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Background and purpose Chorea-acanthocytosis, a rare neurodegenerative disease, affects both the striatum and the medial temporal lobe which may cause involuntary movements and epilepsy, respectively. We examined the imaging changes of the hippocampus/amygdala and the striatum as well as clinical symptoms. Materials and methods We retrospectively reviewed 29 MRI and 13 SPECT studies and the clinical findings of seven genetically confirmed chorea-acanthocytosis patients. We evaluated the time-dependent imaging changes of the hippocampus/amygdala and striatum and examined the relationships among these images and symptoms. Results The initial symptom was epilepsy in four patients and involuntary movements in three patients. These symptoms were eventually noted in five and all seven patients, respectively. On MRI, most patients showed striatum atrophy before a hippocampus/amygdala abnormality emerged, but one patient showed a hippocampus/amygdala abnormality before striatum atrophy. Abnormal MRI findings of hippocampus/amygdala were noted in five patients and atrophy of striatum in all seven patients. SPECT demonstrated hypoperfusion of hippocampus/amygdala in three patients and that of striatum in all five available patients. Four patients demonstrated hypoperfusion of striatum earlier than that of hippocampus/amygdala and one patient showed hypoperfusion of both simultaneously. Many imaging abnormal lesions were accompanied by their corresponding symptoms, but not always so. Conclusion Striatum abnormalities were the initial imaging findings in many chorea-acanthocytosis patients, but epilepsy or hippocampus/amygdala imaging abnormalities may be the only findings at the early stage. It is important to understand the detailed clinical and imaging time courses for the diagnosis of chorea-acanthocytosis.

Purpose To clarify brain abnormalities on magnetic resonance imaging (MRI) and its clinical implications in lissencephaly/subcortical band heterotopia (LIS/SBH) spectrum patients. Methods The clinical severity and classification according to Di Donato were retrospectively reviewed in 23 LIS/SBH spectrum patients. The morphological and signal abnormalities of the brainstem, corpus callosum, and basal ganglia were also assessed. The brainstem distribution pattern of the corticospinal tract (CST) was analyzed by diffusion tensor imaging (DTI) and categorized into two types: normal pattern, in which the CST and medial lemniscus (ML) are separated by the dorsal portion of the transverse pontine fiber, and the abnormal pattern, in which the CST and ML are juxtaposed on the dorsal portion of a single transverse pontine fiber. Correlations between MR grading score and potential additional malformative findings of the brain and clinical symptoms were investigated. Results All patients with grade 3 (n = 5) showed brainstem deformities, signal abnormalities of pontine surface and had a tendency of basal ganglia deformity and callosal hypoplasia whereas those abnormalities were rarely seen in patients with grade 1 and 2 (n = 18). For DTI analysis, the patients with grade 3 LIS/SBH had typically abnormal CST, whereas the patients with grade 1 and 2 LIS/SBH had normal CST. The classification was well correlated with CST and brainstem abnormalities and clinical severity. Conclusion MR assessment including DTI analysis may be useful in assessing the clinical severity in LIS/BH spectrum and may provide insight into its developmental pathology.

Background Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a progressive neurodegenerative disorder with stroke-like lesions. The common MRI findings are gyral swelling and high signal intensity on T2WI/FLAIR images crossing the vascular territories. We have observed a linear cystic lesion and a laminar necrosis in the affected cortices of MELAS patients. Herein, we evaluated these cortical MRI findings in each subtype of mitochondrial disease. Patients and methods We retrospectively reviewed the MRI findings of 71 consecutive patients with clinically and genetically confirmed mitochondrial diseases. The cortical cystic lesions and laminar necrotic lesions were evaluated on T1, T2, and FLAIR images in each subtype of mitochondrial disease, as were their clinical and other imaging characteristics. Results The cortical cystic lesion was observed in 21 of the 71 patients (29.6%) with mitochondrial diseases. Laminar necrosis was detected in only three patients (4.2%). MELAS was the most frequent subtype with cortical cystic lesions, accounting for 81.0%, and all showed the linear pattern except for one patient whose pattern was beaded-like. Conclusion A cortical linear cystic lesion was a common MRI finding in our series of patients with mitochondrial disease, especially in those with MELAS, but laminar necrosis was not. These findings can help differentiate MELAS from infarction.

BACKGROUND AND PURPOSE: Leptomeningeal glioneuronal heterotopia of the brain stem and cerebral migration abnormality were pathologically reported in Fukuyama congenital muscular dystrophy, but the radiologic assessments of the brain stem and cerebral venous system (which may be involved in the development of the anomaly) were insufficient. Here, we evaluated the brain stem and cerebral veins on MR imaging in patients with Fukuyama congenital muscular dystrophy. MATERIALS AND METHODS: We retrospectively reviewed the MR imaging findings of 27 patients with Fukuyama congenital muscular dystrophy. We visually assessed the hypoplasia, superficial structures, and signal intensity of the brain stem on T2WI, FLAIR, and double inversion recovery images and the cerebral, superficial, and deep veins with and without hemorrhage on T2WI and SWI. RESULTS: Brain stem fluffy structures were seen in 96.3% of the cases on T2WI. Superficial high signal intensity on T2WI and FLAIR images was seen in 96.3% and 92.6%, respectively. Abnormally located superficial vessels beneath the cortex were seen in 11.1% on T2WI. Hypoplasia of the superficial cerebral veins was noted in all patients who underwent SWI. Dilated and tortuous subependymal veins were seen in 40.0% on SWI. Hemorrhages were seen in 11.1% on T2WI and in 60.0% on SWI. CONCLUSIONS: Superficial brain stem structural and signal abnormalities would be useful MR imaging findings to diagnose Fukuyama congenital muscular dystrophy as well as venous system abnormalities. Clinicians must keep in mind that this disease has a high risk of hemorrhage.

Focal cortical dysplasia (FCD) is a congenital developmental anomaly that is one of the leading causes of refractory epilepsy. MRI is an essential examination and T1WI, T2WI, and FLAIR images are commonly used MR sequences for delineating FCD.1 However, these MRI findings are often insufficiently clear. We experienced two FCD cases that were much better visualized by using T1WI with chemical shift selective (CHESS) than with T2WI and FLAIR images. CHESS is the most frequently used fat suppression pulse in clinical practice. We report two cases in which CHESS clearly demonstrated FCD, and compare the cases9 pathology and MRI findings.

Objective The underlying neural correlates of psychogenic non-epileptic seizures (PNES) are still unknown and their identification would be helpful for clinicians and patients. This study aimed to reveal details of white matter microstructure and alterations in brain structural networks in patients with PNES by using diffusion tensor imaging (DTI) and graph theoretical connectivity analysis. Methods Seventeen patients with PNES and 26 age- and sex-matched healthy controls were enrolled. All participants underwent DTI on a 3.0-T MRI scanner, and fractional anisotropy (FA) and mean diffusivity (MD) maps were compared by tract-based spatial statistics. Additionally, the structural networks derived from DTI data were analyzed using graph theory and two different parcellation schemes. Results Patients with PNES showed widespread decreases in FA and increases in MD, particularly in the deep white matter. In addition, graph theoretical analysis revealed impaired brain networks in PNES, including increased path length, decreased network efficiency, altered nodal topology, and reduced regional connectivity in the right posterior areas. Conclusion We found widely impaired white matter integrity and impaired brain structural networks in Japanese patients with PNES. These findings contribute to the accumulation of evidence on PNES and may improve understanding of this condition.

