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Background: Although the pulvinar is known for its visual function and extensive connections with cortical areas, the volumetric change and functional connectivity of the pulvinar in posterior cortical atrophy (PCA) remain unclear., Objective: To identify functional and volumetric changes of the pulvinar in PCA patients and the relevant associations with higher visual dysfunction., Methods: A total of 29 patients with PCA and 30 normal controls were recruited. Each participant underwent a comprehensive neuropsychological assessment and both structural and resting-state functional MRI scanning. Voxel-based morphometry (VBM) and seed-based functional connectivity analyses were conducted to assess pulvinar gray matter volume as well as functional connectivity between the pulvinar and whole brain regions. A partial correlation analysis was performed to analyze neuropsychological tests and pulvinar imaging data., Results: Cognitive and visual functions including visuospatial processing, visual perception, episodic memory, and naming were impaired among PCA patients. Marked pulvinar atrophy was noted in PCA patients. Furthermore, functional connectivity between the pulvinar and precuneus was significantly decreased in PCA patients as compared to normal controls (FWE corrected; P < .001). Gray matter volume of the left pulvinar was found to associate with object agnosia (r = .53, P = .005) and prosopagnosia (r = .54, P = .005) among PCA patients. Gray matter volume of the right pulvinar was found to be associated with the Clinical Dementia Rating scale (r = -.52, P = .006) and Activities of Daily Living (r = -.59, P = .002) scores. Prosopagnosia correlated positively to the functional connectivity of the left pulvinar and left middle temporal., Conclusion: Our findings support pulvinar degeneration and its contributions in PCA., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background and Purpose: Thalamic alterations have been reported as a major feature in presymptomatic and symptomatic patients carrying the C9orf72 mutation across the frontotemporal dementia-amyotrophic lateral sclerosis (ALS) spectrum. Specifically, the pulvinar, a high-order thalamic nucleus and timekeeper for large-scale cortical networks, has been hypothesized to be involved in C9orf72-related neurodegenerative diseases. We investigated whether pulvinar volume can be useful for differential diagnosis in ALS C9orf72 mutation carriers and noncarriers and how underlying functional connectivity changes affect this region., Methods: We studied 19 ALS C9orf72 mutation carriers (ALSC9+) accurately matched with wild-type ALS (ALSC9-) and ALS mimic (ALSmimic) patients using structural and resting-state functional magnetic resonance imaging data. Pulvinar volume was computed using automatic segmentation. Seed-to-voxel functional connectivity analyses were performed using seeds from a pulvinar functional parcellation., Results: Pulvinar structural integrity had high discriminative values for ALSC9+ patients compared to ALSmimic (area under the curve [AUC] = 0.86) and ALSC9- (AUC = 0.77) patients, yielding a volume cutpoint of approximately 0.23%. Compared to ALSmimic, ALSC9- showed increased anterior, inferior, and lateral pulvinar connections with bilateral occipital-temporal-parietal regions, whereas ALSC9+ showed no differences. ALSC9+ patients when compared to ALSC9- patients showed reduced pulvinar-occipital connectivity for anterior and inferior pulvinar seeds., Conclusions: Pulvinar volume could be a differential biomarker closely related to the C9orf72 mutation. A pulvinar-cortical circuit dysfunction might play a critical role in disease progression and development, in both the genetic phenotype and ALS wild-type patients., (© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background: Patients with pulvinar area lesions may develop hydrocephalus at any stage. The role of endoscopic third ventriculostomy (ETV) in this setting remains unclear., Method: We retrospectively enrolled 15 patients with a mean age of 43 years who underwent endoscopic resection of pulvinar area lesions using the supracerebellar infratentorial approach (SCITA). We compared the different modalities of hydrocephalus management and their outcomes., Results: Nine of 15 patients (60.0%) had preoperative obstructive hydrocephalus. Five patients underwent ETV before tumor resection, and none developed postoperative hydrocephalus. Four patients underwent one-stage surgery for tumor removal, and one patient with a polymorphous low-grade neuroepithelial tumor of the young required postoperative ETV. Another patient with diffuse astrocytoma and hydrocephalus underwent concurrent lamina terminalis fenestration and endoscopic resection via the SCITA, which resulted in the resolution of hydrocephalus. The preoperative ETV group had no major postoperative complications, while the non-ETV group had three (0/5 vs. 3/4, P = 0.048). The ETV group also had a shorter intensive care unit stay; however, the difference was not significant (1.2 vs. 2.8; P = 0.188). ETV was effective in alleviating symptoms of postoperative hydrocephalus in patients with midbrain-invading tumors., Conclusion: Endoscopic surgery via the SCITA can address both tumor and hydrocephalus issues in some cases but has a higher surgical risk and postoperative hydrocephalus rate. Preoperative ETV can prevent these complications and improve postoperative outcomes., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Purpose: We have reported the relationship between low pulvinar nuclei (PN) intensity in susceptibility-weighted imaging and the appearance of visual hallucinations and cognitive function. The aim of the study was to examine the changes in the quantitative susceptibility mapping (QSM) in patients with Parkinson's disease (PD) who underwent deep brain stimulation (DBS) and verify whether the PN susceptibility value (SV) on QSM can predict visual hallucination and cognitive changes after DBS., Methods: This study examined 24 patients with PD who underwent DBS along with QSM imaging on magnetic resonance imaging (MRI). All MRIs were performed within 3 months before surgery. The PN SV was further assessed based on the QSM. Then, associations were examined among cognitive changes, hallucination, and PN SV. The cognitive function of the patient was compared immediately before surgery and at 1 year postoperatively., Results: Visual hallucinations were observed in seven patients during the follow-up period. The PN SV was ≥0.045 ppm in nine patients with PD, and six of them had visual hallucinations, whereas only one of 15 patients with PD with SV of <0.045 ppm had visual hallucinations (Fisher's exact test, p = .0037)., Conclusions: The SV of >0.045 ppm at the PN in QSM in patients with PD may provide useful information suggesting visual hallucination and cognitive deterioration after DBS treatment., (© 2023 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Background: Although a central role of the thalamus for sleep regulation is undisputed, the exact localization of the crucial structures within the thalamus remains controversial., Case Presentation: Here we report a 35 year old woman with no prior comorbidities who developed severe and persistent hypersomnia with long sleep time after a small right-sided MRI-verified thalamic stroke affecting the dorsal part of the pulvinar and the dorsolateral boarders of the dorsomedial nuclei., Conclusion: The observed symptoms suggest a crucial role of posterior thalamus but not the midline parts of the thalamus in sleep-wake control.

