Cranial MRI Research Articles

Forecasting stroke-like episodes and outcomes in mitochondrial disease.

In this retrospective, multicentre, observational cohort study, we sought to determine the clinical, radiological, EEG, genetics and neuropathological characteristics of mitochondrial stroke-like episodes and to identify associated risk predictors.Between January 1998 and June 2018, we identified 111 patients with genetically determined mitochondrial disease who developed stroke-like episodes. Post-mortem cases of mitochondrial disease (n = 26) were identified from Newcastle Brain Tissue Resource. The primary outcome was to interrogate the clinico-radiopathological correlates and prognostic indicators of stroke-like episode in patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome (MELAS). The secondary objective was to develop a multivariable prediction model to forecast stroke-like episode risk.The most common genetic cause of stroke-like episodes was the m.3243A>G variant in MT-TL1 (n = 66), followed by recessive pathogenic POLG variants (n = 22), and 11 other rarer pathogenic mitochondrial DNA variants (n = 23). The age of first stroke-like episode was available for 105 patients [mean (SD) age: 31.8 (16.1)]; a total of 35 patients (32%) presented with their first stroke-like episode ≥40 years of age. The median interval (interquartile range) between first and second stroke-like episodes was 1.33 (2.86) years; 43% of patients developed recurrent stroke-like episodes within 12 months. Clinico-radiological, electrophysiological and neuropathological findings of stroke-like episodes were consistent with the hallmarks of medically refractory epilepsy. Patients with POLG-related stroke-like episodes demonstrated more fulminant disease trajectories than cases of m.3243A>G and other mitochondrial DNA pathogenic variants, in terms of the frequency of refractory status epilepticus, rapidity of progression and overall mortality. In multivariate analysis, baseline factors of body mass index, age-adjusted blood m.3243A>G heteroplasmy, sensorineural hearing loss and serum lactate were significantly associated with risk of stroke-like episodes in patients with the m.3243A>G variant. These factors informed the development of a prediction model to assess the risk of developing stroke-like episodes that demonstrated good overall discrimination (area under the curve = 0.87, 95% CI 0.82–0.93; c-statistic = 0.89). Significant radiological and pathological features of neurodegeneration were more evident in patients harbouring pathogenic mtDNA variants compared with POLG: brain atrophy on cranial MRI (90% versus 44%, P < 0.001) and reduced mean brain weight (SD) [1044 g (148) versus 1304 g (142), P = 0.005].Our findings highlight the often idiosyncratic clinical, radiological and EEG characteristics of mitochondrial stroke-like episodes. Early recognition of seizures and aggressive instigation of treatment may help circumvent or slow neuronal loss and abate increasing disease burden. The risk-prediction model for the m.3243A>G variant can help inform more tailored genetic counselling and prognostication in routine clinical practice.

Association of Serum Neurofilament Light Chain Concentration and MRI Findings in Older Adults: The Cardiovascular Health Study.

Neurofilament light chain (NfL) in blood is a sensitive but nonspecific marker of brain injury. This study sought to evaluate associations between NfL concentration and MRI findings of vascular brain injury in older adults. A longitudinal cohort study included 2 cranial MRI scans performed about 5 years apart and assessed for white matter hyperintensities (WMH) and infarcts. About 1 year before their second MRI, 1,362 participants (median age 77 years, 61.4% women) without a history of TIA or stroke had measurement of 4 biomarkers: NfL, total tau, glial fibrillary acidic protein (GFAP), and ubiquitin carboxyl-terminal hydrolase L1. Most (n = 1,279) also had the first MRI scan, and some (n = 633) had quantitative measurements of hippocampal and WMH. In primary analyses, we assessed associations of NfL with a 10-point white matter grade (WMG) and prevalent infarcts on second MRI and with worsening WMG and incident infarct comparing the 2 scans. A p value <0.0125 (0.05/4) was considered significant for these analyses. We also assessed associations with hippocampal and WMH volume. In fully adjusted models, log2(NfL) concentration was associated with WMG (β = 0.27; p = 2.3 × 10-4) and worsening WMG (relative risk [RR] 1.24; p = 0.0022), but less strongly with prevalent brain infarcts (RR 1.18; p = 0.013) and not with incident brain infarcts (RR 1.18; p = 0.18). Associations were also present with WMH volume (β = 2,242.9, p = 0.0036). For the other 3 biomarkers, the associations for log2 (GFAP) concentration with WMG and worsening WMG were significant. Among older adults without a history of stroke, higher serum NfL concentration was associated with covert MRI findings of vascular brain injury, especially the burden of WMH and its worsening. Whether these results offer opportunities for the use of NfL as a noninvasive biomarker of WMH or to control vascular risk factors remains to be determined.