Backgrounds Although neurofibrillary tangles (NFTs) mainly accumulate in the medial temporal lobe with human aging, only a few imaging studies have investigated correlations between NFT accumulation and gray matter (GM) volume in cognitively normal older adults. Here, we investigated the correlations between 18F-THK5351 accumulation and GM volume at the voxel level. Material and methods We recruited 47 amyloid-negative, cognitively normal, older adults (65.0 ± 7.9 years, 26 women), who underwent structural magnetic resonance imaging, 11C-Pittsburgh compound-B and 18F-THK5351 PET scans, and neuropsychological assessment. The magnetic resonance and 18F-THK5351 PET images were spatially normalized using Statistical Parametric Mapping 12. Voxel-wise correlations between 18F-THK5351 accumulation and GM volume were evaluated using the Biological Parametric Mapping toolbox. Results A significant negative correlation (p

In amyotrophic lateral sclerosis (ALS), the "zebra sign" in the precentral gyrus on phase difference enhanced magnetic resonance imaging (PADRE) recently has been reported as a possible imaging biomarker for upper motor neuron (UMN) involvement. A previous study has shown that the "zebra sign" allowed us to differentiate patients with ALS from healthy subjects with excellent accuracy. We validated the usefulness of the sign for differentiating patients with ALS from healthy subjects and investigated whether the "zebra sign" can be observed other neurodegenerative disorders with UMN involvement. The "zebra sign" on PADRE was assessed in 26 patients with ALS, 26 with multiple system atrophy (MSA) and 26 healthy controls, and the sign was observed in 50%, 23%, and no subjects, respectively. ALS patients with the "zebra sign" demonstrated a higher UMN burden score than those without the sign. The "zebra sign" on PADRE is not specific to ALS, also present in MSA, but might reflect the degeneration of the UMN within the motor cortex in neurodegenerative disorders.

BACKGROUND AND PURPOSE: The transmantle sign is a characteristic MR imaging finding often seen in focal cortical dysplasia type IIb. The transmantle sign is typically hyperintense on T2WI and FLAIR and hypointense on T1WI. However, in some cases, it shows T1 high signal. We evaluated the imaging and pathologic findings to identify the causes of the T1 high signal in the transmantle sign. MATERIALS AND METHODS: We retrospectively reviewed the preoperative imaging data of 141 consecutive patients with histologically proved focal cortical dysplasia. We selected 25 patients with focal cortical dysplasia with the transmantle sign and divided them into groups based on the pathologic focal cortical dysplasia subtype and T1 signal of the transmantle sign. We evaluated the clinical, radiologic, and pathologic findings, including the number of balloon cells and dysmorphic neurons and the severity of gliosis or calcifications and compared them among the groups. RESULTS: Nine of the 25 patients had a T1-high-signal transmantle sign; the other 16 patients did not. All 9 patients with a T1-high-signal transmantle sign were diagnosed as type IIb (group A). Of the 16 patients with no T1-high-signal transmantle sign, 13 were diagnosed as having type IIb (group B), and the other 3 patients, as type IIa (group C). The number of balloon cells was significantly higher in group A than in the other groups, but there were no differences regarding dysmorphic neurons, the severity of gliosis, or calcifications. CONCLUSIONS: Approximately 6% (9/141) of this patient series had a T1-high-signal transmantle sign, and all were type IIb. The signal may reflect a rich density of balloon cells. This finding could support the differentiation of subtypes, especially type IIb.

The objectives of this study were 1) to histologically validate the hippocampal subfield volumetry based on T2-weighted MRI, and 2) to explore its clinical impact on postsurgical memory function and seizure outcome in temporal lobe epilepsy (TLE) with hippocampal sclerosis (HS). We analyzed the cases of 24 patients with medial TLE (12 left, 12 right) and HS who were preoperatively examined with T2-weighted high-resolution MRI. The volume of each hippocampal subfield was calculated with an automatic segmentation of hippocampal subfields (ASHS) program. Hippocampal sclerosis patterns were determined pathologically, and the cross-sectional area and neuronal cell density of the CA1 and CA4 subfields were calculated using tissue specimens. Pre- and postoperative memory evaluations based on the Wechsler Memory Scale-Revised (WMS-R) were performed. We compared the presurgical MRI-based volumes with the pathological measurements in each subfield and then compared them with the change in the patients' neurocognitive function. As a result, there was a significant relationship between the presurgical MRI-based volume of CA4/dentate gyrus (DG) and the cross-sectional area of CA4 calculated with tissue specimens (Spearman's rs = 0.482, p = 0.023), and a similar trend-level correlation was observed in CA1 (rs = 0.455, p = 0.058). Some of MRI-based or pathology-based parameters in the subfields preliminarily showed relationships with the postsurgical memory changes. In conclusion, automated subfield volumetry for patients with hippocampal sclerosis moderately reflects their subfield atrophy and might be useful to predict the postsurgical change of memory function in these patients.

Significance Multiple sclerosis (MS) can transition from a relapsing-remitting form (RRMS) into a chronic progressive form (secondary progressive MS, SPMS). SPMS pathogenesis remains poorly understood with diagnosis based entirely on retrospective clinical monitoring. We show that T helper cells expressing the transcription factor Eomes (Eomes+ Th cells) were significantly increased in peripheral blood from SPMS patients versus healthy subjects or RRMS and other progressive MS patients. Moreover, Eomes+ Th cells expressing granzyme B were found infiltrating brain tissues in SPMS autopsy samples, providing support for a pathogenic role. Analysis of clinical status on follow-up indicated the value of measuring Eomes+ Th cells for SPMS diagnosis and prognostic monitoring. This study may contribute to future development of biomarker-assisted “precision medicine” for MS., Multiple sclerosis (MS), a putative autoimmune disease of the central nervous system (CNS), commonly presents as relapsing-remitting MS (RRMS), characterized by recurrent episodes of peripheral disabling symptoms resulting from inflammatory CNS damage. Many RRMS patients transition to a chronic disease course with progressive neurological dysfunctions (secondary progressive MS, SPMS), with the progression rate varying between patients and over time. SPMS pathogenesis is now linked to immune-cell–mediated processes, although the mechanisms driving SPMS transition and progression remain elusive, and SPMS lacks biomarkers and effective treatments. We report the crucial involvement of cytotoxic CD4+ T cells expressing Eomes (Eomes+ Th cells) in SPMS pathogenesis—a Th cell subset previously identified in a mouse model of late/chronic autoimmune CNS inflammation. Few Eomes+ Th cells circulate in RRMS patient peripheral blood (n = 44), primary progressive MS (PPMS) patients (n = 25), or healthy controls (n = 42), but Eomes+ Th cells were significantly increased in SPMS (n = 105, P < 0.0001). Strikingly, lymphocytes isolated from SPMS autopsy brain samples revealed CD4+ T cells infiltrating CNS that coexpressed Eomes and the cytotoxic molecule granzyme B. In particular, the Eomes+ Th cell levels were increased in SPMS patients in progressive disease phases versus SPMS patients without current disability increases (P < 0.0001). Moreover, Eomes level acted as a biomarker to predict SPMS patients at risk of disease worsening with over 80% accuracy (ROC-AUC = 0.8276). Overall, our results indicate that granzyme B-expressing Eomes+ T helper cells are involved in the pathogenesis of SPMS, with significant implications for SPMS biomarkers and therapeutic targets.