Objective: Thalamic atrophy is among the earliest brain changes detected in patients with multiple sclerosis (MS) and the degree of thalamic atrophy is a strong predictor of disability progression. The causes of thalamic atrophy are not fully understood. Here, we investigate the contributions of thalamic demyelinated lesions, thalamic neuronal loss, and cerebral white matter (WM) lesions to thalamic volume., Methods: We used postmortem in situ magnetic resonance imaging (MRI) scans of 95 subjects with MS to correlate thalamic lesion volumes with global MRI metrics. We histologically characterized thalamic demyelination patterns and compared neuronal loss and neuritic pathology in the thalami with the extremes of volume., Results: Grossly apparent thalamic discolorations in cm-thick brain slices were T2/fluid-attenuated inversion recovery (FLAIR) hyperintense, T1-hypointense, and appeared as perivascular demyelinated lesions with dystrophic neurons/axons. Subependymal demyelinated lesions with axonal loss and microglial/macrophage activation were also observed. The 12 subjects with the least thalamic volume had a 17.6% reduction of median neuronal density in the dorsomedial/ventrolateral and pulvinar nuclei compared with the 14 subjects with the greatest thalamic volume (p = 0.03). After correcting for age, disease duration, sex, and T2 lesion volume, the total (p = 0.20), ovoid (p = 0.31), or subependymal (p = 0.44) MRI thalamic lesion volumes correlated with thalamic volume. Thalamic volume correlated with cerebral T2 lesion volume (Spearman's rho = -0.65, p < 0.001; p < 0.0001 after correcting for age, disease duration, and sex)., Interpretation: Our findings suggest the degeneration of efferent/afferent thalamic projections and/or a neurodegenerative process as greater contributors to thalamic atrophy than thalamic demyelinating lesions. ANN NEUROL 2020 ANN NEUROL 2020;88:81-92., (© 2020 American Neurological Association.)

Thalamic atrophy is a common feature across all forms of FTD but little is known about specific nuclei involvement. We aimed to investigate in vivo atrophy of the thalamic nuclei across the FTD spectrum. A cohort of 402 FTD patients (age: mean(SD) 64.3(8.2) years; disease duration: 4.8(2.8) years) was compared with 104 age-matched controls (age: 62.5(10.4) years), using an automated segmentation of T1-weighted MRIs to extract volumes of 14 thalamic nuclei. Stratification was performed by clinical diagnosis (180 behavioural variant FTD (bvFTD), 85 semantic variant primary progressive aphasia (svPPA), 114 nonfluent variant PPA (nfvPPA), 15 PPA not otherwise specified (PPA-NOS), and 8 with associated motor neurone disease (FTD-MND), genetic diagnosis (27 MAPT, 28 C9orf72, 18 GRN), and pathological confirmation (37 tauopathy, 38 TDP-43opathy, 4 FUSopathy). The mediodorsal nucleus (MD) was the only nucleus affected in all FTD subgroups (16-33% smaller than controls). The laterodorsal nucleus was also particularly affected in genetic cases (28-38%), TDP-43 type A (47%), tau-CBD (44%), and FTD-MND (53%). The pulvinar was affected only in the C9orf72 group (16%). Both the lateral and medial geniculate nuclei were also affected in the genetic cases (10-20%), particularly the LGN in C9orf72 expansion carriers. Use of individual thalamic nuclei volumes provided higher accuracy in discriminating between FTD groups than the whole thalamic volume. The MD is the only structure affected across all FTD groups. Differential involvement of the thalamic nuclei among FTD forms is seen, with a unique pattern of atrophy in the pulvinar in C9orf72 expansion carriers., (© 2019 The Authors. Human Brain Mapping published by Wiley Periodicals, Inc.)

Objective: Thalamic gliomas in children are less suitable for surgical resection because of their location. In cases of unavoidable resection, careful surgical planning in addition to histology and extent of resection affects prognosis., Methods: A cohort of 10 pediatric patients with thalamic glioma underwent surgical resection at our department. The predominant location of tumor origins in the thalamus was defined in imaging studies. Histopathology was determined (retrospectively in a subset) according to the World Health Organization classification 2016, including the newly established type of "diffuse midline glioma, H3 K27M-mutant.", Results: Three low-grade gliomas (grade I/II) and 7 high-grade gliomas (grade III/IV) were identified. The mean follow-up period was 49.8 months. All 3 low-grade gliomas did not recur (progression-free survival, 58.3 months). Six of 7 high-grade gliomas recurred, and the patients died of the primary disease (overall survival, 28.1 months). Poor outcomes, especially when located at the pulvinar region, were noticeable, with strong predictive power for poor prognosis (P = 0.0018). The presence of H3 K27M mutation and pulvinar location were closely associated (P = 0.0036). Four of 5 patients with pulvinar region tumors developed dissemination and died of the primary disease., Conclusions: Pulvinar location is specifically associated with a high rate of malignancy in histology, the presence of H3 K27M mutation, and dissemination at an early disease stage. This association suggests that a distinct biological profile affects prognosis depending on location within the thalamus, especially the pulvinar. We report that tumor location is highly relevant to prognosis and should be taken into consideration when planning treatment., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: Executive dysfunction is common in dementia with Lewy bodies (DLB). The pulvinar nucleus plays a role in executive control and synchronizes with cortical regions in the salience network that are vulnerable to Lewy pathology., Objective: We investigated the pulvinar subregions in patients with mild DLB and their associations with executive function., Methods: The sample consisted of 38 DLB patients and 38 age- and sex-matched normal controls. We evaluated cognitive function using the Consortium to Establish a Registry for Alzheimer's Disease Assessment Packet. We obtained four pulvinar nuclei using preprocessed T1-weighted magnetic resonance images. We compared volumes and textures of the DLB patients and the normal controls for each nucleus. We used a linear regression to determine the association of textures and neuropsychological test scores., Results: The DLB patients showed comparable volumes to the normal controls in all pulvinar nuclei. However, the DLB patients showed different texture of the left medial pulvinar (PuM) from the normal controls. The entropy, contrast, and cluster shade were lower but autocorrelation of left PuM was higher in the DLB patients compared to the normal controls. These texture features of the left PuM were associated with the set-shifting performance measured by the Trail Making Test., Conclusions: In DLB, the left PuM may be altered from early stage, which may contribute to the development of executive dysfunction., (© 2020 S. Karger AG, Basel.)

Traditionally, the dorsal lateral geniculate nucleus (LGN) and the inferior pulvinar (IPul) nucleus are considered as anatomically and functionally distinct thalamic nuclei. However, in several primate species it has also been established that the koniocellular (K) layers of LGN and parts of the IPul have a shared pattern of immunoreactivity for the calcium-binding protein calbindin. These calbindin-rich cells constitute a thalamic matrix system which is implicated in thalamocortical synchronisation. Further, the K layers and IPul are both involved in visual processing and have similar connections with retina and superior colliculus. Here, we confirmed the continuity between calbindin-rich cells in LGN K layers and the central lateral division of IPul (IPulCL) in marmoset monkeys. By employing a high-throughput neuronal tracing method, we found that both the K layers and IPulCL form comparable patterns of connections with striate and extrastriate cortices; these connections are largely different to those of the parvocellular and magnocellular laminae of LGN. Retrograde tracer-labelled cells and anterograde tracer-labelled axon terminals merged seamlessly from IPulCL into LGN K layers. These results support continuity between LGN K layers and IPulCL, providing an anatomical basis for functional congruity of this region of the dorsal thalamic matrix and calling into question the traditional segregation between LGN and the inferior pulvinar nucleus., (© 2019 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.)

