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Neuroinfections and related diseases are a life‐threatening disease. The practical progression of the clinical management in these diseases in Japan was reviewed. The guidelines for the clinical management of bacterial meningitis in Japan were published in 2014 which were established based on the current knowledge of microbiological evidence in Japan. The guidelines for the clinical management of herpes simplex virus encephalitis (HSVE) in Japan were also published in 2017. Aciclovir should be initiated in all patients with suspected encephalitis. Polymerase chain reaction (PCR) for HSVE has high sensitivity and specificity and is the gold standard diagnostic tool. However, when the negative result of PCR is obtained at the acute stage with the highly suspicious patients with HSVE, our clinical guidelines recommend to continuing aciclovir and repeating PCR. The viral resistance to aciclovir has been reported. In the case of aciclovir‐nonresponding and treatment‐resistant patients, an alternative treatment with foscarnet is recommended. Autoimmune encephalitis (AIE) is considered one of the most common causes of noninfectious acute encephalitis. As a series of anti‐neuronal surface antibodies (NSAs) were found, the diagnostic approach to AIE has undergone a paradigm shift. Different techniques are currently available to detect NSAs: tissue‐based assays (TBAs) with rodent brain sections, cell‐based assays (CBAs), and assays for primary cultured neurons of rodent. The diagnosis of AIE in Japan could previously be delayed owing to limited access to specialized laboratory; the assay system used by TBAs and CBAs for AIE was established and started to use in Japan since 2019. [ABSTRACT FROM AUTHOR]

In recent years, global warming has become a worldwide problem. Carbon dioxide (CO2) is one of the primary substances causing global warming, and its emission should be reduced. A promising technology is the conversion of CO2 to carbon monoxide (CO) using plasma treatment, so that the CO itself or its conversion to methane can be used as fuel for combustion engines. In this article, a CO2 dissociation or reduction treatment system using an adsorbent and nonthermal plasma (NTP) flow is investigated. To improve the conversion efficiency, the conversion efficiency for Ar or He plasma is evaluated. In the physical adsorption process, ∼10% CO2 gas with a flow rate of 10 L/min is prepared, and then, it is introduced into the flow channel; CO2 is adsorbed by the adsorbent. After the adsorption process, the circulation flow channel is set, and then, the Ar or He NTP flow is generated with a blower. As a result, the adsorbed CO2 is desorbed with CO2 concentrations of 20% and 23% by the Ar NTP flow and the He NTP flow, respectively. The CO2 conversion efficiency reaches 21%, and the energy efficiencies reach 13% and 10% with plasma in the cases using the Ar and He NTP flows, respectively. The energy efficiency with regard to CO is better with the Ar plasma, but more atomic carbon can be generated using the He plasma. [ABSTRACT FROM AUTHOR]

Purpose: Acute retinal necrosis (ARN) is a severe necrotizing retinitis caused by varicella-zoster virus or herpes simplex virus (HSV) that often results in blindness. Occasionally, HSV-caused ARN develops after herpes simplex encephalitis (HSE). It remains unknown, however, when and how often ARN develops after HSE. To investigate the actual conditions of patients with ARN following HSE and the interval period between the prior HSE and the onset of ARN, a retrospective nationwide survey of the Japanese population was performed. Study design: Retrospective. Methods: Questionnaires were sent out to the neurology and ophthalmology departments of teaching hospitals in Japan. They inquired about HSE patients in neurology departments and ARN patients diagnosed with HSV in ophthalmology departments. The proportion of the HSV-ARN patients with a history of HSE and their interval periods were obtained from the questionnaires returned by the ophthalmology departments. Results: Neurology departments of 324 hospitals responded to the questionnaires (response proportion: 40.9%), and 53 HSE cases were reported. Ophthalmology departments of 535 hospitals responded the questionnaires (response proportion: 54.3%), and 67 HSV-ARN cases were reported. Among the 67 HSV-ARN cases, 16 (23.9%) had histories of prior HSE. Although the interval periods from the prior HSE to the onset of HSV-ARN varied among cases, nearly half developed HSV-ARN within 2 years. Conclusions: This nationwide survey of the Japanese population showed that HSV-ARN develops after HSE in higher frequency within 2 years. Neurologists and ophthalmologists should be aware that HSE survivors have a risk of contracting HSV-ARN. [ABSTRACT FROM AUTHOR]

The aim of this study was to assess the efficacy, safety, and concentration of meropenem in cerebrospinal fluid when meropenem (2 g every 8 h) was administered to Japanese adult patients with bacterial meningitis. Five Japanese patients (mean age 60.6 years [range 35–71]) were enrolled. Infection with Streptococcus pneumoniae (three patients), Streptococcus salivarius (one patient), and Staphylococcus aureus (one patient) was confirmed by cerebrospinal fluid culture. Meropenem (2 g every 8 h) was administered to all five patients. Treatment duration ranged from 14 to 28 days (mean 22.6 days). All the patients were successfully treated. The concentration of meropenem in cerebrospinal fluid ranged from 0.27 to 6.40 μg/ml up to 8.47 h and was over 1 μg/ml 3 h after starting meropenem infusion. In each patient, the present study confirmed for the first time that the concentration of meropenem in cerebrospinal fluid exceeded the minimal inhibitory concentration for these pathogens. Eleven clinical and laboratory adverse events considered to be related to meropenem were observed in all patients, but no serious adverse event and no discontinuance of treatment due to adverse events occurred. Thus meropenem appeared to be a well-tolerated and effective agent for Japanese adult patients with bacterial meningitis. 2 g every 8 h of meropenem was delivered to CSF and its concentration was exceed in MICs for the detected pathogens. [ABSTRACT FROM AUTHOR]

Background: Quantitative electroencephalogram (qEEG) changes in chronic hepatitis C patients treated with interferon-α (lFN-α) have previously been reported. However, whether lFN-α-induced depression is related to changes in qEEG during IFN-α treatment remains unclear. Method: Fifty chronic hepatitis C patients were enrolled and lFN-α was administered intramuscularly at 9 x 106 IU daily for the first 4 weeks and then 3 times a week for the next 20 weeks. Serial EEGs obtained before and at 4 weeks after treatment were assessed. The absolute power for each frequency band was determined using qEEG techniques. Differences in the rate of change in absolute power for each of 6 frequency bands (δ,ϑ1, ϑ2, α1, α2 and β) were assessed between patients with and without major depression using the Mann-whitney U test. When significant differences in the rate of change in absolute power for each frequency band were observed, differences in the rate of change were also assessed between patients with and without psychological complications using the Mann-Whitney U test. Results: Major depression due to psychological complications during IFN-α treatment was reported in 10 out of 50 patients. In the 01 band, the difference in the rate of change was demonstrated to be significant (p = 0.0036). Moreover, at the central, frontal, parietal, and temporal locations, the rates of change were also significantly different. Conclusion: In IFN-α-treated chronic hepatitis C patients who were diagnosed with major depression, qEEG changes were more obvious and widely distributed. [ABSTRACT FROM AUTHOR]