Accurate histological diagnosis and molecular testing using a sufficient tumor sample of advanced lung cancer, especially non-small cell lung cancer (NSCLC), are crucial for precision medicine. The aim of this study was to assess the feasibility and safety of surgical biopsy for intrathoracic lesions, and, in addition, overall survival after surgical biopsy. One hundred-one patients who underwent surgical biopsy for intrathoracic lesions of lung cancer at our hospital between 2011 and 2019 were retrospectively reviewed. Their clinical and pathologic records were reviewed. In addition to evaluating the oncologic safety of the surgical biopsy, the overall survival based on the biopsy results was estimated. The total number of surgical sites of the 101 patients was 131, and common biopsy sites were the lungs (82, 62.6%) followed by hilar/mediastinal lymph nodes (27, 20.6%). There were 13 postoperative complications (12.9%) without surgery-related deaths. The median time from surgical biopsy to the initiation of treatment was 27 days. Appropriate amounts of specimens for diagnosis and molecular testing were obtained from all patients (100%). When limited to treatment-naive patients with stage IV adenocarcinoma, patients treated with tyrosine kinase inhibitors (TKIs) or immune checkpoint inhibitors (ICIs) based on molecular testing had a better prognosis. Surgical biopsy for intrathoracic lesions of lung cancer may be a safe and effective method to make a definitive diagnosis, including companion diagnostics for advancing precision therapy in selected patients with inoperable advanced NSCLC.

Background: Despite the importance of psychosis as a comorbidity of temporal lobe epilepsy (TLE), the underlying neural mechanisms are still unclear. We aimed to investigate abnormalities specific to psychosis in TLE, using diffusion MRI parameters and graph-theoretical network analysis. Material and Methods: We recruited 49 patients with TLE (20 with and 29 without interictal schizophrenia-like psychosis) and 42 age-/gender-matched healthy controls. We performed 3-tesla MRI scans including 3D T1-weighted imaging and diffusion tensor imaging in all participants. Among the three groups, fractional anisotropy (FA), mean diffusivity (MD), and global network metrics were compared by analyses of covariance. Regional connectivity strength was compared by network-based statistics. Results: Compared to controls, TLE patients showed significant temporal and extra-temporal changes in FA, and MD, which were more severe and widespread in patients with than without psychosis. We observed distinct differences between TLE patients with and without psychosis in the anterior thalamic radiation (ATR), inferior fronto-occipital fasciculus (IFOF), and inferior longitudinal fasciculus (ILF). Similarly, for network metrics, global, and local efficiency and increased path length were significantly reduced in TLE patients compared to controls, but with more severe changes in TLE with psychosis than without psychosis. Network-based statistics detected significant differences between TLE with and without psychosis mainly involving the left limbic and prefrontal areas. Conclusion: TLE patients with interictal schizophrenia-like psychosis showed more widespread and severe white matter impairment, involving the ATR, IFOF and ILF, as well as disrupted network connectivity, particularly in the left limbic and prefrontal cortex, than patients without psychosis.

Background and purpose Recent studies suggest that the autoantibodies against adrenergic/muscarinic receptors might be one of the causes and potential markers of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). The purpose of this study was to investigate the structural network changes related to autoantibody titers against adrenergic/muscarinic receptors in ME/CFS by performing a single-subject gray matter similarity-based structural network analysis. Methods We prospectively examined 89 consecutive right-handed ME/CFS patients who underwent both brain MRI including 3D T1-wighted images and a blood analysis of autoantibodies titers against β1 adrenergic receptor (β1 AdR-Ab), β2 AdR-Ab, M3 acetylcholine receptor (M3 AchR-Ab), and M4 AchR-Ab. Single-subject gray matter similarity-based structural networks were extracted from segmented gray matter images for each patient. We calculated local network properties (betweenness centrality, clustering coefficient, and characteristic path length) and global network properties (normalized path length λ, normalized clustering coefficient γ, and small-world network value δ). We investigated the correlations between the autoantibody titers and regional gray matter/white matter volumes, the local network properties, and the global network properties. Results Betweenness centrality showed a significant positive correlation with β1-AdR-Ab in the right dorsolateral prefrontal cortex. The characteristic path length showed a significant negative correlation with β2-AdR-Ab in the right precentral gyrus. There were no significant correlations between the antibody titers and the regional gray matter/white matter volumes, and the global network properties. Conclusions Our findings suggest that β1 AdR-Ab and β2 AdR-Ab are potential markers of ME/CFS.

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder caused by a CAG nucleotide repeat expansion in atrophin 1. A previous report described cerebellar white matter lesions on magnetic resonance imaging (MRI) in elderly-onset DRPLA patients, but this finding has not been fully investigated in a total population of DRPLA patients, including juvenile or early-adult onset patients. Herein, we attempted to determine the frequency, distribution pattern, and features of the cerebellar white matter lesions in 30 consecutive DRPLA patients. We also assessed the relationships between the cerebellar white matter lesions and clinical parameters and other MRI findings. The cerebellar white matter lesions were found in 43% of the 30 DRPLA patients, and in 70% of the late adult-onset DRPLA patients. In approx. Two-thirds of the patients with cerebellar white matter lesions, the lesions were localized in the paravermal area (paravermal lesions). Multiple logistic regression analyses revealed that the Fazekas grade of 'cerebral' white matter lesions was independently associated with 'cerebellar' white matter lesions. In conclusion, cerebellar white matter lesions are one of the distinctive MRI features in DRPLA patients, especially in patients with older age at onset. Cerebellar white matter lesions, as well as cerebral white matter lesions, might originate from the disease process of DRPLA itself, and they often have a characteristic distribution of paravermal lesions.

Background Chemoradiotherapy (CRT) is the standard treatment for c-stage IIIB non-small cell lung cancer (NSCLC); however, patients who respond to CRT are at risk of developing fatal complications such as massive hemoptysis or infection. In such cases, surgery is an alternative option. Currently, there are limited reports on surgery for complications arising during definitive CRT for locally advanced NSCLC. We report a case of hemoptysis after definitive CRT for c-T4N2M0 stage IIIB NSCLC that was successfully treated with lower bilobectomy combined with left atrial resection. Case presentation A 72-year-old man with c-T4N2M0 stage IIIB NSCLC with left atrial invasion developed hemoptysis during CRT, which was discontinued to control hemoptysis. Chest computed tomography revealed a regressed and cavitated tumor. Three weeks after discontinuation of CRT, surgery was performed to avoid fatal complications and secure radicality. We performed lower bilobectomy combined with partial left atrial resection, which was performed using an automatic tri-stapler. The bronchial stump was covered with an omental flap. The resected specimen pathologically showed complete response with fistula between the intermediate bronchus and necrotic cavity in the tumor. His postoperative course was uneventful, and the patient was disease free at 10 months after surgery. Conclusions We successfully performed surgery after definitive CRT in a patient with c-T4N2M0 stage IIIB NSCLC. Partial left atrial resection was safely performed with an automatic tri-stapler. A complete pathological response to CRT was achieved. In a case with a chance of complete (R0) resection, when the risk of developing fatal complications might outweigh the risk of post-CRT surgery perioperative complications, surgery should be considered as a treatment option.