Posterior thalamic pulvinar nuclei have been implicated in different aspects of spatial attention, but their exact role in humans remain unclear. Most neuropsychological studies of attention deficits after pulvinar lesion have concerned single patients or small samples. Here we examined a group of 13 patients with focal damage to posterior thalamus on a visual search task with faces, allowing us to test several hypotheses concerning pulvinar function in controlling attention to visually salient or emotionally significant stimuli. Our results identified two subgroups of thalamic patients with distinct patterns of attentional responsiveness to emotional and colour features in face targets. One group with lesions located in anterior and ventral portions of thalamus showed intact performance, with a normal facilitation of visual search for faces with emotional (fearful or happy) expressions on both side of space, similar to healthy controls. By contrast, a second group showed a slower and poorer detection of face targets, most severe for neutral faces, but with a paradoxically enhanced facilitation by both colour and emotional features. This second group had lesions centred on the pulvinar, involving mainly the dorso-medial sectors in patients showing enhanced effects of colour features, but extending to more dorso-lateral sectors in those with enhanced effects of emotional features. These findings reveal that pulvinar nuclei are not critical for orienting attention to emotionally or visually salient features, but instead provide new evidence in support of previous hypotheses suggesting an important role in controlling attention in visual scenes with distracting information., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background and Purpose: Elevated signal intensity (SI) in the dentate nucleus (DN), globus pallidus (GP) and pulvinar (PUL) was reportedly observed on unenhanced T1-weighted (T1WI) magnetic resonance (MR) images in patients receiving multiple enhanced MR examinations. We aimed to clarify whether this phenomenon influences the long-term neurological status of patients., Materials and Methods: We studied 196 radiosurgically treated patients undergoing ≥10 MR examinations using a single dose of gadodiamide and the same 1.5 Tesla MR unit. SI ratios were calculated by referencing the brainstem (BS) for the DN and the thalamus (TH) for the GP and PUL. We compared the SI ratios at the first, fifth, and 10th, and at the most recent examinations. The neurological symptoms of all 196 patients were assessed at each MR examination by one of the authors (MY)., Results: The DN/BS and GP/TH SI ratios were significantly increased at the fifth examination ( p < .0001, p = 0.0094) and, thereafter, gradually increased. Although the PUL/TH SI ratio was not significantly increased at the fifth examination ( p = 0.2515), a significant increase was noted at the 10th examination ( p < .0001). There were no significant predictive factors for DN/BS SI increases. Younger age, no brain metastasis, and normal estimated glomerular filtration rate were related to GP/TH SI ratio increases ( p = 0.0308, p = 0.0001, p = 0.0306). Higher age and total bilirubin level were related to an increased PUL/TH SI ratio ( p = 0.0276, p = 0.0097). No patients experienced gadodiamide-related health problems., Conclusions: Although the SI ratios rose as numbers of gadodiamide administrations increased, no adverse health effects have developed to date.

Background: Posterior cortical atrophy (PCA) is a clinical variant of Alzheimer's disease (AD) that presents with progressive visuospatial symptoms. While amnestic AD is characterized by disrupted default mode network (DMN) connectivity with corresponding increases in salience network (SN) connectivity, a visuospatial network appears to be disrupted early in PCA. Based on PCA patients' clinical features, we hypothesized that, in addition to early decreased integrity within the visuospatial network, patients with PCA would show increases in SN connectivity despite relative preservation of DMN. As the lateral pulvinar nucleus of the thalamus has direct anatomical connections with striate and extrastriate cortex and DMN, and the medial pulvinar is anatomically interconnected with SN, we further hypothesized that lateral and medial pulvinar nuclei might be implicated in intrinsic connectivity changes in PCA., Methods: 26 patients with PCA and 64 matched controls were recruited through UCSF Memory and Aging Center research programs. Each completed a standardized neuropsychological battery, structural MRI, and task-free fMRI. Seed-based functional correlations were used to probe networks of interest, including those seeded by the medial and lateral pulvinar thalamic nuclei, across the whole brain, and functional data analyses were adjusted for brain atrophy., Results: Patients with PCA showed disproportionate deficits in the visuospatial domain; they also showed preserved social sensitivity and endorsed more depressive symptoms than HCs. PCA patients had significant parietooccipital atrophy accompanied by widespread connectivity decreases within the visuospatial network, enhanced connectivity between some structures in SN, and enhanced connectivity between key nodes of the DMN compared to controls. Increased SN connectivity correlated with a measure of social sensitivity, and increased DMN connectivity correlated with short-term memory performance. Medial pulvinar connectivity increases in PCA were topographically similar to SN (anterior insula) connectivity increases, while lateral pulvinar connectivity increases were similar to DMN (posterior cingulate) connectivity increases., Conclusions: PCA is characterized by preserved to heightened connectivity in the SN and DMN despite decreased visuospatial network connectivity. The spatial similarity of medial and lateral pulvinar connectivity changes to those seen in the SN and DMN suggests a role for the pulvinar in intrinsic connectivity network changes in PCA., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

The thalamus is believed to play crucial role in processing viscero-sensory information, and regulating the activity of amygdala in patients with panic disorder (PD). Previous functional neuroimaging studies have detected abnormal activation in the thalamus in patients with PD compared with healthy control subjects (HC). Very few studies, however, have investigated for volumetric abnormalities in the thalamus in patients with PD. Furthermore, to the best of our knowledge, no previous study has investigated for shape abnormalities in the thalamus in patients with PD. Twenty-five patients with PD and 25 HC participants (all female) were recruited for the study. A voxel-wise volume comparison analysis and a vertex-wise shape analysis were conducted to evaluate structural abnormalities in the PD patients compared to HC. The patients with PD demonstrated significant gray matter volume reductions in the thalamus bilaterally, relative to the HC. The shape analysis detected significant inward deformation in some thalamic regions in the PD patients, including the anterior nucleus, mediodorsal nucleus, and pulvinar nucleus. PD patients showed shape deformations in key thalamic regions that are believed to play a role in regulating emotional and cognitive functions., Competing Interests: The authors have declared that no competing interests exist.

Background: Dementia with Lewy bodies is characterized by transient clinical features, including fluctuating cognition and visual hallucinations, implicating dysfunction of cerebral hub regions, such as the pulvinar nuclei of the thalamus. However, the pulvinar is typically only mildly affected by Lewy body pathology in dementia with Lewy bodies, suggesting additional factors may account for its proposed dysfunction., Methods: We conducted a comprehensive analysis of postmortem pulvinar tissue using whole-transcriptome RNA sequencing, protein expression analysis, and histological evaluation., Results: We identified 321 transcripts as significantly different between dementia with Lewy bodies cases and neurologically normal controls, with gene ontology pathway analysis suggesting the enrichment of transcripts related to synapses and positive regulation of immune functioning. At the protein level, proteins related to synaptic efficiency were decreased, and general synaptic markers remained intact. Analysis of glial subpopulations revealed astrogliosis without activated microglia, which was associated with synaptic changes but not neurodegenerative pathology., Discussion: These results indicate that the pulvinar, a region with relatively low Lewy body pathological burden, manifests changes at the molecular level that differ from previous reports in a more severely affected region. We speculate that these alterations result from neurodegenerative changes in regions connected to the pulvinar and likely contribute to a variety of cognitive changes resulting from decreased cortical synchrony in dementia with Lewy bodies. © 2018 International Parkinson and Movement Disorder Society., (© 2018 International Parkinson and Movement Disorder Society.)

