Emotion processing impairments are common in patients undergoing brain surgery for fronto‐temporal tumour resection, with potential consequences on social interactions. However, evidence is ...controversial concerning side and site of lesions causing such deficits. This study investigates visual and auditory emotion recognition in brain tumour patients with the aim of clarifying which lesion sites are related to impairments in emotion processing from different modalities. Thirty‐four patients were evaluated, before and after surgery, on facial expression and emotional prosody recognition; voxel‐based lesion–symptom mapping (VLSM) analyses were performed on patients’ post‐surgery MRI images. Results showed that patients’ performance decreased after surgery in both visual and auditory modalities, but, in general, recovered 3 months after surgery. In facial expression recognition, left brain‐damaged patients showed greater post‐surgery deterioration than right brain‐damaged ones, whose performance specifically decreased for sadness and fear. VLSM analysis revealed two segregated areas in the left hemisphere accounting for post‐surgery scores for happy (fronto‐temporo‐insular region) and surprised (middle frontal gyrus and inferior fronto‐occipital fasciculus) facial expressions. Our findings demonstrate that surgical removal of tumours in the fronto‐temporal region produces impairment in facial emotion recognition with an overall recovery at 3 months, suggesting a partially different representation of positive and negative emotions in the left and right hemispheres for visually – but not auditory – presented emotions; moreover, we show that deficits in specific expression recognition are associated with discrete lesion locations.
Hypomyelination is observed in the context of a growing number of genetic disorders that share clinical characteristics. The aim of this study was to determine the possible role of magnetic resonance ...imaging pattern recognition in distinguishing different hypomyelinating disorders, which would facilitate the diagnostic process. Only patients with hypomyelination of known cause were included in this retrospective study. A total of 112 patients with Pelizaeus–Merzbacher disease, hypomyelination with congenital cataract, hypomyelination with hypogonadotropic hypogonadism and hypodontia, Pelizaeus–Merzbacher-like disease, infantile GM1 and GM2 gangliosidosis, Salla disease and fucosidosis were included. The brain scans were rated using a standard scoring list; the raters were blinded to the diagnoses. Grouping of the patients was based on cluster analysis. Ten clusters of patients with similar magnetic resonance imaging abnormalities were identified. The most important discriminating items were early cerebellar atrophy, homogeneity of the white matter signal on T2-weighted images, abnormal signal intensity of the basal ganglia, signal abnormalities in the pons and additional T2 lesions in the deep white matter. Eight clusters each represented mainly a single disorder (i.e. Pelizaeus–Merzbacher disease, hypomyelination with congenital cataract, hypomyelination with hypogonadotropic hypogonadism and hypodontia, infantile GM1 and GM2 gangliosidosis, Pelizaeus–Merzbacher-like disease and fucosidosis); only two clusters contained multiple diseases. Pelizaeus–Merzbacher-like disease was divided between two clusters and Salla disease did not cluster at all. This study shows that it is possible to separate patients with hypomyelination disorders of known cause in clusters based on magnetic resonance imaging abnormalities alone. In most cases of Pelizaeus–Merzbacher disease, hypomyelination with congenital cataract, hypomyelination with hypogonadotropic hypogonadism and hypodontia, Pelizaeus–Merzbacher-like disease, infantile GM1 and GM2 gangliosidosis and fucosidosis, the imaging pattern gives clues for the diagnosis.
Introduction
Sporadic Creutzfeldt‐Jakob disease (sCJD) comprises multiple subtypes (MM1, MM2, MV1, MV2C, MV2K, VV1, and VV2) with distinct disease durations and spatiotemporal cascades of brain ...lesions. Our goal was to establish the ante mortem diagnosis of sCJD subtype, based on patient‐specific estimates of the spatiotemporal cascade of lesions detected by diffusion‐weighted magnetic resonance imaging (DWI).
Methods
We included 488 patients with autopsy‐confirmed diagnosis of sCJD subtype and 50 patients with exclusion of prion disease. We applied a discriminative event‐based model (DEBM) to infer the spatiotemporal cascades of lesions, derived from the DWI scores of 12 brain regions assigned by three neuroradiologists. Based on the DEBM cascades and the prion protein genotype at codon 129, we developed and validated a novel algorithm for the diagnosis of the sCJD subtype.
