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Catheter ablation is an important non-pharmacological intervention for atrial fibrillation (AF), but its effect on the incidence of asymptomatic cerebral emboli and long-term effects on cognitive function remain unknown. We prospectively enrolled 101 patients who underwent AF ablation. Brain magnetic resonance imaging (MRI) (72 patients) and neuropsychological assessments (66 patients) were performed 1–3 days (baseline) and 6 months after ablation. Immediately after ablation, diffusion-weighted MRI and 3-dimensional double inversion recovery (3D-DIR) detected embolic microinfarctions in 63 patients (87.5%) and 62 patients (86.1%), respectively. After 6 months, DIR lesions disappeared in 41 patients. Microbleeds (MBs) increased by 17%, and 65% of the de novo MBs were exactly at the same location as the microinfarctions. Average Mini-Mental State Examination scores improved from 27.9 ± 2.4 to 28.5 ± 1.7 (p = 0.037), and detailed neuropsychological assessment scores showed improvement in memory, constructional, and frontal lobe functions. Ejection fraction, left atrial volume index and brain natriuretic peptide level improved from baseline to 3–6 months after ablation. Despite incidental microemboli, cognitive function was preserved 6 months after ablation.

Calcified amorphous tumor (CAT) is a non-neoplastic tumor composed of calcified nodules consisting of amorphous fibrous material, and it may eventually cause cerebral infarction (CI). We experienced a 67-year-old woman with CAT who had recurrent CI. After excision of the CAT, the CI did not show recurrence. A review of previous papers on CI due to CAT in Pubmed revealed that 7 of 13 studies originated in Japan and that CI can occur even with small CAT. Surgical treatment is recommended to prevent CI recurrence, especially when CAT is accompanied by mitral annular calcification or has marked mobility.

Introduction Several reports have shown that neuromelanin-sensitive magnetic resonance imaging (NMI) using 3T magnetic resonance imaging is useful for the differential diagnosis of Parkinson's disease (PD), progressive supranuclear palsy (PSP), and other neurological diseases. However, the number of cases in previous studies has been insufficient. We aimed to determine the relationship between NMI and severity of PD and related disorders, and thereby establish the diagnostic utility of NMI for diagnosing neurological diseases. Methods We enrolled 591 patients (531 subjects after removal of duplicates) with parkinsonism who underwent NMI. The contrast ratio of the locus coeruleus (LC-CR) and the area of the substantia nigra pars compacta (SNc) were analyzed in each patient. Results The patients’ clinical diagnoses were as follows: 11 patients in the disease control group (DCG), 244 patients with PD, 49 patients with PSP, and 19 patients with multiple system atrophy with predominant parkinsonism. Additionally, some patients were diagnosed with dementia with Lewy bodies, vascular parkinsonism, and drug-induced parkinsonism. SNc in the patients with PD and PSP was significantly smaller than that in DCG. LC-CR in the patients with PD was lower than that in DCG; furthermore, LC-CR in the patients with PD was significantly lower than that in the patients with PSP. We found that an area under the receiver-operating characteristic curve, indicating diagnostic efficacy, of 0.85 for LC-CR is a promising biomarker for differentiating PD from PSP. Conclusion NMI effectively contributes to differentiating neurodegenerative diseases, such as PD and PSP.

Background Miyazaki syndrome is overshunting-associated myelopathy, which is a rare complication of ventriculoperitoneal shunt. We present the first case of Miyazaki syndrome caused by cystoperitoneal (CP) shunt for an arachnoid cyst (AC) in this report. Case Description We report a case of a 42-year-old man with 12-year progressive spastic paraplegia, who underwent CP shunt for an AC at the age of 15 years. Although few findings suggested overshunting on symptoms and head computed tomography, contrast-enhanced magnetic resonance imaging revealed the engorgement of the cervical spinal epidural venous plexus compressing the spinal cord. Shunt valve replacement with a pressure-adjustable valve was performed. Postoperatively, the cervical cord compression by the enlarged spinal epidural venous plexus was completely improved, but, possibly due to delayed diagnosis and treatment, the patient's symptoms only partially improved. Conclusions When patients with a history of any kind of shunt surgery develop myelopathy, Miyazaki syndrome should be suspected and, for early diagnosis, cervical and/or contrast-enhanced magnetic resonance imaging should be performed.

