Brain Magnetic Resonance Imaging Changes Research Articles

Clinical features of neurotoxicity after CD19 CAR T-cell therapy in mantle cell lymphoma

AbstractCD19 chimeric antigen receptor (CAR) T-cell therapy has proven highly effective for treating relapsed/refractory mantle cell lymphoma (MCL). However, immune effector cell–associated neurotoxicity syndrome (ICANS) remains a significant concern. This study aimed to evaluate the clinical, radiological, and laboratory correlatives associated with ICANS development after CD19 CAR T-cell therapy in patients with MCL. All patients (N = 26) who received standard-of-care brexucabtagene autoleucel until July 2022 at our institution were evaluated. Laboratory and radiographic correlatives including brain magnetic resonance imaging (MRI) and electroencephalogram (EEG) were evaluated to determine the clinical impact of ICANS. Seventeen (65%) patients experienced ICANS after treatment, with a median onset on day 6. Ten (38%) patients experienced severe (grade ≥3) ICANS. All patients with ICANS had antecedent cytokine release syndrome (CRS), but no correlation was observed between ICANS severity and CRS grade. Overall, 92% of EEGs revealed interictal changes; no patients experienced frank seizures because of ICANS. In total, 86% of patients with severe ICANS with postinfusion brain MRIs demonstrated acute neuroimaging findings not seen on pretreatment MRI. Severe ICANS was also associated with higher rates of cytopenia, coagulopathy, increased cumulative steroid exposure, and prolonged hospitalization. However, severe ICANS did not affect treatment outcomes of patients with MCL. Severe ICANS is frequently associated with a range of postinfusion brain MRI changes and abnormal EEG findings. Longer hospitalization was observed in patients with severe ICANS, especially those with abnormal acute MRI or EEG findings, but there was no discernible impact on overall treatment response and survival.

Brain magnetic resonance imaging as predictors in pediatric anti-N-methyl-D-aspartate receptor encephalitis

ObjectiveTo investigate the associations between brain magnetic resonance imaging (MRI) changes and clinical profiles in children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. MethodsClinical data and brain MRI results of children diagnosed with anti-NMDAR encephalitis in Guangzhou Women and Children's Medical Center from October 2014 to June 2022 were retrospectively studied. ResultsA total of 143 children (Male: female 54:89) were enrolled, with a mean onset age of 6.8 years (6.8 ± 3.1). 40.6 % (58/143) of patients had abnormal initial brain MRI. Lesions in temporal lobe (34.5 %, 20/58) and frontal lobe (25.9 %, 15/58) were relatively common. Children with abnormal initial brain MRI were prone to have fever (P = 0.023), dystonia (P = 0.037), positive MOG antibodies (P = 0.015), higher cerebrospinal fluid (CSF) white blood cell count (WBC) (P = 0.019) and to receive rituximab treatment (P = 0.037). There were no significant differences in modified Rankin Scale (mRS) scores before immunotherapy, after immunotherapy and at last follow-up between the normal initial brain MRI group and abnormal group. No initial brain MRI changes were found to be associated with relapses. Brain MRI was reviewed in 72 patients at last follow-up with a median follow-up time of 25.5 months and 48.6 % (35/72) of patients had abnormal brain MRI. The mRS score of the group with normal brain MRI at last follow-up was significantly lower than that of the abnormal group. ConclusionsAbout 40.0 % of children with anti-NMDAR encephalitis had abnormal initial brain MRI. Initial brain MRI was associated with certain clinical profiles, but not with relapse and prognosis. Around half of patients had abnormal brain MRI at last follow-up and were prone to have higher mRS score.