BACKGROUND Diffusional kurtosis imaging (DKI) and neurite orientation dispersion and density imaging (NODDI) metrics provide more specific information regarding pathological changes than diffusion tensor imaging (DTI). PURPOSE To detect microstructural abnormalities in myalgic encephalomyelitis (ME) / chronic fatigue syndrome (CFS) patients by using DKI and NODDI metrics. STUDY TYPE Prospective. POPULATION Twenty ME/CFS patients and 23 healthy controls were recruited. FIELD STRENGTH/SEQUENCE Three-b value DWI (b-values = 0, 1000, and 2000 sec/mm2 ) and 3D T1 -weighted images were at 3.0T. ASSESSMENT Mean kurtosis (MK), neurite density index (NDI), orientation dispersion index (ODI), fractional anisotropy (FA), and mean diffusivity (MD) were calculated. STATISTICAL TESTING The two-sample t-test analysis in SPM12 software was used to compare the differences between ME/CFS and control groups. RESULTS In the ME/CFS patients, we observed significant FA decreases in the genu of the corpus callosum and the anterior limb of the right internal capsule (P

Background Despite recent advances in diffusion MRI (dMRI), there is still limited information on neurite orientation dispersion and density imaging (NODDI) in temporal lobe epilepsy (TLE). This study aimed to demonstrate neurite density and dispersion in TLE with and without hippocampal sclerosis (HS) using whole-brain voxel-wise analyses. Material and methods We recruited 33 patients with unilateral TLE (16 left, 17 right), including 14 patients with HS (TLE-HS) and 19 MRI-negative 18F-fluorodeoxyglucose positron emission tomography (FDG-PET)-positive patients (MRI-/PET+ TLE). The NODDI toolbox calculated the intracellular volume fraction (ICVF) and orientation dispersion index (ODI). Conventional dMRI metrics, that is, fractional anisotropy (FA) and mean diffusivity (MD), were also estimated. After spatial normalization, all dMRI parameters (ICVF, ODI, FA, and MD) of the patients were compared with those of age- and sex-matched healthy controls using Statistical Parametric Mapping 12 (SPM12). As a complementary analysis, we added an atlas-based region of interest (ROI) analysis of relevant white matter tracts using tract-based spatial statistics. Results We found decreased neurite density mainly in the ipsilateral temporal areas of both right and left TLE, with the right TLE showing more severe and widespread abnormalities. In addition, etiology-specific analyses revealed a localized reduction in ICVF (i.e., neurite density) in the ipsilateral temporal pole in MRI-/PET+ TLE, whereas TLE-HS presented greater abnormalities, including FA and MD, in addition to a localized hippocampal reduction in ODI. The results of the atlas-based ROI analysis were consistent with the results of the SPM12 analysis. Conclusion NODDI may provide clinically relevant information as well as novel insights into the field of TLE. Particularly, in MRI-/PET+ TLE, neurite density imaging may have higher sensitivity than other dMRI parameters. The results may also contribute to better understanding of the pathophysiology of TLE with HS., Highlights • We examined temporal lobe epilepsy (TLE) with or without hippocampal sclerosis (HS). • Neurite orientation dispersion and density imaging (NODDI) was used. • Ipsilateral reduction of neurite density was found in MRI-negative PET-positive TLE. • More extensive abnormalities were presented in TLE with HS. • NODDI may provide clinical relevance and novel insights into the field of TLE.

The aim of the current study was to investigate the postoperative changes of high-signal lesions at the surgical margin on serial T2WI/FLAIR images in non-neoplastic patients. Seventy-one postoperative MR images in 27 patients (17 focal cortical dysplasia and 10 hippocampal sclerosis) who underwent surgery for intractable epilepsy were evaluated. T2WI/FLAIR-high-signal lesions in size and shape were visually assessed using a 3-point grading system. Associations of postoperative seizures or electroencephalography(EEG)spikes with advancing grade were also evaluated. As a result, follow-up showed enlarged T2WI/FLAIR-high-signal lesions in 6 of 27 patients (22%). The presence of post-operative seizures or EEG spikes was significantly associated with enlargement of the T2WI/FLAIR-high-signal lesion. Enlargement of the T2WI/FLAIR-high-signal lesion was observed in some non-neoplastic patients, and this finding might be correlated with remnant epileptogenesis.

Objective Previous studies have demonstrated structural brain network abnormalities in patients with temporal lobe epilepsy (TLE) using cortical thickness or gray matter (GM) volume. However, no studies have applied single-subject GM network analysis. Here, we first applied an analysis of similarity-based single-subject GM networks to individual patients with TLE. Materials and methods We recruited 51 patients with TLE and unilateral hippocampal sclerosis (22 left, 29 right TLE) and 51 age- and gender- matched healthy controls. Single-subject structural networks were extracted from three-dimensional T1-weighted magnetic resonance images for each subject. In this method, nodes were defined as small cortical regions and edges representing connecting regions that have high statistical similarity. The constructed graphs were analyzed using the graph theoretical approach. The following global and local network properties were calculated: betweenness centrality, clustering coefficient, and characteristic path length. In addition, small world properties (normalized path length λ, normalized clustering coefficient γ, and small-world network value σ) were obtained and compared with those for the controls. Results Although the small-world configurations were retained, impaired global clustering coefficient was observed in left and right TLE. At a regional level, patients with left TLE showed a widespread decrease of the clustering coefficient beyond the ipsilateral temporal lobe and a decreased characteristic path length in the ipsilateral temporal pole. On the other hand, patients with right TLE showed a localized decrease of the clustering coefficient in the ipsilateral temporal lobe. Conclusions Our findings suggest that global and local network properties disrupted and moved toward randomized networks in TLE patients in comparison to controls. This network alteration was more extensive in left TLE than in right TLE patients. Single-subject GM networks may contribute to a better understanding of the pathophysiology of TLE.

Objective The main objective of this study was to determine the ability of double inversion recovery (DIR) data coupled with machine-learning algorithms to distinguish normal individuals from epileptic subjects and to identify the laterality of the focus side in MRI-negative, PET-positive temporal lobe epilepsy (TLE) patients. Materials and methods We used whole-brain DIR data as the input features with which to train a linear support-vector machine model in 63 participants who underwent high-resolution structural MRI and DIR scans. The subjects included 20 left TLE patients, 19 right TLE patients, and 24 healthy controls (HCs). Results Using the DIR data, we achieved a robust accuracy of 87.30% for discriminating among the left TLE, right TLE, and HC groups as well as 84.61%, 97.72%, and 93.02% prediction accuracies for distinguishing left TLE from right TLE, HC from right TLE, and HC from left TLE, respectively. Interpretation Our experimental results suggest that DIR data coupled with machine-learning algorithms provide a promising approach to identifying MRI-negative TLE patients, especially when fluorodeoxyglucose-PET is not available.

We report the case of a 49-year-old patient who developed brain, sternal, and spine metastases almost simultaneously after the radical resection of a yp-T4N0M0 pulmonary pleomorphic carcinoma of the right upper lobe following induction chemotherapy. The left occipital brain metastasis was surgically removed and followed by radiation therapy. The sternal and vertebral metastases were treated with radiation therapy. Concurrently, the immune checkpoint inhibitor nivolumab was administered. After 12 cycles of nivolumab, the two bone metastases were well-controlled. However, the brain metastasis recurred and was surgically removed again. We were able to investigate the tumor-infiltrating lymphocytes in brain metastases resected before and after radio-immunotherapy. The results revealed the increased number of CD8- and CD68-positive cells after the combined therapy compared with before the therapy. In addition, the high-level expression of program death-ligand 1 was maintained in the brain metastasis.

