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Herpes simplex encephalitis (HSE) is an acute form of encephalitis that can lead to poor neurological outcomes. Although the exact pathogenesis of HSE remains elusive, recent reports suggest a significant role for postinfectious immune‐inflammatory processes in the central nervous system (CNS). This study aimed to clarify the association between CNS autoimmune responses and clinical presentation in patients with HSE, focusing on cerebrospinal fluid (CSF) characteristics, particularly the IgG index. We retrospectively analyzed 176 consecutive patients suspected of having aseptic meningitis /encephalitis for chronological changes in CSF findings and clinical presentations. These patients underwent PCR screening for herpesviruses (HV) in their CSF. We identified seven patients positive for herpes simplex virus type 1 (HSV‐1), 20 patients positive for varicella‐zoster virus, and 17 patients who met the criteria for aseptic meningitis but were PCR‐negative for HV. Patients in the HSV‐1‐positive group exhibited a significant increase in the IgG index at the time of PCR‐negative conversion compared with on admission (p = 0.0156), while such a change was not observed in the other two groups. Additionally, all patients in the HSV‐1‐positive group tested negative for anti‐neural autoantibodies in CSF and serum samples collected approximately 3 weeks after onset. This study, therefore, highlights that CSF IgG index elevation occurs even after PCR‐confirmed HSV‐1 clearance, which might indicate immunopathogenesis that is independent of antibody‐mediated mechanisms. [ABSTRACT FROM AUTHOR]

Background and purpose: Multiple system atrophy (MSA) is a neurodegenerative disease with characteristic motor and autonomic symptoms. Impaired brain serotonergic innervation can be associated with various clinical indices of MSA; however, the relationship between clinical symptoms and cerebrospinal fluid (CSF) levels of 5‐hydroxyindole acetic acid (5‐HIAA), a main serotonin metabolite, has not been fully elucidated. Methods: To compare CSF 5‐HIAA levels between patients with MSA and healthy controls, we included 33 controls and 69 MSA patients with either predominant parkinsonian or cerebellar ataxia subtypes. CSF 5‐HIAA levels were measured using high‐performance liquid chromatography. Additionally, we investigated correlations between CSF 5‐HIAA and various clinical indices in 34 MSA patients. Results: CSF 5‐HIAA levels were significantly lower in MSA patients than in controls (p < 0.0001). Probable MSA patients had lower CSF 5‐HIAA levels than possible MSA patients (p < 0.001). In MSA patients, CSF 5‐HIAA levels were inversely correlated with scores in Parts 1, 2, and 4 of the Unified Multiple System Atrophy Rating Scale, and with systolic and diastolic blood pressure in Part 3. Structural equation modeling revealed significant paths between serotonin and clinical symptoms, and significance was highest for activities of daily living, walking, and body sway. Conclusions: Serotonin dysfunction, as assessed by CSF 5‐HIAA levels, may implicate greater MSA severity. [ABSTRACT FROM AUTHOR]

Materials exhibiting irreversible phase transitions, leading to changes in their properties, have a potential for novel application in electronic components such as a non‐rewritable high‐security memory. Here, we focused on the two salts, [(9‐triptycylammonium)([18]crown‐6)][Ni(dmit)2] (1) and [(9‐triptycylammonium)([15]crown‐5)][Ni(dmit)2] (2), which featured 2D sheet structures with alternately stacked cation and anion layers. Both salts exhibit similar cation arrangements, however, their anion arrangements differ significantly. The temperature‐dependent magnetic susceptibilities of 1 and 2 were well reproduced by the alternating chain model (JAC1/kB=−306(8), JAC2/kB=−239(3) K) and the Curie‐Weiss model (θ=−3.9(1) K), respectively. 1 experience a reversible phase transition around 40–60 K, causing anomalies in magnetic behavior. Moreover, an irreversible single‐crystal‐to‐single‐crystal phase transition to 1′ undergo at ~381 K, inducing a rearrangement of [Ni(dmit)2]− anions and a resistivity decrease from 6.5×106 to 6.5×102 Ω cm. The susceptibility curve of 1′ was reproduced by a combination of the Curie‐Weiss and dimer models (Jdimer/kB=−407(5), θ=−26.7(5) K). The irreversible transition of 1 is the first example for such supramolecule and [Ni(dmit)2]− system to our knowledge, in opening potential new‐type materials. [ABSTRACT FROM AUTHOR]