Volumetric changes in brain MRI of infants with hypoxic-ischemic encephalopathy and abnormal neurodevelopment who underwent therapeutic hypothermia

BackgroundHypoxic-ischemic encephalopathy (HIE) is a severe neonatal complication that can result in 40–60 % of long-term morbidity. Magnetic Resonance Imaging (MRI) is a noninvasive method which is usually performed before discharge to visually assess acquired cerebral lesions associated with HIE and severity of lesions possibly providing a guide for detecting adverse outcomes. This study aims to evaluate the impact of HIE on brain volume changes observed in MRI scans performed at a mean 10 days of life, which can serve as a prognostic indicator for abnormal neurodevelopmental (ND) outcomes at 18–24 months among HIE infants. MethodsWe retrospectively identified a cohort of HIE patients between June 2013 and March 2017. The inclusion criteria for therapeutic hypothermia (TH) were a gestational age ≥35 weeks, a birth weight ≥1800 g, and the presence of ≥ moderate HIE. Brain MRI was performed at a mean 10 days of life and brain volumes (total brain volume, cerebral volume, cerebellar volume, brain stem volume, and ventricle volume) were measured for quantitative assessment. At 18–24 months, the infants returned for follow-up evaluations, during which their cognitive, language, and motor skills were assessed using the Bayley Scales of Infant and Toddler Development III. ResultsThe study recruited a total of 240 infants between 2013 and 2017 for volumetric brain MRI evaluation. Among these, 83 were normal control infants, 107 were TH-treated HIE infants and 37 were HIE infants who did not receive TH due to contraindications. Clinical evaluation was further proceeded. We compared the brain volumes between the normal control infants (n = 83) with normal ND but TH-treated HIE infants (n = 76), abnormal ND TH-treated HIE infants (n = 31), and the severe HIE MRI group with no TH (n = 37). The abnormal ND TH-treated HIE infants demonstrated a significant decrease in brainstem volume and an increase in ventricle size (p < 0.001) (Table 4). Lastly, the severe brain MRI group who did not receive TH showed significantly smaller brain stem (p = 0.006), cerebellar (p = 0.006) and cerebrum volumes (p = 0.027), accompanied by larger ventricular size (p = 0.013) compared to the normal control group (Table 5). ConclusionIn addition to assessing the location of brain injuries in MRI scans, the reduction in brain stem volume coupled with an increase in ventricular volume in HIE infants may serve as a biomarker indicating severe HIE and adverse long-term ND outcomes among HIE infants who either received therapeutic hypothermia (TH) treatment or not.

High serum neurofilament light chain levels correlate with brain atrophy and physical disability in multiple sclerosis.

Serum neurofilament light chain (sNfL) is a promising biomarker of neuroaxonal damage in persons with multiple sclerosis (pwMS). In cross-sectional studies, sNfL has been associated with disease activity and brain magnetic resonance imaging (MRI) changes; however, it is still unclear to what extent in particular high sNfL levels impact on subsequent disease evolution. sNfL was quantified by an ultrasensitive single molecule array (Simoa) in 199 pwMS (median age=34.2 years, 64.3% female) and 49 controls. All pwMS underwent 3-T MRI to assess global and compartmental normalized brain volumes, T2-lesion load, and cortical mean thickness. Follow-up data and serum samples were available in 144 pwMS (median follow-up time=3.8 years). Linear and binary logistic models were used to estimate the independent contribution of sNfL for changes in MRI and Expanded Disability Status Scale (EDSS). Age-corrected sNfL z-scores from a normative database of healthy controls were used for sensitivity analyses. High sNfL levels at baseline were associated with atrophy measures of the whole brain (standardized beta coefficient βj= -0.352, p< 0.001), white matter (βj= -0.229, p= 0.007), thalamus (βj= -0.372, p= 0.004), and putamen (βj= -1.687, p= 0.012). pwMS with high levels of sNfL at baseline and follow-up had a greater risk of EDSS worsening (p= 0.007). Already single time point elevation of sNfL has a distinct effect on brain volume changes over a short-term period, and repeated high levels of sNfL indicate accumulating physical disability. Serial assessment of sNfL may provide added value in the clinical management of pwMS.