The objective of this multicenter cross-sectional study was to determine the prevalence of fatigue and factors contributing to it in a large sample of Japanese patients with Parkinson's disease (PD). We used the 16-item Parkinson Fatigue Scale (PFS-16), which was designed to assess fatigue exclusively associated with PD. We carried out this study using PFS-16, the Unified Parkinson's Disease Rating Scale, Zung's Self-Rating Depression Scale, Parkinson's Disease Sleep Scale (PDSS), and the PD quality of life (QOL) scale (PDQ-39) by interview using questionnaires and physical examination by neurologists in 361 nondemented PD patients. Fatigue (an average PFS score of 3.3 or greater) was revealed in 151 patients (41.8%). Multiple logistic regression analysis indicated that the significant independent variables related to the presence of fatigue were the scores of PDSS and PDQ-39. Depression score was not a significant contributing factor. Our study revealed that the prevalence of fatigue in Japanese PD patients is as high as that in Western countries, and that fatigue is a relatively independent symptom, although sleep disturbance may be associated with fatigue. Since fatigue is significantly related to QOL reduction, therapeutic interventions including treatment of sleep disturbance are important. © 2009 Movement Disorder Society [ABSTRACT FROM AUTHOR]

Abstract: A recent trial suggested that corticosteroid was beneficial in herpes simplex virus encephalitis (HSVE), but that precise role remains unclear. We assessed the differences of cerebrospinal fluid (CSF) cytokine changes between different outcomes and between patients with and without corticosteroid administration at the acute stage of HSVE. Interleukin (IL)-1β, IL-2, IL-6, IL-10, interferon (IFN)-γ, and tumor necrosis factor-α were measured in 56 serial CSFs taken from 20 adult HSVE patients. Their outcomes were poor in 7 and good in 13 patients, and corticosteroid was administered in 10. The differences in the initial and maximum cytokine values were assessed among the different outcomes. The decline rate of cytokine values between the initial and second CSF samples was also assessed between patients with and without corticosteroid. The initial IFN-γ and maximum IL-6 with a poor outcome were higher than those with a good outcome (p =0.019 for IFN-γ and p =0.013 for IL-6). The decline rate of IL-6 in patients with corticosteroid was higher than that without corticosteroid (p =0.034). The initial IFN-γ and maximum IL-6 CSF values represented prognostic biomarkers in HSVE. One pharmacological mechanism related to corticosteroid in HSVE is apparently inhibition of pro-inflammatory cytokines such as IL-6. [Copyright &y& Elsevier]

The objective of this study was to evaluate the executive dysfunction (ExD) in Parkinson's disease (PD) using the Behavioral Assessment of the Dysexecutive Syndrome (BADS), which provides a wide-range assessment of ExD. The BADS and the Unified Parkinson's Disease Rating Scale (UPDRS) were investigated in 63 nondemented PD patients who revealed scores of ≥24 points on the Mini-Mental State Examination based on the DSM-IV. Multiple logistic regression analysis was performed to evaluate the predisposing factors to ExD, which was defined as <70 points on the age-controlled standardized score. The total score on the UPDRS was a significant independent predisposing factor to ExD. Among the various parts of the UPDRS, part II was the significant factor for ExD. The profile scores of all subtests on the BADS in patients with ExD were significantly lower than those of patients without ExD. All profile scores decreased with severity of PD, but the changes among these scores differed. ExD in nondemented PD predisposed to a greater severity of PD, particularly as regards the activity of daily living impairment. Nondemented PD revealed wide-range components of ExD. All components of ExD were impaired with severity of PD, but the patterns of each component exhibited variety. © 2007 Movement Disorder Society [ABSTRACT FROM AUTHOR]

We evaluated the vulnerability of central nervous system (CNS) in patients with systemic lupus erythematosus (SLE) using exploratory eye movement analysis and random number generation (RNG), and compared the tests in evaluating CNS vulnerability. Nineteen patients received the tests more than a month after SLE onset in nonpsychotic status. Exploratory eye movements were analyzed using an eye-mark recorder that detects corneal reflection of infrared light, and numbers of eye fixations were counted to calculate responsive search score (RSS). Using digits 0 through 9, 100 numbers were vocally generated at a random fashion. “Seriality score” was calculated from the recorded 100 numbers. RSS of SLE patients was similar to that of normal individuals, irrespective of neuropsychiatric lupus history. Seriality score of patients having a history of neuropsychiatric lupus was higher than that of never having it ( p < 0.05). No relations were confirmed between RSS and seriality score. The current study suggested heterogeneous nature of SLE in CNS vulnerability when evaluating with seriality score, but not with RSS. There seemed to be a difference between exploratory eye movement analysis and RNG in evaluating CNS vulnerability. Each test seemed to evaluate different aspects of brain function. [ABSTRACT FROM AUTHOR]

Abstract: Constructibility is a combinatorial property of simplicial complexes. In general, it requires a great deal of time to decide whether a simplicial complex is constructible or not. In this paper, we consider sufficient conditions for nonconstructibility of simplicial 3-balls to investigate efficient algorithms for the decision problem. [Copyright &y& Elsevier]

Autonomous distributed systems comprising an overlay network on the Internet are proliferating as a new technology responsible for the next-generation Web. Due to the properties of such a large-scale system, it is difficult to measure the characteristics of the entire system without modifying the internal protocol of the applications. In this paper, by using a measurement method applicable to P2P applications and measured results obtained by applying this measurement method in part, a general-purpose method for estimating the size and behavior of the entire P2P network is proposed. Further, the present method is applied to a real P2P network and its effectiveness is presented by a specific example. © 2006 Wiley Periodicals, Inc. Electron Comm Jpn Pt 1, 90(3): 54–63, 2007; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/ecja.20318 [ABSTRACT FROM AUTHOR]

