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1.
Artículo en Inglés | MEDLINE | ID: mdl-33547757

RESUMEN

OBJECTIVE: Increasing reports suggest a role for immunological mechanisms in febrile infection-related epilepsy syndrome (FIRES). The objective of this study was to elucidate the efficacy and safety of intrathecal dexamethasone therapy (IT-DEX). METHODS: We assessed six pediatric patients with FIRES who were administered add-on IT-DEX in the acute (n = 5) and chronic (n = 1) phases. We evaluated clinical courses and prognosis. We measured cytokines/chemokines in cerebrospinal fluid (CSF) from FIRES patients at several points, including pre- and post-IT-DEX, and compared them with control patients with chronic epilepsy (n = 12, for cytokines/chemokines) or with noninflammatory neurological disease (NIND, n = 13, for neopterin). RESULTS: Anesthesia was weaned after a median of 5.5 days from IT-DEX initiation (n = 6). There was a positive correlation between the duration from the disease onset to the introduction of IT-DEX and the length of ICU stay and the duration of mechanical ventilation. No patient experienced severe adverse events. Seizure spreading and background activities on electroencephalography were improved after IT-DEX in all patients. The levels of CXCL10, CXCL9, IFN-γ, and neopterin at pre-IT-DEX were significantly elevated compared to levels in epilepsy controls, and CXCL10 and neopterin were significantly decreased post-IT-DEX, but were still higher compared to patients with chronic epilepsy. IL-6, IL-8, and IL-1ß were significantly elevated before IT-DEX compared to epilepsy controls, though there was no significant decrease post-treatment. INTERPRETATION: IT-DEX represents a therapeutic option for patients with FIRES that could shorten the duration of the critical stage of the disease. The effect of IT-DEX on FIRES might include cytokine-independent mechanisms.

2.
Brain Dev ; 2021 Feb 17.
Artículo en Inglés | MEDLINE | ID: mdl-33610339

RESUMEN

OBJECTIVE: To elucidate the clinical characteristics of Japanese pediatric patients with acquired demyelinating diseases (ADS), positive for myelin oligodendrocyte glycoprotein antibody (MOG-IgG), we conducted a nation-wide survey. METHODS: Information about pediatric patients under 18 years old with ADS was solicited with surveys sent to 323 facilities. In an initial survey, we asked whether the center had any patients with ADS, and the MOG-IgG serostatus of the patients. In a follow-up survey, we requested more precise information on patients with ADS. RESULTS: Initial survey: 263 replies providing information on 175 patients were received. MOG-IgG were examined in 78 patients and 54 of those (69%) were positive for MOG-IgG. Follow-up survey: The characteristic involvement was optic neuritis, with visual disturbance and optic pain as characteristic symptoms. The relapse rate was 44% in patients positive for MOG-IgG, which was higher than that in seronegative patients (38%). For acute phase treatments, corticosteroid (CS), plasma exchange, and intravenous immunoglobulin (IVIG) were useful. To prevent relapse, CS, intermittent IVIG, immunosuppressants, and monoclonal antibodies were useful, but the efficacies of disease modifying drugs were uncertain. Sequelae such as visual disturbance, cognitive impairment, motor dysfunction, and epilepsy were observed in 11% of patients with MOG-IgG. CONCLUSIONS: MOG antibody-associated diseases were found to be common among pediatric ADS patients. Since a variety of sequelae were observed in these patients, it is important to identify the appropriate treatment to ensure the best outcome. The presence of the MOG autoantibody should be taken into consideration as part of the diagnostic criteria for pediatric ADS.

3.
Brain Dev ; 2021 Jan 05.
Artículo en Inglés | MEDLINE | ID: mdl-33419638

RESUMEN

BACKGROUND: Incontinentia pigmenti (IP) is an X-linked neurocutaneous disorder that can present with cerebral arteriopathy during early infancy. However, no previous reports have demonstrated arteriopathic manifestations during postinfantile childhood in patients with IP. PATIENT DESCRIPTION: We describe a case of IP in a 2-year-old girl who developed encephalopathic manifestations associated with influenza A infection. She presented diffuse magnetic resonance imaging abnormalities involving the cortices, subcortical white matter, corpus callosum, basal ganglia, and thalami, resembling the findings in early infantile cases reported in the previous literatures. Magnetic resonance angiography demonstrated attenuation of the cerebral arteries. Proinflammatory cytokines and chemokines were upregulated in the cerebrospinal fluid. Left hemiplegia remained following the remission of the arteriopathic manifestations. Genetic analyses revealed a novel type of mutation in the IKBKG gene. CONCLUSION: Our findings indicate that patients with IP can develop destructive cerebral arteriopathy even after early infancy. The similarities in magnetic resonance imaging abnormalities between our patient and the previously reported infantile patients may be explained by the underlying immunologic pathophysiology of IP.