Outcome of non-cooled asphyxiated infants with under-recognised or delayed-onset encephalopathy

ObjectiveTo describe the clinical characteristics, MRI findings and neurodevelopmental outcome of infants with documented perinatal asphyxia and seizure onset within 24 hours after birth who were not selected for therapeutic...

Sneddon’s Syndrome as rare and underdiagnosed cause of stroke: about two cases / A Síndrome de Sneddon como causa rara e subdiagnosticada de AVC: cerca de dois casos

Case 1. Female, 27 years old, with migraine with aura. Use of combined contraceptive therapy. She presented visual and cutaneous complaints for eight months, using prednisone 60mg/day due to the hypothesis of systemic vasculitis. On examination, right temporal hemianopsia, normal fundus of the eye, Raynaud ‘s phenomenon with necrosis stitches in fingers and livedo were noted. Brain MRI with diffusion restriction in the left occipital lobe, hypersignal foci in the parietal lobe, and head of the left caudate nucleus. Reticular Livedo (RL) compatible biopsy. Anticardiolipin IgM (13.3 U/mL), ESR (47mm/h in the 1st hour and 52mm/h in the 2nd hour), and VDRL 1:8 reagents. Normal CSF. Initially treated with warfarin, she has been using rivaroxaban for two years, remaining asymptomatic. Case 2. Female, 39 years old, with a report of sudden dysarthria and right hemiparesis, with a previous episode of vertigo. A lumbosacral spine MRI was performed, presenting no alterations. Normal CSF. Antiphospholipid antibodies: Beta-2-glycoprotein I IgM 21 UR/mL and IgG 9 UR/mL, lupus anticoagulant 0.98, anticardiolipin IgG 9.4 GPL and IgM 31 MPL. Cranial MRI showed old ischemic gaps and hyperintense areas on T2/FLAIR with diffusion restriction affecting the left paramedian portion of the pons, related to acute stroke. Presence of livedo in the distal region of the limbs. In current use of warfarin 7.5mg/day, presenting remission of symptoms. Discussion: Sneddon Syndrome (SS) is a non-inflammatory thrombotic vasculopathy characterized by the concomitant presence of livedo reticularis and stroke. Neurological manifestations are present in up to 77% of cases. It is more prevalent in women between 20 and 42 years old. Treatment is aimed at preventing potentially serious, disabling, and even fatal new vascular events. Final Comments: SS is a rare syndrome that probably results from a series of acquired or congenital hemostatic abnormalities that preferentially involve the cutaneous and cerebral vascular beds. Although it has no specific biomarker, there are reports associating SS with antiphospholipid antibodies and VDRL reactor. Clinical presentation, laboratory findings and skin biopsy associated with an excellent response to anticoagulant treatment were fundamental in the diagnostic elucidation.

Three novel mutations of the BCKDHA, BCKDHB and DBT genes in Chinese children with maple syrup urine disease.

Maple syrup urine disease (MSUD) is a rare metabolic autosomal recessive disorder caused by deficiency of the branched-chain α-ketoacid dehydrogenase complex. Mutations in the BCKDHA, BCKDHB and DBT genes are responsible for MSUD. This study presents the clinical and molecular characterizations of four MSUD patients. Clinical data of patients were retrospectively analyzed, and genetic mutations were identified by whole-exome sequencing. CLUSTALX was employed to analyzed cross-species conservation of the mutant amino acid. The impact of the mutations was analyzed with PolyPhen-2 software. The I-TASSER website and PyMOL software were used to predict the protein three-position structure of the novel mutations carried by the patients. Vomiting, irritability, feeding difficulties, seizures, dyspnoea, lethargy and coma were the main clinical presentations of MSUD. Cranial MRI showed abnormal symmetrical signals in accordance with the presentation of inherited metabolic encephalopathy. Seven mutations were detected in four patients, including three novel pathogenic mutations in the BCKDHA (c.656C>A), BCKDHB (deletion of a single-copy of BCKDHB) and DBT (c.1219dup) genes. Structural changes were compatible with the observed phenotypes. Different types of MSUD can display heterogeneous clinical manifestations. Exhaustive molecular studies are necessary for a proper differential diagnosis. The newly identified mutation will play a key role in the prenatal diagnosis of MSUD in the future.

