: 262.5 mmH2O, range: 150-380 mmH2O), cerebrospinal fluid (CSF) leukocyte count (median: 128×106/L, range: 1-610×106/L), and protein level (median: 0.48 g/L) were also observed. The median CSF anti-NMDAR antibody titer was 1:10 (1:1-1:32), while the median serum MOG antibody titer was 1:32 (1:10-1:1024). Seven cases exhibited unilateral cortical FLAIR hyperintensity, and five cases (42%) had bilateral cortical FLAIR hyperintensity, including four cases involving the bilateral medial frontal lobes. Of the 12 patients, five showed lesions at other sites (e.g., the brainstem, corpus callosum, or frontal orbital gyrus) before or after the development of cortical encephalitis. EEG showed slow waves in four cases, spike-slow waves in two cases, an epileptiform pattern in one case, and normal waves in two cases. The median number of relapses was two. Over a mean follow-up period of 18.5 months, only one patient experienced residual visual impairment, while the remaining 11 patients had good prognoses. FLAMES alone is difficult to distinguish from overlap syndrome based on clinical features. However, FLAMES with bilateral medial frontal lobe involvement suggests the presence of the overlap syndrome.

Acute disseminated encephalomyelitis following the COVID-19 vaccine Ad26.COV2.S, a case report

BackgroundThe severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) pandemic has been leading to dramatic health, social and economic problems around the world. It was necessary to introduce worldwide vaccination program against SARS-CoV-2 virus. Vaccination of billions of people around the world leads to many questions about risk of vaccines and possible side effects. It is well known that acute disseminated encephalomyelitis (ADEM) is a rare, but possible complication of vaccines. Previously, cases of ADEM following various COVID-19 vaccines, including the vaccines from AstraZenica, Pfizer, Sputnik V, SinoVac, Moderna, Sinopharm, have been described. In this case report, we present the first documented case of ADEM following the COVID-19 vaccine Ad26.COV2.S from Johnson & Johnson.Case presentationWe present the case of a 31-year-old female with gradually progression of right-sided weakness and numbness during a three-week period. Four weeks prior to symptom onset, the patient received the single-dose SARS-CoV-2 vaccine Ad26.COV2.S. Neuroimaging revealed five large juxtacortical T2 FLAIR hyperintense lesions with incomplete contrast enhancement on post-contrast T1 images located supratentorial: one in the right cerebral hemisphere and four in left cerebral hemisphere. The patient was followed up for four months. Symptom debut, clinical picture and MRI were typical for ADEM and the patient completely recovered after high dose intravenous methylprednisolone treatment.ConclusionsThis is, to the best of our knowledge, the first case report of ADEM following the COVID-19 vaccine Ad26.COV2.S. This case illustrates, although ADEM is a rare complication following SARS-CoV-2 vaccines, the necessity of maintaining a vaccine safety monitoring system to identify patients at high risk from developing severe complications from the vaccines.

Absence of cortical lesions after the first seizure in a patient with anti-myelin oligodendrocyte glycoprotein-associated cortical encephalitis

A 31-year-old man developed headache and generalized convulsions. At the time of the first seizure, there was no distinct MRI abnormality. He was admitted to the hospital with repeated seizures, left-sided hemiparesis, and left-sided neglect. He had a slight fever, elevated cerebrospinal fluid (CSF) pressure, and increased CSF cell count with predominance of mononuclear cells. A repeat MRI scan on day 8 after the recurrent seizure showed cortical edema in the right cerebral hemisphere on fluid-attenuated inversion recovery (FLAIR), abnormal high signal on DWI, and decreased apparent diffusion coefficient. The patient was diagnosed with aseptic meningoencephalitis and treated with antiviral drugs and methylprednisolone pulse therapy. Serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was subsequently detected, and prednisolone was added to treat the FLAIR-hyperintense lesions in anti-MOG antibody associated encephalitis with seizures (FLAMES). It is important to identify the clinical picture and typical images of FLAMES to allow early treatment.

A unilateral FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) case from a developing country: A case report

The new clinical and radiological entity of the myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is known as the FLAMES syndrome. It will add to the literature and enhance the understanding of this disease. Our case presented a 25-year-old male patient with no known comorbid presented with a generalized sudden headache of moderate intensity for 10 days who came to our hospital. A right-sided upper motor neuron facial palsy was found on the examination. The diagnosis was further confirmed utilizing the MRI scan and the presence of MOG-IgG antibodies. The patient was started on intravenous methylprednisolone which lead to improvement of his symptoms. In the follow-up contrast-enhancing MRI of the brain, the findings suggested near resolution as compared to the initial MRI. The FLAMES have been categorized as a subtype of the MOGAD. It is commonly found in males as compared to females and the mean age in which it commonly affects is 29 years with the common age group of 11-46 years. The clinical symptoms and physical signs along with the findings of unilateral cortical FLAMES on MRI suggest the distinctive findings of the FLAMES syndrome. Patients commonly present with symptoms like fever, headache, and cortical symptoms like aphasia. There is a dire need of improving the understanding of this clinic-radiographic syndrome which makes it critically important to ensure the timely diagnosis and prompt consideration of the required medications.