Objective: The main syndrome of anti-N-methyl-d-aspartate receptor encephalitis (NMDARE) is well-characterized, however, the difference in main symptoms between the initial episode and relapses and patient-oriented long-term outcomes has not been previously described. Methods: To investigate the difference in syndrome symptoms between episodes and patient-oriented outcomes, we administered a structured questionnaire survey for the patients with anti-NMDARE or their family members. From the answers, we analyzed the frequency of main symptoms (e.g., prodromes, abnormal behaviors, memory deficit, speech disorders, involuntary movements, hypo-ventilation) between episodes and patient-oriented outcomes that included the recovery rate for return to previous work or school. Results: Fifty-six patients were enrolled, and 14 (25%) showed clinical relapse. Details of symptoms at relapse were obtained from 11 patients. Prodrome (27% vs. 96%, p < 0.001), decreased level of consciousness (55% vs. 88%, p = 0.021), seizures (36% vs. 77%, p = 0.012), and involuntary movements (27% vs. 84%, p < 0.001) were significantly less frequent at relapse than initial episode. Although 80% (35/44) of the patients achieved favorable long-term outcomes (modified Rankin Scale score, 0–2), only 61% (27/44) returned to their previous work or school life. Conclusion: Anti-NMDARE can relapse with milder and more limited symptoms than those of initial episode, and some patients did not return to their previous activities even after achieving a neurologically favorable outcome. [ABSTRACT FROM AUTHOR]

Since the discovery of a series of antineuronal surface antibodies (NSAs), diagnostic approach to antibody‐mediated encephalitis, and other disorders has undergone a "paradigm shift." Currently, over ten specific NSAs, whose antigens include synaptic proteins (eg, anti‐N‐methyl‐d‐aspartate receptor) and proteins expressed on the neuronal surface (eg, dipeptidyl‐peptidase‐like protein 6), have been reported. Noble diagnostic implementations that preserve the conformation of the target epitope for NSA detection were developed to optimize the tests' sensitivity and specificity. NSAs can be detected through the following three techniques: tissue‐based assay (TBA) with rodent brain sections, cell‐based assay (CBA), and assays for rodent primary cultured neurons. TBA is a robust screening test that can identify most of the NSAs but cannot confirm specific antibodies. Meanwhile, CBA can confirm the specific antigen of NSAs, thereby suitable for the screening of positive patients. Assays with cultured neurons are also available not only for antibody screening but also for immunoprecipitation to identify an unknown antibody and analyze the antibody effects. Examining the distinctive syndrome caused by NSAs facilitates proper and rapid autoantibody detection, and combining different assays using cerebrospinal fluid minimizes misinterpretation of the tests. The up‐to‐date diagnostic algorithm for NSA‐associated autoimmunity recommends that the diagnostic process of autoantibodies involves screening by TBA/cultured neurons and confirmation by CBA sequentially. Intensive immunosuppressive therapies can be considered when the results of screening assays or confirming tests for NSAs are positive. [ABSTRACT FROM AUTHOR]

Abstract Background Knowledge of adult patients with influenza-associated acute encephalopathy (IAE) is limited. We conducted a detailed survey to investigate the prevalence, clinical features, associated outcomes, and prognostic factors in adult IAE patients. Method A nationwide questionnaire on IAE patients was sent to the departments of Internal Medicine, Neurology, Neurosurgery, and Emergency and Critical Care at all hospitals with ≥200 beds in Japan. Results 118 patients were diagnosed with IAE during the 2013/14 to 2015/16 influenza seasons, and the estimated annual incidence of IAE in Japanese adults was 0.98/1000,000 population. 44 patients were subsequently enrolled in the detailed study. 93% of patients exhibited disturbance of consciousness. Convulsions and delirious behavior were present in 26% and 40% of patients, respectively. 65% of patients received pulse corticosteroid therapy with methylprednisolone and 21% of patients received intravenous gamma-globulin therapy. 63% of patients achieved a good recovery, but 7% died. Multiple logistic regression analysis revealed that plasma glucose level demonstrated a statistically significant association with poor outcome. Conclusion: This nationwide survey provided data for the annual incidence and clinical features of IAE in Japanese adults. Hyperglycemia was indicated as an independent predictor of poor prognosis in IAE patients and reflected systemic hypercytokinemia in IAE pathogenesis. Highlights • Knowledge of influenza-associated acute encephalopathy (IAE) in adults is limited. • The annual incidence of IAE was 0.98/1000,000 adult population in Japan. • Approximately 63% of patients achieved a good recovery, but 7% died. • Plasma glucose level was significantly associated with poor outcome. [ABSTRACT FROM AUTHOR]

In this study, we aimed to clarify the risk factors associated with unfavorable outcomes in adults with pneumococcal meningitis (PnM). Surveillance was conducted between 2006 and 2016. Adults with PnM (n = 268) were followed up for outcomes within 28 days after admission using the Glasgow Outcome Scale (GOS). After classifying the patients into the unfavorable (GOS1–4) and favorable (GOS5) outcome groups, i) the underlying diseases, ii) biomarkers at admission, and iii) serotype, genotype, and antimicrobial susceptibility for all isolates were compared between both groups. Overall, 58.6% of patients with PnM survived,15.3% died, and 26.1% had sequelae. The number of living days in the GOS1 group was highly heterogeneous. Motor dysfunction, disturbance of consciousness, and hearing loss were the commonest sequelae. Of the underlying diseases identified in 68.9% of the PnM patients, liver and kidney diseases were significantly associated with unfavorable outcomes. Of the biomarkers, creatinine and blood urea nitrogen, followed by platelet and C-reactive protein had the most significant associations with unfavorable outcomes. There was a significant difference in the high protein concentrations in the cerebrospinal fluid between the groups. Serotypes 23F, 6C, 4, 23A, 22F, 10A, and 12F were associated with unfavorable outcomes. These serotypes were not penicillin-resistant isolates possessing three abnormal pbp genes (pbp1a , 2x , and 2b), except for 23F. The expected coverage rate of the pneumococcal conjugate vaccine (PCV) was 50.7% for PCV15 and 72.4% for PCV20. In the introduction of PCV for adults, the risk factors for underlying diseases should be prioritized over age, and serotypes with unfavorable outcomes should be considered. [ABSTRACT FROM AUTHOR]