Peripheral and central smell regions in children with epilepsy: An MRI evaluation

ObjectivesWe investigated the peripheral and central olfactory regions in children with epilepsy using cranial MRI. MethodsIn this retrospective study, cranial MRI images were obtained from 23 children with epilepsy and 23 healthy controls. Olfactory bulb (OB) volume and olfactory sulcus (OS) depth in the peripheral olfactory region and insular cortex and corpus amygdala areas in the central olfactory region were measured. ResultsThere was no significant difference in the OB volume and OS depth in the peripheral olfactory regions in the two groups (p > 0.05). In the central olfactory region, the insular cortex and corpus amygdala areas in the epilepsy group were significantly smaller than those in the control group (p < 0.05). In both groups, the OS depth on the right side was significantly higher than that on the left side (p < 0.05). In the epilepsy group, there were positive correlations between each of the OB volumes, OS depths, insular cortex areas, and corpus amygdala areas bilaterally (p < 0.05). In both groups, there were positive correlations between the OB volume and OS depth, OS depth and insular cortex area and insular cortex area and corpus amygdala areas (p < 0.05). ConclusionA change in the central olfactory region in epileptic patients may be related to central tissue damage due to epilepsy. This finding has important implications for epilepsy patients, with early diagnosis and treatment potentially preventing a reduction in the volumes/depths of components of the central olfactory region in the pediatric population.

Bevacizumab-induced isolated oculomotor nerve palsy in glioblastoma multiforme

Bevacizumab, a monoclonal antibody against the vascular endothelial growth factor receptor, is the standard treatment of recurrent glioblastoma multiforme. In addition to common systemic side effects of bevacizumab, there are rare cases of cranial nerve palsy. We report a case of transient oculomotor nerve palsy after systemic administration of bevacizumab. Twenty-four hours after the systemic infusion of bevacizumab, transient oculomotor nerve palsy developed in a 49-year-old male patient. In the cranial MRI, there was no malignancy-related progression. Bevacizumab treatment was discontinued. Methylprednisolone was started considering that bevacizumab increased the inflammatory response. Oculomotor nerve palsy resolved in 14 days. There are many side effects of bevacizumab whose mechanisms of action have not been fully explained. Cranial nerve involvement is rarely reported. Our case is the first reported case of bevacizumab-induced oculomotor nerve palsy.

Rare Occurrence of Microsporidial Myositis Involving Masticatory Muscles.

A 45-year-old woman with AIDS on antiretroviral therapy (tenofovir disoproxil, lamivudine, dolutegravir) presented with painful mastication and generalized musculoskeletal pain for 6 weeks. She had bilateral hand contractures, with tender masseters, temporalis, and gastrocnemii. Absolute eosinophil count was 500 cells/mm3, creatine phosphokinase 66 U/L, and CD4 count 147 cells/mm3. Cranial MRI showed infiltration of masticatory muscles (Figure 1). Muscle biopsy revealed microsporidial myositis (Figure 2). Microsporidia are obligate intracellular fungi classically affecting immunocompromised hosts.1 We present findings of rare microsporidial myositis affecting masticatory muscles. Muscle biopsy is diagnostic and excludes potential clinical (polymyalgia rheumatica, temporal arteritis), medication-related, and histologic mimics.2

Study on the pathogenesis of Holmes tremor by multimodal 3D medical imaging: case reports of three patients