Background and Purpose: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity., Materials and Methods: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls., Results: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables., Conclusions: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease., (© 2017 by American Journal of Neuroradiology.)

Anorexia nervosa (AN) is a psychiatric disorder, in which the prognosis for some patients is poor. The etiology and effective treatments for AN have not been established. We examined morphometric changes in the brain of AN and clarified how the changes were associated with symptoms and pathophysiology. We enrolled 52 participants: 7 with the restrictive type of AN, 13 with the binge-eating/purging type, 3 with eating disorder not otherwise specified, and 29 healthy controls. Participants underwent T1-weighted MRI. Group differences between patients and controls in gray matter volume (GMV) were analyzed using voxel-based morphometry. Age and body mass index (BMI) were considered covariates. Correlations between regional GMVs and drive for thinness and body dissatisfaction were examined. Patients had decreased GMV in the superior/middle temporal gyrus (STG/MTG), pulvinar, and superior frontal gyrus after correction for age and BMI, and in the STG/MTG, middle frontal gyrus, and cingulate after correction for age. A correlational group difference was detected for body dissatisfaction and GMV in the STG. Our findings suggest that decreased GMV in the STG is related to body dissatisfaction that could come from impaired visuospatial perception, together with GMV decreases in several regions, which may be involved in development of AN., (Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.)

Dysarthria is a frequent symptom in patients with stroke. The anatomical structures responsible for dysarthria have been reported in patients with lacunar infarcts, but the related lesions in patients with basal ganglia hemorrhage (BGH) have not been investigated. The aim of this study was to identify associations between the lesion location and the presence/absence of dysarthria in patients with BGH using voxel-based lesion symptom mapping (VLSM) analyses. A retrospective analysis was conducted on 26 patients with acute BGH (mean age, 54.0 years; men:women, 14:12) who underwent conservative management. The patients were classified into groups based on the presence or absence of dysarthria at the time of admission, which was determined by reviewing the patients' medical records. Brain lesions were traced on magnetic resonance images that were acquired within the first 3 weeks after BGH onset, and then separate high-resolution region-of-interest images were generated. Associations between dysarthria and the lesion location were determined with the VLSM analyses. The average volume of the delimited lesions was 7.38±5.75cm 3 . The VLSM analyses identified several voxel clusters, mainly in the pulvinar nucleus of the left thalamus, that were significantly related to the presence of dysarthria at admission. These findings suggest that patients with BGH extending into the left pulvinar nucleus should be monitored for dysarthria., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Complex visual hallucinations occur in 70%-80% of dementia with Lewy bodies patients and significantly affect well-being. Despite the prevalence of visual hallucinations in dementia with Lewy bodies, the neuropathological basis of this phenomenon is poorly understood. The pulvinar nucleus of the thalamus has not previously been neuropathologically examined, but has been linked to visual hallucinations in dementia with Lewy bodies. The objective of this study was to investigate whether neuropathological or morphometric changes occur in the pulvinar nucleus in dementia with Lewy bodies cases that may contribute to visual hallucinations., Methods: Postmortem pulvinar tissue was acquired from 8 individuals with dementia with Lewy bodies, 8 with Alzheimer's disease, and 8 control cases and was analyzed using stereological and quantitative neuropathological techniques., Results: Lewy body pathology was present throughout the pulvinar in dementia with Lewy bodies but was most severe in the medial pulvinar. Neuronal loss was found in the lateral pulvinar in dementia with Lewy bodies and Alzheimer's disease but was more severe in dementia with Lewy bodies., Conclusions: The pulvinar has an important role in visual attention, visual target selection and affective visual perception. These functions are thought to be deficient in dementia with Lewy bodies and may contribute a vulnerability to visual hallucinations. Therefore, this study has demonstrated neuropathological changes that may promote the manifestation of visual hallucinations in dementia with Lewy bodies. © 2017 International Parkinson and Movement Disorder Society., (© 2017 International Parkinson and Movement Disorder Society.)

This article reports the case of a 68-year-old patient with anti-HU antibodies paraneoplastic encephalitis. The clinical manifestations were atypical and the paraclinical work-up, notably the magnetic resonance imaging (MRI) showing bilateral posterior thalamic hyperintensities (pulvinar sign), misleadingly pointed towards a variant Creutzfeld-Jakob disease. After presenting the case, the differential diagnosis of the pulvinar sign is discussed along with other important diagnostic considerations., (© The Author(s) 2016.)

The aim of this study was to analyze the pattern of magnetic resonance diffusion-weighted imaging (DWI) findings in status epilepticus in terms of clinical characteristics. Participants comprised 106 patients with status epilepticus who were admitted to our hospital and underwent DWI. Forty-five patients (42.5 %) showed abnormal findings on DWI and were divided into two groups, comprising 26 patients (24.5 %) with cortex lesions alone and 19 patients (17.9 %) with cortex and pulvinar lesions in the same hemisphere. A long duration of status epilepticus (>120 min) tended to be more prevalent among patients with cortex and pulvinar lesions (57.9 %) than among patients with cortex lesions alone (30.8 %) by univariate and multivariate analyses. Todd's palsy tended to be more frequent in patients with abnormalities on DWI (24/45, 53.3 %) than in patients with normal DWI (21/61, 34.4 %). Six of the 26 patients with cortex lesions alone (23.1 %) had taken anti-epileptic drugs before the attack compared to none of the 19 patients with both cortex and pulvinar lesions. The trend toward a longer duration of status epilepticus in patients with both cortex and pulvinar lesions favors a spreading pattern of seizure discharge from cortex to pulvinar via cortico-pulvinar pathways, and anti-epileptic drugs might, to some extent, prevent spreading of seizure discharge from cortex to pulvinar. In addition, existence of high-intensity areas on DWI at the onset of epilepsy may be a predictive factor for the occurrence of Todd's palsy.

