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Brain Sciences
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Neuroinflammation in Neurological Diseases
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Neuroinflammation in Neurological Diseases

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Neuropharmacology and Neuropathology".

Deadline for manuscript submissions: closed (20 July 2023) | Viewed by 19360

Special Issue Editors

Dr. Lindsay T. Michalovicz

E-Mail Website
Guest Editor
Health Effects Laboratory Division, Centers for Disease Control and Prevention, National Institute for Occupational Safety and Health, Morgantown, WV 26505, USA
Interests: neuroinflammation; neurotoxicology; Gulf War illness; sickness behavior; glia; cytokine signaling
Dr. Kimberly A. Kelly

E-Mail Website
Guest Editor
Health Effects Laboratory Division, Centers for Disease Control and Prevention, National Institute for Occupational Safety and Health, Morgantown, WV 26505, USA
Interests: animal models; neurotoxicity; Gulf War illness; amphetamines; neuroinflammation

Special Issue Information

Dear Colleagues,

Neuroinflammation has become a major topic of interest and focus area in understanding the development, persistence, and consequences of many neurological disorders and diseases. Inflammation in the brain has been associated with the development of sickness behavior and sickness-behavior-related disorders, as well as neurological dysfunction and neurodegeneration. Moreover, illness-related neuroinflammation has the potential to result from direct, central inflammation or be the consequence of circulated peripheral inflammatory signals.

In this Special Issue of Brain Sciences, we aim to highlight current research that focuses on the role of neuroinflammation and neuroinflammatory signaling in the development and perpetuation of neurological disorders and disease. Authors are invited to submit their cutting-edge research related to neuroinflammation in neurological disorders and diseases, including sickness behavior, mood and sleep disorders, chemotherapy-induced cognitive impairment (i.e., chemo brain), myalgic encephalomyelitis/chronic fatigue syndrome, Gulf War illness, traumatic brain injury, neurodegenerative disease (e.g., Alzheimer’s and Parkinson’s), stroke, neurotoxic exposures, etc. These papers may include both clinical and basic science research studies.

Dr. Lindsay T. Michalovicz
Dr. Kimberly A. Kelly
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Brain Sciences is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2200 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • neuroinflammation
  • neurodegeneration
  • neurotoxicology
  • sickness behavior
  • brain disorders

Published Papers (10 papers)