Cortical superficial siderosis (cSS) is a rare condition that is regarded as a potential magnetic resonance marker of cerebral amyloid angiopathy (CAA). We describe the case of a 68-year-old man with cSS and Parkinson's disease (PD) who subsequently exhibited incidental microhemorrhages, which were only detected on magnetic resonance imaging (MRI), at one week after deep brain stimulation (DBS) surgery. cSS is now considered to be a significant risk factor for CAA and future bleeding. Therefore, because DBS surgery is invasive and may increase the risk of intracerebral hemorrhage, the procedure should be performed carefully when managing patients with PD and CAA.

Background: Some patients with Parkinson’s disease (PD) develop peri-lead brain edema after deep brain stimulation (DBS) surgery. The influence of edema on neurological function is not well characterized. We investigated the relationship of brain edema after DBS surgery with motor and cognitive function. Methods: Thirteen patients with PD (6 males and 7 females; mean age: 61.2 years) who underwent bilateral subthalamic nucleus (STN) DBS surgery were included. All patients underwent magnetic resonance imaging (MRI) examination on day 6 post-DBS surgery. The volume of edema was measured either in the frontal white matter or STN on fluid attenuated inversion recovery (FLAIR) images. We examined the relationship between these volumes and changes in cognitive and motor function. Results: Patients were divided into those with frontal subcortical edema (FE) ≥3,000 mm 3 (FE+ group; n = 7) and

Article, Annals of clinical and translational neurology. 6(12): 2595-2600 (2019)

Low-loss passive components for RF signals compatible with the on-wafer process are essential for realizing integrated circuits with high-frequency and high-power operations. We successfully fabricate thick-metal-film-based coplanar waveguides (CPWs) and inductors (INDs) by directly bonding a 17- $\mu \text{m}$ -thick Al foil to a sapphire (0001) substrate and wet etching. The surface-activated bonding (SAB) technologies at room temperature are used. RF characteristics of the foil-based passive components are compared with those of components made of 1- $\mu \text{m}$ -thick evaporated Al layers. We obtain a better insertion loss and a higher ${Q}$ -factor for foil-based CPWs and INDs, respectively. The measured characteristics are compared with those obtained by an analysis based on the equivalent circuit scheme. Impacts of side etching of foils and surface oxidation on their characteristics are observed. Characteristics of virtual components made of 1- $\mu \text{m}$ -thick Al foils, i.e., 1- $\mu \text{m}$ -thick Al films with the same resistivity as that of foils, are analytically investigated.

Background: Since the advent of magnetic resonance imaging technology, cerebral microbleeds can be diagnosed in vivo. However, the underlying mechanism of cerebral microbleed formation is not fully understood. Objectives: This study aimed to identify the factors associated with cerebral microbleeds after carotid artery stenting (CAS). Method: We retrospectively examined 125 patients who underwent CAS for carotid stenosis. Cerebral microbleeds were investigated using T2*-weighted gradient-echo (GRE) imaging before and after CAS. We analyzed the possible association of new microbleeds with the following risk factors: the number of baseline microbleeds and ischemic cerebral lesions, the occurrence of cerebral hyperperfusion syndrome, and new ischemic cerebral lesions after CAS. Results: Baseline cerebral microbleeds were detected in 53 patients (42.4%). New cerebral microbleeds after CAS were observed in 13 of 125 patients (10.4%) and were exclusively associated with new ischemic lesions but not with other risk factors. No patient showed a merged image of a new cerebral microbleed on GRE imaging or a new ischemic lesion on diffusion-weighted imaging. Lobar and deep microbleeds were noted in 12/13 (92.3%) and 1 patient (7.7%), respectively. Of 12 patients with new microbleeds, 10 (76.9%) and 2 (15.4%) had a new microbleed in the ipsilateral and contralateral hemispheres, respectively. Conclusions: We found that new cerebral microbleeds developed after CAS and that these might be associated with new ischemic lesions, mostly in the territory of the treated carotid artery. We speculate that these microbleeds result from the deoxygenation of hemoglobin in the embolus or, alternatively, small hemorrhagic transformation of ischemic lesions.