Background and Purpose: The aim of this study was to identify characteristic 3.0 T brain MRI findings in patients with aspartylglucosaminuria (AGU), a rare lysosomal storage disorder. Previous AGU patient material imaged at 1.0 and 1.5 T was also re-evaluated., Materials and Methods: Twenty-five brain MRI examinations from 20 AGU patients were included in the study. Thirteen patients underwent a prospective 3.0 T MRI (5 male, 8 female, aged 9-45 years). Twelve examinations from nine patients (4 male, 5 female, aged 8-33 years) previously imaged at 1.0 or 1.5 T were re-evaluated. Two patients were included in both the prospective and the retrospective groups. Visual analysis of the T1- and T2-weighted images was performed by two radiologists., Results: The previously reported signal intensity changes in T2-weighted images were visible at all field strengths, but they were more distinct at 3.0 T than at 1.0 or 1.5 T. These included signal intensity decrease in the thalami and especially in the pulvinar nuclei, periventricular signal intensity increase and juxtacortical high signal foci. Poor differentiation between gray and white matter was found in all patients. Some degree of cerebral and/or cerebellar atrophy and mild ventricular dilatation were found in nearly all patients. This study also disclosed various unspecific findings, including a higher than normal incidence of dilated perivascular spaces, arachnoid cysts, pineal cysts and mildly dilated cavum veli interpositi., Conclusion: This study revealed particular brain MRI findings in AGU, which can raise the suspicion of this rare disease in clinical practice., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Patients with striate cortex lesions experience visual perception loss in the contralateral visual field. In few patients, however, stimuli within the blind field can lead to unconscious (blindsight) or even conscious perception when the stimuli are moving (Riddoch syndrome). Using functional magnetic resonance imaging (fMRI), we investigated the neural responses elicited by motion stimulation in the sighted and blind visual fields of eight patients with lesions of the striate cortex. Importantly, repeated testing ensured that none of the patients exhibited blindsight or a Riddoch syndrome. Three patients had additional lesions in the ipsilesional pulvinar. For blind visual field stimulation, great care was given that the moving stimulus was precisely presented within the borders of the scotoma. In six of eight patients, the stimulation within the scotoma elicited hemodynamic activity in area human middle temporal (hMT) while no activity was observed within the ipsilateral lesioned area of the striate cortex. One of the two patients in whom no ipsilesional activity was observed had an extensive lesion including massive subcortical damage. The other patient had an additional focal lesion within the lateral inferior pulvinar. Fiber-tracking based on anatomical and functional markers (hMT and Pulvinar) on individual diffusion tensor imaging (DTI) data from each patient revealed the structural integrity of subcortical pathways in all but the patient with the extensive subcortical lesion. These results provide clear evidence for the robustness of direct subcortical pathways from the pulvinar to area hMT in patients with striate cortex lesions and demonstrate that ipsilesional activity in area hMT is completely independent of conscious perception., (© 2014 Wiley Periodicals, Inc.)

Increased brain iron levels may be a risk factor for age-related neurologic disorders. Little is known about factors other than age and sex potentially affecting brain iron concentration. We investigated dietary habits (iron and calcium supplements, dairy products, vegetables, and red meat) as a potential modifiable predictor of brain iron levels using 3-T susceptibility-weighted magnetic resonance imaging. One hundred ninety volunteers were scanned, and mean phase and mean phase of low-phase voxels were determined for deep gray-matter (DGM) structures, including the caudate, putamen, thalamus, pulvinar, hippocampus, amygdala, red nucleus, and substantia nigra. There was a trend for lower mean phase (suggestive of high iron levels) in individuals taking iron supplements (p = 0.075). Among men, both increased dairy and vegetable intakes were significantly associated with lower DGM mean phase (p < 0.05) and mean phase of low-phase voxels (p < 0.05) in the thalamus, pulvinar, and red nucleus. In contrast, among women, iron levels were not associated with dairy consumption (p > 0.05) in the DGM but were inversely associated with vegetable intake in the thalamus (p = 0.006). Brain iron levels appear to be modulated by diet, with effects being highly dependent on gender., (Published by Elsevier Inc.)

A 76-year-old man was admitted to our hospital due to gait difficulty. Brain imaging indicated bilateral pulvinar lesions and moderate white matter lesions. The serum α-galactosidase A levels were measured for the differential diagnosis of bilateral pulvinar lesions and were found to be abnormally low. Therefore, the patient was suspected to have variant Fabry disease. A GLA mutation analysis showed the p.E66Q mutation, which is speculated to be a functional polymorphism rather than a disease-causing mutation of Fabry disease. Enzyme replacement therapy did not result in a marked improvement, however, the disease progression stopped.

Recent work has identified disruption of several brain networks involving limbic and cortical regions that contribute to the generation of diverse symptoms of major depressive disorder (MDD). Of particular interest are the networks anchored on the right anterior insula, which binds the cortical and limbic regions to enable key functions that integrate bottom-up and top-down information in emotional and cognitive processing. Emotional appraisal has been linked to a presumed hierarchy of processing, from sensory percepts to affective states. But it is unclear whether the network level dysfunction seen in depression relates to a breakdown of this presumed hierarchical processing system from sensory to higher cognitive regions, mediated by core limbic regions (e.g. insula). In 16 patients with current MDD, and 16 healthy controls, we investigated differences in directional influences between anterior insula and the rest of the brain using resting-state functional magnetic resonance imaging (fMRI) and Granger-causal analysis (GCA), using anterior insula as a seed region. Results showed a failure of reciprocal influence between insula and higher frontal regions (dorsomedial prefrontal cortex) in addition to a weakening of influences from sensory regions (pulvinar and visual cortex) to the insula. This suggests dysfunction of both sensory and putative self-processing regulatory loops centered around the insula in MDD. For the first time, we demonstrate a network-level processing defect extending from sensory to frontal regions through insula in depression. Within limitations of inferences drawn from GCA of resting fMRI, we offer a novel framework to advance targeted network modulation approaches to treat depression., (Copyright © 2014 Elsevier B.V. and ECNP. All rights reserved.)

Hexanucleotide repeat expansion in C9orf72 represents the most common genetic cause of familial and sporadic behavioural variant frontotemporal dementia. Previous studies show that some C9orf72 carriers with behavioural variant frontotemporal dementia exhibit distinctive atrophy patterns whereas others show mild or undetectable atrophy despite severe behavioural impairment. To explore this observation, we examined intrinsic connectivity network integrity in patients with or without the C9orf72 expansion. We studied 28 patients with behavioural variant frontotemporal dementia, including 14 C9orf72 mutation carriers (age 58.3 ± 7.7 years, four females) and 14 non-carriers (age 60.8 ± 6.9 years, four females), and 14 age- and sex-matched healthy controls. Both patient groups included five patients with comorbid motor neuron disease. Neuropsychological data, structural brain magnetic resonance imaging, and task-free functional magnetic resonance imaging were obtained. Voxel-based morphometry delineated atrophy patterns, and seed-based intrinsic connectivity analyses enabled group comparisons of the salience, sensorimotor, and default mode networks. Single-patient analyses were used to explore network imaging as a potential biomarker. Despite contrasting atrophy patterns in C9orf72 carriers versus non-carriers, patient groups showed topographically similar connectivity reductions in the salience and sensorimotor networks. Patients without C9orf72 expansions exhibited increases in default mode network connectivity compared to controls and mutation carriers. Across all patients, behavioural symptom severity correlated with diminished salience network connectivity and heightened default mode network connectivity. In C9orf72 carriers, salience network connectivity reduction correlated with atrophy in the left medial pulvinar thalamic nucleus, and this region further showed diminished connectivity with key salience network hubs. Single-patient analyses revealed salience network disruption and default mode network connectivity enhancement in C9orf72 carriers with early-stage or slowly progressive symptoms. The findings suggest that patients with behavioural variant frontotemporal dementia with or without the C9orf72 expansion show convergent large-scale network breakdowns despite distinctive atrophy patterns. Medial pulvinar degeneration may contribute to the behavioural variant frontotemporal dementia syndrome in C9orf72 carriers by disrupting salience network connectivity. Task-free functional magnetic resonance imaging shows promise in detecting early-stage disease in C9orf72 carriers and may provide a unifying biomarker across diverse anatomical variants., (© The Author (2014). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Objective: Recently, magnetic resonance imaging (MRI) abnormalities of the pulvinar in patients with epilepsy have received greater attention, but their occurrence and features have not been fully elucidated. Therefore, we investigated the clinical and radiological features of patients with epilepsy who presented MRI abnormalities of the pulvinar., Patients and Methods: We retrospectively investigated 225 consecutive patients who came to our institute because of seizures and underwent an MRI within 24h. The patients who exhibited pulvinar MRI abnormalities, their profile, seizure type, efficacy of medication, and chronological changes of MRI findings were examined., Results: Out of the 225 patients who underwent MRI within 24h of seizure, 17 exhibited MRI abnormalities of the pulvinar. All of these 17 patients presented status epilepticus. Bilateral pulvinar diffusion-weighted imaging (DWI) hyperintensity was observed in 3 patients and unilateral pulvinar DWI hyperintensity in the other 14. Out of these 14 patients, 7 exhibited DWI hyperintensity in the ipsilateral cerebral cortex, and 10 patients presented an old lesion due to stroke or trauma., Conclusions: Our results demonstrated that the involvement of the pulvinar in status epilepticus is more frequent than expected and consisted of unilateral or bilateral DWI hyperintensities that may completely normalize. These pulvinar MRI abnormalities possibly reflect the epileptogenic hyperexcitation of different cortical areas through their connections with the pulvinar., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