Abstract: A 72‐year‐old woman with slight pulmonary interstitial reticular markings was initially diagnosed with microscopic polyangiitis (MPA). Two years later, cavitated pulmonary masses appeared, and a biopsy specimen revealed granulomas. Granulomatosis with polyangiitis (GPA) was diagnosed. The masses resolved with treatment. Ten years later, the usual interstitial pneumonia (UIP) pattern appeared on chest computed tomography (CT). The diagnosis of lung toxicity from methotrexate (MTX) or cyclophosphamide (CYC) was precluded by the clinical course. Despite treatment with prednisolone (PSL), the UIP progressed. The change of pulmonary pathology from masses to UIP is rare in patients with GPA. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate the clinical characteristics of acute ischemic stroke (AIS) patients who experienced hypoesthesia as the initial symptom. We retrospectively analyzed the medical records of 176 hospitalized AIS patients who met our inclusion and exclusion criteria and evaluated their clinical features and MRI findings. Among this cohort, 20 (11%) patients presented with hypoesthesia as the initial symptom. MRI scans of these 20 patients identified lesions in the thalamus or pontine tegmentum in 14 and brain lesions at other sites in 6. The 20 hypoesthesia patients had higher systolic (p = 0.031) and diastolic blood pressure (p = 0.037) on admission, and a higher rate of small-vessel occlusion (p < 0.001) than patients without hypoesthesia. The patients with hypoesthesia had a significantly shorter average hospital stay (p = 0.007) but did not differ significantly from those without hypoesthesia in National Institutes of Health Stroke Scale scores on admission (p = 0.182) or the modified Rankin Scale scores for neurologic disability on discharge (p = 0.319). In the patients with acute onset hypoesthesia, high blood pressure, and neurological deficits were more likely to be due to AIS than other causes. Since most of the lesions in AIS patients with hypoesthesia as the initial symptom were found to be small, we recommend performing MRI scans with such patients to confirm AIS. [ABSTRACT FROM AUTHOR]

Regional progression of neurofibrillary tangles (NFTs) around the hippocampus was traced on thick sections double immunofluorolabeled with RD3 and RD4 antibodies, specific for three- and four-repeat tau, respectively. As reported, the cubic density of all tau-positive neurons was predominant in the entorhinal cortex and cornu ammonis (CA)1, and decreased progressively to the CA2-4 subregions. Among the three isoform profiles (RD3+/4−, RD3+/4+, and RD3−/4+), this regional gradient was replicated with RD3+/4− and RD3+/4+ neurons, while RD3−/4+ neurons exhibited the reverse gradient. Comparison of the subregion pairs confirmed a consistent profile shift along this gradient in every case regardless of the abundance of NFTs. To clarify the underlying mechanism of this regional profile shift, intraneuronal intensity of RD3 and RD4 immunoreactivity (IR) was quantified. Although their intensities were both lower in dendrites than in the soma, this gradient was steeper with RD4, leaving RD3 IR in dendrites. Dendritic arborization was abundant in RD3−/4+ pretangles, attenuated in RD3+/4+ neurons, and further attenuated in RD3+/4− ghost tangles. These findings suggest that dendritic RD4 IR retracts first, leaving RD3 IR in the dendrites. Taken together, this dendrite-oriented retraction initiates the gradual shift from RD3−/4+ pretangle neurons to RD3+/4− ghost tangles by way of RD3+/4+ NFTs. This intraneuronal profile shift may be a basis for the regional gradation featured by the similar profile shift during progression of NFT pathology. [ABSTRACT FROM AUTHOR]

• Parkinson's disease patients explored less efficiently than age-matched controls. • The patients showed smaller saccade size than the controls on the antisaccade task. • Effects of aging were not observed in the variables showing the disease effects. Parkinson's disease (PD) reportedly show disturbed visual exploration. However, whether this disturbance is due to dysfunctional visual information processing remains unclear. To clarify the effects of PD on visual information processing when exploring for targets and to compare disease effects with aging effects, we used an infrared eye-movement assessment system. Cognitively normal PD patients (n = 13), healthy age-matched (n = 17) and young controls (n = 36) participated in this study, and were evaluated using two figure-matching tasks representing visual information processing (clock-matching and inverted clock-matching tasks) and saccade tasks representing oculomotor function. With figure-matching tasks, PD patients showed significantly larger numbers of images watched in a single trial compared to healthy age-matched controls on the inverted clock-matching task. No aging effects was found in these variables. In contrast, no disease effect was apparent for reaction time, which was significantly longer in healthy age-matched controls than in healthy young controls. For saccade tasks, PD patients showed significantly smaller saccade size than healthy age-matched controls on the antisaccade task, but no aging effects were evident. Our approaches highlighted that visual exploration disturbance in PD may be due to dysfunctional visual information processing in addition to dysfunctional oculomotor processing. These disease effects may differ from aging effects. [ABSTRACT FROM AUTHOR]

An adult female complained of enlargement of right eyes in other people. Diffusion-weighted imaging detected an abnormal high-intensity area in the region from the splenium of the corpus callosum to the major forceps on the right side. The patient reported that right eyes appeared larger in size, which suggested prosopometamorphopsia. Adichotic listening test identified left-ear deficit. Acombination of prosopometamorphopsia and left-ear deficit was not identified in the reported patients. Prosopometamorphopsia in most of the reported patients included the eye as did that in our patient. This result suggested the importance of information on the eye in recognizing faces. [ABSTRACT FROM AUTHOR]

The article discusses the role of a frame shift mutation in fused in sarcoma (FUS) gene in causing lower motor neuron disease. It also presents a case study of a Japanese man who was suffering from distal weakness of the right leg. After being put through nerve conduction study it was found to have a decrease in F-wave frequency in the right extremities.

A letter to the editor is presented which discusses the case of a 65-year-old woman who was admitted to the hospital of neurology ward and was diagnosed of acute meningo-encephalitis associated with carcinosarcoma with neuroendocrine differentiation of the uterus.

Background: A unique patient with MELAS syndrome, who initially masqueraded as having acute encephalitis and was eventually diagnosed with MELAS syndrome harboring a mtDNA 14453G → A mutation, is described.Case Presentation: A 74-year-old Japanese man was admitted to another hospital due to acute onset of cognitive impairment and psychosis. After 7 days he was transferred to our hospital with seizures and deteriorating psychosis. The results of primary ancillary tests that included EEG, CSF findings, and brain MRI supported the diagnosis of an acute encephalitis. HSV-DNA and antibodies against neuronal surface antigens in the CSF were all negative. With the assistance of the lactate peak on the brain lesions in the magnetic resonance spectroscopy image and genetic analysis of the biopsied muscle, he was eventually diagnosed with MELAS syndrome harboring mtDNA 14453G → A mutation in the ND6 gene.Conclusions: This case provides a caveat that MELAS syndrome can manifest in the symptoms and ancillary tests masquerading as an acute encephalitis caused by infection or autoimmunity. This is the first adult patient seen to harbor the mtDNA14453G → A with a unique onset, which broadens the phenotypic spectrum of MELAS syndrome associated with ND6 gene mutation. [ABSTRACT FROM AUTHOR]

The article discusses a study to detect Alzheimer disease (AD)-specific four repeat tau proteins polyclonal antibody with a deamidated asparagine residue at N279 to yield aspartic acid found in AD and mentions immunoabsorption of this 4R tau polyclonal antibody with Nitrogen-peptide.