4.
J Neuroimmunol ; 352: 577475, 2021 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-33454554

RESUMEN

In this study, we assessed circulating immune cells and plasma cytokine levels in 15 pediatric patients with drug-resistant epilepsy (DRE). DRE patients had a significantly higher percentage of CD14+ monocytes positive for IL-1ß, IL-1 receptor antagonist, IL-6, and TNF-α than controls. Significantly higher intracellular levels of IFN-γ in CD4+ T cells and NK cells were also found in DRE patients. The level of IL-1ß+ CD14+ monocytes correlated with seizure frequency, and intracellular levels of IFN-γ in NKT-like cells were negatively correlated with the duration of epilepsy. Peripheral immune cells might be involved in the pathogenesis of DRE.

5.
Exp Neurol ; 337: 113552, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33309748

RESUMEN

Alzheimer's disease (AD), a progressive neurodegenerative disorder, is a major societal, scientific, and economic problem. Several early-life factors associated with an increased risk for the clinical diagnosis of AD have recently been identified. In the present study, we investigated the involvement of early-life stress in the pathogenesis of AD using heterozygous amyloid precursor protein (APP) mutant mice (AppNL-G-F/wt) and wild-type (Appwt/wt) mice. We found that maternal-separated Appwt/wt mice showed narrowing of vessels and decreased pericyte coverage of capillaries in the prefrontal cortex, while maternal-separated AppNL-G-F/wt mice additionally showed the impairment of cognitive function, earlier formation of Aß plaques, increased vessel-associated microglia, and disruption of the blood-brain barrier. Substantial activation of microglia was detected in the maternal-separated AppNL-G-F/wt mice and maternal-separated Appwt/wt mice. At an early stage, morphological changes and inflammatory responses were observed in the microglia of the maternal-separated AppNL-G-F/wt mice and maternal-separated Appwt/wt mice, and morphological changes in the microglia were observed in the non-maternal-separated AppNL-G-F/wt mice. Microglia activation induced by maternal separation in combination with the APP mutation may impair the vascular system, leading to AD progression. These findings therefore suggest that maternal separation results in the early induction of AD-related pathology via angiopathy.

6.
Brain Dev ; 43(1): 2-31, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32829972

RESUMEN

The cardinal symptom of acute encephalopathy is impairment of consciousness of acute onset during the course of an infectious disease, with duration and severity meeting defined criteria. Acute encephalopathy consists of multiple syndromes such as acute necrotizing encephalopathy, acute encephalopathy with biphasic seizures and late reduced diffusion and clinically mild encephalitis/encephalopathy with reversible splenial lesion. Among these syndromes, there are both similarities and differences. In 2016, the Japanese Society of Child Neurology published 'Guidelines for the Diagnosis and Treatment of Acute Encephalopathy in Childhood', which made recommendations and comments on the general aspects of acute encephalopathy in the first half, and on individual syndromes in the latter half. Since the guidelines were written in Japanese, this review article describes extracts from the recommendations and comments in English, in order to introduce the essence of the guidelines to international clinicians and researchers.

7.
Cytokine ; 137: 155324, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33032108

RESUMEN

Patients with hemorrhagic shock and encephalopathy syndrome (HSES) have a high early mortality rate, which may be caused by a 'cytokine storm'. However, there is little information on how cytokines and chemokines change over time in these patients. We aimed to describe the characteristics of HSES by examining changes in serum biomarker levels over time. Six patients with HSES were included. We retrospectively evaluated their clinical course and imaging/laboratory data. We measured serum levels of multiple cytokines [interleukin 1ß (IL-1ß), IL-2, IL-4, IL-6, IL-10, IL-17, interferon-gamma, and tumor necrosis factor alpha], chemokines (IL-8, monocyte chemoattractant protein-1, interferon-inducible protein-10), and growth and differentiation factor (GDF)-15. The highest cytokine and chemokine levels were noted in the first 24 h, and decreased thereafter. The GDF-15 level was markedly high. Cytokine, chemokine, and GDF-15 levels were significantly higher in patients with HSES than in controls in the first 24 h, except for IL-2 and IL-4. Patients with HSES have high inflammatory cytokine and chemokine levels, a high GDF-15 level in the first 24 h, and high lactate levels. Our study provides new insights on the pathophysiology of HSES, a detailed clinical picture of patients with HSES, and potential biomarkers.