Article, Annals of clinical and translational neurology. 6(5): 974-978 (2019)

ObjectiveThe severity of cerebral small vessel disease (SVD) is assessed through neuroimaging findings, including hypertensive arteriopathy (HA)-SVD and cerebral amyloid angiopathy (CAA)-SVD. HA-SVD and CAA-SVD have been collectively estimated as total scores: the HA-SVD and CAA-SVD scores, respectively. Previous reports suggest that HA-SVD scores are associated with cognitive function; however, the relationship between CAA-SVD scores and cognitive function remains unclear. Therefore, we examined the association between CAA-SVD scores and cognitive function. Furthermore, we developed a modified CAA-SVD score considering cortical microinfarcts and posterior dominant white matter hyperintensities, which are imaging findings of CAA, and examined the association between these scores and cognitive function in the same patient group.DesignProspective study.SettingSingle centre study from a memory clinic.ParticipantsSubjects were diagnosed with mild cognitive impairment (MCI) or mild dementia in our memory clinic between February 2017 and July 2019 and underwent clinical dementia rating scale and brain MRI assessment. A total of 42 patients (aged 75.3±9.12 years) were registered prospectively.Primary and secondary outcome measuresWe evaluated intellectual function, memory, frontal lobe function and constructional ability. Furthermore, the relationship between each score and cognitive function was examined.ResultsThe CAA-SVD score showed significant associations with cognitive function (R2=0.63, p=0.016), but the HA-SVD score did not (R2=0.41, p=0.35). The modified CAA-SVD score was also significantly associated with cognitive function (R2=0.65, p=0.008).ConclusionCognitive function is associated with the CAA-SVD score, and more efficiently with the modified CAA-SVD score, in memory clinic patients. Although we have not validated the weighting of the modified CAA-SVD score, these scores can be a predictor of cognitive deterioration in patients with MCI and mild dementia.

Even with postmortem pathological examination, only limited information is provided of the foci of in vivo clinical information. Cerebral small vessel disease, which is associated with ageing, dementia and stroke, highlights the difficulty in arriving at a definitive diagnosis of the lesions detected on in vivo radiological examination. We performed a radiological−pathological comparative study using ex vivo MRI to examine small cerebral lesions. Four patients with small vessel disease lesions detected on in vivo MRI were studied. Exact pathological findings of in vivo MRI-detected lesions were revealed. The ischaemic lesion after 17 days from onset showed positivity for peroxiredoxin, cluster of differentiation 204 and glial fibrillary acidic protein, indicating sterile inflammation and neuroprotective reaction. Cortical microinfarcts beneath the cortical superficial siderosis were associated with inflammation from the superficial layer in a patient with cerebral amyloid angiopathy; in this patient, a bilinear track-like appearance of the cortical superficial siderosis on the ex vivo MRI was compatible with iron deposition on the pia matter and within cortical layers II–III. An in vivo MRI-detected cerebral microbleed was revealed to be heterogeneous. An in vivo MRI-detected cerebral microbleed was revealed to be a venous angioma. Furthermore, a neuropathologically confirmed embolic cerebral microbleed was firstly detected using this method. Our results suggest that in vivo MRI-detected lobar cerebral microbleeds can be caused by non-cerebral amyloid angiopathy aetiologies, such as microembolism and venous angioma. Venous angioma and embolic microbleeds may mimic cerebral amyloid angiopathy markers on in vivo MRI. To clarify the clinical importance of these lesions, we should investigate their rate and frequency in a large cohort of healthy individuals and patients with cardiac risk factors. Thus, we provide evidence that ex vivo micro-MRI improves the clinical diagnosis of small vessel diseases., Graphical Abstract Graphical Abstract

Background: Hypertensive arteriopathy (HA) and cerebral amyloid angiopathy (CAA) may contribute to the development of mixed cerebral microbleeds (CMBs). Recently, the total small vessel disease (SVD) scores for HA and CAA were proposed, which are determined by a combination of MRI markers to reflect overall severity of these microangiopathies. Objective: We investigated whether or not total HA-SVD and CAA-SVD scores could be used to predict overlap of HA and CAA in patients with mixed CMBs. Methods: Fifty-three subjects with mixed CMBs were retrospectively analyzed. MRI markers (CMBs, lacunes, perivascular space, white matter hyperintensity [WMH] and cortical superficial siderosis [cSS]) were assessed. The HA-SVD score and CAA-SVD score were obtained for each subject. Anterior or posterior WMH was also assessed using the age-related white matter changes scale. Results: The two scores were positively correlated (ρ= 0.449, p