Background: Postural abnormalities in Parkinson's disease (PD) patients and unimpaired elderly are not well differentiated. Factors related to postural abnormality associated with PD are controversial. Objective: We assessed differences in postural change between PD patients and unimpaired elderly and elucidated factors related to abnormal posture in PD patients. Methods: We measured the dropped head angle (DHA), anterior flexion angle (AFA), and lateral flexion angle (LFA) of the thoracolumbar spine of an unprecedented 1,117 PD patients and 2,732 general population participants (GPPs) using digital photographs. Two statistical analyses were used for elucidating factors related to these angles. Results: In GPPs, age was correlated with DHA, AFA, and LFA. DHAs, AFAs, and LFAs of PD patients and age‐matched GPPs were 21.70° ± 14.40° and 13.13° ± 10.79°, 5.98° ± 12.67,°and − 3.82° ± 4.04°, and 0.86° ± 4.25° and 1.33° ± 2.16°, respectively. In PD patients, factors related to DHA were age, male sex, and H & Y stage during ON time. Factors related to AFA were age, duration of disease, H & Y stage during ON and OFF times, pain, vertebral disease, and bending to the right. A factor related to LFA was AFA. Conclusions: DHA and AFA of GGPs correlated with age and were larger in PD patients than those with in GPPs. Some PD patients showed angles far beyond the normal distribution. Thus, factors associated with disease aggravation affected postural abnormality in PD patients. [ABSTRACT FROM AUTHOR]

Background Crowned dens syndrome (CDS) is a radioclinical entity defined by calcium deposition on the transverse ligament of atlas (TLA). In this study, the novel semi-quantitative diagnostic criteria for CDS to evaluate the degree of calcification on TLA by cervical CT are proposed. Method From January 2010 to September 2014, 35 patients who were diagnosed with CDS by cervical CT were adopted as subjects in this study. Based on novel criteria, calcium deposition on TLA was classified into “Stage” and “Grade”, to make a score, which was evaluated semi-quantitatively. The correlation between calcification score and CRP level or pain score, and the effects of treatments, such as NSAIDs and corticosteroids, were statistically analyzed. Results The total calcification score from added “Stage” and “Grade” scores demonstrated a significantly strong and linear correlation with CRP level ( R 2 = 0.823, ** p < 0.01). In the multiple comparison test for the treatment effects, significant improvement of the CRP level and pain score were demonstrated after corticosteroid therapy (** p < 0.01) compared with NSAIDs. In the conditional logistic regression analysis, the rapid end of corticosteroid therapy was an independent risk factor for relapse of cervico-occipital pain [OR = 50.761, * p = 0.0419]. Conclusion The degree of calcification on TLA evaluated by the novel semi-quantitative criteria significantly correlated with CRP level. In the treatment of CDS, it is recommended that a low dosage (15–30 mg) of corticosteroids be used as first-line drugs rather than conventional NSAID therapy. Additionally, it is also recommended to gradually decrease the dosage of corticosteroids. [ABSTRACT FROM AUTHOR]

Background: Cardiac myxoma is known to cause repeated events of cerebral embolism. Soft and irregularly shaped myxomas with high mobility are associated with a higher occurrence of cerebral embolism. In contrast, nonmobile cardiac myxomas with a round regular shape are rarely considered to be a cause of cerebral embolism. In this case, we present a patient with recurrent cerebral embolism associated with a small and nonmobile cardiac myxoma of round regular shape.Case Presentation: A 76-year-old Japanese man presented to our hospital with weakness in his right upper extremity. He had a history of right frontal lobe infarction in the previous month. T2-weighted magnetic resonance imaging revealed an area of hyperintensity in the left precentral gyrus, indicating acute cerebral infarction. Transthoracic echocardiography revealed normal left ventricular function and no abnormalities. However, transesophageal echocardiography showed a small and nonmobile left atrial tumor with round regular shape attached to the ostium secundum of the atrial septum. Based on these findings, we diagnosed recurrent cerebral infarction due to embolization caused by left atrial myxoma, and cardiac tumor extraction was performed on hospitalization day 36. The excised tumor measured 0.6 × 0.6 × 0.5 cm and was diagnosed as cardiac myxoma by histologic examination.Conclusions: Even small and nonmobile cardiac myxomas with a round regular shape may cause recurrent cerebral infarction. The diagnosis of this type of atrial myxoma is elusive and transesophageal echocardiography was an effective method of detection. In a clinical situation, this type of cardiac myxoma may be overlooked as a cause of cerebral infarction. [ABSTRACT FROM AUTHOR]

Background and Aim Therapeutic management of bacterial meningitis worldwide has been established based on the patient age and risk factors. Aim The present study investigated the distribution of pathogens and outcomes in adult bacterial meningitis with underlying disease in Tokyo, Japan. Methods Participants in this retrospective study were 131 adult cases diagnosed with having bacterial meningitis based on their clinical symptoms and laboratory data. All patients had been admitted to our hospitals (Nihon University Itabashi Hospital and Surugadai Hospital) in Tokyo, Japan, between 1984 and 2013. Bacterial meningitis was classified with underlying disease into three groups, as follows: group 1, invasive procedures or complicated head trauma within the preceding 3 months ( n = 35); group 2, immunocompromised status ( n = 37); or group 3, both conditions ( n = 31). Poor outcome was defined as a Glasgow Outcome Scale score of 1-3. Results The distribution of pathogens differed significantly in each group. The most common pathogen in each group was Staphylococcus epidermidis (23.7%) in group 1; penicillin-intermediate Streptococcus pneumoniae (12.8%) in group 2; and Staphylococcus epidermidis (13.9%) in group 3. The highest rate of mortality and poor outcome was shown in group 2, and followed by group 3 and 1. The rate of antibiotic-resistant bacteria was 68.9% for all pathogens in bacterial meningitis with underlying disease, 70.6% in group 1, 55.6% in group 2 and 80.6% in group 3. Conclusion This is the first retrospective study of the distribution of pathogens and outcomes in adult bacterial meningitis with underlying disease in Japan. [ABSTRACT FROM AUTHOR]

We report a rare case of infarction limited in the region from the declive to the tuber of the posterior lobe in the cerebellar vermis. A 47‐year‐old woman complained of dizziness with acute onset. Mild incoordination of the left upper extremity and mild truncal and gait ataxia was noted. Brain magnetic resonance imaging showed an infarct of the region from the declive to the tuber on the left side. In the posterior lobe, the region from the declive to the tuber is associated with coordination of the hands. The dorsal spinocerebellar tract and the ventral spinocerebellar tract convey unconsciousness proprioceptive signals from the trunk and leg and project to the anterior and posterior lobes in the cerebellar vermis. Symptoms in our patient indicated that the lesion of this region was related to the impairment of these two tracts and incidence of incoordination of the lateral upper extremity. [ABSTRACT FROM AUTHOR]