Objective: Recent studies have revealed an association between Parkinson's disease (PD) and Fabry disease, a lysosomal storage disorder; however, the underlying mechanisms remain to be elucidated. This study aimed to investigate the enzymatic properties of serum alpha‐galactosidase A (GLA) and compared them with the clinical parameters of PD. Methods: The study participants consisted of 66 sporadic PD patients and 52 controls. We measured serum GLA activity and calculated the apparent Michaelis constant (Km) and maximal velocity (Vmax) by Lineweaver–Burk plot analysis. Serum GLA protein concentration was measured by enzyme‐linked immunosorbent assay. We examined the potential correlations between serum GLA activity and GLA protein concentration and clinical features and the plasma neurofilament light chain (NfL) level. Results: Compared to controls, PD patients showed significantly lower serum GLA activity (P < 0.0001) and apparent Vmax (P = 0.0131), but no change in the apparent Km value. Serum GLA protein concentration was lower in the PD group (P = 0.0168) and was positively associated with GLA activity. Serum GLA activity and GLA protein concentration in the PD group showed a negative correlation with age. Additionally, serum GLA activity was negatively correlated with the motor severity score and the level of plasma NfL, and was positively correlated with the score of frontal assessment battery. Interpretation: This study highlights that the lower serum GLA activity in PD is the result of a quantitative decrement of GLA protein in the serum and that it may serve as a biomarker of disease severity. [ABSTRACT FROM AUTHOR]

We present a novel zinc oxide (ZnO) optically transparent electrode (OTE) prepared by the spin‐spray technique for spectroelectrochemistry. The spin‐spray technique can deposit ZnO film at a low cost, high rate deposition, and at a low temperature (<100 °C) in a single step. This new technique provides good optical transparency and electrical conductivity for ZnO. The electrochemical and spectroelectrochemical properties of the ZnO electrode were investigated for varying thicknesses of ZnO using methylene blue as a redox indicator. A ZnO OTE chip that includes three electrodes on a glass chip was developed for thin‐layer spectroelectrochemistry. Moreover, the ZnO films were successfully applied in an electrochemical‐localized surface plasmon resonance (LSPR) method for methylene blue detection by using them as a transparent conducting substrate for loading gold nanoparticles. [ABSTRACT FROM AUTHOR]

The syntheses, aggregation motifs and solid‐state emissions of clothespin‐shaped binuclear trans‐bis(salicylaldiminato)platinum(II) complexes 1–4 bearing halogen atoms (X) at the para positions of phenoxy moieties are described and compared with those of the non‐substituted analog 5. Crystalline (±)‐4 (X = I, polymorph B) obtained by recrystallization from benzene exhibits intense phosphorescent emission under UV irradiation at ambient temperature, while crystals of (±)‐1 (X = F), (±)‐2 (X = Cl), (±)‐3 (X = Br) and (±)‐4 (X = I, polymorph A), obtained from poor solvents including hexanes, are less emissive under the same conditions. The emission intensities of these crystals increase in the order of (±)‐4 (B) > (±)‐5 > (±)‐1–3, (±)‐4 (A). XRD analysis shows that the less emissive crystals (±)‐1–3 and (±)‐4 (polymorph A) form specific aggregates in which (R)‐ and (S)‐units with "A"‐shaped conformations are aligned in an alternating, shuttlecock‐like interpenetrating manner as a result of one‐dimensional infinite X⋯π interactions. The emissive polymorph 4 (B) exhibits a significantly different non‐interpenetrating alignment of "A"‐shaped conformational units, which are firmly bound as a result of a two‐dimensional constraint network consisting of I⋯I and Pt⋯Pt interactions. The present variations in solid‐state emission are therefore explained on the basis of the constraint dimensionality of the packing units of each complex. [ABSTRACT FROM AUTHOR]