Background and purpose Cortical microinfarcts (CMIs) are frequently found in the brains of patients with advanced cerebral amyloid angiopathy (CAA) at autopsy. The small vessel disease (SVD) score for CAA (i.e., the CAA-SVD score) has been proposed to evaluate the severity of CAA-associated vasculopathic changes by a combination of magnetic resonance imaging (MRI) markers. The aim of this study was to examine the association between total CAA-SVD score and features of CMIs on in vivo 3-Tesla MRI. Methods Eighty patients with probable CAA were retrospectively analyzed. Lobar cerebral microbleeds, cortical superficial siderosis, enlargement of perivascular space in the centrum semiovale and white matter hyperintensity were collectively assessed, and the total CAA-SVD score was calculated. The presence of CMI was also examined. Results Of the 80 patients, 13 (16.25%) had CMIs. CMIs were detected more frequently in the parietal and occipital lobes. A positive correlation was found between total CAA-SVD score and prevalence of CMI (ρ = 0.943; p = 0.005). Total CAA-SVD score was significantly higher in patients with CMIs than in those without (p = 0.009). In a multivariable logistic regression analysis, the presence of CMIs was significantly associated with total CAA-SVD score (odds ratio 2.318 [95% confidence interval 1.228-4.376]; p = 0.01, per each additional point). Conclusions The presence of CMIs with a high CAA-SVD score could be an indicator of more severe amyloid-associated vasculopathic changes in patients with probable CAA.

Corticobasal syndrome (CBS) is characterized by unilateral atrophy of the brain. New diagnostic criteria for CBS include intermediate somatosensory dysfunction. Here, we aimed to carefully examine intermediate somatosensory function to identify tests which can assess impairment in CBS patients. Using voxel-based morphometry (VBM), we also aimed to show the anatomical bases of these impairments. Subjects included 14 patients diagnosed with CBS and 14 patients with Parkinson's disease (PD). Patients were evaluated using intermediate somatosensory tests and neuropsychological assessments. VBM was used to analyze differences in gray matter volumes between CBS and PD patients. In the PD group, no tests showed a significant difference between the dominant-side onset and the non-dominant-side onset. In the CBS group, all tests showed worse scores on the affected side. For detecting intermediate somatosensory dysfunction in CBS, two tests are recommended: tactile object naming and 2-point discrimination. VBM analysis showed that the volume of the left post- and pre-central gyrus, and both sides of the supplementary motor area were significantly decreased in the CBS group compared to the PD group. Although CBS remains untreatable, early and correct diagnosis is possible by performing close examination of intermediate somatosensory function.

An 85-year-old woman diagnosed with amyotrophic lateral sclerosis died of pneumonia and was autopsied. Magnetic resonance imaging (MRI) performed 16 days before death revealed an intracortical high-intensity lesion in her right temporal cortex on three-dimensional (3D)-double inversion recovery (DIR) and 3D-fluid-attenuated inversion recovery (FLAIR) images. Histopathological examination indicated a cortical microinfarct (CMI) juxtaposed to cerebral amyloid angiopathy. Recently, in vivo detection of CMIs using 3D-DIR and 3D-FLAIR on 3-tesla MRI has been reported, and postmortem MRI study confirmed the presence of CMIs. This is the first case study to compare CMI findings detected upon premortem MRI to the CMI itself discovered upon postmortem neuropathological examination.

Purpose Istradefylline is useful in treating the wearing-off state in Parkinson’s disease (PD). We investigated the effectiveness of istradefylline (ISD) in improving arousal, sleep, and gait deficits in patients with PD. Methods We examined 14 patients with PD treated with ISD. We assessed the patients using the Unified Parkinson's Disease Rating Scale, Parkinson's Disease Questionnaire, Timed Up-and-Go test (TUG), Freezing of Gait Questionnaire (FOG-Q), Epworth Sleepiness Scale (ESS), and Parkinson’s Disease Sleep Scale (PDSS) before and 1 month after ISD use. Results ESS scores were significantly lower 1 month after the start of ISD treatment (6.79 ± 6.50) than before the intervention (8.14 ± 6.15, Wilcoxon signed-rank test, p = 0.0033). PDSS scores were not significantly different 1 month after beginning the treatment (112 ± 23 mm) when compared to those before the intervention (110 ± 27 mm, Wilcoxon signed-rank test, p = 0.40). TUG scores were not changed after 1 month of ISD use (14.9 ± 8.3 s) when compared to those before the intervention (21.3 ± 30.0 s, Wilcoxon signed-rank test, p = 0.59). Although these measures were not significantly affected by ISD treatment, some patients remarkably improved after the treatment. FOG-Q scores were significantly lower 1 month after the beginning of treatment (9.79 ± 7.16) than those before the intervention (12.14 ± 5.82, Wilcoxon signed-rank test, p = 0.030). Conclusions ISD may improve daytime sleepiness and FOG in patients with PD.