8.
Molecules ; 25(14)2020 Jul 19.
Artículo en Inglés | MEDLINE | ID: mdl-32707694

RESUMEN

In this study, we evaluated the magnetization properties of a magnetic alloy with single-crystalline cubic nanostructures, in order to clarify its magnetocrystalline anisotropy. Upon applying a specific annealing treatment to the CuNiFe base material, the precipitated magnetic particles grew into cubic granules, resulting in the formation of nanometric cubic single crystals of magnetic CuNiFe in a nonmagnetic Cu-rich matrix. The cubic nanostructures of CuNiFe were oriented along their crystallographic axis, in the <100> direction of the face-centered-cubic structure. We evaluated the static magnetization properties of the sample, which originated primarily from the CuNiFe nanocubes precipitated in the Cu-rich matrix, under an applied DC magnetic field. The magnetocrystalline anisotropy was readily observed in the magnetization curves. The <111> axis of the CuNiFe was observed to be the easy axis of magnetization. We also investigated the dynamic magnetization properties of the sample under an AC magnetic field. By subtracting the magnetic signal induced by the eddy current from the magnetization curves of the sample, we could obtain the intrinsic AC magnetization properties of the CuNiFe nanocubes.

9.
Brain Dev ; 42(7): 508-514, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32360071

RESUMEN

BACKGROUND: We previously reported the nationwide, epidemiological data of acute encephalopathy in Japan during 2007-2010. Here we conducted the second national survey of acute encephalopathy during 2014-2017, and compared the results between the two studies to elucidate the trends in the seven years' interval as well as the influence of changes in pediatric viral infections and guidelines for acute encephalopathy in Japan. METHODS: The Research Committee on Acute Encephalopathy supported by the Japanese Government sent a questionnaire to 507 hospitals throughout Japan, and collected the responses by mail. RESULTS: A total of 1115 cases from 267 hospitals reportedly had acute encephalopathy during April 2014-June 2017. In this study, the age at onset was younger, the ratios of recently established syndromes, such as acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) and clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS), were higher, and the ratio of influenza-associated encephalopathy was lower, than in the previous study. The age at onset of influenza-associated encephalopathy was lower, and that of HHV-6/7-associated encephalopathy higher, compared to the first survey. The outcomes of entire acute encephalopathy remained unchanged. CONCLUSION: Some of these changes reflected the recent trends of viral infectious diseases including 2009 influenza pandemic, and others the standardization of the diagnosis of acute encephalopathy in Japan.

11.
Biomed J ; 43(3): 205-210, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32330681

RESUMEN

Febrile infection-related epilepsy syndrome (FIRES) is an intractable neurological disease characterized by an unexplained refractory status epilepticus triggered by febrile infection. A Consensus definition of FIRES was proposed in 2018, and its clinical features and prognosis are gradually being clarified. However, the development of effective treatments has been hindered as the etiology of this rare disease is as yet unelucidated. The basic approach to the management of FIRES, like other forms of epilepsy, is based on the control of seizures, however seizures are extremely intractable and require intravenous administration of large doses of anticonvulsants, mainly barbiturates. This treatment strategy produces various complications including respiratory depression and drug hypersensitivity syndrome, which make it more difficult to control seizures. Consequently, it is crucial to predict these events and to formulate a planned treatment strategy. As well, it is important to grow out of conventional treatment strategies that rely on only anticonvulsants, and alternative therapies are gradually being developed. One such example is the adoption of a ketogenic diet which may lead to reduced convulsions as well as improve intellectual prognosis. Further, overproduction of inflammatory cytokines in the central nervous system has been shown to be strongly related to the pathology of FIRES which has led to attempts at immunomodulation therapy including anti-cytokine therapy.