ABSTRACT Background The aim of this study was to investigate the cortical and subcortical brain structures in Parkinson's disease (PD) with visual hallucination (VH), and to elucidate the association between the proposed hypothesis of VH in PD and regional brain volume changes. Methods We used 3T magnetic resonance imaging (MRI) and voxel-based morphometry (VBM) to investigate the brain structures of PD patients with VH (PD-VH; n = 13) and PD patients without VH (PD-C; n = 13). Results The PD-VH patients showed significant cortical atrophy compared to the PD-C patients in the bilateral dorsolateral prefrontal cortex, left rostral region of the prefrontal cortex, left ventral section of the cingulate cortex, bilateral primary visual cortex, and secondary visual cortex including the left inferior occipital gyrus, right lingual cortex, right supramarginal gyrus, and left fusiform gyrus. Significant subcortical atrophic changes were observed in the white matter of the right parahippocampal gyrus, the bilateral posterior part of the cingulate gyrus, the left lingual gyrus, and the right middle occipital gyrus. Conclusions VH in PD can occur due to distinctive neuroanatomical involvement. © 2013 International Parkinson and Movement Disorder Society [ABSTRACT FROM AUTHOR]

We delineated the effects of magnetic field strength on signal intensities to facilitate the specific findings of multiple system atrophy (MSA). Fifteen patients with probable MSA were imaged by 0.35T fast spin-echo (FSE), 1.5T FSE, and 3.0T FSE using a consistent protocol, testing all field strengths on the same day. Sixty patients with probable Parkinson's disease (PD) also underwent imaging. Moderate or marked hyperintensity at the dorsolateral outer putaminal margin, hyperintensity of the putaminal body, hypointensity relative to the globus pallidus at the dorsolateral putaminal margin, and infratentorial signal changes were evaluated as specific findings for MSA. As the field strength increased, the occurrence of hyperintensity both at the dorsolateral outer putaminal margin and of the putaminal body decreased, while the occurrence of hypointensity at the dorsolateral putaminal margin increased in MSA. The occurrence of uniform mild hyperintensity of the outer putaminal margin was evident in 7% at 0.35T, 40% at 1.5T, and 47% at 3.0T in MSA and in 5% at 0.35T, 60% at 1.5T, and 75% at 3.0T in PD. However, no PD patients showed hyperintensity at the dorsolateral outer putaminal margin and that of the putaminal body. Putaminal magnetic resonance imaging (MRI) findings in MSA were altered considerably by magnetic field strength. The severity and distribution of signal changes are important for assessing putaminal MRI findings in MSA. © 2010 Movement Disorders Society [ABSTRACT FROM AUTHOR]

Angioinvasive aspergillus can lead to acute infarction. A 74-year old man complained about mild weakness of the left limbs; based on diffusion-weighted magnetic resonance imaging, he was diagnosed with acute infarction in the right caudate and anterior limb of the internal capsule. After admission, he had a fever with disturbance of consciousness. Elevated serum β-D-glucan, elevated galactomannan antigen titers in cerebrospinal fluid and histopathological analysis on a biopsy specimen of the right sphenoid sinus enabled a diagnosis of Aspergillus infection. Serial magnetic resonance angiography showed the progressing stenosis of the major cerebral artery by angioinvasive aspergillosis with the spread of the cerebral infarction. Our case suggests that serial magnetic resonance angiography might be useful for monitoring progression of aspergillus vasculopathy. [ABSTRACT FROM AUTHOR]