Long-term intake of potential zinc-chelating drugs may cause zinc deficiency. We postulated that zinc deficiency in Parkinson’s disease (PD) patients was related to the intake of drugs such as levodopa. We investigated the relationship between zinc levels and levodopa administration period, dosage, and symptoms of zinc deficiency in PD patients. We measured serum zinc levels and analyzed correlations between serum zinc levels, the levodopa oral administration period, dosage, dosing frequency, and zinc deficiency symptoms including taste disorders. Data analyses were performed using Spearman’s rank correlation coefficient. The mean serum zinc level was 60.5 ± 11.6 μg/dL. The mean administration period for levodopa was 8.0 ± 5.5 years, mean administration frequency 3.4 ± 0.9 times/d, and mean administration dose 420.6 ± 237.1 mg/d. Negative correlations between zinc levels and levodopa dosage and dosing frequency were found. Multiple regression analysis showed a significant correlation with the frequency of levodopa (β = −0.360, p = 0.007). No significant change in clinical symptoms was observed after zinc administration, but anxiety tended to improve. Our results indicated that frequent levodopa administration strongly influenced serum zinc levels which may have alleviating effects on psychiatric symptoms, therefore, preventing zinc deficiency can be important during PD treatment.

Objective When patients take neuroleptics, the distinction between Parkinson's disease (PD) and drug-induced parkinsonism (DIP) based solely on clinical features can become difficult. At present, 123I-FP-CIT SPECT (DAT-SPECT) and 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy are widely used to supplement the differential diagnosis of parkinsonism. This study assessed the clinical symptoms and neurological findings in the patients suspected of having DIP based on DAT-SPECT findings. Methods Twenty-three patients (11 men, 12 women, age: 52-81 years old) presenting with DIP were recruited. All patients underwent neurological examinations, including brain magnetic resonance imaging and DAT-SPECT. Patients who showed abnormal DAT-SPECT results underwent MIBG myocardial scintigraphy. Results Eleven patients showed a reduction in the ligand uptake on DAT-SPECT (DAT-positive group), and nine of these patients showed a low delayed heart-to-mediastinum (H/M) ratio on MIBG myocardial scintigraphy. The remaining 12 patients showed normal results on DAT-SPECT (DAT-negative group). All patients in the DAT-positive group had asymmetric motor symptoms, whereas only 4 in the DAT-negative group exhibited this clinical feature (p=0.001). A detailed medical history showed that 7 of the 11 patients in the DAT-positive group had prodromal symptoms for PD. However, only 1 patient in the DAT-negative group exhibited these symptoms (p= 0.009). Although two patients in the DAT-negative group showed poor improvement, they showed a normal H/M ratio on MIBG and no response to levodopa. Conclusion The patients in the DAT-positive group might have prodromal symptoms that were worsened by neuroleptic drugs. The results of detailed history-taking and neurological findings seem to indicate cases of compromised dopaminergic transmission before the administration of neuroleptic drugs.