Magnetic resonance imaging of human variegated squirrel bornavirus 1 (VSBV-1) encephalitis reveals diagnostic pattern indistinguishable from Borna disease virus 1 (BoDV-1) encephalitis but typical for bornaviruses

ABSTRACT Human bornavirus encephalitis is an emerging disease caused by the variegated squirrel bornavirus 1 (VSBV-1) and the Borna disease virus 1 (BoDV-1). While characteristic brain magnetic resonance imaging (MRI) changes have been described for BoDV-1 encephalitis, only scarce diagnostic data in VSBV-1 encephalitis exist. We systematically analysed brain MRI scans from all known VSBV-1 encephalitis patients. Initial and follow-up scans demonstrated characteristic T2 hyperintense lesions in the limbic system and the basal ganglia, followed by the brainstem. No involvement of the cerebellar cortex was seen. Deep white matter affection occurred in a later stage of the disease. Strict symmetry of pathologic changes was seen in 62%. T2 hyperintense areas were often associated with low T1 signal intensity and with mass effect. Sinusitis in three patients on the first MRI and an early involvement of the limbic system suggest an olfactory route of VSBV-1 entry. The viral spread could occur per continuitatem to adjacent anatomical brain regions or along specific neural tracts to more distant brain regions. The number and extent of lesions did not correlate with the length of patients’ survivals. The overall pattern closely resembles that described for BoDV-1 encephalitis. The exact bornavirus species can thus not be deduced from imaging results alone, and molecular testing and serology should be performed to confirm the causative bornavirus. As VSBV-1 is likely of tropical origin, and MRI investigations are increasingly available globally, imaging techniques might be helpful to facilitate an early presumptive diagnosis of VSBV-1 encephalitis when molecular and/or serological testing is not available.

Evaluation of neurologically symptomatic and asymptomatic patients in childhood Wilson's disease with central nervous system involvement: A retrospective observational study

Abstract Objective: The aim was to evaluate the clinical and laboratory characteristics of children with Wilson's disease (WD) with and without neurological presentation with abnormalities on brain magnetic resonance imaging (MRI) and to describe the relationship of these observations with disease severity and functional outcome and their impact on prognosis. Materials and methods: Demographic, neuropsychiatric findings, laboratory, disease severity and functional results of 48 children with neurologically symptomatic and asymptomatic WD were evaluated retrospectively. Results: A significant positive correlation was found between the neurologic symptom score and functional outcome in WD with neurological presentation (p &lt;0.001). A significant positive correlation was found between disease severity scores (PELD, MELD, Child Pugh and Dhwin score) and modified Rankin scores (mRS) in WD without neurological presentation (p &lt;0.001, 0.004, &lt;0.001, 0.001, respectively). In addition, a significant positive correlation was found between total bilirubin, direct bilirubin and Internationel Normalized Ratio (INR) values and mRS scores (p= 0.006, 0.012, 0.004, respectively). Kayser-Fleischer Ring sign in the eye was higher in the group with neurological symtomatic WD (p &lt;0.001). The number of patients presenting with clinic of fulminant hepatitis and hepatic encephalopathy was significantly higher in the neurological asymptomatic group (p &lt;0.001) Conclusion: Brain MRI changes may occur even in hepatic WD and presymptomatic cases, although infrequently, regardless of the presence of neurological symptoms. It should be known that neurological symptoms in children and adolescents may occur without significant liver disease. Even without neurological symptoms, all children with WD should have a brain MRI before treatment.

Recurrent generalized seizures as the prominent manifestation in a patient with CADASIL: a case report and literature review

BackgroundCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited arteriopathy typically caused by mutations in the NOTCH-3 gene. Few detailed descriptions of recurrent generalized seizures in CADASIL has been reported.Case presentationThis article details a case of recurrent generalized seizures, which eventually be diagnosed as CADASIL with a heterozygous variant, c.1630 C > T (p. Arg544Cys), in exon 11 of the Notch 3 gene. Here, we discussed the possible pathogenesis underlying the epilepsy associated with CADASIL through the brain magnetic resonance imaging changes and the captured epileptiform waves in the electroencephalography during the patient’s follow-up period. Related literatures were also reviewed to discuss the etiology of the epilepsy.ConclusionsRecurrent generalized seizures may be a presenting neurological manifestation of CADASIL in the absence of other discernible causes. Clinicians should comprehensively seek the possible etiology of patients with recurrent generalized seizures, considering the possible diagnosis of CADASIL.