Parkinson’s disease (PD) is caused by a selective degeneration of dopamine neurons. The relationship between dopamine transporter (DAT) density and gray matter volume has been unclear. Here we investigated the voxelwise correlation between gray matter volume and DAT binding measured by 123I-N-ω-fluoropropyl-2β-carboxymethoxy-3β-(4-iodophenyl)nortropane (123I-FP-CIT) single-photon emission computed tomography (SPECT; DaTscan™ imaging) in PD. Thirty-one male patients with PD were examined with MRI and DaTscan. To measure nigrostriatal dopaminergic degeneration in PD, the specific binding ratio (SBR) of the striatum was obtained by DaTscan. Voxel-based morphometry (VBM) of 3D T1-weighted images was used to evaluate the relationships between the regional gray matter volume and the SBR in the striatum. There were significant positive correlations between the SBR and the gray matter volume in the right pulvinar and posterior middle temporal gyrus and a trend level in the left pulvinar, all of which are associated with the second visual pathway. The nigrostriatal dopaminergic degeneration might affect the secondary visual pathway, leading to visual dysfunctions in PD.

Neuronal intranuclear inclusion disease is a neurodegenerative disorder pathologically characterized by eosinophilic hyaline intranuclear inclusions. A high-intensity signal along the corticomedullary junction on DWI has been described as a specific MR imaging finding of the cerebrum in neuronal intranuclear inclusion disease. However, MR imaging findings of the cerebellum in neuronal intranuclear inclusion disease have not been fully evaluated. Here, we review MR imaging findings of the cerebellum in a series of 8 patients with pathologically confirmed neuronal intranuclear inclusion disease. The MR imaging results showed cerebellar atrophy (8/8 patients) and high-intensity signal on FLAIR images in the medial part of the cerebellar hemisphere right beside the vermis (the “paravermal area”) (6/8) and in the middle cerebellar peduncle (4/8). The paravermal abnormal signals had a characteristic distribution, and they could be an indicator of the diagnosis of neuronal intranuclear inclusion disease even when using the results of past MR imaging examinations in which DWI findings were not examined.

A dural arteriovenous fistula (DAVF) with spinal perimedullary venous drainage can cause progressive myelopathy, and it is sometimes incorrectly diagnosed as another spinal cord disease. Here we report the cases of three individuals with a DAVF (one craniocervical junction DAVF and two tentorial DAVFs) with progressive myelopathy showing unique magnetic resonance (MR) imaging findings. MR T2*WI or susceptibility-weighted imaging (SWI) demonstrated symmetrical dark signal intensity lesions predominantly in the dorsal aspect of medulla and the central gray matter of cervical spinal cord that showed the "black butterfly" silhouette. Cerebral angiography revealed DAVFs draining into anterior and posterior spinal veins. Dark signals on T2*WI and SWI were presumed to be hemorrhages, which were probably caused by prolonged venous congestion. Identifying this "black butterfly" sign can facilitate the diagnosis of DAVF, differentiating DAVF from other spinal cord diseases such as demyelinating lesions and neoplasms.

Background: White matter hyperintensities (WMH) in the cholinergic pathways are associated with cognitive performance in Alzheimer's disease. This study aimed to evaluate the relationship between the volume reduction of cholinergic pathways and cognitive function in patients with multiple sclerosis (MS). Methods: Thirty-two MS patients underwent a brain MRI and cognitive measurements including the Mini-Mental State Examination (MMSE) and the Japanese version of the Montreal Cognitive Assessment (MoCA-J). The extent of WMH within the cholinergic pathways was assessed using the Cholinergic Pathways Hyperintensities Scale (CHIPS). Computerized WMH volumes were also obtained. FreeSurfer was used to measure regional volumes including the cortical and subcortical volumes. The correlations among the CHIPS, the WMH volume, and the clinical data were assessed, in addition to the correlations between the cognitive scores and regional volumes measured by FreeSurfer. Results: The CHIPS score and the WMH volume were strongly positively correlated with each other (r=0.87, P

Objective Dementia with Lewy bodies (DLB) is often cited as the second most common dementia after Alzheimer’s disease (AD). It is clinically important to distinguish DLB from AD because specific side effects of antipsychotic drugs are limited to DLB. The relative preservation of cingulate glucose metabolism in the posterior cingulate gyri versus that in the precuni, known as the cingulate island sign (CIS), in patients with DLB compared with AD is supposed to be highly specific for diagnosing DLB. In a previous study, using brain perfusion SPECT, the largest value (0.873) for the area under the receiver operating characteristic (ROC) curve (AUC) for differentiating DLB from AD was obtained with the ratio of the posterior cingulate gyri from an early Alzheimer’s disease-specific hypoperfusion volume of interest (VOI) versus the medial occipital lobe. Two purposes of this study are as follows: one is optimization of VOI setting for calculating CIS values and the other is to evaluate their accuracy and simultaneously to retest the method described in our previous paper. Methods We conducted a retest of this SPECT method with another cohort of 13 patients with DLB and 13 patients with AD. Furthermore, we optimized VOIs using contrast images obtained from group comparisons of DLB and normal controls; the same 18 patients with DLB and 18 normal controls examined in our previous study. We obtained DLB-specific VOIs from areas where brain perfusion was significantly decreased in DLB. As the numerators of these ratios, early Alzheimer’s disease-specific VOIs were used after subtracting DLB-specific VOIs. The DLB-specific VOIs were used as the denominator. Results In retest, the obtained AUC was 0.858 and the accuracy, sensitivity, and specificity were 84.6, 84.6, and 84.6%, respectively. The ROC curve analysis with these optimized VOIs yielded a higher AUC of 0.882; and the accuracy, sensitivity, and specificity of these new CIS ratios were 84.6, 92.3, and 76.9%, respectively, with a threshold value of 0.281. Conclusion Optimized CISs using brain perfusion SPECT are clinically useful for differentiating DLB from AD.

Gerstmann-Straussler-Scheinker syndrome caused by the P102L mutation in the prion protein gene (GSS102) is usually characterized by the onset of slowly progressive cerebellar ataxia, with dementia occurring much later. Because of the relatively long disease course and the prominence of progressive cerebellar ataxia in the early stage, GSS102 is often misdiagnosed as other neurodegenerative disorders. We present two cases of genetically proven GSS102L, both of which present with atrophy and decreased blood flow of the thalamus as determined by voxel-based specific regional analysis system for Alzheimer's disease (VSRAD) advance software and easy Z-score analysis for 99mTc-ethyl cysteinate dimer-SPECT, respectively. These thalamic abnormalities have not been fully evaluated to date, and detecting them might be useful for differentiating GSS102 from other neurodegenerative disorders.

Static encephalopathy of childhood with neurodegeneration in adulthood (SENDA) or β-propeller protein-associated neurodegeneration (BPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA), causing abnormal iron accumulation in the globus pallidus and substantia nigra through autophagic dysfunction due to WDR45 mutations.1,2 SENDA/BPAN is often difficult to diagnose because brain MRI does not detect the characteristic findings in childhood. We found transient hyperintensity and swelling in the globus pallidus and substantia nigra due to infections during childhood as characteristic findings.

PurposeThe pathophysiology of idiopathic generalized epilepsy (IGE) is still unclear, but graph theory may help to understand it. Here, we examined the graph-theoretical findings of the gray matter network in IGE using anatomical covariance methods.Materials and methodsWe recruited 33 patients with IGE and 35 age- and sex-matched healthy controls. Gray matter images were obtained by 3.0-T 3D T1-weighted MRI and were normalized using the voxel-based morphometry tools of Statistical Parametric Mapping 12. The normalized images were subjected to graph-theoretical group comparison using the Graph Analysis Toolbox with two different parcellation schemes. Initially, we used the Automated Anatomical Labeling template, whereas the Hammers Adult atlas was used for the second analysis.ResultsThe resilience analyses revealed significantly reduced resilience of the IGE gray matter networks to both random failure and targeted attack. No significant between-group differences were found in global network measures, including the clustering coefficient and characteristic path length. The IGE group showed several changes in regional clustering, including an increase mainly in wide areas of the bilateral frontal lobes. The second analysis with another region of interest (ROI) parcellation generated the same results in resilience and global network measures, but the regional clustering results differed between the two parcellation schemes.ConclusionThese results may reflect the potentially weak network organization in IGE. Our findings contribute to the accumulation of knowledge on IGE.