A 55-year-old man with no mental retardation had presented a history of frequent transient clumsiness of his right upper and lower extremities for about 20 years. He was admitted to a general hospital with weakness of right side of the body, and first-ever generalized seizure attack occurred the next day. Brain CT showed calcification in the left cerebral cortices. So he was referred to our hospital. On neurological examination, he had mild clumsiness of his right upper limb and right pyramidal tract sign. He had neither facial port-wine stain nor glaucoma. The blood test and cerebrospinal fluid analysis were unremarkable. Electroencephalogram showed slowing and reduction of activity at the left frontal and parietal areas with no epileptic activities. Brain CT showed "tram-track calcification" and lobar atrophy in the left fronto-parietal cortices. Susceptibility weighted imaging (SWI) on MRI revealed enlarged transmedullary veins in the left periventricular white matter and low intensity lesions along the cortical gyri. Post gadolinium fluid-attenuated inversion recovery imaging (FLAIR-Gd) showed leptomeningeal enhancement in the left fronto-parietal lobes more extensively than those by post gadolinium T1-weighted image. Brain perfusion single photon emission computed tomography with a technetium-99m-ethyl cysteinate dimer (99mTc-ECD SPECT) revealed hypoperfusion in the fronto-parietal lobes. These clinical and neuroimaging findings were compatible with type III Sturge-Weber syndrome (SWS). His condition was improved after treatment with oral levetiracetam (1,000 mg daily). Although adult-onset type III SWS is very rare, it is important to perform SWI and post-contrast FLAIR for assessing leptomeningeal angioma in patients with seizure with focal cortical calcification even if they have no facial nevus.

Tuberous sclerosis complex (TSC) 1 or TSC2 is mutated in most TSC patients. TSC2 mutations are more frequently associated with worse outcomes, earlier age at seizure onset, more severe intellectual disability, and higher tuber load than TSC1. The degree of white matter involvement is thought to be associated with the severity of neurological impairment. At present, genotype-phenotype correlations and relationship between tuber burden and neurological disability in TSC are debatable. We presented a 43-year-old patient with TSC2 mutation, whose symptom was only incomplete quadrantic visual field deficit in spite of multiple brain tubers. The visual field deficit was thought to be due to a small lesion in the upper medial part of the optic radiation revealed by diffusion tensor imaging. Her brain tubers showed normal findings in magnetic resonance spectroscopy. Our case suggested that neurological and neuropsychiatric manifestations of TSC are affected by the quality rather than number of the lesions. In addition, MRS may be useful to identify the correlation between brain tubers and neurological disability in TSC patients.

Neuro-Sweet disease (NSD) is defined as Sweet disease with central nervous system (CNS) involvement. Also known as acute febrile neutrophilic dermatosis, NSD is characterised by painful erythematous plaques and multisystem neutrophilic infiltration.1 Familial Mediterranean fever (FMF), characterised by recurrent fever and serositis, is caused by mutations of the Mediterranean fever ( MEFV ) gene. FMF is rarely reported in patients with Sweet’s syndrome.2 In this report, we discuss three cases of possible NSD. Our findings indicate that some mutations in the MEFV gene may act as additional susceptibility factors in autoimmune inflammatory neurological diseases, including NSD. We conducted MEFV mutation analyses on genomic DNA samples from three patients diagnosed with possible NSD between 1 April 2014 and 31 October 2016. Patients were diagnosed with NSD in accordance with previously described criteria.1 In all patients, infection, malignancy and other autoimmune diseases were clinically excluded. All patients provided written informed consent to participate in this study. MEFV gene analyses were performed via PCR (see online supplementary file 1). ### Supplementary file 1 [SP1.pdf] ### Patient 1 A 62-year-old man presented with headache and difficulty understanding spoken language in June 2014, following which he experienced a generalised seizure and was transferred to our hospital. Dark-red, mottled erythema was observed on the surface of his skin. High levels of protein and interleukin (IL)-6 were observed in the cerebrospinal fluid (CSF). MRI revealed a tumefactive lesion in the left temporo-occipital region on T1, gadolinium-enhanced, fluid-attenuated inversion recovery (FLAIR) sequences (figure 1A,B). Brain biopsy of the lesion demonstrated perivascular inflammation (figure 1C), while skin biopsy revealed mild …