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15 pages, 4121 KiB  
Article
A Projectile Concussive Impact Model Produces Neuroinflammation in Both Mild and Moderate-Severe Traumatic Brain Injury
by Lindsay T. Michalovicz, Kimberly A. Kelly, Travis J. A. Craddock and James P. O’Callaghan
Brain Sci. 2023, 13(4), 623; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci13040623 - 06 Apr 2023
Cited by 1 | Viewed by 1385
Abstract
Traumatic brain injury (TBI) is a major cause of death and disability and is experienced by nearly 3 million people annually as a result of falls, vehicular accidents, or from being struck by or against an object. While TBIs can range in severity, [...] Read more.
Traumatic brain injury (TBI) is a major cause of death and disability and is experienced by nearly 3 million people annually as a result of falls, vehicular accidents, or from being struck by or against an object. While TBIs can range in severity, the majority of injuries are considered to be mild. However, TBI of any severity has the potential to have long-lasting neurological effects, including headaches, cognitive/memory impairments, mood dysfunction, and fatigue as a result of neural damage and neuroinflammation. Here, we modified a projectile concussive impact (PCI) model of TBI to deliver a closed-head impact with variable severity dependent on the material of the ball-bearing projectile. Adult male Sprague Dawley rats were evaluated for neurobehavioral, neuroinflammatory, and neural damage endpoints both acutely and longer-term (up to 72 h) post-TBI following impact with either an aluminum or stainless-steel projectile. Animals that received TBI using the stainless-steel projectile exhibited outcomes strongly correlated to moderate-severe TBI, such as prolonged unconsciousness, impaired neurobehavior, increased risk for hematoma and death, as well as significant neuronal degeneration and neuroinflammation throughout the cortex, hippocampus, thalamus, and cerebellum. In contrast, rats that received TBI with the aluminum projectile exhibited characteristics more congruous with mild TBI, such as a trend for longer periods of unconsciousness in the absence of neurobehavioral deficits, a lack of neurodegeneration, and mild neuroinflammation. Moreover, alignment of cytokine mRNA expression from the cortex of these rats with a computational model of neuron–glia interaction found that the moderate-severe TBI produced by the stainless-steel projectile strongly associated with the neuroinflammatory state, while the mild TBI existed in a state between normal and inflammatory neuron–glia interactions. Thus, these modified PCI protocols are capable of producing TBIs that model the clinical and experimental manifestations associated with both moderate-severe and mild TBI producing relevant models for the evaluation of the potential underlying roles of neuroinflammation and other chronic pathophysiology in the long-term outcomes associated with TBI. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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13 pages, 1876 KiB  
Article
Protective Effects of Sodium Para-Aminosalicylic Acid on Lead and Cadmium Co-Exposure in SH-SY5Y Cells
by Jian-Chao Peng, Yue Deng, Han-Xiao Song, Yuan-Yuan Fang, Cui-Liu Gan, Jun-Jie Lin, Jing-Jing Luo, Xiao-Wei Zheng, Michael Aschner and Yue-Ming Jiang
Brain Sci. 2023, 13(3), 382; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci13030382 - 22 Feb 2023
Cited by 3 | Viewed by 1500
Abstract
Background: Combined exposure to lead and cadmium is common in occupational environments. However, the effects of co-exposure to Pb-Cd on neurotoxicity have not been fully clarified. Sodium para-aminosalicylic acid (PAS-Na) has previously been shown to protect neurons from Pb-induced toxicity. This study aimed [...] Read more.
Background: Combined exposure to lead and cadmium is common in occupational environments. However, the effects of co-exposure to Pb-Cd on neurotoxicity have not been fully clarified. Sodium para-aminosalicylic acid (PAS-Na) has previously been shown to protect neurons from Pb-induced toxicity. This study aimed to investigate the beneficial effect of PAS-Na against co-exposure to Pb-Cd-induced neurodegeneration in SH-SY5Y cells. Methods: The MTT assay was used to detect the effects of Pb and Cd alone, or in combination, on SH-SY5Y cell survival. The effects of Pb and Cd alone or in combination on oxidative stress were assessed by reactive oxygen species (ROS) level. Nrf2, the master switch for antioxidant responses, was detected by immunofluorescence. Protein expression levels of PI3K, Akt, p-Akt, Nrf2 and HO-1 were determined by Western blot analysis. Results: MTT assay results established that the survival rate of SH-SY5Y cells was not significantly affected by exposure to 1 μmol/L lead, 0.25 μmol/L cadmium, and 1-fold Pb-Cd mixture (1 μmol/L Pb + 0.25 μmol/L Cd), while 10-fold Pb-Cd combined exposure (10 μmol/L Pb + 2.5 μmol/L Cd) significantly reduced the survival rate of SH-SY5Y cells. Combined Pb-Cd exposure significantly increased intracellular ROS levels, and N-Acetyl-L-cysteine (NAC) treatment in the 10 μmol/L Pb + 2.5 μmol/L Cd group significantly decreased ROS expression levels, attenuating the levels of oxidative stress. Protein expression of PI3K and p-Akt