Epilepsy is a diverse brain disorder, and the pathophysiology of its various forms and comorbidities is largely unknown. A recent machine learning method enables us to estimate an individual’s “brain-age” from MRI; this brain-age prediction is expected as a novel individual biomarker of neuropsychiatric disorders. The aims of this study were to estimate the brain-age for various categories of epilepsy and to evaluate clinical discrimination by brain-age for (1) the effect of psychosis on temporal lobe epilepsy (TLE), (2) psychogenic nonepileptic seizures (PNESs) from MRI-negative epilepsies, and (3) progressive myoclonic epilepsy (PME) from juvenile myoclonic epilepsy (JME). In total, 1196 T1-weighted MRI scans from healthy controls (HCs) were used to build a brain-age prediction model with support vector regression. Using the model, we calculated the brain-predicted age difference (brain-PAD: predicted age—chronological age) of the HCs and 318 patients with epilepsy. We compared the brain-PAD values based on the research questions. As a result, all categories of patients except for extra-temporal lobe focal epilepsy showed a significant increase in brain-PAD. TLE with hippocampal sclerosis presented a significantly higher brain-PAD than several other categories. The mean brain-PAD in TLE with inter-ictal psychosis was 10.9 years, which was significantly higher than TLE without psychosis (5.3 years). PNES showed a comparable mean brain-PAD (10.6 years) to that of epilepsy patients. PME had a higher brain-PAD than JME (22.0 vs. 9.3 years). In conclusion, neuroimaging-based brain-age prediction can provide novel insight into or clinical usefulness for the diverse symptoms of epilepsy.

This study aimed to quantitatively compare the signals from double inversion recovery (DIR) and fluid-attenuated inversion recovery (FLAIR) in temporal lobe epilepsy (TLE) with a focus on anterior temporal lobe white matter abnormal signal (ATLAS) lesions. We recruited 59 patients with TLE (32 left, 27 right) and 24 healthy controls (HCs). All patients underwent 3T-MRI scans including 3D DIR and FLAIR images, and the images were normalized and compared among the three groups by the software program SPM 12. We also explored the association of the ATLAS with disease duration, seizure types, and the existence of hippocampal sclerosis (HS). As a result, compared to the HCs, there were significantly increased DIR signals in the ipsilateral anterior temporal white matter of both the left and right TLE patients. There was no significant signal difference in FLAIR images between the HCs and patients except for a trend-level increase in left TLE. There was also no significant association between the ATLAS and disease duration, seizure type, or HS. These results quantitatively confirmed the significant signal increases of DIR in the ipsilateral anterior temporal lobe in both left and right TLE, whereas FLAIR revealed no significant between-group differences. These findings may indicate greater usefulness of DIR compared to FLAIR for detecting ATLAS lesions.

Graph theory is an emerging method to investigate brain networks. Altered cerebral blood flow (CBF) has frequently been reported in temporal lobe epilepsy (TLE), but graph theoretical findings of CBF are poorly understood. Here, we explored graph theoretical networks of CBF in TLE using arterial spin labeling imaging. We recruited patients with TLE and unilateral hippocampal sclerosis (HS) (19 patients with left TLE, and 21 with right TLE) and 20 gender- and age-matched healthy control subjects. We obtained all participants' CBF maps using pseudo-continuous arterial spin labeling and analyzed them using the Graph Analysis Toolbox (GAT) software program. As a result, compared to the controls, the patients with left TLE showed a significantly low clustering coefficient (p=0.024), local efficiency (p=0.001), global efficiency (p=0.010), and high transitivity (p=0.015), whereas the patients with right TLE showed significantly high assortativity (p=0.046) and transitivity (p=0.011). The group with right TLE also had high characteristic path length values (p=0.085), low global efficiency (p=0.078), and low resilience to targeted attack (p=0.101) at a trend level. Lower normalized clustering coefficient (p=0.081) in the left TLE and higher normalized characteristic path length (p=0.089) in the right TLE were found also at a trend level. Both the patients with left and right TLE showed significantly decreased clustering in similar areas, i.e., the cingulate gyri, precuneus, and occipital lobe. Our findings revealed differing left-right network metrics in which an inefficient CBF network in left TLE and vulnerability to irritation in right TLE are suggested. The left-right common finding of regional decreased clustering might reflect impaired default-mode networks in TLE.

Introduction In temporal lobe epilepsy (TLE), an uncoupling of cerebral blood flow (CBF) and metabolism is indicated. The aim of this study was to clarify this relationship by investigating intraventricular brain temperature (BT) and CBF in TLE using a noninvasive MRI technique. Material and methods We recruited 38 subjects with left TLE and 39 subjects with right TLE. All patients underwent an MRI examination including diffusion-weighted imaging (DWI) and pseudo-continuous arterial spin labeling (pCASL). Intraventricular BT was calculated by a DWI thermometric technique, and regional CBF was obtained from pCASL images, and we analyzed the relationship between them using the statistical parametric mapping 8 software (SPM8). Results In only the left TLE group, there was a significant negative correlation between CBF and BT in the left inferior frontal gyrus. Positive correlations in the bilateral posterior poles were also found at the trend level in left TLE group. The patients with right TLE showed no significant correlation. Conclusion Our results may reflect a regional uncoupling of blood flow and metabolism in those brain areas, and they may indicate that TLE has different metabolic regulation systems in the brain.

Background and purpose Automated subfield volumetry of hippocampus is desirable for use in temporal lobe epilepsy (TLE), but its utility has not been established. Automatic segmentation of hippocampal subfields (ASHS) and the new version of FreeSurfer software (ver.6.0) using high-resolution T2-weighted MR imaging are candidates for this volumetry. The aim of this study was to evaluate hippocampal subfields in TLE patients using ASHS as well as the old and new versions of FreeSurfer. Materials and methods We recruited 50 consecutive unilateral TLE patients including 25 with hippocampal sclerosis (TLE-HS) and 25 without obvious etiology (TLE-nonHS). All patients and 45 healthy controls underwent high-resolution T2-weighted and 3D-volume T1-weighted MRI scanning. We analyzed all of their MR images by FreeSurfer ver.5.3, ver.6.0 and ASHS. For each subfield, normalized z-scores were calculated and compared among groups. Results In TLE-HS groups, ASHS and FreeSurfer ver.6.0 revealed maximal z-scores in ipsilateral cornu ammonis (CA) 1, CA4 and dentate gyrus (DG), whereas in FreeSurfer ver.5.3 ipsilateral subiculum showed maximal z-scores. In TLE-nonHS group, there was no significant volume reduction by either ASHS or FreeSurfer. Conclusions ASHS and the new version of FreeSurfer may have an advantage in compatibility with existing histopathological knowledge in TLE patients with HS compared to the old version of FreeSurfer (ver.5.3), although further investigations with pathological findings and/or surgical outcomes are desirable., Highlights • Automated hippocampal subfield analysis is needed in temporal lobe epilepsy (TLE). • Automatic segmentation hippocampal subfields (ASHS) and FreeSurfer are available. • 50 TLE patients (25 with hippocampal sclerosis (HS)) were analyzed. • ASHS and new version of FreeSurfer may have an advantage in compatibility. • High-resolution T2-weighted images might help better automatic segmentation in TLE.