A 47-year-old man presented with sudden visual loss, optic disk edema, retinal ischemia, and limited upgaze in the left eye. Initial MRI revealed thickened, enhancing left optic nerve. Extensive work-up for an inflammatory and infiltrative etiology was positive only for Borrelia burgdorferi IgM by Western blot. Six weeks later the patient had numbness and weakness on his left side. MRI showed enhancing lesions extending from the left optic nerve to the optic chiasm, along the visual pathways bilaterally, mainly on the right side from optic tract to lateral geniculate body and pulvinar. Stereotactic biopsy of the right pulvinar lesion revealed glioblastoma. The tumor progressed rapidly, and the patient died 11 weeks after the onset of first symptoms., (Copyright © 2014 Elsevier Inc. All rights reserved.)

A 39-year-old woman developed right hemiparesis in a few days. Magnetic resonance images revealed cerebral infarction in the territory of the left lenticulostriate artery, and MR angiography showed severe stenosis of the middle and anterior cerebral arteries and moderate one of the vertebral arteries. Bilateral and symmetric T1 hyperintensity in the pulvinar (T1-weighted imaging-pulvinar sign; "T1 pulvinar sign") was detected, which is recognized as a key imaging of Fabry disease. The α-galactosidase A gene analysis, however, showed no mutation. Although specific physical symptoms were solely short stature and oligomenorrhea, the diagnosis of Turner syndrome was confirmed by the chromosome analysis which showed mosaicism of 45XO and 46X,r(X) (60%:40%). To our knowledge, this is the first report of Turner syndrome with "T1 pulvinar sign".

Purpose: To define the neuropathological findings of pulvinar degeneration seen in long duration status epilepticus., Methods: We review the clinical, radiologic, neurophysiologic, investigational and neuropathological findings on a 27 year old woman who died after 162 days of prolonged refractory status epilepticus., Results: Continuous EEG monitoring confirmed recurrent uncontrolled seizure activity bilaterally and independently, most frequent in the right fronto-temporal region. Initial MRI of the brain was normal. Repeat study until on day 127 of admission showed advanced changes, with bilateral pulvinar T2/FLAIR hyperintensities. The autopsy revealed sharply defined, grey, soft, granular nodules in each medial pulvinar nucleus. Microscopically these consisted of sharply defined paucicellular areas with loss of neurons and myelin and with numerous macrophages in their centers, surrounded by reactive astrocytes with relatively spared of axons. The spinal cord at cervical and thoracic levels showed symmetric spongy vacuolation in the central part of the dorsal columns and lateral corticospinal tracts, with mild myelin loss, relatively preserved axons. The pathological lesions found in this case in thepulvinar are somewhat similar to the pathologic lesions described in Wernicke's encephalopathy. Those found in the spinal cord of our patient resemble characteristic features of B12 related subacute combined degeneration., Conclusion: Characteristic pulvinar degeneration may be found as an acquired phenomenon in prolonged refractory status epilepticus. We hypothesize that the neuropathological findings result from an excessive focal metabolic demand, secondary to neuronal network over activation in the setting of prolonged, frequent bi-temporal seizures., (Copyright © 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: To report on iodine-125 (I125) interstitial irradiation in the treatment of low grade brain stem gliomas in adults., Patients and Methods: Ten patients with well-circumscribed lesions of the brainstem and histological confirmation of low grade glioma treated with stereotactically implanted I-125 seed in our department between 1995 and 2012 were retrospectively analyzed., Results: In 9 patients the lesion was treated with one I-125 seed and in one patient, 2 spatial separated lesions were implanted, therefore a total of 11 I-125 seeds were implanted. The mean volume of the 11 lesions was 2.76 ml (range: 0.5-7.2 ml), mean activity of the seeds was 6.23 mCi (range: 1.5-11.1 mCi), mean duration of irradiation was 28.5 days (range: 21-41 days) and mean effective dose rate was 9.16 cGy/h (range: 6.2-12 cGy/h). The 30 days perioperative morbidity and mortality rate was 0%. Median follow up was 72.5 month (range 5-168 months). Six of ten patients were free of progression until last follow up., Conclusion: In our experience at the University Clinic in Freiburg Germany, interstitial radiosurgery based on MRI is a safe and effective method to diagnose and treat low grade gliomas of the brain stem. Furthermore randomized studies are needed to confirm the therapeutic impact of this method in comparison to external beam radiation of brain stem gliomas., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Background and Purpose: Low glucose values are often seen in term infants with NE, including HIE, yet the contribution of hypoglycemia to the pattern of neurologic injury remains unclear. We hypothesized that MR features of neonatal hypoglycemia could be detected, superimposed on the predominant HIE injury pattern., Materials and Methods: Term neonates (n = 179) with NE were prospectively imaged with day-3 MR studies and had glucose data available for review. The predominant imaging pattern of HIE was recorded as watershed, basal ganglia, total, focal-multifocal, or no injury. Radiologic hypoglycemia was diagnosed on the basis of selective edema in the posterior white matter, pulvinar, and anterior medial thalamic nuclei. Clinical charts were reviewed for evidence of NE, HIE, and hypoglycemia (<46 mg/dL)., Results: The predominant pattern of HIE injury imaged included 17 watershed, 25 basal ganglia, 10 total, 42 focal-multifocal, and 85 cases of no injury. A radiologic diagnosis of hypoglycemia was made in 34 cases. Compared with laboratory-confirmed hypoglycemia, MR findings had a positive predictive value of 82% and negative predictive value of 78%. Sixty (34%) neonates had clinical hypoglycemia before MR imaging. Adjusting for 5-minute Apgar scores and umbilical artery pH with logistic regression, clinical hypoglycemia was associated with a 17.6-fold higher odds of MR imaging identification (P < .001). Selective posterior white matter and pulvinar edema were most predictive of clinical hypoglycemia, and no injury (36%) or a watershed (32%) pattern of injury was seen more often in severe hypoglycemia., Conclusions: In term infants with NE and hypoglycemia, specific imaging features for both hypoglycemia and hypoxia-ischemia can be identified.