significantly decreased in the 10 μmol/L Pb + 2.5 μmol/L Cd group, while the expression of PI3K and p-Akt protein increased after PAS-Na intervention. Immunofluorescence analysis showed that levels of Nrf2 in the nucleus increased in the 10 μmol/L Pb + 2.5 μmol/L Cd group, along with Nrf2 protein levels, suggesting that Nrf2 was translocated from the cytoplasm into the nucleus upon combined Pb-Cd exposure. In addition, HO-1 protein expression level, a downstream gene product of Nrf2, was increased. In response to NAC intervention, HO-1 protein expression levels significantly decreased. PAS-Na had the same intervention effect as NAC. Conclusion: Combined exposure to Pb-Cd induced oxidative stress and cytotoxicity in SH-SY5Y cells. PAS-Na displayed antagonistic effects on neurodegenerative changes induced by combined Pb-Cd exposure; hence, it may afford a novel treatment modality for exposure to these metals. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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13 pages, 2759 KiB  
Article
Chloride Intracellular Channel Protein 2 Promotes Microglial Invasion: A Link to Microgliosis in the Parkinson’s Disease Brain
by Mohammed E. Choudhury, Saya Ozaki, Noriyuki Miyaue, Taisei Matsuura, Kanta Mikami, Afsana Islam, Madoka Kubo, Rina Ando, Hajime Yano, Takeharu Kunieda, Masahiro Nagai and Junya Tanaka
Brain Sci. 2023, 13(1), 55; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci13010055 - 28 Dec 2022
Viewed by 1473
Abstract
Activated microglia potentially cause neurodegeneration in Parkinson’s disease (PD). Matrix metalloproteinase (MMP)-9 plays a crucial role in the pathogenesis of PD, but the modulator of microglial release of MMP-9 remains obscure. Given the modulatory effect of chloride intracellular channel protein 2 (CLIC2) on [...] Read more.
Activated microglia potentially cause neurodegeneration in Parkinson’s disease (PD). Matrix metalloproteinase (MMP)-9 plays a crucial role in the pathogenesis of PD, but the modulator of microglial release of MMP-9 remains obscure. Given the modulatory effect of chloride intracellular channel protein 2 (CLIC2) on MMPs, we aimed to determine the role of CLIC2 in regulating microglial MMP expression and activation. We found that CLIC2 is expressed in microglia and neurons in rat brain tissue and focused on the function of CLIC2 in primary cultured microglia. Exposure to recombinant CLIC2 protein enhanced microglial invasion activity, and its knockdown abolished this activity. Moreover, increased activation of MMP-9 was confirmed by the addition of the CLIC2 protein, and CLIC2 knockdown eliminated this activation. Additionally, increased expression of CLIC2 was observed in PD-modeled tissue. In conclusion, CLIC2 increases MMP-9 activity in the microglia, which are involved in PD pathogenesis. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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13 pages, 1336 KiB  
Article
Clinical Manifestation, Auxiliary Examination Features, and Prognosis of GFAP Autoimmunity: A Chinese Cohort Study
by Lei Liu, Boyan Fang, Zhixin Qiao, Xiaomeng Di, Qiuying Ma, Jingxiao Zhang and Jiawei Wang
Brain Sci. 2022, 12(12), 1662; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci12121662 - 03 Dec 2022
Cited by 5 | Viewed by 2233
Abstract
Objective: This paper reports the clinical manifestation and auxiliary examination features of 15 Chinese patients with glial fibrillary acidic protein (GFAP) autoimmunity. Methods: From June 2016 to December 2019, patients suspected to have neurological autoimmune disease after having their serum and cerebrospinal fluid [...] Read more.
Objective: This paper reports the clinical manifestation and auxiliary examination features of 15 Chinese patients with glial fibrillary acidic protein (GFAP) autoimmunity. Methods: From June 2016 to December 2019, patients suspected to have neurological autoimmune disease after having their serum and cerebrospinal fluid (CSF) tested for conventional neural antibodies were scanned for additional autoantibodies by immunohistochemistry. Samples that showed a characteristic immunoreactive pattern reminiscent of the GFAP of astrocytes were selected and confirmed by cell-based assay using cells-expressing human GFAPα. Results: A total of 15 patients (eight male and seven female) with a median age at onset of 53 years (range 28–72) were identified as GFAP-IgG-positive. Fourteen cases had GFAP-IgG detected in the CSF, while serum GFAP-IgG was detected in 11 cases. Eleven of the fifteen patients (73.3%) presented with an acute monophasic course, of which 10 (90.9%) had antecedent flu-like symptoms. The predominant phenotype was meningoencephalitis (46.7%), followed by meningoencephalomyelitis in 40% of the cases. The most common clinical features included long tract signs, brainstem symptoms, tremors, headaches, and psychiatric symptoms. Magnetic resonance imaging (MRI) revealed the enhancement of the meninges, the surface of the brainstem, the cerebellum, and the spinal cord as predominant. Inflammatory CSF showed mild lymphocyte-predominant pleocytosis with a median of 51/μL and elevated protein with a median of 87.5 mg/dL. Five patients had coexisting antibodies, including NMDAR-IgG in three patients and Yo and MOG-IgG in one patient each. One patient underwent a