BackgroundWe examined for the first time the imaging characteristics of Holmes tremor (HT) through multimodal 3D medical imaging.Case presentationThree patients with Holmes tremor who visited the Affiliated Hospital of Chengdu University of TCM from August 2018 to April 2021 were retrospectively investigated to summarize their clinical and imaging data.ResultsHolmes tremor in two of the three patients was caused by hypertensive cerebral hemorrhage and in the third patient induced by hemorrhage due to ruptured brain arteriovenous malformations. HT occurred 1 to 24 months after the primary disease onset and manifested as a tremor in the contralateral limb, mostly in the upper portion. Cranial MRI showed that the lesions involved the thalamus in all three patients. The damaged thalamic nuclei included the ventral anterior nucleus, ventral lateral nucleus and ventromedial lateral nucleus, and the damaged nerve fibers included left thalamocortical tracts in one patient. In the other two patients, the damaged thalamic nuclei included the centromedian and dorsomedial nucleus, and the damaged nerve fibers included left cerebellothalamic and thalamocortical tracts. One patient showed significant improvement after treatment with pramipexole while the other two patients exhibited a poor response, one of whom had no response to the treatment with pramipexole and was only significantly relieved by clonazepam.ConclusionWe used multimodal 3D medical imaging for the first time to analyze the pathogenesis of HT and found that multiple thalamic nuclei were damaged. The damaged nuclei and nerve fiber tracts of two patients were different from those of the third patient, with different clinical manifestations and therapeutic effects. Therefore, it is speculated that there may be multiple pathogeneses for HT.

An Endoscopic Intraventricular Approach in Glioblastoma: A Case Report of an Adult Filipino with Periventricular Mass

In this study, we present a case of a 58-year-old female with a 1-year history of a generalized headache who suddenly developed left-sided weakness accompanied by disorientation and changes in mood and behaviour. A cranial MRI plain and contrast revealed a right thalamo-mesencephalic mass with beginning hydrocephalus. The patient underwent endoscopic transventricular septostomy, biopsy of thalamic mass with ventriculoperitoneal shunt insertion under endoscopic guidance. The official histopathology results revealed a WHO Grade IV glioblastoma. The patient was subsequently treated with chemoradiation.

Potential risk factors of persistent postural-perceptual dizziness: a pilot study.

Persistent postural-perceptual dizziness (PPPD) unifies the main characteristics of chronic subjective dizziness, visual vertigo and related diseases, which is a common chronic disease in neurology. At present, the pathology of PPPD is not fully understood. In this single-center retrospective case series review, we aim to investigate the potential risk factors of PPPD. Eighty inpatients diagnosed with PPPD were recruited with 81 apparently healthy controls. Patient-specific clinico-radiological data were collected from both groups. Conditions of hypertension, diabetes, smoking, and drinking were derived from medical history. Blood test results were recorded including total cholesterol, triglyceride, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, fibrinogen, vitamin B12, folic acid, total cholesterol, triglyceride, and folate level. The subjects were examined by carotid artery CTA and cranial MRI, and the imaging findings of carotid atherosclerosis (CAS), white matter hyperintensities (WMHs) and lacunar infarction (LI) were recorded. Binary logistic regression analysis was used to investigate the difference between the case and control groups. Significance was defined as p value less than 0.05. The prevalence rate of hypertension in the case group was significantly higher than that in the control group, and the detection rates of CAS, WMHs, and LI in the case group were significantly higher than those in the control group (p < 0.05 for all). Hypertension, CAS, WMHs, and LI are associated with PPPD, which may be potential risk factors for its development.

Relationship between the Quantitative Indicators of Cranial MRI and the Early Neurodevelopment of Preterm Infants.