The pulvinar is the largest nucleus of the thalamus. Its lateral and inferior areas have rich connections with the visual- and dorsolateral parietal cortices. Several cells in the medial and upper area connect the anterior cingulum and the premotor and prefrontal association areas. This neuronal network was considered to organize the saccades and visual attention. Other cells in the medial nucleus have axonal connections with paralimbic-, insular and higher order association-cortices. The medial structure integrates complex sensory information with limbic reactivity settings, transmitting these to the temporal and parieto-occipital centres. The pulvinar is supplied by the posterior chorioideal artery. Visual salience is considered to be an important function of the pulvinar. Visual selection enables subjects to choose the actually adequate behavioral act. To serve the visual salience the pulvinar may also inhibit inappropriate eye movements. The pulvinar appears to be a key structure of the EEG's alpha rhythm generator, acting together with the parietooccipital and temporal cortices. Dynamic fluctuation of BOLD signals on fMRI correlates well with the change of alpha power even in resting state. We presume that the pulvinar is part of a closed cortico-subcortical circuit, analogous with the striatum, but the output of the pulvinar initiates complex behavioral reactions, including perception, selective attention and emotions. Damage of the pulvinar may elicit contralateral visual neglect, because of the dissociation of the neuronal network integrated by the superior temporal area. Increased activity of the pulvinar was found during abrupt reaction to fearful visual signals; and also in the etiopathology of endogenous depressions through the alteration of serotonin transporters. Increased bilateral signal intensity of the pulvinar on MRI was detected in cases of the new variants of Creutzfeldt-Jakob- and Fabry diseases.

Characteristic clinical manifestations of AFD such as acroparesthesias, angiokeratoma, corneal opacity, hypo/ and anhidrosis, gastrointestinal symptoms, renal and cardiac dysfunctions can occur in male and female patients, although heterozygous females with AFD usually seem to be less severely affected. The most prominent CNS manifestations consist of cerebrovascular events such as transient ischaemic attacks (TIAs) and (recurrent) strokes. For the most part, CNS complications in AFD have been attributed to cerebral vasculopathy, including anatomical abnormalities. The natural history of Fabry patients includes transitory cerebral ischaemia and strokes, even in very young persons of both genders. The mechanism is partly due to vascular endothelial accumulation of Gb-3. White matter lesions (WML) on occur MRI. Both males and females can be safely treated with enzyme replacement; and thus screening for Fabry disease of young stroke populations should be considered. There are, however, no hard data of treatment effect on mortality and morbidity. Stroke in Anderson-Fabry disease study of 721 patients with cryptogenic stroke, aged 18-55 years, showed a high prevalence of Fabry disease in this group: 5% (21/432) of men and 3% (7/289) of women. Combining results of both sexes showed that 4% of young patients with stroke of previously unknown cause had Fabry disease, corresponding to about 1-2% of the general population of young stroke patients. Cerebral micro- and macro-vasculopathy have been described in Fabry disease. Neuronal globotriaosylceramide accumulation in selective cortical and brain stem areas including the hippocampus has been reported by autopsy studies in FD, but clinical surrogates as well as the clinical relevance of these findings have not been investigated so far. Another Neurologic hallmark of Fabry disease (FD) includes small fiber neuropathy as well as cerebral micro- and macroangiopathy with premature stroke. Cranial MRI shows progressive white matter lesions (WML) at an early age, increased signal intensity in the pulvinar, and tortuosity and dilatation of the larger vessels. Conventional MRI shows a progressive load of white matter lesions (WMLs) due to cerebral vasculopathy in the course of FD. Another study has been conducted to quantify brain structural changes in clinically affected male and female patients with FD. The peripheral neuropathy in Fabry disease manifests as neuropathic pain, reduced cold and warm sensation and possibly gastrointestinal disturbances. Patients with Fabry disease begin having pain towards the end of the first decade of life or during puberty. Children as young as 6 years of age have complained of pain often associated with febrile illnesses with reduced heat and exercise tolerance. The patients describe the pain as burning that is often associated with deep ache or paresthesiae. Some patients also have joint pain. A high proportion of patients with Fabry disease is at increased risk of developing neuropsychiatric symptoms, such as depression and neuropsychological deficits. Due to both somatic and psychological impairment, health-related quality of life (QoL) is considerably reduced in patients with Fabry disease. Targeted screening for Fabry disease among young individuals with stroke seems to disclose unrecognized cases and may therefore very well be recommended as routine in the future. Furthermore, ischemic stroke is related to inflammation and arterial stiffness and no study had addressed this relationship in patients with AF disease and cerebrovascular disease, so this topic could represent a possible future research line.

Objective: We hypothesized that prominent pulvinar hypointensity in brain MRI represents the disease process due to iron accumulation in Alzheimer disease (AD). We aimed to determine whether or not the pulvinar signal intensity (SI) on the fluid-attenuated inversion recovery (FLAIR) sequences at 3.0T MRI differs between AD patients and normal subjects, and also whether the pulvinar SI is correlated with the T2(*) map, an imaging marker for tissue iron, and a cognitive scale., Materials and Methods: Twenty one consecutive patients with AD and 21 age-matched control subjects were prospectively included in this study. The pulvinar SI was assessed on the FLAIR image. We measured the relative SI ratio of the pulvinar to the corpus callosum. The T2(*) values were calculated from the T2(*) relaxometry map. The differences between the two groups were analyzed, by using a Student t test. The correlation between the measurements was assessed by the Pearson's correlation test., Results: As compared to the normal white matter, the FLAIR signal intensity of the pulvinar nucleus was significantly more hypointense in the AD patients than in the control subjects (p < 0.01). The pulvinar T2(*) was shorter in the AD patients than in the control subjects (51.5 ± 4.95 ms vs. 56.5 ± 5.49 ms, respectively, p = 0.003). The pulvinar SI ratio was strongly correlated with the pulvinar T2(*) (r = 0.745, p < 0.001). When controlling for age, only the pulvinar-to-CC SI ratio was positively correlated with that of the Mini-Mental State Examination (MMSE) score (r = 0.303, p < 0.050). Conversely, the pulvinar T2(*) was not correlated with the MMSE score (r = 0.277, p = 0.080)., Conclusion: The FLAIR hypointensity of the pulvinar nucleus represents an abnormal iron accumulation in AD and may be used as an adjunctive finding for evaluating AD.