Objective. To assess the relation between executive dysfunction (ED) in Parkinson’s disease (PD) and resting state functional connectivity evaluated using electroencephalography (EEG) coherence. Methods. Sixty-eight nondemented sporadic PD patients were assessed using the Behavioural Assessment of the Dysexecutive Syndrome (BADS) to evaluate executive function. EEG coherence in the left frontoparietal electrode pair (F3-P3) and the right frontoparietal electrode pair (F4-P4) was analyzed in the alpha and theta range. The BADS scores were compared across the coherence groups, and the multiple logistic regression analysis was performed to assess the contribution of confounders. Results. The standardized BADS score was significantly lower in the low F3-P3 coherence group in the alpha range (Mann-Whitney U test, p=0.032), though there was no difference between F4-P4 coherence group in the alpha range, F3-P3, and F4-P4 coherence groups in the theta range and the standardized BADS score. The multiple logistic regression analysis revealed the significant relation between the F3-P3 coherence group in alpha range and age-controlled standardized BADS score (p=0.039, 95% CI = 1.002–1.062). Conclusion. The decrease in resting state functional connectivity between the frontal and parietal cortices especially in the left side is related to ED in PD. [ABSTRACT FROM AUTHOR]

Postural deformities and executive dysfunction (ED) are common symptoms of Parkinson’s disease (PD); however, the relationship between postural deformities and ED in patients with PD remains unclear. This study assessed the relationship between postural deformities and ED in patients with PD. Sixty-five patients with sporadic PD were assessed for the severity of postural deformities and executive function. The severity of postural deformities was scored using the United Parkinson’s Disease Rating Scale item 28 score: no postural deformity (0), mild postural deformities (1), or severe postural deformities (2–4). Executive function was assessed using the Behavioral Assessment of the Dysexecutive Syndrome (BADS) and an age-controlled standardized BADS score <70 was defined as ED. Age-controlled standardized BADS scores were compared across the three groups using the Kruskal-Wallis test. Relationship between ED and the severity of postural deformities was assessed using the Mann-Whitney U test. Age-controlled standardized BADS score significantly differed among the three groups P=0.005. ED was significantly related to the severity of postural deformities P=0.0005. The severity of postural deformities was associated with a lower age-controlled standardized BADS score and ED, and these findings suggest that postural deformities were associated with frontal dysfunction in patients with PD. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 70-year old woman having a subarachnoid hemorrhage, small bowel obstruction, and ischemic colitis. Topics include detection of hepatitis C antibody and hepatitis B antigens being negative, diagnosis of subarachnoid hemorrhage through Cerebral angiography, and interlobular artery injury's impact on chronic renal ischemia.

Introduction Freezing of gait (FOG) is a common symptom of Parkinson's disease (PD). Although the pathophysiological mechanism of FOG is unknown, previous studies have suggested that frontal dysfunction is associated with FOG. The Behavioral Assessment of the Dysexecutive Syndrome (BADS) battery, which is wide-ranging neurological battery composed of six subtests, evaluates frontal function and is more sensitive to executive dysfunction (ED) than other tools in PD patients. This is the first study to assess the relation between FOG in the ‘on’ state and frontal dysfunction evaluated using BADS. Methods Subjects were 65 patients with PD. Multiple logistic regression analysis was used to compare the age-controlled standardized BADS score, age, disease duration, Hoehn and Yahr (HY) stage, levodopa-equivalent daily dose, and Mini-Mental State Examination (MMSE) score across patients with FOG ( n = 43) and patients without FOG ( n = 22). Score on each of the six BADS subtests were compared across patients with and without FOG using the Mann–Whitney U test. Results Multiple logistic regression analysis revealed that FOG was related to lower age-controlled standardized BADS score ( P = 0.022) and higher HY stage ( P = 0.009) but not to disease duration, levodopa equivalent daily dose, or MMSE score. Among the six BADS subtests, score on the Zoo Map Test, which evaluates problem solving and planning, was lower in patients with FOG than in patients without FOG. Conclusion These results support a relation between on-state FOG and frontal dysfunction in PD patients. [ABSTRACT FROM AUTHOR]

Background: The central visual field is projected to the region from the occipital tip to the posterior portion of the medial area in the striate cortex. However, central visual field disturbances have not been compared with the location of the lesions in the striate cortex. Methods: Thirteen patients with visual field defects caused by partial involvement of the striate cortex were enrolled. The lesions were classified according to their location into the anterior portion, the posterior portion of the medial area, and the occipital tip. Visual field defects were examined by the Goldmann perimetry, the Humphrey perimetry and the auto-plot tangent screen. We defined a defect within the central 10° of vision as a central visual field disturbance. The visual field defects in 13 patients were compared with the location of their lesions in the striate cortex. Results: The medial area was involved in 7 patients with no involvement of the occipital tip. In 2 of them, peripheral homonymous hemianopia without central visual field disturbance was shown, and their lesions were located only in the anterior portion. One patient with a lesion in the posterior portion alone showed incomplete central homonymous hemianopia. Three of 4 patients with lesions located in both the anterior and posterior portions of the medial area showed incomplete central homonymous hemianopia and peripheral homonymous hemianopia. The occipital tip was involved in 6 patients. Five of them had small lesions in the occipital tip alone and showed complete central homonymous hemianopia or quadrantanopia. The other patient with a lesion in the lateral posterior portion and bilateral occipital tip lesions showed bilateral slight peripheral visual field disturbance in addition to complete central homonymous hemianopia on both sides. Conclusions: Lesions in the posterior portion of the medial area as well as the occipital tip caused central visual field disturbance in our study, as indicated in previous reports. Central homonymous hemianopia tended to be incomplete in patients with lesions in the posterior portion in the medial area. In contrast, complete central homonymous hemianopia and quadrantanopia were shown in patients with occipital tip lesions. Our study suggested that the fibers related to the central visual field were sparse in the posterior portion of the medial area in contrast to the occipital tip, and approached the occipital tip with a high concentration of fibers. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Introduction: The paravermal zone of the right rostral cerebellar hemisphere is an important area for speech function. Recent functional MRI (fMRI) studies reported the significance of lobulus simplex within the rostral cerebellum for speech function. Here, we assess the responsible lesion for dysarthria within the rostral cerebellum. Patients and methods: In order to evaluate the lesion in the rostral cerebellum responsible for cerebellar dysarthria, we compared the locations of infarcts from 4 reported patients with isolated dysarthria caused by cerebellar infarction as well as an additional newly encountered patient in whom cerebellar infarction caused isolated dysarthria. Results: The paravermal zone of the rostral cerebellar hemisphere was involved in all 5 patients. The lesions were located in the lobulus quadrangularis and lobulus simplex in 3 of the patients, and the lobulus simplex and lobulus semilunaris superior in 2 of the patients. The infarcts in 4 of the patients were located on the right side. Conclusion: The lesions in 4 of the patients included the right paravermal zone of the rostral cerebellar hemisphere, which is regarded as an important area for speech function. The lobulus simplex was most highly involved in all 5 patients. We present the importance of lobulus simplex for speech function, as in the fMRI data of previous reports, based on the localization of cerebellar infarcts in patients with isolated dysarthria. [ABSTRACT FROM AUTHOR]