In this paper, we assessed the reliability of glucose metabolic brain data for identifying lateralization of magnetic resonance imaging (MRI)-negative temporal lobe epilepsy (TLE) patients. We designed and developed an efficacious and automatic metabolic-wise lateralization framework. The proposed lateralization framework comprises three main systematic levels. In the first stage of our investigation, we pre-processed interictal fluorodeoxyglucose positron emission tomography images to extract glucose metabolic brain data. In the second stage, we used a voxel selection method involving a feature-ranking strategy to select the most discriminative metabolic voxels. Finally, we used a support vector machine followed by a 10-fold cross-validation strategy to assess the proposed lateralization framework in 27 patients with right MRI-negative TLE and 29 patients with left MRI-negative TLE. The proposed lateralization framework achieved an excellent accuracy of 96.43 % concordance with experienced PET interpreter. Thus, we show that pattern analysis of glucose metabolic brain data can accurately lateralize MRI-negative TLE patients in the clinical setting.

Background and purpose Chorea-acanthocytosis is clinically difficult to distinguish from Huntington's disease because these disorders have similar symptoms and MR imaging findings. We evaluated the usefulness of single-case voxel-based morphometry (VBM) analysis for differentiating the two diseases as well as VBM analysis. Materials and methods We examined five genetically proven chorea-acanthocytosis patients and 11 Huntington's disease patients to detect differences in the gray and white matter atrophic pattern by using single-case VBM analysis in each patient and their clinical findings. We also evaluated VBM analysis for a group comparison in both disease and control groups. Results The single-case VBM analysis results demonstrated a gray matter volume loss in caudate nucleus in all 16 patients. A characteristic symmetrical white matter volume loss was detected in globus pallidus, putamen, and thalamus on both sides in all the chorea-acanthocytosis patients, but this pattern of atrophy was not seen in any of the Huntington's disease patients. With the VBM analysis, a significant gray matter volume loss was noted in caudate nucleus on both sides in chorea-acanthocytosis patients compared with Huntington's disease patients, and a more extensive white matter volume loss around the basal ganglia and thalamus was observed in chorea-acanthocytosis patients compared to Huntington's disease patients, consistent with the single-case VBM analysis results. Genetic testing identified two novel pathogenic mutations, exon 1 c.16_22delGTGGTCG and exon 55 c.7736-7739delGAGA in a chorea-acanthocytosis patient. Conclusions Single-case VBM analysis may be useful to differentiate chorea-acanthocytosis from Huntington's disease with a focus on white matter atrophy.

A 62-year-old woman was admitted with a 20 mm diameter tumor in the left upper lobe of the lung. Preoperative computed tomography (CT) revealed a displaced anomalous B1+2 arising from the left main bronchus. In addition, an accessory fissure was detected between the apicoposterior (S1+2) and anterior (S3 ) segments. As the lesion was entirely contained in the S1+2, we performed video-assisted thoracic surgical S 1+2 segmentectomy with systematic lymph node dissection. During the operation, we easily detected and successfully divided the displaced B1+2 located behind the left main pulmonary artery. The pathological diagnosis was invasive adenocarcinoma with T1bN0M0 (TNM 8th edition). Histologically, neither lymphatic invasion nor vascular invasion was detected in the invasive area of the tumor. If regional lymph node dissection with appropriate intraoperative histologic examination by frozen section could be made, segmentectomy may be an acceptable, optional procedure with curative intent for cancer patients with such an anomaly.

Cerebellar dysmaturation and injury is associated with a wide range of neuromotor, neurocognitive and behavioral disorders as well as with preterm birth. We used diffusion tensor MR imaging to investigate a disruption in structural cortico-ponto-cerebellar (CPC) connectivity in children with infantile-onset severe epilepsy.We performed CPC tract reconstructions in 24 hemimegalencephaly (HME) patients, 28 West syndrome (WS) of unknown etiology patients, and 25 pediatric disease control subjects without a history of epilepsy nor brain abnormality on MRI. To identify the CPC tract, we placed a seeding ROI separately in each right and left cerebral peduncle. We evaluated the distribution patterns of the CPC tracts to the cerebellum and their correlation with clinical findings.In control and WS of unknown etiology groups, both sides' CPC tracts descended to bilateral hemispheres in 20 (80.0%) and 21 (75.0%); mixed (bilateral on one side and unilateral on the other side) in five (20.0%) and five (17.9%); and unilateral in zero (0.0%) and two (7.1%), respectively. However, in the HME, both sides' CPC tracts descended to bilateral hemispheres in four (16.7%); mixed in 13 (54.1%); and unilateral in seven (29.2%). These CPC patterns differed significantly between the HME and other groups (p 0.001). Among HME patients, those with a unilateral cerebellar distribution on both sides had significantly earlier seizure onset (p = 0.049) and more frequent seizures (p = 0.052) at a trend level compared to those with bilateral and mixed distributions.Disrupted CPC tracts were observed more frequently in HME patients than in WS of unknown etiology patients and controls, and they may be correlated with earlier seizure onset and more frequent seizures in HME patients. DTI is a useful and non-invasive method for speculating the pathology in the developing brain.

Dentatorubral–pallidoluysian atrophy (DRPLA) is an autosomal dominant spinocerebellar ataxia caused by CAG triplet expansion in atrophin 1 and is frequently associated with cerebral white matter lesions. To elucidate the clinical features of elderly onset DRPLA and the key radiological findings for differentiating DRPLA from physiological white matter lesions in healthy elderly subjects, we reviewed the clinical and magnetic resonance imaging (MRI) features of ten patients with elderly onset genetically confirmed DRPLA (> 60 years) and compared their MRI findings with those of age- and sex-matched ten healthy subjects with asymptomatic cerebral white matter lesions. The initial symptom was cerebellar ataxia in all DRPLA patients, and five of them did not have any symptoms other than ataxia at the time of MRI examination. Atrophy of the brainstem, superior cerebellar peduncle, and cerebellum was detected in all DRPLA patients and none of the healthy subjects. Abnormal signals in the brainstem (inferior olive, pons, and midbrain), thalamus, and cerebellar white matter were frequently observed in elderly onset DRPLA patients but not in healthy subjects. In conclusion, elderly onset DRPLA presents as cerebellar ataxia alone in the early stage of disease. Atrophy of the brainstem, superior cerebellar peduncle, and cerebellum and abnormal signals in the brainstem, cerebellum, and thalamus are key findings for differentiating elderly onset DRPLA from asymptomatic cerebral white matter lesions in healthy subjects.

This study aimed to investigate abnormalities in structural covariance network constructed from gray matter volume in myotonic dystrophy type 1 (DM1) patients by using graph theoretical analysis for further clarification of the underlying mechanisms of central nervous system involvement. Twenty-eight DM1 patients (4 childhood onset, 10 juvenile onset, 14 adult onset), excluding three cases from 31 consecutive patients who underwent magnetic resonance imaging in a certain period, and 28 age- and sex- matched healthy control subjects were included in this study. The normalized gray matter images of both groups were subjected to voxel based morphometry (VBM) and Graph Analysis Toolbox for graph theoretical analysis. VBM revealed extensive gray matter atrophy in DM1 patients, including cortical and subcortical structures. On graph theoretical analysis, there were no significant differences between DM1 and control groups in terms of the global measures of connectivity. Betweenness centrality was increased in several regions including the left fusiform gyrus, whereas it was decreased in the right striatum. The absence of significant differences between the groups in global network measurements on graph theoretical analysis is consistent with the fact that the general cognitive function is preserved in DM1 patients. In DM1 patients, increased connectivity in the left fusiform gyrus and decreased connectivity in the right striatum might be associated with impairment in face perception and theory of mind, and schizotypal-paranoid personality traits, respectively.