Objective: The neurobiological basis of inattentiveness, a core feature of attention-deficit/hyperactivity disorder (ADHD), is not yet well understood. Structural abnormalities in thalamus, especially the pulvinar nuclei, have recently been reported in ADHD. Pulvinar nuclei maintain reciprocal connections with cortical/subcortical areas, and play a central coordinating role during visual attention processing. The objective of this study was to test the hypothesis that children and young adolescents with ADHD would show atypical pulvinar-cortical functional pathways during sustained attention performance, and that these functional abnormalities would be associated with the inattentive symptoms of the disorder., Method: Visual attention task-based functional magnetic resonance imaging (fMRI) data from 22 children and young adolescents with ADHD and 22 demographically matched, normal control subjects were analyzed. Cortical activation maps and temporal correlations of activity patterns between pulvinar nuclei and the remainder of brain were constructed for each participant. Correlations between activation magnitude of pulvinar and diagnostic measures were calculated in subjects with ADHD., Results: Compared to controls, subjects with ADHD showed significantly reduced pulvinar activations bilaterally, significantly decreased functional connectivity between bilateral pulvinar and right prefrontal regions, and significantly increased connectivity between the right pulvinar and bilateral occipital regions. In addition, the activation magnitude in the left pulvinar was negatively correlated with the DSM-IV inattentive index in ADHD group., Conclusions: Allied with previous evidence of structural abnormalities in pulvinar, the current data suggest that inappropriate development of pulvinar may lead to disrupted functional circuits for visual attention processing, and that these disruptions contribute significantly to the pathophysiological mechanisms of the inattentiveness symptoms in ADHD., (Copyright © 2012 American Academy of Child and Adolescent Psychiatry. Published by Elsevier Inc. All rights reserved.)

Background: Previous research showed acute diffusion-weighted imaging changes in pulvinar after extensive cortical injury from neonatal stroke. The authors used diffusion tensor imaging (DTI) to see how separate regions of ipsilateral thalamus are directly affected after a primary hit to their connected cortex in neonatal stroke., Methods: The authors analysed DTI images of three term infants with acute unilateral cortical arterial ischaemic stroke. Probabilistic tractography was used to define separate thalamic regions of interests (ROIs). The authors evaluated the three eigenvalues (EV) and apparent diffusion coefficient (ADC) values in the ROIs., Results: The ADC and EV in voxels of ROIs placed within the nuclei corresponding to ischaemic cortex were significantly lower than those in the unaffected contralesional thalamic nuclei., Conclusions: Our findings support the concept of acute network injury in neonatal stroke. ADC and EV were altered in specific thalamic regions that corresponded to the specific cortical areas affected by the primary ischaemic injury.

Crossed cerebellar diaschisis and bright pulvinar are rare in patients with status epilepticus. We present a case of a 53-year-old man who was found confused, incontinent, and nonverbal. The EEG findings were consistent with non-convulsive status epilepticus. The brain MR images showed findings consistent with crossed cerebellar diaschisis and bright pulvinar. We report and discuss this case to increase the awareness of these MRI signs in confused and obtunded patients.

Background: Thalamic volume deficits are associated with psychosis but it is unclear whether the volume reduction is uniformly distributed or whether it is more severe in particular thalamic regions., Aims: To quantify whole and regional thalamic volume in males with early-onset psychosis and healthy male controls., Method: Brain scans were obtained for 80 adolescents: 46 individuals with early-onset psychosis with a duration of positive symptoms less than 6 months and 34 healthy controls. All participants were younger than 19 years. Total thalamic volumes were assessed using FreeSurfer and FSL-FIRST, group comparisons of regional thalamic volumes were studied with a surface-based approach., Results: Total thalamic volume was smaller in participants with early-onset psychosis relative to controls. Regional thalamic volume reduction was most significant in the right anterior mediodorsal area and pulvinar., Conclusions: In males with minimally treated early-onset psychosis, thalamic volume deficits may be most pronounced in the anterior mediodorsal and posterior pulvinar regions, adding strength to findings from post-mortem studies in adults with psychosis.

Introduction: Creutzfedt-Jakob Disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Early diagnosis and the determination of its form are epidemiologically important, with strong impact on public health. Bilateral pulvinar hyperintensity, either alone (pulvinar sign) or in association with the dorsomedial nucleus of the thalamus (double hockey stick sign) on T2, FLAIR and diffusion weighted imaging (DWI), is a criterion for the probable diagnosis of the variant CJD (vCJD). Bilateral hyperintensity of the caudate, putamina and cortex is the usual pattern found in the sporadic CJD (sCJD)., Objective: Analysis of the imaging aspects on a sCJD patient showing T2 hyperintensity of the pulvinar and dorsomedial thalamic nucleus, in order to assess the magnetic resonance imaging (MRI) accuracy in the discrimination between vCJD and sCJD, when this lesion pattern is present., Methods: We performed a MRI on a 62-year-old female with definitive diagnosis of sCJD made by anatomopathologic study of the brain tissue. Qualitative analysis of MRI, including DWI, T2 and FLAIR sequences, as well as lesional patterns found., Results: Brain MRI showed hyperintensity of the caudate, putamina, pulvinar and dorsomedial nucleus of the thalamus, in DWI, T2 and FLAIR sequences; hypersignal of the caudate and putamina was greater than the signal intensity of the thalami. Hyperintensity of the hippocampus and frontal, temporal and parietal cortex were more obvious in FLAIR and DWI., Comment: Hyperintensity of the pulvinar and dorsomedial nucleus of the thalamus on sCJD may complicate the differential diagnosis with vCJD. True pulvinar sign and double hockey stick sign, consistent with vCJD, must only be considered if the hyperintensity is greater than signal intensity of the caudate and putamina.

We analyzed the phenomenon of transient regional diffusion-weighted MRI (DWI) hyperintensity in a series of status epilepticus (SE) patients with respect to seizure type, epileptogenic lesions and EEG findings. A prospective series of 54 patients (30 men, 24 women, mean age 61.5 years) admitted with SE was analyzed with regard to clinical semiology, EEG and MRI findings including DWI and EEG recordings in the acute peri-ictal phase. DWI abnormalities occurred most frequently in patients with complex-partial SE (27/50%) and generalized SE (18/33.3%). Forty patients (74.1%) had symptomatic, 13/24.1% cryptogenic and 1/1.9% idiopathic epilepsies. On DWI, the hippocampus was affected in 37/68.5% cases, often in combination with other brain areas (15/40.5%), in particular the pulvinar was affected in 14/25.9% patients. Bilateral DWI changes were found in 8/14.8% patients. No correlation with a specific seizure type was observed. In 21/38.9%, DWI changes were ipsilateral to the epileptogenic brain lesion (p<0.001) and in 5/9.3% contralateral, whereas in the majority of patients, either bilateral changes or no specific epileptogenic lesion were found. EEG abnormalities correlated with lateralization of DWI abnormalities in 44/81.5% (p<0.001). The most common localization of DWI abnormalities associated with ictal activity was the hippocampus and the pulvinar. Combined DWI-MRI and EEG analysis provides clues to seizure localization and propagation, as well as to identify brain structures affected by continuous or frequent ictal activity. This large series of patients with different features (SE type and cause, various epileptogenic lesions) demonstrates the heterogeneity of the phenomenon of peri-ictal DWI changes., (Copyright © 2011 Elsevier B.V. All rights reserved.)