We investigated the neurological and MRI findings of 21 patients with pure midbrain infarction. The locations of the infarcts were classified into paramedian (PM) area (13 patients), lateral area (6 patients), and PM and lateral areas (1 patient), based on the distribution of each penetrating artery of the midbrain. The infarction of 1 patient showed bilateral multiple lesions. In the PM area group, 12 patients showed eye movement disorders and truncal and gait ataxia. Eye movement disorders consisted of medial longitudinal fasciculus syndrome, oculomotor nerve palsy, and bilateral vertical gaze palsy. The infarcts of the 2 patients with bilateral vertical gaze palsy were located in the PM area on both sides. Eye movement disorders and truncal and gait ataxia were prominent in the PM area group. Ataxic hemiparesis was shown in 2 patients of the PM area group and 4 patients of the lateral area group. We showed that ataxic hemiparesis also occurred in PM area infarction, although ataxic hemiparesis was a prominent feature of lateral area infarction. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The article presents a case study of a 61-year-old woman who complained of sleeplessness and easily became confused while working her job. It states that the woman became unresponsive for the period lasting 5 to 10 minutes on admission and neurological examinations were performed while she was non-responsive. Results show that the female patient has a probable Creutzfeldt-Jakob disease (CJD) who showed complex partial seizure following sleeplessness and cognitive impairment.

We report the first current perception threshold (CPT) examination of sensory disturbance in subacute myelo-optico-neuropathy (SMON). SMON patients experience serious neurological symptoms, including dysesthesia, sensory loss, motor weakness, and visual impairment. During CPT examination, 5 Hz, 250 Hz, and 2,000 Hz stimulations were used to stimulate C fibers, A-δ fibers, and A-β fibers, respectively. Ten SMON patients (mean age, 73.8 ± 8.4 years) and ten age-matched controls (72.3 ± 6.3 years) were studied using CPT measured at the index finger and near the external malleolus. The CPTs to 250 Hz and 2,000 Hz stimulations near the external malleolus were significantly higher and the CPT to 5 Hz stimulation was significantly lower in the SMON group than in the control group. Although peripheral nerve impairment is mild in SMON, pathological examination shows a decrease of large fibers. This is thought to increase the CPTs to 250 Hz and 2,000 Hz stimulations. The center of the gate control of pain exists in the posterior horn receiving information from the dorsal root ganglion. The dorsal root ganglion at the lumber cord is strongly impaired in SMON; therefore, the gate control may not work effectively, and decreases CPT to 5 Hz stimulation. [ABSTRACT FROM AUTHOR]

We report a 77-year-old man showing bilateral superior altitudinal hemianopia (BSAH) caused by bilateral occipital lobe infarction. The lesions existed into the lingual gyri and involved V1, V2 and V3 in the visual cortex on both sides. The location of the lesions correlated well with visual field defects in our patient. There have been 8 case reports of BSAH caused by occipital lobe lesions, however, the extent of the lesions inside the visual cortex was not evaluated in the previously reported patients. The analysis in our patient showed a close clinico-radiological correlation of BSAH. [ABSTRACT FROM AUTHOR]

Abstract: Depression and nocturnal disturbances are frequent in patients with Parkinson''s disease (PD). The aim of this study was to determine the correlation between depressive symptoms and nocturnal disturbances in patients with PD in Japan. The subjects of this multi-center cross-sectional study were 188 patients with PD and 144 age-matched controls who were assessed for nocturnal disturbances by the Parkinson''s disease sleep scale (PDSS) and for depressive symptoms by Zung Self-Rating Depression Scale (SDS). Depressive symptoms (SDS score of ≥40) were identified in 122 patients (64.9%). The SDS was significantly higher in PD patients than control subjects. The stepwise regression model identified PDSS (p <0.001) and Unified Parkinson''s Disease Rating Scale I (mental state) (p =0.002) as significant determinants of SDS. Stepwise regression analysis identified item 15 (daytime sleepiness) (p =0.002), item 13 (early morning tremor) (p =0.008), item 12 (nocturnal dystonia) (p =0.015), and item 3 (sleep maintenance insomnia) (p =0.026) as significant predictors of SDS. Our results indicated that depressive symptoms in PD correlate significantly with nocturnal disturbances, and that daytime sleepiness, dystonia, tremor and sleep fragmentation are the most common nocturnal disturbances in depressed patients with PD. [Copyright &y& Elsevier]

Abstract: In Parkinson''s disease (PD), sudden unexpected sleep episodes and excessive daytime sleepiness (EDS) while driving and engaging in social activities are important problems. We conducted a multi-center study to clarify the prevalence and contributing factor of EDS and sleep episodes in Japanese patients with PD. We evaluated 188 patients with PD (85 men, 103 women) and 144 age-matched controls for sleepiness. EDS was defined as an Epworth sleepiness scale (ESS) score of ≥10. ESS score was significantly higher (6.6±4.2 vs. 5.6±3.8) and prevalence of sleep episodes was higher in PD than in controls (6.4% vs. 0.7%). PD patients with EDS were more likely to have sleep episodes (22.5% vs. 2.0%), higher score for disease severity and depressive symptoms, and on higher dose of dopaminergic agents than those without EDS. However, there were no differences in nocturnal disturbances between the two groups. ESS score was not different between patients taking ergot and non-ergot dopamine agonists. Logistic regression analysis demonstrated that mental state, total dose of dopaminergic agents, and ESS score were significant predictors of sleep episodes. ESS score of ≥10 had 75% sensitivity and 82.4% specificity for sleep episodes. These results suggest that sleepiness in PD is dependent on disease itself and dopaminergic treatment rather than nocturnal disturbances. [Copyright &y& Elsevier]