Aim To explore the relationship between the quantitative indicators (biparietal width, interhemispheric distance) of the cranial MRI for preterm infants at 37 weeks of postmenstrual age (PMA) and neurodevelopment at 6 months of corrected age. Methods A total of 113 preterm infants (gestational age < 37 weeks) delivered in the Obstetrics Department of the First People's Hospital of Lianyungang from September 2018 to February 2020 and directly transferred to the Neonatology Department for treatment were enrolled in this study. Based on their development quotient (DQ), the patients were divided into the normal (DQ ≥ 85, n = 76) group and the abnormal (DQ < 85, n = 37) group. Routine cranial MRI (cMRI) was performed at 37 weeks of PMA to measure the biparietal width (BPW) and interhemispheric distance (IHD). At the corrected age of 6 months, Development Screening Test (for children under six) was used to assess the participants' neurodevelopment. Results Univariate analysis showed statistically significant differences in BPW, IHD, and the incidence of bronchopulmonary dysplasia between the normal and the abnormal groups (P < 0.05), while no statistically significant differences were found in maternal complications and other clinical conditions between the two groups (P > 0.05). Binary logistic regression analysis demonstrated statistically significant differences in IHD and BPW between the normal and the abnormal groups (95% CI: 1.629-12.651 and 0.570-0.805, respectively; P = 0.004 and P < 0.001, respectively), while no significant differences were found in the incidence of bronchopulmonary dysplasia between the two groups (95% CI: 0.669-77.227, P = 0.104). Receiver operating characteristic curve revealed that the area under the curve of BPW, IHD, and the joint predictor (BPW + IHD) were 0.867, 0.805, and 0.881, respectively (95% CI: 0.800-0.933, 0.710-0.900, and 0.819-0.943, respectively; all P values < 0.001). Conclusion BPW and IHD, the two quantitative indicators acquired by cMRI, could predict the neurodevelopmental outcome of preterm infants at the corrected age of 6 months. The combination of the two indicators showed an even higher predictive value.

Compound Heterozygous ROBO3 Mutation in Two Siblings Presenting with Horizontal Gaze Palsy without Scoliosis: Case-Based Review.

Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare, autosomal recessively inherited disorder characterized by a congenital absence of conjugated horizontal eye movements with progressive scoliosis developing in childhood and adolescence. HGPPS is caused by mutations of the ROBO3 gene that disrupts the midline crossing of the descending corticospinal and ascending lemniscal sensory tracts in the medulla. We present two siblings, 5-year-old and 2-year-old boys with HGPPS, from non-consanguineous parents. The older brother was brought for the evaluation of moderate psychomotor retardation. He had bilateral horizontal gaze palsy with preserved vertical gaze and convergence. Scoliosis was absent. Cranial MRI showed brainstem abnormalities, and diffusion tensor imaging showed absent decussation of cortico-spinal tracts in the medulla. Clinical diagnosis of HGPPS was confirmed by sequencing of ROBO3 gene, IVS4-1G > A (c.767-1G > A) and c.328_329delinsCCC (p.Asp110Profs*57) compound heterozygous variations were found, and segregated in parents. The younger boy was first reported at 16 months of age and had the same clinical and neuroradiological findings, unlike mild psychomotor retardation. ROBO3 gene analysis showed the same variants in his brother. Our cases show the importance of evaluating eye movements in children with neurodevelopmental abnormalities and looking for brainstem abnormalities in children with bilateral horizontal gaze palsy.

Isolated CNS Hemophagocytic Lymphohistiocytosis in Children:What We Know, What We Don't Know ?

The presence of central nervous system involvement has a profound impact on the prognosis, treatment, and clinical outcome of the primary hemophagocytic lymphohistiocytosis (pHLH). However, isolated CNS-HLH is a challenging disease with a high mortality and morbidity , possibly resulting from a spsecific neuroinflammation that leads to isolated disease only without systemic activation under some additional genetic modifiers. In this study, we retrospectively reviewed our isolated CNS-HLH cases and there were 73 patients (36 male, 37 female) with a median age of 20 months (range, 1- 226 months) diagnosed as primary hemophagocytic lymphohistiocytosis at Hacettepe University Faculty of Medicine, between January 2005 and June 2021. Among these, 39 (53%) patients had central nervous system involvement either on admission or during the recurrence. On admission, the number of patients who had both CNS and sytemic involvement was 19 (49%), moreover 2 had CNS infiltration both initially and during the course of relapse. 8 patient did show CNS involvement only during the relapse. Ten (25%) patients (5 male, 5 female) with isolated CNS involvement are the main subject of this study and none of them had infectious trigger. What we know is they were presented with mostly unexplained neurological findings and /or cranial nerve paralysis. In this group median age at presentation was 101 months ( range 6 - 180 months). They all had primary HLH associated patogenic mutation and in some of them diagnosed was also confirmed by brain biopsy. Neither family history and /or consanguinity nor HLH criteria are fullfilling in this devastating disorder. Cranial MRI gives many clues during admission in experienced hands. Two of our published cases were initially diagnosed as lymphomatoid granulomatosis and acute disseminated encephalomyelitis ; they were diagnosed as hemophagocytic lymphohistiocytosis after developing systemic symptoms 3 and 12 months later. Interestingly 6 of 10 patients in this group never developed systemic symptomps, 7 patients underwent allogeneic bone marrow transplantation. Spinal cord involvement was determined in 8 patients(20%), including 4 at diagnosis and 4 during follow up ; including one previously published case, 4 out of 8 had isolated cases did show spinal involvement as well. Even though few number of cases with isolated CNS-HLH has been reported in the recent years, we believe that the number of such cases is not limited to those who have been reported because it is rather difficult to diagnose patients with isolated CNS symptoms, which leads to misdiagnosis and/or mistreatment. What we don't know is how to specifically treat patients with CNS directed therapy, and exactly which mutations are associated with isolated CNS-HLH or whether there is a known tendency in this group and perhaps unknown mutations ? Does it have a facilitating effect ? DisclosuresNo relevant conflicts of interest to declare.