12.
Eur J Immunol ; 50(2): 205-219, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31549730

RESUMEN

The NLRP3 inflammasome is a molecular complex that translates signals from pathogens and tissue damage into inflammatory responses, and plays crucial roles in numerous neurological diseases. Activation of the NLRP3 inflammasome leads to caspase-1 dependent cleavage of pro-IL-1ß to form mature IL-1ß. By acting on the P2X7 purinergic receptor, extracellular ATP is one of the major stimuli that activates the NLRP3 inflammasome. Although microglia express multiple purinergic receptors, their roles in inflammasome-mediated inflammation are largely unknown. We studied the role of the P2Y12 receptor, a metabotropic P2Y receptor enriched in microglia, on inflammation in vitro. Inhibition of the microglial P2Y12 receptor by PSB0739 or siRNA knockdown suppressed IL-1ß release. P2Y12 receptor-deficient microglia displayed markedly attenuated IL-1ß mRNA expression and release. P2Y12 receptor blockade also suppressed IL-6 production. Both IL-1ß and IL-6 responses were augmented by extracellular ADP or ADP-ßS and were abrogated by PSB0739. Mechanistically, ADP-ßS potentiated NF-κB activation. In addition, ADP altered mitochondrial membrane potential in combination with ATP and increased the number of caspase-1 positive cells through the P2Y12 receptor. These results elucidate a novel inflammatory mechanism by which extracellular ADP acts on the P2Y12 receptor to activate NF-κB and the NLRP3 inflammasome to enhance microglial inflammation.


Asunto(s)
Adenosina Difosfato/metabolismo , Inflamasomas/metabolismo , FN-kappa B/metabolismo , Receptores Purinérgicos P2Y12/metabolismo , Animales , Caspasa 1/metabolismo , Línea Celular , Citocinas/metabolismo , Regulación de la Expresión Génica/fisiología , Inflamación/metabolismo , Mediadores de Inflamación/metabolismo , Interleucina-1beta/metabolismo , Macrófagos/metabolismo , Ratones , Especies Reactivas de Oxígeno/metabolismo , Transducción de Señal/fisiología
13.
Brain Dev ; 42(3): 264-269, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31843295

RESUMEN

OBJECTIVE: Some pediatric patients with autoimmune encephalitis (AE) experience sequelae in spite of immunotherapy. In this study, we aimed to evaluate the association of serum matrix metallopeptidase-9 (MMP-9) and tissue inhibitor of metalloproteinase-1 (TIMP-1) levels with the neurological prognosis of AE. METHODS: We retrospectively included 13 patients with AE who had been referred to Saitama Children's Medical Center from February 2011 to May 2019. We compared serum MMP-9 levels, TIMP-1 levels, and MMP-9/TIMP-1 ratio in the acute period (within 30 days from the onset of AE) and subacute period (30-day period following the acute period). We also compared these biomarker levels between patients with (group A) and without sequelae (group B). Sequelae were evaluated at discharge or the last visit. RESULTS: Group A (median age, 7.8 years; range, 5.3-10.7 years) and group B (median age, 13.3 years; range, 11.1-15.4 years) had 6 patients each; 1 patient was excluded because the time of AE onset was unknown. In the acute period, there were no significant differences in MMP-9 levels, TIMP-1 levels, and MMP-9/TIMP-1 ratio between groups A and B. In the subacute period, serum MMP-9/TIMP-1 ratio was higher in group A than in group B (p < 0.01). There were no significant differences in MMP-9 and TIMP-1 levels between groups A and B. CONCLUSIONS: Patients with sequelae of AE showed a high MMP-9/TIMP-1 ratio in the subacute period. Our study demonstrates that elevation of serum MMP-9/TIMP-1 ratio in the subacute period may be a predictive factor of sequelae of AE.


Asunto(s)
Enfermedades Autoinmunes del Sistema Nervioso/sangre , Encefalitis/sangre , Metaloproteinasa 9 de la Matriz/sangre , Inhibidor Tisular de Metaloproteinasa-1/sangre , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Retrospectivos
14.
Nat Commun ; 10(1): 4896, 2019 10 25.
Artículo en Inglés | MEDLINE | ID: mdl-31653859

RESUMEN

Salinity, pH, and redox states are fundamental properties that characterize natural waters. These properties of surface waters on early Mars reflect palaeoenvironments, and thus provide clues on the palaeoclimate and habitability. Here we constrain these properties of pore water within lacustrine sediments of Gale Crater, Mars, using smectite interlayer compositions. Regardless of formation conditions of smectite, the pore water that last interacted with the sediments was of Na-Cl type with mild salinity (~0.1-0.5 mol/kg) and circumneutral pH. To interpret this, multiple scenarios for post-depositional alterations are considered. The estimated Na-Cl concentrations would reflect hyposaline, early lakes developed in 104-106-year-long semiarid climates. Assuming that post-depositional sulfate-rich fluids interacted with the sediments, the redox disequilibria in secondary minerals suggest infiltration of oxidizing fluids into reducing sediments. Assuming no interactions, the redox disequilibria could have been generated by interactions of upwelling groundwater with oxidized sediments in early post-depositional stages.