Background: Cortical microinfarctions (CMI) are often detected in patients with cerebral amyloid angiopathy (CAA) and cerebral microembolism. Purpose: To evaluate whether the size, location and distribution of CMIs differ between CAA and microembolism on 3T MRI. Methods: We retrospectively screened 657 patients who consulted our memory clinic from Jan 2011 to Mar 2018 and underwent detection protocol for CMIs (3-dimensional double inversion recovery (DIR), 3-dimensional fluid attenuated inversion recovery (FLAIR) and susceptibility-weighted imaging (SWI) on 3T MRI). There were 120 patients which had DIR-positive and SWI-negative lesion less than 10 mm in diameter. These patients were classified to embolic stroke group and CAA group which fulfilled the modified Boston criteria for possible or probable CAA. We analyzed the size, location and distribution of small cortical lesions on each group. Result: We enrolled 44 patients as embolic stroke group and 27 patients as CAA group. A total of 175 small cortical lesions were identified in the embolic stroke group and 63 in the CAA group. Mean number of small cortical lesions in the embolic stroke group was significantly higher than those of the CAA group (4.0 vs 2.3; p = 0.001). In the CAA group, the frequency of cortical lesions less than 5mm and localized in the cortical gray matter; ie. CMI, was higher than in the embolic stroke group (92% vs 69%; p < 0.001). By contrast, the frequency of cortical lesions larger than 5mm was significantly higher in the embolic stroke group than in the CAA group (21% vs 8%; p = 0.004). Furthermore, the frequency of cortical lesions involving subcortical area was significantly higher in the embolic stroke group than in the CAA group (30% vs 8%; p < 0.001). Regarding their distribution, the CAA group showed occipital lobe dominance compared to the embolic stroke group (30% vs 10%; p < 0.01). Conclusion: Our results suggest that CMIs caused by CAA tend to be less numerous, localized in the cortical gray matter and smaller than 5mm, and show occipital dominance compared to those by microembolism. These characteristics may be helpful for differentiating CMIs due to CAA from those caused by microembolism.

Objectives Levodopa–carbidopa intestinal gel (LCIG) infusion is a useful therapy for the wearing‐off phenomenon of advanced Parkinson's disease (PD) patients. Recently, we found three PD patients that may have had a zinc deficiency after the LCIG infusion, possibly due to the zinc‐chelating action of levodopa. This study aims to evaluate changes in serum zinc levels in three patients that received LCIG treatment and to determine possible remedies for zinc deficiency during treatment. Materials and Methods We performed a prospective blood analysis of serum zinc levels before, when possible, and after LCIG treatment in our three PD patients. Results The serum zinc levels of the first patient before treatment and 4 months after beginning LCIG treatment were 69 and 58 μg/dl, respectively. For the second patient, serum zinc levels before treatment and two months after starting LCIG treatment were 87 and 46 μg/dl, respectively. The baseline serum zinc level for the third patient was not examined, but was 48 μg/dl 5 months after starting the LCIG infusion. Conclusions Levodopa–carbidopa intestinal gel infusion might have caused a zinc deficiency through levodopa zinc chelation. Zinc deficiency with LCIG infusion has not yet been reported, though preventing zinc deficiency may be an important factor in future LCIG treatment strategies.

Purpose Arterial spin-labeling (ASL) perfusion MRI, a noninvasive method of assessing cerebral blood flow, is becoming a diagnostic tool of epilepsy. This study was undertaken to evaluate the diagnostic validity of ASL in patients with status epilepticus (SE) in a periictal state. Method Twenty cases with SE were studied. Patients were imaged at a 3 T MRI including ASL and diffusion-weighted imaging (DWI), and were also examined using electroencephalography (EEG). The abnormal findings of ASL were compared with those obtained from DWI and EEG. Result Focal hyperperfusion was found in the cortical territory of 13 cases (65%). In 10 of those 13 cases, the ASL hyperperfusion region corresponded to DWI high intensity and EEG abnormality. Two cases showed hyperperfusion corresponding to EEG abnormalities in ASL despite the absence of high intensity in DWI. The remaining single case showed hyperperfusion in ASL despite the absence of high intensity in DWI and EEG abnormalities. Hyperperfusion in the subcortical territory was observed in the ipsilateral thalamus in three cases and in the contralateral cerebellum in one case. Conclusion Our results suggest that ASL is a useful tool to diagnose status epilepticus and localization of the epilepsy focus.