Brain MRI in the Decision for Liver Transplantation in Pediatric Neurological Wilson's Disease.

Neurological Wilson's disease (WD) presentation in the pediatric population is rare, and liver transplantation (LT) in these patients remains controversial. The aim of the present study was to assess the role of brain magnetic resonance imaging (MRI) in predicting reversion of brain lesions and neurological outcomes in pediatric WD patients after LT. Patients with confirmed WD (Leipzig score ≥4), disease onset in pediatric age (<18 years), neurological involvement, and submitted to LT were selected. Clinical records and pre- and post-LT brain MRI were evaluated. Six patients met the pre-defined inclusion criteria, one of whom died shortly after LT and was excluded. The indication for LT was end-stage liver disease in two patients and neurological worsening despite optimized treatment in three patients. After LT, the neurological picture progressively improved in all patients. Pre-LT brain MRI showed T1-weighted hyperintensities in four patients, which quickly resolved afterward. T2-weighted hyperintensities were observed in four patients before LT, completely resolving in one patient, stabilizing in two, and improving in one after LT. A direct correlation could not be found between clinical and neuroradiological improvement. Progressive clinical improvement was observed even in patients with irreversible brain MRI changes. Conversely, some patients with normal MRI had only slight neurological improvement. The pattern of T2-weighted hyperintensities after LT was unpredictable and did not correlate with neurological outcomes, suggesting that these changes may not entail irreversible clinical damage. Therefore, brain MRI does not seem to have prognostic value for assessing clinical response to LT.

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease

Patient: Male, 17-month-old Final Diagnosis: Fluid attenuated inversion recovery (FLAIR) imaging showing cortical hyperintense lesions in myelin-oligodendrocyte-glycoprotein (MOG) associated encephalitis with seizures (FLAMES Symptoms: Encephalopathy • fever • left sided weakness • seizures Medication: — Clinical Procedure: Plasmapharesis Specialty: Neurology Objective: Rare disease Background:The relatively new autoimmune disorder, anti-myelin oligodendrocyte glycoprotein (MOG) disease is particularly interesting because of its broad range of presentations. This entity’s appearance on magnetic resonance imaging (MRI) of the brain often makes identifying this disease a challenging process. Younger patients tend to present with an acute disseminated encephalomyelitis picture, with encephalopathy and multifocal neurological signs, while older patients are more likely to present with optic neuritis. We, however, report an atypical case of a patient who presented with encephalopathy, seizures, and significant cortical and subcortical gray matter involvement and was found to have anti-MOG positivity in serum.Case Report:A 17-month-old previously healthy boy presented to Emergency Department with fever, lethargy, vomiting, and left-sided weakness. Eventually, he required intubation due to a prolonged seizure. Continuous electroencephalogram captured several focal seizures, and MRI of the brain showed cortical and subcortical T2 hyperintensities. After extensive laboratory evaluation, he tested positive for anti-MOG antibody. He was empirically started on high-dose intravenous pulse methylprednisolone, followed by plasma exchange, given the poor response to the intravenous steroids. At the 5-month follow-up, the results of the neurological examination had dramatically improved, and MRI findings had largely resolved.Conclusions:This case highlights the importance of suspecting anti-MOG antibody-mediated encephalitis, even while ruling out infectious etiologies, in children presenting with encephalopathy, seizures and MRI abnormalities. Prompt recognition would allow for less delay in treatment and hopefully improve prognosis.

Pregnancy-induced brain magnetic resonance imaging changes in women with multiple sclerosis.