15.
ACS Appl Mater Interfaces ; 11(31): 27954-27963, 2019 Aug 07.
Artículo en Inglés | MEDLINE | ID: mdl-31348636

RESUMEN

Rosette-like layered double hydroxide (roseLDH) crystals with interlayer CO32- anions were synthesized by the reaction of Mg(NO3)2, Al(NO3)3, and hexamethylenetetramine (HMT) at 140 °C over 4 days. Crystals as large as 20 µm were produced when using a specific range of HMT concentrations. The substitution of CO32- interlayer ions with ClO4- or Cl- anions was achieved by the addition of perchloric acid or hydrochloric acid, respectively, to dispersion of material in methanol. The products were denoted as CO32-roseLDH, ClO4-roseLDH, and Cl-roseLDH, respectively. These LDHs were characterized using X-ray diffraction under controlled relative humidity, as well as by Fourier transform infrared spectroscopy and scanning electron microscopy. Adsorption experiment with anions such as phosphate (HPO42-) and nitrate (NO3-) was conducted by using ClO4-roseLDH and Cl-roseLDH. The results indicate that both anions were adsorbed through an ion-exchange mechanism. The maximum HPO42- adsorption capacity at equilibrium on ClO4-roseLDH was 1.6 mmol g-1 (49.6 mg P g-1), which corresponds to approximately 75% of the total positive layer charge. Cl-roseLDH showed a similar adsorption capability. Commercially available platelike LDH particles were essentially impermeable to water flow due to clogging, while the roseLDH crystals showed excellent permeability, an order of magnitude higher than that exhibited by the platelike LDH synthesized using a homogeneous precipitation method with different growth conditions. Anion adsorption during batch and flow-through test with the ClO4-roseLDH (mean particle diameter ∼ 38 µm) in a packed bed showed good uptake of HPO42- and NO3- from aqueous solutions. These results demonstrate the potential of roseLDH materials to serve as a column filler adsorbent of the hazardous anions.

16.
Pediatr Neurol ; 100: 60-66, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31272782

RESUMEN

BACKGROUND: Ataxia telangiectasia is an autosomal recessive disorder characterized by cerebellar ataxia, telangiectases, immune defects, and a predisposition to malignancy. Quality of life is severely impaired by neurological symptoms. However, curative options for the neurological symptoms are limited. Recent studies have demonstrated short-term improvement in neurological symptoms with betamethasone therapy. However, the long-term and adverse effects of betamethasone are unclear. The aim of this study was to evaluate the long-term effects, benefits, and adverse effects of low-dose betamethasone in ataxia telangiectasia. METHODS: Six patients with ataxia telangiectasia received betamethasone at 0.02 mg/kg/day for two years. After cessation of betamethasone, the patients were observed for two additional years. Neurological assessments were performed, and adverse effects were monitored every three months throughout the four-year study period. RESULTS: Transient improvement of neurological symptom was observed in five of the six patients. However, after two years betamethasone treatment, only one of the six patients showed a slight improvement in the neurological score, one patient showed no change, and the neurological scores of the remaining four patients deteriorated. After the cessation of betamethasone treatment, neurological symptoms worsened in all patients. As an adverse effect of betamethasone, transient adrenal dysfunction was observed in all cases. CONCLUSIONS: Although these findings are in agreement with previous studies suggesting that short-term betamethasone treatment transiently benefits patients with ataxia telangiectasia, the long-term benefits and risks should be carefully considered.


Asunto(s)
Ataxia Telangiectasia/tratamiento farmacológico , Betametasona/farmacología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Glucocorticoides/farmacología , Evaluación de Resultado en la Atención de Salud , Adolescente , Enfermedades de las Glándulas Suprarrenales/inducido químicamente , Betametasona/administración & dosificación , Betametasona/efectos adversos , Niño , Femenino , Glucocorticoides/administración & dosificación , Glucocorticoides/efectos adversos , Humanos , Estudios Longitudinales , Masculino , Enfermedades del Sistema Nervioso Periférico/inducido químicamente
17.
Int Cancer Conf J ; 8(1): 24-28, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31149542