Results
Cascades of MM1, MM2, MV1, MV2C, and VV1 originated in the parietal cortex and, following subtype‐specific orderings of propagation, went toward the striatum, thalamus, and cerebellum; conversely, VV2 and MV2K cascades showed a striatum‐to‐cortex propagation. The proposed algorithm achieved 76.5% balanced accuracy for the sCJD subtype diagnosis, with low rater dependency (differences in accuracy of ± 1% among neuroradiologists).
Discussion
Ante mortem diagnosis of sCJD subtype is feasible with this novel data‐driven approach, and it may be valuable for patient prognostication, stratification in targeted clinical trials, and future therapeutics.
Highlights
Subtype diagnosis of sporadic Creutzfeldt‐Jakob disease (sCJD) is achievable with diffusion MRI.
Cascades of diffusion MRI abnormalities in the brain are subtype‐specific in sCJD.
We proposed a diagnostic algorithm based on cascades of diffusion MRI abnormalities and demonstrated that it is accurate.
Our method may aid early diagnosis, prognosis, stratification in clinical trials, and future therapeutics.
The present approach is applicable to other neurodegenerative diseases, enhancing the differential diagnoses.
Purpose
We evaluated the relationship between
11
C-methionine PET (
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C-METH PET) findings and molecular biomarkers in patients with supratentorial glioma who underwent surgery.
Methods
A ...consecutive series of 109 patients with pathologically proven glioma (64 men, 45 women; median age 43 years) referred to our Institution from March 2012 to January 2015 for tumour resection and who underwent preoperative
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C-METH PET were analysed. Semiquantitative evaluation of the
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C-METH PET images included SUVmax, region of interest-to-normal brain SUV ratio (SUVratio) and metabolic tumour volume (MTV). Imaging findings were correlated with disease outcome in terms of progression-free survival (PFS), and compared with other clinical biological data, including IDH1 mutation status, 1p/19q codeletion and MGMT promoter methylation. The patients were monitored for a mean period of 16.7 months (median 13 months).
Results
In all patients, the tumour was identified on
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C-METH PET. Significant differences in SUVmax, SUVratio and MTV were observed in relation to tumour grade (
p
< 0.001). IDH1 mutation was found in 49 patients, 1p/19q codeletion in 58 patients and MGMT promoter methylation in 74 patients. SUVmax and SUVratio were significantly inversely correlated with the presence of IDH1 mutation (
p
< 0.001). Using the 2016 WHO classification, SUVmax and SUVratio were significantly higher in patients with primary glioblastoma (IDH1-negative) than in those with other diffuse gliomas (
p
< 0.001). Relapse or progression was documented in 48 patients (median PFS 8.7 months). Cox regression analysis showed that SUVmax and SUVratio, tumour grade, tumour type on 2016 WHO classification, IDH1 mutation status, 1p/19q codeletion and MGMT promoter methylation were significantly associated with PFS. None of these factors was found to be an independent prognostic factor in multivariate analysis.
Conclusion
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C-METH PET parameters are significantly correlated with histological grade and IDH1 mutation status in patients with glioma. Grade, pathological classification, molecular biomarkers, SUVmax and SUVratio were prognostic factors for PFS in this cohort of patients.
The trial was registered with ClinicalTrials.gov (registration: NCT02518061).
Lesions in the ventrolateral region of the dominant frontal lobe have been historically associated with aphasia. Recent imaging results suggest that frontal language regions extend beyond classically ...defined Broca’s area to include the ventral precentral gyrus (VPCG) and the arcuate fasciculus (AF). Frontal gliomas offer a unique opportunity to identify structures that are essential for speech production. The aim of this prospective study was to investigate the correlation between language deficits and lesion location in patients with gliomas.