The effect of pregnancy on brain changes and radiological disease activity in women with multiple sclerosis (MS) is not well understood. This study was undertaken to describe the dynamics of lesion activity and brain volume changes during the pregnancy and postpartum periods. This observational study of 62 women with relapsing-remitting MS included magnetic resonance imaging (221 scans) as well as clinical visits at baseline (<24 and >6months before pregnancy), prepregnancy (<6months before pregnancy), postpartum (<3months after delivery), and follow-up (>12 and <24months after delivery) periods. The majority of women had a mild disability and a short disease duration (median = 5.5years). Eighteen (29.0%) women had a relapse during the year preceding pregnancy onset, nine (14.5%) during pregnancy, and 20 (32.3%) in the year following delivery. Disability status remained unchanged during follow-up. Women in the postpartum period (n=62) had higher T2 lesion volume (median = 1.18ml vs. 0.94ml), greater annualized T2 lesion volume increase (0.23ml vs. 0.0ml), lower brain parenchymal fraction (85.6% vs. 86.4%), and greater annualized brain volume loss (-1.74% vs. -0.16%) compared with the prepregnancy period (all p<0.001). At 12-24months after delivery, women (n=41) had higher T2lesion volume (1.16ml vs. 1.0ml) and lower brain parenchymal fraction (86.0% vs. 86.5%) compared to the prepregnancy period (both p<0.001). The postpartum period was associated with an increase in T2 lesion volume and accelerated brain volume loss in a considerable proportion of women. This should be considered in treatment decision-making and designing clinical trials.

Multiparameter magnetic resonance imaging in the diagnosis of herpes encephalitis in children

Introduction. This study is determined with the high prevalence of encephalitis in children, as well as the severe course and the possible disability. Herpes encephalitis occurs in almost half of cases of viral encephalitis in children. It is known that changes on the brain magnetic resonance imaging (MRI) in the acute stage of the disease are detected more often than on brain computed tomography (CT), but the clarification of this brain MRI changes is needed.Objectives of the study. To assess the features of brain multiparametric MRI changes in herpes encephalitis in children.Materials and methods. Two groups of children were examined, the first group included 25 children aged 6,0±4,8 years with laboratory-confirmed acute EH, the second group (control) — 23 children without signs of central nervous system damage at the comparable gender and age. Structural changes were assessed using MRI of the brain in T1-WI, T2-WI, Flair, T1-WI modes post contrast, DWI, DTI and MR spectroscopy.Results. In 40% of cases revealed panencephalitis, 36% — leukoencephalitis, 24% — polioencephalitis. Most often, the process involved the cerebral hemispheres — 72%, the brain stem — 44%, the thalamus — 40% and basal nuclei — 36%. In 52% of cases changes in DWI were found, in 20% of cases the lesions accumulated contrast agent. There was a statistically significant decrease of FA both in the focus and in the intact area in children with EH compared with the control group.Conclusion. The application of multiparametric MRI using DWI, DTI, and MR spectroscopy methods are statistically significant for the detection and assessment of focal brain lesions in children with herpes encephalitis.

A brief report on incidence, radiographic feature and prognostic significance of brain MRI changes after anti-PD-1/PD-L1 therapy in advanced non-small cell lung cancer.

Neurologic immune-related adverse events (nirAEs) are uncommon but potentially lethal complications of immune checkpoint inhibitor (ICI) treatment. However, the incidence, radiographic features and prognostic significance of brain magnetic resonance imaging (MRI) changes after ICI treatment remain largely unknown. Consecutive patients with advanced non-small cell lung cancer (NSCLC) at three participating institutions receiving anti-PD-1/PD-L1 therapy from June 2017 to September 2020 were screened, and those who received brain MRI within 6weeks before ICI initiation and at least one follow-up brain MRI after ICI treatment were included. Serial brain MRI images were independently reviewed by two experienced radiologists. With a median follow-up of 13.2months, 27 (20.0%) of the 135 enrolled patients developed certain kind of brain MRI aberration. The 1-, 2- and 3-year cumulative incidence of brain MRI aberration was 17.1%, 36.3% and 52.2%, respectively. Brain MRI aberration indicative of stroke, mimicking typical white matter lesions and presenting as T2-hyperintensity suggestive of CNS vasculitis or encephalitis, was documented in 11, 9 and 4 patients, respectively. Patients with brain MRI aberration had higher clinical benefit rate (p = 0.030), longer progression-free survival (p = 0.015) and a tendency of improved overall survival (p = 0.054). Brain MRI aberrations developed after ICI treatment are not uncommon, and their manifestations vary a lot. Patients developing brain MRI aberrations tended to have better prognosis, which needed to be further investigated.