Objective Spinal and bulbar muscular atrophy (SBMA) is a hereditary motor neuron disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR). Animal studies have shown that the pathogenesis of SBMA is dependent on serum testosterone level. This study is aimed at evaluating the efficacy and safety of androgen deprivation by leuprorelin acetate in patients with SBMA. Methods Fifty SBMA patients underwent subcutaneous injections of leuprorelin acetate or placebo in a randomized, placebo-controlled trial for 48 weeks, followed by an open-label trial for an additional 96 weeks, in which 19 patients of the leuprorelin group and 15 of the placebo group received leuprorelin acetate. The patients who did not participate in the open-label trial were also followed up for the 96-week period (UMIN000000474). Results Leuprorelin acetate significantly extended the duration of cricopharyngeal opening in videofluorography and decreased mutant AR accumulation in scrotal skin biopsy. The patients treated with leuprorelin acetate for 144 weeks exhibited significantly greater functional scores and better swallowing parameters than those who received placebo. Autopsy of one patient who received leuprorelin acetate for 118 weeks suggested that androgen deprivation inhibits the nuclear accumulation or stabilization, or both, of mutant AR in the motor neurons of the spinal cord and brainstem. Interpretation These observations suggest that administration of leuprorelin acetate suppresses the deterioration of neuromuscular impairment in SBMA by inhibiting the toxic accumulation of mutant AR. The results of this phase 2 trial support the start of large-scale clinical trials of androgen deprivation for SBMA. Ann Neurol 2009;65:140-150 [ABSTRACT FROM AUTHOR]

Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease caused by a CAG repeat expansion in the androgen receptor gene. Because the progression of SBMA is slow, it is plausible to identify biomarkers that monitor disease course for therapeutic development. To verify whether the 6-min walk test (6MWT) is a biomarker of SBMA, we performed the 6MWT in 35 genetically confirmed patients and in 29 age-matched healthy controls. The walk distance covered within 6 min (6MWD) was significantly less in SBMA than it was in controls (323.3 ± 143.9 m and 637.6 ± 94.2 m, respectively; P < 0.001). In test-retest analysis, the intraclass correlation coefficient for the 6MWD was high in SBMA patients ( r = 0.982). In a 1-year follow-up the 6MWD significantly decreased at a rate of 11.3% per year. Our observations suggest that the 6MWT is a biomarker that can be used to monitor progression of motor impairment in SBMA. Muscle Nerve, 2008 [ABSTRACT FROM AUTHOR]

The article presents a case study of a 53 year old woman with an unusual presentation characterized by acute onset of generalized sensory symptoms associated with anhidrosis, anosmia, and ageusia. After intravenous immunoglobulin therapy, a remarkable improvement in the pain and temperature sensation was noted in her face, trunk, and extremities. The impaired pain and temperature sensation remained distributed along cranial and spinal dermatomes.

Highlights from the article: Cover Feature: Variations in the Solid-State Emissions of Clothespin-Shaped Binuclear trans-Bis(salicylaldiminato)platinum(II) with Halogen Functionalities (Eur. J. Inorg. B The Cover Feature b shows the contrasting emission properties of crystal polymorphs of a clothespin-shaped bimetallic platinum(II) complex bearing iodine atoms. B The Cover Feature b shows the contrasting emission properties of crystal polymorphs of a clothespin-shaped bimetallic platinum(II) complex bearing iodine atoms.

Pneumocephalus develops due to various reasons; however, pneumocephalus caused by factors other than head trauma is rare, and a few cases of infections have been reported. We encountered a case of pneumocephalus caused by Enterobacter cloacae meningoencephalitis following cerebral infarction. Although we could not perform cerebrospinal fluid analysis and culture, E. cloacae was cultured from blood. We conducted an autopsy and confirmed meningoencephalitis in the cerebral infarction lesion. Thus, we considered that E. cloacae meningoencephalitis caused the pneumocephalus. Because bacterial meningoencephalitis is a treatable disease, we should strictly consider that meningoencephalitis due to bacteria such as E. cloacae causes pneumocephalus. [ABSTRACT FROM AUTHOR]