To evaluate brain parenchymal high-signal-intensity abnormalities within the drainage territory of developmental venous anomalies (DVAs) identified by susceptibility-weighted imaging (SWI) at 3 T. One hundred and thirty patients with 137 DVAs identified by SWI were retrospectively studied. 3D fluid-attenuated inversion recovery (FLAIR) images were reviewed for parenchymal high-signal-intensity abnormalities and SWI images were reviewed for hypointense foci (microhemorrhages or cavernous malformations) adjacent to DVAs. Patient age, the degree of underlying white matter disease, DVA location (supratentorial or infratentorial), and the presence or absence of hypointense foci were compared across DVAs with and without high-signal-intensity abnormalities. The correlation between patient age and the size of any high-signal-intensity abnormality was analyzed using linear regression. Forty-two of 137 DVAs (30.7 %) had high-signal-intensity abnormalities. An adjusted prevalence of 18/71 (25.4 %) was obtained after excluding patients with considerable underlying white matter disease. Only DVA location (supratentorial) was associated with the presence of high-signal-intensity abnormalities (p

Somatosensory functions are subdivided into two large groups: the elementary somatosensory functions, which consist of light touch, pain, thermal sensation, joint position sense and vibration sense, and the intermediate somatosensory functions, which include two-point discrimination, tactile localisation, weight, texture and shape perception. It is generally accepted that the functional localisation of somatosensory function is the postcentral gyrus, but some researchers suggested that the insula is also involved in somatosensory function.1 Insula is a multimodal area and has a major role as a convergence zone implicated in the coordination between internal and external information through emotional subjective awareness. However, the subregional specificity of somatosensory functions in the insula has not been fully elucidated.2 We experienced three cases of stroke restricted exactly to the subregions of the insula which showed heterogeneous somatosensory dysfunction. For these patients, we performed a close examination of the intermediate somatosensory function, and discussed the relation between the insula and intermediate somatosensory function. ### Patient 1 An 81-year-old, right-handed woman who had difficulty speaking in February 2017 was transferred to our hospital. MRI revealed a lesion in the right middle insula (figure 1A). The symptom improved at the time of hospital arrival and she underwent the best medical treatment. The symptom disappeared on the next day. Intelligence and elementary somatosensory functions were normal. The results of the intermediate somatosensory examinations showed that two-point discrimination, tactile localisation and graphesthesia were significantly impaired. Figure 1 MRI findings of the cases presented. (A) Patient 1 had a cortical lesion in the right middle insula. (B) Patient 2 had a cortical lesion in the …

Pigmented neurons in the substantia nigra pars compacta (SNc) and locus coeruleus (LC) show decreased numbers differentially in Parkinson's disease (PD) and multiple system atrophy (MSA). Recent reports have described that fast spin-echo T1-weighted magnetic resonance imaging (MRI) by a 3-tesla machine can visualize neuromelanin-related contrast of the noradrenergic and dopaminergic neurons respectively in the LC and the SNc. Using neuromelanin MRI at 3 T, we investigated possible alterations of these catecholaminergic neurons in 32 PD and 9 MSA patients, and compared the results with those of 23 normal volunteers. The contrast ratio of the LC and SNc was decreased in MSA and PD patients, most prominently in the LC in MSA patients. The contrast ratio of the SNc was correlated with the Hoehn-Yahr stage of PD and the severity of neuroradiological abnormalities in MSA. These results indicate a potential diagnostic value of neuromelanin MRI to distinguish MSA patients from normal and PD patients.

Neuromelanin-sensitive MR imaging (NMI) is an increasingly powerful tool for the diagnosis of Parkinson's disease (PD). This study was undertaken to evaluate longitudinal changes on NMI in PD patients.We examined longitudinal changes on NMI in 14 PD patients. The area and contrast ratio (CR) of the substantia nigra pars compacta (SNc) were comparatively analyzed.The total area and CR of the SNc upon follow-up NMI were significantly smaller than those on initial NMI (from 33.5±18.9 pixels and 6.35±2.86% to 21.5±16.7 pixels and 4.19±2.11%; Wilcoxon signed-rank test, p0.001 and p=0.022, respectively). The area and CR of the dominant side SNc upon initial NMI were significantly greater than those on follow-up NMI (from 15.3±9.1 pixels and 6.5±2.7% to 7.9±8.5 pixels and 3.7±2.9%; Wilcoxon signed-rank test, p=0.002 and p=0.007, respectively). On a case-by-case basis, the area of the SNc invariably decreased upon follow-up NMI in all patients. We further demonstrated that the total area and CR of the SNc negatively correlated with disease duration (Pearson correlation coefficient, r=-0.63, p0.001 and r=-0.41, p=0.031, respectively). In area analyses, our results demonstrated very high intraclass correlation coefficients for both intra- and inter-rater reliability.NMI is a useful and reliable tool for detecting neuropathological changes over time in PD patients.