Gordon Holmes syndrome – first time diagnosed in Croatia

We present a 38-year-old woman with clinical presentation of ataxia, cognitive impairment, and secondary amenorrhea with remarkable brain hyperintensities on brain magnetic resonance imaging (MRI). Clinical symptoms began at the age of 20 years with the development of secondary amenorrhea, instability, and cognitive dysfunction. Although this set of medical symptoms of ataxia, primary/secondary amenorrhea due to hypogonadotropic hypogonadism, and cognitive impairment are known as Gordon Holmes syndrome, such has not been described in Croatia so far. Because of this, the patient was unsuccessfully diagnostically investigated in various neurological institutions on different neurodegenerative diseases, primarily based on ataxia, cognitive impairment, and remarkable brain hyperintensities on brain MRI. We started to analyze a large group of possible genetically triggered autosomal-recessive degenerative ataxias, and noticed compatibility of clinical symptoms: cerebellar ataxia, cognitive impairment, and hypogonadotropic hypogonadism, along with brain MRI changes characterized with significant subcortical white matter, thalamic, and brain steam confluent patchy areas of hyperintensity and cerebellar atrophy, that constitute a syndrome caused by ATM RNF216 gene mutation, Gordon Holmes syndrome (GHS). Genetic analysis in Variantyx laboratory in the USA performing whole-exome sequencing showed compound heterozygous for RNF 216 mutation, which confirmed the diagnosis of GHS, for the first time diagnosed in Croatia.

Changes in brain MRI under different lunar cycles: a cross-sectional study

ABSTRACT Physiologic changes in mammals, including humans, are known to vary as a function of different lunar cycle phases. Importantly, the structure of the central nervous system (CNS) affects its functional status. A total of 15 healthy volunteers underwent magnetic resonance imaging (MRI) in each of the lunar cycles, with each subject undergoing a total of four imaging sessions. Size and volume of structures such as the hippocampus, subarachnoid space, lentiform nucleus, thalamus, and trigon, were measured by expert radiologists. Inter-phase differences in size and volume were determined. There was a significant difference between ventricular volume, whole-brain volume, hippocampal volume, lentiform nuclei volume, and thalamus diameter between the four lunar phases. The new moon had the lowest values than any other lunar phase in any reported structures. Importantly, the difference between the new moon and the third quarter lunar phase was always significant. There was no significant difference in subarachnoid space volume and trigone diameter of the ventricles in different lunar phases. The trigone was the smallest in the third quarter lunar phase. Our results suggest that lunar phases affect brain structures.

Circulating angiopoietin-2 and angiogenic microRNAs associate with cerebral small vessel disease and cognitive decline in older patients reaching end-stage renal disease.

The prevalence of end-stage renal disease (ESRD) is increasing worldwide, with the majority of new ESRD cases diagnosed in patients >60 years of age. These older patients are at increased risk for impaired cognitive functioning, potentially through cerebral small vessel disease (SVD). Novel markers of vascular integrity may be of clinical value for identifying patients at high risk for cognitive impairment. We aimed to associate the levels of angiopoietin-2 (Ang-2), asymmetric dimethylarginine and a selection of eight circulating angiogenic microRNAs (miRNAs) with SVD and cognitive impairment in older patients reaching ESRD that did not yet initiate renal replacement therapy (n = 129; mean age 75.3 years, mean eGFR 16.4 mL/min). We assessed brain magnetic resonance imaging changes of SVD (white matter hyperintensity volume, microbleeds and the presence of lacunes) and measures of cognition in domains of memory, psychomotor speed and executive function in a neuropsychological test battery. Older patients reaching ESRD showed an unfavourable angiogenic profile, as indicated by aberrant levels of Ang-2 and five angiogenic miRNAs (miR-27a, miR-126, miR-132, miR-223 and miR-326), compared with healthy persons and patients with diabetic nephropathy. Moreover, Ang-2 was associated with SVD and with the domains of psychomotor speed and executive function, while miR-223 and miR-29a were associated with memory function. Taken together, these novel angiogenic markers might serve to identify older patients with ESRD at risk of cognitive decline, as well as provide insights into the underlying (vascular) pathophysiology.