RESUMEN

Molecular targeted drugs, such as everolimus and sunitinib, have shown efficacy against advanced pancreatic neuroendocrine tumors. Octreotide, a somatostatin analogue, improves the hormone-related symptoms in patients with neuroendocrine tumors. Furthermore, it has been reported that octreotide has antitumor activity in patients with metastatic midgut neuroendocrine tumors. However, whether octreotide has anti-proliferative effects in patients with advanced pancreatic neuroendocrine tumors is not fully understood. We report a 71-year-old man with multiple liver metastases of pancreatic neuroendocrine tumor. He was treated with everolimus 10 mg daily and sunitinib 25 mg daily on days 1-14 every 3 weeks at the physician's discretion. However, these molecular targeted drugs were discontinued due to disease progression or severe adverse effects. Octreotide long-acting repeatable was administered continuously from the initiation of everolimus treatment. The tumor marker level markedly decreased and the metastatic liver lesions showed shrinkage with octreotide treatment. Immunohistochemistry of tumor specimens obtained before treatment showed that somatostatin receptor 2, a high-affinity receptor for octreotide, was highly expressed. The clinical course of this patient suggested that octreotide long-acting repeatable may be a treatment option for advanced pancreatic neuroendocrine tumors after failure of everolimus and sunitinib. Further clinical trials are warranted to determine whether the expression of somatostatin receptor 2 in tumor tissues is predictive of octreotide efficacy.

18.
Case Rep Pediatr ; 2018: 6047318, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30345135

RESUMEN

Immune-mediated central nervous system manifestations of group A ß-hemolytic Streptococcus (GABHS) infection include Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)-which includes tic and obsessive compulsive disorders-and a variety of neurobehavioral disorders. We report a case of Streptococcus dysgalactiae subspecies equisimilis (group G Streptococcus) (GGS) infection associated with involuntary movements, complex tics, and emotional lability in an 11-year-old Japanese girl. Serum IgM and IgG antibodies to lysoganglioside were positive, and she responded rapidly to intravenous immunoglobulin treatment. Neuropsychiatric disorder associated with GGS infection was ultimately diagnosed. The present findings suggest that neuropsychiatric disorders can result from GGS infection and that the pathogenic mechanism is similar to that of GABHS infection. Future large-scale studies should examine the relation between GGS infection and onset of neuropsychiatric disorder.

19.
Gan To Kagaku Ryoho ; 45(7): 1113-1116, 2018 Jul.
Artículo en Japonés | MEDLINE | ID: mdl-30042284

RESUMEN

We report a case ofrecurrent sigmoid cancer in which long-term disease control was achieved by intermittent oxaliplatin (L-OHP)administration. A 58-year-old woman underwent first-line chemotherapy with capecitabine and L-OHP(CapeOX) following peritoneal lymph node recurrence of sigmoid cancer. Tumor shrinkage was confirmed by a computed tomography (CT)scan following 4 courses of CapeOX treatment. However, L-OHP administration was discontinued by the 9 course due to peritoneal neuropathy. L-OHP was reintroduced following tumor progression confirmed by CT or elevation of carcinoembryonic antigen levels detected by a blood test. This stop-and-go strategy controlled lymph node recurrence effectively for over 5 years and was not associated with the development ofperitoneal neuropathy. We suggest that the intermittent administration ofL -OHP-containing chemotherapy is an important treatment option for some patients with advanced colorectal cancer, achieving effective long-term disease control with no associated peripheral neuropathy.


Asunto(s)
Antineoplásicos/uso terapéutico , Compuestos Organoplatinos/uso terapéutico , Neoplasias del Colon Sigmoide/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Femenino , Humanos , Persona de Mediana Edad , Oxaliplatino , Enfermedades del Sistema Nervioso Periférico , Factores de Tiempo , Resultado del Tratamiento
20.
Brain Dev ; 40(10): 947-951, 2018 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-29929751

RESUMEN

Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most common pediatric encephalopathy in Japan, however, the exact neuropathology remains uncertain. The postmortem neuropathology in a patient with AESD revealed reduction of myelinated axons with early stage of astrocytosis in the absence of neuronal loss, which suggests the primary pathological damage in AESD involves myelinated axons and astrocytes rather than cortical neurons. An increased number of gemistocytic astrocytes at the corticomedullary junction may cause reduced diffusion, leading to the so-called bright tree appearance on magnetic resonance imaging, characteristic to AESD.


Asunto(s)
Anomalías Múltiples/patología , Axones/patología , Epilepsia/patología , Gliosis/patología , Síndrome de la Trisomía 18/patología , Autopsia , Epilepsia/fisiopatología , Resultado Fatal , Humanos , Lactante , Masculino
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