Predictors of fever response in tuberculous meningitis: A clinical, MRI and biomarker study.

Central nervous system (CNS) has a different immune surveillance system; therefore, fever at admission and timeline of fever response after antitubercular treatment (ATT) may follow a different course in CNS infection. We report the predictors of fever response in tuberculous meningitis (TBM) including the effect of tumour necrosis factor-α (TNF-α) in cerebrospinal fluid (CSF) and its gene expression at mRNA of peripheral blood mononuclear cells (PBMCs). Fifty-seven patients with TBM were prospectively evaluated. Their clinical findings and severity of meningitis were recorded. The expression of TNF-α gene in PBMCs was quantified by real-time polymerase chain reaction and TNF-α concentration in CSF by cytokine bead array both in the patients and 14 matched controls. All the patients had history of fever for a median duration of 75days. The admission temperature ranged between 37.2°C and 40°C and correlated with CSF cell counts (p<0.05). Cranial MRI was abnormal in 54 (94.7%) and revealed exudates in 33(57.9%), hydrocephalus in 27(47.4%), infarction in 27(47.4%) and tuberculoma in 33(57.9%) patients. Fever subsided after a median duration of 18 (2 60)days of treatment. Twelve (21.8%) patients only became afebrile within 10days. The expression of TNF-α gene correlated with CSF concentration of TNF-α (p=0.02) and independently predicted duration of defervescence [adjusted hazard ratio 1.02 (95% CI 1.00-1.04; p=0.01). In the patients with TBM, defervescence takes longer time, and TNF-α gene expression predicts the duration of defervescence. Future studies are needed to evaluate the role of TNF-α-modifying drugs in TBM.

Peripheric smell regions in patients with semicircular canal dehiscence: An MRI evaluation

ObjectivesWe investigated the peripheric smell regions in patients with semicircular canal dehiscence (SCD) by cranial MRI. MethodsIn this retrospective study, cranial MRI images of 186 adult patients were included. Group 1 consisted of 83 adult patients with SCD. The control group (Group 2) consisted of 83 healthy subjects without central vertigo. Olfactory bulb (OB) volume and olfactory sulcus (OS) depth were measured in all groups. ResultsIn group 1, SCD was detected on the right (33.7%), left (26.5%) sides and bilateral (39.8%). Localization of dehiscence was at superior SC (75.9%), posterior SC (21.7%), lateral SC (1.2%), and posterior + superior SCs (1.2%). OB volumes of the SCD group were significantly lower than the control group bilaterally (p < 0.05). There were no significant differences between OS depths of groups 1 and 2 (p > 0.05). In SCD group, there were positive correlations between OB volumes; OS depths; and OB volumes and OS depths (p < 0.05). In older patients, bilateral OS depth values got lower (p < 0.05). In females, left OB volume values were lower than males (p < 0.05). In right SCD (+) patients, left OS depth values got lower (p < 0.05). ConclusionWe concluded that possible changes in CSF dynamics may cause the development of SCD at thin bone segments; and a decrease in the OB volume. CSF leaks into the perineural sheet of the olfactory bulb (OB) maybe responsible for the decrease in the OB volume. In addition, minor trauma, infection, and inflammation may also be responsible for both coexistences of SCD development and OB volume decrease.