Introduction In Parkinson's disease (PD) patients, magnetic resonance (MR) imaging studies using phase difference enhanced imaging (PADRE) and susceptibility-weighted imaging (SWI) showed the obscuration of the boundary between the crural fibers and substantia nigra, and the absence of dorsolateral nigral hyperintensity, respectively. PADRE images have not been evaluated in other types of neurodegenerative parkinsonism, and PADRE and SWI images have not been compared. Here we evaluated PADRE and SWI images in patients with progressive supranuclear palsy (PSP), multiple system atrophy (MSA), or PD and controls, and we compared the diagnostic values. Methods PADRE and SWI-like MR images were visually assessed focusing on the substantia nigra in 39 PD patients, eight with PSP, 13 with MSA, and 34 normal controls. Results The obscuration of the boundary between the crural fibers and substantia nigra on PADRE was observed in: the PD group, 62%; PSP, 100%; MSA, 60%, and controls, 19%. The overall collect classification for neurodegenerative parkinsonism was 74%. The absence of dorsolateral nigral hyperintensity on SWI-like images was present in: PD, 97%; PSP, 100%; MSA, 67%; and controls, 6%, resulting in the overall correct classification of 96%. Conclusions The MR feature on PADRE was observed not only in PD but also in other neurodegenerative parkinsonism, especially in PSP with high sensitivity. The finding in substantia nigra on SWI had greater discrimination power than that of PADRE in neurodegenerative parkinsonism, especially in PD.

PURPOSE The purpose of the present retrospective study was to evaluate the sensitivity of susceptibility-weighted imaging (SWI) compared to conventional spin-echo T2-weighted and T2*-weighted images in detecting iron deposition in the motor cortex of amyotrophic lateral sclerosis (ALS) patients in comparison with age-matched normal controls. We also investigated the etiology of the low signal referring to the pathology of one autopsy case. METHODS This retrospective magnetic resonance (MR) study included 23 ALS patients and 28 age-matched normal controls. The signal intensity of the motor cortex was scored by SWI, conventional T2-weighted images and T2*-weighted images. A postmortem study of one patient was also performed. RESULTS On SWI, there was a significant difference between the precentral cortical signal intensity scores in the ALS patients and the controls (P < .0001). The total scores of signal intensities of the precentral cortex were positively correlated with age in the normal controls (r = .494), but no correlation was observed in the ALS patients. The postmortem study showed intensely stained microglias and macrophages after antiferritin antibody staining in the precentral cortices. CONCLUSIONS Decreased signal intensity of the motor cortex on SWI may serve a useful role in ALS diagnoses, particularly in young patients. MR images were also helpful for speculating on the etiology of ALS.

The objective of this study was to compare the diagnostic accuracy of dual-time-point 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) to single-time-point 18F-FDG PET for staging of preoperative lung cancer. Between November 2008 and December 2009, 107 patients who were diagnosed as having lung cancer or strongly suspected of having lung cancer were enrolled. They underwent dual-time-point 18F-FDG PET following conventional imaging. Dual-time-point 18F-FDG PET imaging (whole body) was performed at 1-h (early) post-FDG injection and repeated (2 h delayed) after injection. The diagnostic accuracy of pre-PET staging and post-PET staging was retrospectively evaluated, and the diagnostic accuracy of dual-time-point 18F-FDG PET was compared to that of single-time-point 18F-FDG PET. In 100 patients, the early 18F-FDG PET scan resulted in upstaging of the tumor in ten (10 %) and down-staging of the tumor in five (5 %) compared to the conventional scan. The delayed phase of 18F-FDG PET provided no additional information on staging for lung cancer patients. The remaining seven patients were diagnosed as not having lung cancer. This study confirmed that dual-time-point 18F-FDG PET is useful for differential diagnosis between benign and malignant lesions, but has no major impact on staging and therapeutic management of patients with pathologically proven lung cancer.

BACKGROUND AND PURPOSE Amygdala enlargement (AE) has been reported as an epileptogenic focus in subtypes of temporal lobe epilepsy (TLE). The purpose of this study was to investigate the clinical, morphological, and pathological characteristics of AE. METHODS We retrospectively reviewed the clinical data and imaging findings of 23 TLE patients with ipsilateral AE. We performed morphological MR analyses using FreeSurfer and voxelbased morphometry (VBM) in 14 of the 23 patients and in 20 controls whose images were obtained by a 3.0-Tesla MRI. A pathological study was also performed in 2 patients who underwent operations. RESULTS All patients became seizure free or shSowed dramatic improvement by medical therapy except for two. They received operations and their pathology revealed that both patients had cortical dysplasia in from the amygdala to the ipsilateral temporal pole. The FreeSurfer analysis showed a significant difference in the amygdala volumes between the affected and nonaffected sides. VBM revealed significant increases of gray matter volumes of the temporal pole on the side of AE in seven of the 14 patients with AE (50%). CONCLUSIONS Cortical dysplasia may be one of the pathological diagnoses in AE, and in some patients it may extend to the temporal pole.

Background: Despite the increasing use of arterial spin labeling (ASL) in patients with epilepsy, little is known about its brain regional distribution pattern, including diaschisis, and its correspondence with FDG-PET. Here, we investigated the regional match and mismatch between FDG-PET and ASL in temporal lobe epilepsy (TLE). Methods: We recruited 27 patients with unilateral TLE, who underwent inter-ictal ASL and FDG-PET scans. These images were spatially normalized using Statistical Parametric Mapping 12, and the regional values in both ASL and FDG-PET were calculated using PMOD software within 20 volumes of interest (VOIs), including the temporal lobe, adjacent cortices, subcortical structures, and cerebellum. ASL images of 37 healthy controls were also analyzed and compared. Results: Whereas, ASL showed significant side differences, mainly in the temporal and frontal lobes, the significant abnormalities in FDG-PET were more widespread and included the insula and supramarginal gyrus. Ipsilateral thalamic reduction was found in FDG-PET only. The detectability of the focus side compared with the contralateral side was generally higher in FDG-PET. The discriminative values in ASL compared with healthy controls were higher in temporal neocortex and amygdala VOIs. Conclusions: There are similar and differing regional distributions between FDG-PET and ASL in TLE, possibly reflecting regional match and mismatch of cerebral blood flow and metabolism. At this stage, it seems that ASL couldn't present comparable clinical usefulness with FDG-PET. These findings deepen our knowledge of ASL imaging and are potentially useful for its further application.

It is implicated that diabetic patients are more resistant to aspirin therapy than patients with other diseases or healthy individuals. We evaluated the inhibitory effects of aspirin on aggregation and the cyclooxygenase activity of platelets of 10 patients with severe type-2 diabetes mellitis (DM) and compared the results with those of healthy individuals. Although platelet aggregation had a tendency to be more resistant to aspirin with the DM group, there was no significant difference in half maximal inhibitory concentration 50 values of aspirin on the cyclooxygenase activity between the patients with DM and healthy individuals. Thus, the residual platelet aggregability uninhibited by aspirin appears to be independent of the cyclooxygenase activity. Since adenosine diphosphate (ADP) receptor blocking almost completely inhibited the residual platelet aggregability, it is suggested that hyperreactivity to ADP is more prevalent in patients with DM.