Invasive Mold Infection of the Central Nervous System in Immunocompromised Children.

Background: Due to the difficulties in the definite diagnosis, data on brain imaging in pediatric patients with central nervous system (CNS)-invasive mold infection (IMD) are scarce. Our aim was to describe brain imaging abnormalities seen in immunocompromised children with CNS-IMD, and to analyze retrospectively whether specific imaging findings and sequences have a prognostic value. Methods: In a retrospective study of 19 pediatric patients with proven or probable CNS-IMD, magnetic resonance imaging (MRI)-findings were described and analyzed. The results were correlated with outcome, namely death, severe sequelae, or no neurological sequelae. Results: 11 children and 8 adolescents (11/8 with proven/probable CNS-IMD) were included. Seven of the patients died and 12/19 children survived (63%): seven without major neurological sequelae and five with major neurological sequelae. Multifocal ring enhancement and diffusion restriction were the most common brain MRI changes. Diffusion restriction was mostly seen at the core of the lesion. No patient with disease limited to one lobe died. Perivascular microbleeding seen on susceptibility weighted imaging (SWI) and/or gradient-echo/T2* images, as well as infarction, were associated with poor prognosis. Conclusions: The presence of infarction was related to poor outcome. As early microbleeding seems to be associated with poor prognosis, we suggest including SWI in routine diagnostic evaluation of immunocompromised children with suspected CNS-IMD.

Prevalence and factors of verbal learning and memory dysfunction in patients with epilepsy – A single centre study

The objective of this study is to determine prevalence and factors leading to verbal learning and memory dysfunction among patients with epilepsy.A total of 211 subjects were recruited. Their verbal memory was assessed by Rey’s Auditory Verbal Learning Test (RAVLT). This test was further subdivided into four major spheres for analysis, namely the verbal learning, interference list, immediate memory and delayed memory. All data collected were analyzed using Statistical Package for Social Sciences.Among the 211 patients, 55% (n = 116) had focal seizures and the remaining 45% (n = 95) had generalized seizures. Prevalence of verbal learning and memory impairment was high at 39.97% overall, and found most commonly in patients with focal impaired awareness seizures. Verbal learning and immediate memory dysfunction were significantly lower in focal impaired awareness group compared to others. Age more than 50 years, exposure to three or more antiepileptic drugs and use of carbamazepine more than 1000 mg a day were the predictors in poor verbal memory outcome. No statistical difference was observed in the mean RAVLT scores among the gender and hand dominance groups. Between patients with and without electroencephalogram changes as well as brain magnetic resonance imaging changes, the mean RAVLT scores showed no statistically significant difference.Verbal learning and memory impairment is prevalent among the epilepsy patients. The consequences of the memory impairment can be as debilitating as the seizure control. RAVLT is a reliable and practical test in the clinical setting.