Cost-Effectiveness of Prophylactic Cranial Irradiation vs. MRI Surveillance for Extensive Stage Small Cell Lung Cancer

Due to conflicting prospective data, controversy exists regarding prophylactic cranial irradiation (PCI) in extensive stage small cell lung cancer (ES-SCLC). We sought to evaluate the cost-effectiveness of PCI versus MRI surveillance alone in the context of modern radiotherapy treatment modalities using available evidence and the CMS alternative payment model (APM).A Markov state transition model was constructed using software to perform a cost-effectiveness analysis comparing MRI surveillance alone with PCI for ES-SCLC. PCI in the base case analysis was assumed to be 25 Gy in 10 fractions delivered with a 3D conformal radiotherapy technique (3D-CRT) without daily image guidance. In the MRI surveillance alone arm, MRI surveillance was assumed to be performed at baseline then every 3 months for the first year and every 6 months for year 2, consistent with the surveillance plan currently being implemented in the SWOG MAVERICK cooperative group trial (NCT04155034). Clinical parameters were obtained from clinical trial data, and cost data were based on 2019 Medicare reimbursement. Strategies were compared using the incremental cost-effectiveness ratio (ICER) with effectiveness in quality-adjusted life years (QALYs) and evaluated with a willingness to pay threshold of $100,000 per QALY gained. One-way and probabilistic sensitivity analyses were performed to consider model uncertainty.In the base case scenario, PCI was not cost-effective with an ICER of $168,456 per QALY gained compared to MRI surveillance alone, assuming that overall survival was 10 months and 12.5 months for MRI surveillance alone and PCI, respectively. One-way sensitivity analysis showed that results were most sensitive to the variation of overall survival and cognitive decline rates between the two groups. In a scenario analysis, when all patients receive hippocampal-avoidance PCI (HA-PCI), the model results become near cost effective with PCI due to presumed lower rate of cognitive decline (ICER $129,307 per QALY gained). If HA-IMRT with memantine was also utilized for salvage treatment in all patients in the MRI surveillance group with the assumption above, PCI was found to be cost effective ($88,096 per QALY gained). In addition, the total radiation therapy reimbursement per episode suggested by APM for radiation therapy for brain metastases ($10,783) is almost identical to the current Medicare reimbursement rate for 10 fractions with HA-IMRT ($10,445). Thus, this scenario analysis also reflected the use of the APM.PCI was not cost effective compared to MRI surveillance alone due to the neurocognition decline impact of PCI based on available evidence. HA-PCI may be a potential cost-effective strategy for ES-SCLC, with expected confirmation following the ongoing SWOG MAVERICK trial, which includes assessments of cognitive function.

Electrical status epilepticus in sleep: The role of thalamus in etiopathogenesis

PurposeIn patients diagnosed with epilepsy, decreased ratio of N-acetyl aspartate to creatine (NAA/Cr) measured in magnetic resonance spectroscopy (MRS) has been accepted as a sign of neuronal cell loss or dysfunction. In this study, we aimed to determine whether a similar neuronal cell loss is present in a group of encephalopathy with electrical status epilepticus in sleep (ESES) patients MethodsWe performed this case-control study at a tertiary pediatric neurology center with patients with ESES. Inclusion criteria for the patient group were as follows: 1) a spike-wave index of at least 50%, 2) acquired neuropsychological regression, 3) normal cranial MRI. Eventually, a total of 21 patients with ESES and 17 control subjects were enrolled in the study. MRI of all control subjects was also within normal limits. 3D Slicer program was used for the analysis of thalamic and brain volumes. LCModel spectral fitting software was used to analyze single-voxel MRS data from the right and left thalamus of the subjects. ResultsThe mean age was 8.0 ± 1.88 years and 8.3 ± 1.70 years in ESES patients and the control subjects. After correcting for the main potential confounders (age and gender) with a linear regression model, NAA/Creatine ratio of the right thalamus was significantly lower in the ESES patient group compared to the healthy control group (p = 0.026). Likewise, the left thalamus NAA/Cr ratio was significantly lower in the ESES patient group than the healthy control group (p = 0.007). After correcting for age and gender, right thalamic volume was not statistically significantly smaller in ESES patients than in healthy controls (p = 0.337), but left thalamic volume was smaller in ESES patients than in healthy controls (p = 0.024). ConclusionIn ESES patients, the NAA/Creatine ratio, which is an indicator of neuronal cell loss or dysfunction in the right and left thalamus, which appears regular on MRI, was found to be significantly lower than the healthy control group. This metabolic-induced thalamic dysfunction, which was reported for the first time up to date, may play a role in ESES epileptogenesis.