Nineteen patients with glioma and 10 healthy subjects were evaluated with diffusion tensor imaging magnetic resonance (MR) tractography, functional MR (verb generation task) and the Aachener Aphasie Test. Patients were divided into two groups according to lesion location with respect to the ventral precentral sulcus: (i) anterior (n=8) with glioma growing in the inferior frontal gyrus (IFG) and underlying white matter; (ii) posterior (n=11) with glioma growing in the VPCG and underlying white matter. Virtual dissection of the AF, frontal intralobar tract, uncinate fasciculus (UF) and inferior frontal occipital fasciculus (IFOF) was performed with a deterministic approach.
Seven posterior patients showed aphasia classified as conduction (4), Broca (1), transcortical motor (1) and an isolated deficit of semantic fluency; one anterior patient had transcortical mixed aphasia. All posterior patients had invasion of the VPCG, however only patients with aphasia had also lesion extension to the AF as demonstrated by tractography dissections. All patients with language deficits had high grade glioma. Groups did not differ regarding tumour volume. A functional pars opercularis was identified with functional MR imaging (fMRI) in 17 patients.
Gliomas growing in the left VPCG are much more likely to cause speech deficits than gliomas infiltrating the IFG, including Broca’s area. Lesion extension to the AF connecting frontal to parietal and temporal regions is an important mechanism for the appearance of aphasia.
Abstract Purpose To evaluate the impact of fluid-attenuated-inversion-recovery MRI (FLAIR/MRI) and Carbon-11-labeled-methionine PET (11C-MET-PET) on high grade glioma (HGG) tumor volume delineation ...for radiotherapy planning. Material and methods Sixty-nine patients with HGG were evaluated. The clinical target volumes (CTV1, generated by adding a 10 mm margin to FLAIRMRI area, CTV2 by adding a 20 mm margin to enhanced T1MRI) and biological target volume (BTV) were delineated on pre-operative MRI images and 11CMETPET respectively. Results The overlap between CTV1 and CTV2 showed a low correlation between the two volumes with CTV1 not always fully included into the CTV2. In all cases the whole BTV was included into the CTV1, while in 35/69 patients (50%) part of BTV was outside the CTV2 despite larger margins were added. In all cases recurrences were within the CTV1 volume and in 19/38 (50%) partially outside the CTV2. In all patients relapse corresponded to the BTV area. Conclusions Our data suggest that the target volume definition using FLAIR–MRI is more adequate compared to enhanced T1MRI. 11C-METPET uptake could help identify microscopic residual areas.
Prion disease research and surveillance can be challenging due to the disease's difficulty to diagnose, rapid progression, and geographic dispersion. Improving accessibility through teleneurology ...could improve the ability to conduct these activities.
The aim of this study was to determine the feasibility of conducting teleneurology assessments for research and surveillance of prion diseases.
Participants were offered in-person visit, medical record review, or teleneurology assessment. Standardized histories and assessments evaluating cognition, functional ability, and neuropsychiatric symptoms were collected. Data regarding participants' satisfaction with teleneurology were collected.
From April 2017 to July 2018, the study received 114 referrals. 45 and 5 participants consented for the teleneurology and medical record review arms of the study, respectively. 29 subjects participated in at least one teleneurology visit. Participants expressed satisfaction with teleneurology and found it easy to participate. Some aspects of the examination were hindered or interrupted due to technological reasons.
We demonstrate the feasibility and preference of teleneurology as a modality in which subjects with prion disease can partake in clinical research. Technological aspects sometimes interfered with research assessments.
Visual neglect has classically been associated with right hemisphere injury in parietal, frontal, or temporal cortex, in the basal ganglia or in the thalamus. More recently, visual neglect has been ...associated with injury extended into fronto-parietal white matter tracts. However, in most published cases white and gray matter injuries were associated. We present the anatomo-clinical study of a patient presenting with severe acute left visual neglect due to ischemic infarct limited to the right cerebral hemisphere white matter. Magnetic resonance diffusion tensor imaging tractography was instrumental to accurately localize the injury to the right arcuate fasciculus that is a component of the large-scale networks controlling visuo-spatial attention. These results add to a growing appreciation that neglect may result from disruption of a distributed attentional network.
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