The present multicenter cross-sectional study was performed using semistructured questionnaires to determine the contributing factors of sleep disturbances in Japanese patients with Parkinson's disease (PD). We used the Parkinson's disease sleep scale (PDSS, Japanese version). All data were obtained by means of interviewed questionnaire and physical examination by neurologists. The study was carried out between April 2005 and December 2005 at eight university hospitals and affiliated facilities in the Kanto area of Japan. A total of 188 (85 men and 103 women) PD patients and 144 controls (64 men and 80 women) were included. Stepwise regression analysis identified complications of treatment, depression, age, and disease duration as significant risk factors of sleep disturbances in PD. Significant differences in total PDSS score were observed between Hoehn & Yahr (H&Y) Stages 1 and 4, between H&Y Stages 2 and 4, and between H&Y stages 3 and 4 (Bonferroni test). The results of this survey suggested that complications due to treatment (dyskinesia, wearing off, on- off), depressive state, and disease stage are significant determinants of sleep disorders in Japanese patients with PD. We speculate that the reduction of neurotransmitters involved in the sleep-wakefulness mechanism and degeneration of neurons progress together in parallel with deterioration of motor function. © 2006 Movement Disorder Society [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in which the authors discuss case study of an 81-year-old female patient having discomfort in her right lower limb, with high serum Toxoplasma antibody levels indicating toxoplasmic encephalitis; and highlight that aging is a risk factor for such infections.

Background: Diabetic chorea appears during the course of poorly-controlled diabetes. While chorea associated with diabetes mellitus usually occurs during hyperglycemic episodes, hypoglycemia can also cause diabetic chorea. Brain magnetic resonance imaging (MRI) is useful for evaluating the pathogenesis of diabetic chorea. However, several diabetic chorea cases have reportedly not shown abnormal high-intensity in the putamen and striatum on T1-weighted images.Case Presentation: We report a 74-year-old woman who was admitted to our hospital for treatment of poorly-controlled type 2 diabetes mellitus. Intensified insulin treatment gradually normalizeed blood glucose, but on the 19th hospital day, after a blood glucose measurement of 49 mg/dL, she showed hemichorea of the left face, shoulder, arm and leg. MRI revealed no abnormalities of either the putamen or the striatum on T1-weighted images. She was treated with dopamine receptor antagonists, which alleviated her hemichorea symptoms and allowed discharge from the hospital. 1 year after the first hospitalization, she had to be readmitted because her glycemic control had markedly deteriorated. Glycemic control improved rapidly, and, because hemichorea did not recur, the dopamine receptor antagonists were stopped. 1 month later, however, hemichorea recurred. She resumed taking the dopamine receptor antagonists, resulting in immediate disappearance of the hemichorea.Conclusions: We herein describe a rare case of diabetes-associated hemichorea occurring after hypoglycemic episodes without abnormal high-intensity findings in the basal ganglia on T1-weighted images. The hemichorea relapsed with cessation of dopamine receptor antagonists. This case also underscores the importance of longitudinal assessment and treatment for hemichorea after hypoglycemic episodes, even in the absence of MRI findings, in elderly diabetic patients. [ABSTRACT FROM AUTHOR]

Neurofibrillary tangles (NFTs), are shared between progressive supranuclear palsy (PSP) and Alzheimer disease (AD). Histological distinction of PSP and AD is possible based on the distribution of NFTs. However, neuropathologists may encounter diagnostic difficulty with comorbidity of PSP and AD. In this study, we tried to circumvent this difficulty by analyzing five autopsied brains harboring both PSP and AD pathology. Tau-positive lesions were sorted based on their cell type (neuron versus glia), and tau isoforms: three-repeat (3R) versus four-repeat (4R) tau. 16 regions were selected to map these lesions throughout the brain. 4R-tau lesions were present in all areas examined. Among them, 3R-tau lesions were absent in some areas. These 4R selective (4R+/3R-) areas dictate prototypic distribution of PSP, not usually found in AD, such as pontine nucleus, red nucleus, inferior olivary nucleus, dentate nucleus, globus pallidus and putamen, each contained both glial and neuronal lesions. In contrast, additional 3R-tau lesions were found in hippocampal formation to neocortex, where 3R immunoreactivity (IR) was predominant over the 4R counterpart mainly in neurons as found in AD but not in PSP. Although tau lesions in central grey matter, substantia nigra and locus coeruleus are found in both AD and PSP, 4R-selectivity with glial component suggests PSP origin. Even if the presence of 3 R IR in these areas suggests AD pathology, it does not exclude the involvement of PSP-type lesion because distinction of 4R IR into PSP or AD is not yet possible. Further demixing may be possible if biochemical difference of 4R tau between PSP and AD is identified. [ABSTRACT FROM AUTHOR]

We report the case of a 79-year-old Japanese woman who developed cerebellar ataxia followed by rigidity, dysautonomia and cognitive disorders, and was thus clinically diagnosed as having possible MSA with dementia. Neuropathological findings demonstrated not only olivopontocerebellar and striatonigral degeneration with frequent glial cytoplasmic inclusions (GCIs), but also degenerative changes in the parahippocampal region, accentuated in the anterior portion of perirhinal cortex, where neuronal cytoplasmic inclusions (NCIs) and NFTs were numerous while GCIs were limited. NCIs were frequent in the deep layer, whereas NFTs were more frequent in superficial cortical layers. Other hippocampal subregions including subiculum, dentate fascia and cornu ammonis were minimally involved. NCIs in the perirhinal cortex showed intense argyrophilia with the Campbell-Switzer silver impregnation method, but not argyrophilic with the Gallyas method. Most neuronal alpha-synuclein aggregates in dendrosomatic fraction formed globular/tadpole-like, and ultrastructurally comprised granular-coated fine fibrils 12-24 nm in diameter. To the best of our knowledge, alpha-synuclein-related neuronal pathology localized in the perirhinal region without hippocampal involvement has not been previously reported in MSA, and may provide clues to elucidate how neuronal pathology evolves in the hippocampal/parahippocampal regions in MSA, particularly in cases with dementia. [ABSTRACT FROM AUTHOR]

The article discusses accompanying of takotsubo cardiomyopathy with amyotrophic lateral sclerosis (ALS) will lead to complications. It renders a casestudy of a 70 year old woman who developed ALS and reports about her respiratory failure also. It describes her normal biochemical, neurological and Xray findings. It informs that excess catecholamine and cardiac nerve activity develops takotsubo cardiomyopathy and she died. It concludes that takotsubo cardiomyopathy is main reason of complication.

A letter to the editor is presented wherein the authors discuss a case of a 69-year-old man who was diagnosed with unique case of beriberi with decreased cerebral blood flow (CBF) and upon treatment with thiamine showed improved CBF.