Decreased Need for Anesthesia during Ultra-Fast Cranial MRI inYoung Children: One-Year Summary.

Rapid volume coverage sequences based on real-time MRI allow for scanning of the entire brain within a few seconds. Movements of children become almost irrelevant due to the ultra-fast acquisition of 30 ms per slice. The adoption of these sequences in a real-time cranial MRI protocol (RT-cMRI) is expected to reduce the frequency of examinations requiring anesthesia in infants and toddlers. The aim of the study was to quantify the reduction in the number of anesthesia examinations in young children after the implementation of the new RT-cMRI protocol. All cMRI studies of children up to 6years in the first 12 months after the establishment of the RT-cMRI 2019/2020 were retrospectively compared to a matched group of the same period in 2017/2018. The frequency of examinations under anesthesia vs. non-sedation examinations was analyzed. In addition, the number of follow-up examinations and the effectiveness of RT-cMRI was determined. The launch of RT-cMRI led to a significant decrease in the proportion of cMRI under anesthesia from 92 % to 55 %. Only 2 % of the RT-cMRI failed and required conventional MRI under sedation in the follow-up. The speed and ease of use of RT-cMRI increased the number of follow-up examinations from 1.3 to 1.4 examinations per child. This innovative real-time MRI examination allows a drastic reduction in the number of studies under anesthesia for suitable cranial pathologies in children under 6 years. However, cautious selection of indications as well as adjustments to the workflow in the radiological department are required. · Real-time MRI sequences are almost unaffected by patient movement. · The application of real-time cranial MRI can spare children from sedation. · Low-threshold access results in more frequent follow-up examinations. · Sorge I, Hirsch FW, Voit D et al. Decreased Need for Anesthesia during Ultra-Fast Cranial MRI in Young Children: One-Year Summary. Fortschr Röntgenstr 2022; 194: 192 - 198.

MRI characteristics of brain edema in preeclampsia/eclampsia patients with posterior reversible encephalopathy syndrome

BackgroundThe neuroimaging manifestations of eclampsia and preeclampsia often overlap, mainly presenting as posterior reversible encephalopathy syndrome (PRES). The purpose of this retrospective study was to compare the extent and nature of brain edema in eclampsia and preeclampsia patients with PRES based on MRI characteristics.MethodsOne hundred fifty women diagnosed with preeclampsia-eclampsia and undergoing cranial MRI were enrolled; 24 of these were diagnosed as having eclampsia. According to clinicoradiologic diagnosis of PRES, eligible patients were classified as having eclampsia with PRES (group E-PRES) and preeclampsia with PRES (group P-PRES). A scale on T2W FLAIR-SPIR images was established to evaluate the extent of brain edema, and the score of brain edema (SBE) of both groups was compared. In patients of the two groups who also underwent DWI sequence, the presence or absence of hyperintensity on DWI and hypointensity on ADC maps were determined to compare the nature of brain edema. Furthermore, clinical and biochemical data of the two groups were compared.ResultsThe incidence of PRES in eclampsia patients was significantly higher than that in preeclampsia patients (87.50% vs. 46.03%, P<0.001). The SBE of all regions and typical regions in group E-PRES patients were significantly higher than those in group P-PRES patients (15.88±8.72 vs. 10.90±10.21, P=0.021; 8.52±3.87 vs. 5.01±4.19, P=0.002; respectively). The presence of hyperintensity on DWI was determined more frequently in group E-PRES patients than group P-PRES patients (71.43% vs. 32.00%, P=0.024). Age, systolic blood pressure, white blood cell count, neutrophil count and percentage of neutrophils were significantly different between the two groups (P<0.05).ConclusionsCertain MRI characteristics that reflect the extent and nature of brain edema were different between eclampsia and preeclampsia patients with PRES. Additional prospective studies are still required to explore whether these MRI characteristics of brain edema may further become a potential predictor for eclamptic seizures in preeclampsia patients with PRES.