Differential value of brain magnetic resonance imaging in multiple system atrophy cerebellar phenotype and spinocerebellar ataxias

Clinically differentiating multiple system atrophy cerebellar (MSA-C) phenotype and spinocerebellar ataxias (SCAs) is challenging especially in the early stage. We assessed diagnostic value of brain magnetic resonance imaging (MRI) in differentiating MSA-C and SCAs based at different disease stages (<3, 3–7, and >7 years of disease duration). Overall, 186 patients with probable MSA-C and 117 with genetically confirmed SCAs were included. Hot cross bun (HCB) signs and middle cerebellar peduncle (MCP) hyperintensities were exclusively prevalent in MSA-C compared to SCAs at <3 years (HCB, 44.6% versus 0.9%; MCP hyperintensities, 38.3% versus 0.9%, respectively). Sensitivity, specificity, and positive predictive value (PPV) for HCB signs to differentiate MSA-C from SCAs were 45%, 99%, and 99% and those for MCP hyperintensities were 68%, 99%, and 99%, respectively; considering both HCB signs and MCP hyperintensities, specificity and PPV were 100%. However, the differential value of MRI signs decreased over time. MCP widths were smaller and showed more significant decrease in MSA-C than in SCAs. In conclusion, pontine and MCP changes were exclusively prominent in early stage MSA-C rather than in SCAs. Therefore, we should consider disease duration when interpreting pontine and MCP changes in brain MRIs, which will help better differentiate MSA-C and SCAs.

Exposure to surgery under general anaesthesia and brain magnetic resonance imaging changes in older adults

BackgroundPreclinical studies suggest that exposure to general anaesthesia (GA) could cause neurodegeneration consistent with Alzheimer's disease (AD) pathology. Brain magnetic resonance imaging (MRI) is useful to study structural brain changes. We tested the hypothesis that exposure to surgery with GA (surgery/GA) is associated with greater cortical thinning and increased frequency of white matter lesions. MethodsThis is a cross-sectional analysis of 70–91-yr-old participants enrolled in the Mayo Clinic Study of Aging who had baseline MRI. The thickness of selected cortical regions, the volume of white matter hyperintensities, and the frequency of cortical infarctions were compared in participants who were and were not exposed to surgery/GA within 20 yr before the first MRI obtained after enrolment. ResultsOf 1410 participants with MRI scans, 932 were exposed to surgery/GA before scanning. In adjusted analyses, cortical thickness in regions vulnerable to AD was significantly less in those exposed to surgery/GA in the prior 20 yr (difference −0.023 mm, [95% confidence interval (CI) −0.041 to −0.005], P=0.014). Those with surgery in the prior 20 yr were more likely to have ‘abnormal thickness’ compared with those without surgery (odds ratio=1.45, [95% CI 1.10–1.90], P=0.009). Exposure was not associated with white matter hyperintensities or the presence of brain infarcts. ConclusionsThis study suggests that exposure of older adults to surgical anaesthesia is associated with thinning in cortical regions implicated in AD. The pathogenesis and mechanisms driving these neurodegenerative changes, and the potential clinical significance of these findings, require further study.

Serial brain MRI changes related to autoimmune pathophysiology in Hashimoto encephalopathy with anti-NAE antibodies: A case-series study

PurposeHashimoto encephalopathy (HE) is an autoimmune-mediated encephalopathy associated with anti-thyroid antibodies. We previously discovered serum autoantibodies against the NH2-terminal of α-enolase (NAE), which serve as a specific diagnostic biomarker for HE and may be involved in the autoimmune pathophysiology of HE, including vasculitis. Although the common findings of brain magnetic resonance imaging (MRI) in HE have been recognized as normal or non-specific white matter lesions, serial MRI changes have been less well studied. The aim of this study was to clarify detailed and longitudinal MRI changes in HE associated with anti-NAE antibodies. MethodsWe investigated serial brain MR images in 12 Japanese patients with HE who had serum anti-NAE antibodies. ResultsBrain MRI showed diffuse white matter abnormalities and/or multiple small subcortical lesions in 10 patients. These lesions were apparently non-specific; however, in 7 of these patients we observed expanding and diminishing white matter lesions, emerging subcortical high-intensity spots on diffusion-weighted images, or reversible limbic lesions, which worsened at relapse and improved after recovery following immunotherapies. ConclusionMRI lesions that fluctuate according to the disease condition were frequently observed in HE patients with anti-NAE antibodies, which suggests that these fluctuation may be associated with the autoimmune pathophysiology of HE.