stereotactic brain biopsy, and the neuropathology diagnosis was diffuse large B-cell lymphoma. One patient had ovarian teratoma. Eleven of the fifteen (73.3%) patients received both intravenous immunoglobulin and steroids. Among them, three patients also received immunosuppressive agents later. During a two-year follow-up, 9 of the 15 (60%) patients achieved complete clinical remission. Conclusions: The clinical presentation of GFAP astrocytopathy is heterogeneous. It can be characterized by an acute monophasic course and a chronic relapsing course. Tremors are a prominent clinical manifestation in patients with an acute monophasic course with GFAP-IgG antibodies only. Most patients responded well to immunotherapy. In patients with GFAP autoimmunity, presenting with a chronic relapsing course, one should actively search for immunogenic factors and the culprit antibodies. In the case of primary central nervous system lymphoma, GFAP autoimmunity does not always equate to autoimmune GFAP astrocytopathy. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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18 pages, 7584 KiB  
Article
Regulation of Microglia-Activation-Mediated Neuroinflammation to Ameliorate Ischemia-Reperfusion Injury via the STAT5-NF-κB Pathway in Ischemic Stroke
by Zhijun Pu, Shengnan Xia, Pengfei Shao, Xinyu Bao, Dan Wu and Yun Xu
Brain Sci. 2022, 12(9), 1153; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci12091153 - 29 Aug 2022
Cited by 7 | Viewed by 1691
Abstract
Inflammatory reaction after ischemia-reperfusion contributes significantly to a worsened prognosis, and microglia activation is the main resource of inflammation in the nervous system. Targeting STAT5 has been shown to be a highly effective anti-inflammatory therapy; however, the mechanism by which the STAT5 signaling [...] Read more.
Inflammatory reaction after ischemia-reperfusion contributes significantly to a worsened prognosis, and microglia activation is the main resource of inflammation in the nervous system. Targeting STAT5 has been shown to be a highly effective anti-inflammatory therapy; however, the mechanism by which the STAT5 signaling pathway regulates neuroinflammation following brain injury induced by ischemia-reperfusion remains unclear. Dauricine is an effective agent in anti-inflammation and neuroprotection, but the mechanism by which dauricine acts in ischemia-reperfusion remained unknown. This study is the first to find that the anti-inflammation mechanism of dauricine mainly occurs through the STAT5-NF-κB pathway and that it might act as a STAT5 inhibitor. Dauricine suppresses the inflammation caused by cytokines Eotaxin, KC, TNF-α, IL-1α, IL-1β, IL-6, IL-12β, and IL-17α, as well as inhibiting microglia activation. The STAT5b mutant at Tyr-699 reverses the protective effect of dauricine on the oxygen-glucose deprivation-reperfusion injury of neurons and reactivates the P-NF-κB expression in microglia. These results suggest that dauricine might be able to suppress the neuroinflammation and protect the neurons from the injury of post-ischemia-reperfusion injury via mediating the microglia activation through the STAT5-NF-κB pathway. As a potential therapeutic target for neuroinflammation, STAT5 needs to be given further attention regarding its role in ischemic stroke. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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13 pages, 3239 KiB  
Article
Lipopolysaccharide Exposure Differentially Alters Plasma and Brain Inflammatory Markers in Adult Male and Female Rats
by Hannah A. Nonoguchi, Timothy Wee Shang Kouo, Sandhya Kortagere, Joshua Hillman, David L. Boyle and Chitra D. Mandyam
Brain Sci. 2022, 12(8), 972; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci12080972 - 24 Jul 2022
Cited by 5 | Viewed by 1845
Abstract
Humans and rodents have sexually dimorphic immune responses, which could influence the brain’s response to a systemic inflammatory insult. Lipopolysaccharide (LPS) is a stimulator of the innate immune system and is routinely used in animal models to study blood–brain barrier (BBB) dysfunction under [...] Read more.
Humans and rodents have sexually dimorphic immune responses, which could influence the brain’s response to a systemic inflammatory insult. Lipopolysaccharide (LPS) is a stimulator of the innate immune system and is routinely used in animal models to study blood–brain barrier (BBB) dysfunction under inflammatory conditions. Therefore, we examined whether inflammatory response to LPS and the associated BBB disruption differed in male and female adult rats. Rats were treated with saline or two injections of 1 mg/kg LPS and studied 24 h after the second LPS injection. Plasma isolated from trunk blood and brain tissue homogenates of the prefrontal cortex (PFC), dorsal striatum (DS), hippocampus, and cerebellum were analyzed for cytokines and chemokines using a 9-plex panel from Meso Scale Discovery. BBB disruption was analyzed with tight junction proteins claudin-5 and VE-cadherin via Western blotting and VEGF by ELISA. This allowed us to compare sex differences in the levels of individual cytokines as well as associations among cytokines and expression of tight junction proteins between the plasma and specific brain regions. Higher levels of interferon-γ, interleukin-10 (IL-10), IL-13, IL-4, CXCL-1, and VEGF in the plasma were revealed compared to the brain homogenates, and higher levels of TNFα, IL-1β, IL-6, and IL-5 in the PFC were seen compared with plasma and other brain regions in males. Females showed higher levels of plasma CXCL1 and VEGF compared to males, and males showed higher levels of PFC TNFα, IL-6, IL-4, and VEGF compared to females. LPS induced significant increases in plasma cytokines and VEGF in both sexes. LPS did not significantly alter cytokines in brain tissue homogenates, however, it increased chemokines in the PFC, DS, and hippocampus. In the PFC, LPS produced BBB disruption, which is evident as reduced expression of claudin-5 in males and reduced expression of VE-cadherin in both sexes. Taken together, our results reveal significant sex differences in pro-inflammatory cytokine and chemokine levels in plasma and brain that were associated with BBB disruption after LPS, and validate the use of multiplex assay for plasma and brain tissue samples. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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14 pages, 1546 KiB  
Article
Kappa Free Light Chains in Cerebrospinal Fluid in Inflammatory and Non-Inflammatory Neurological Diseases
by Franz Felix Konen, Philipp Schwenkenbecher, Konstantin Fritz Jendretzky, Stefan Gingele, Torsten Witte, Kurt-Wolfram Sühs, Matthias Grothe, Malte Johannes Hannich, Marie Süße and Thomas Skripuletz
Brain Sci. 2022, 12(4), 475; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci12040475 - 03 Apr 2022
Cited by 8 | Viewed by 2116
Abstract
Background: Oligoclonal bands represent intrathecal immunoglobulin G (IgG) synthesis and play an important role in the diagnosis of multiple sclerosis (MS). Kappa free light chains (KFLC) are increasingly recognized as an additional biomarker for intrathecal Ig synthesis. However, there are limited data on [...] Read more.
Background: Oligoclonal bands represent intrathecal immunoglobulin G (IgG) synthesis and play an important role in the diagnosis of multiple sclerosis (MS). Kappa free light chains (KFLC) are increasingly recognized as an additional biomarker for intrathecal Ig synthesis. However, there are limited data on KFLC in neurological diseases other than MS. Methods: This study, conducted at two centers, retrospectively enrolled 346 non-MS patients. A total of 182 patients were diagnosed with non-inflammatory and 84 with inflammatory neurological diseases other than MS. A further 80 patients were classified as symptomatic controls. Intrathecal KFLC production was determined using different approaches: KFLC index, Reiber’s diagram, Presslauer’s exponential curve, and Senel’s linear curve. Results: Matching results of oligoclonal bands and KFLC (Reiber’s diagram) were frequently observed (93%). The Reiber’s diagram for KFLC detected intrathecal KFLC synthesis in an additional 7% of the patient samples investigated (4% non-inflammatory; 3% inflammatory), which was not found by oligoclonal band detection. Conclusions: The determination of both biomarkers (KFLC and oligoclonal bands) is recommended for routine diagnosis and differentiation of non-inflammatory and inflammatory neurological diseases. Due to the high sensitivity and physiological considerations, the assessment of KFLC in the Reiber’s diagram should be preferred to other evaluation methods. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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15 pages, 2416 KiB  
Article
Lipoxin A4 Receptor Stimulation Attenuates Neuroinflammation in a Mouse Model of Intracerebral Hemorrhage
by Risa Futokoro, Masanori Hijioka, Moe Arata and Yoshihisa Kitamura
Brain Sci. 2022, 12(2), 162; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci12020162 - 26 Jan 2022
Cited by 6 | Viewed by 2751
Abstract
Intracerebral hemorrhage (ICH) is caused by the rupture of blood vessels in the brain. The excessive activation of glial cells and the infiltration of numerous inflammatory cells are observed during bleeding. Thrombin is a key molecule that triggers neuroinflammation in the ICH brain. [...] Read more.
Intracerebral hemorrhage (ICH) is caused by the rupture of blood vessels in the brain. The excessive activation of glial cells and the infiltration of numerous inflammatory cells are observed during bleeding. Thrombin is a key molecule that triggers neuroinflammation in the ICH brain. In this study, we focused on lipoxin A4 (LXA4), an arachidonic acid metabolite that has been reported to suppress inflammation and cell migration. LXA4 and BML-111, an agonist of the LXA4 receptor/formyl peptide receptor 2 (ALX/FPR2), suppressed microglial activation; LXA4 strongly inhibited the migration of neutrophil-like cells in vitro. ALX/FPR2 was expressed on neutrophils in the ICH mouse brain and the daily administration of BML-111 attenuated the motor coordination dysfunction and suppressed the production of proinflammatory cytokines in the ICH mouse brain. On the other hand, BML-111 did not show a significant reduction in the number of microglia and neutrophils. These results suggest that systemic administration of ALX/FPR2 agonists may suppress the neuroinflammatory response of microglia and neutrophils without a change in cell numbers. Additionally, their combination with molecules that reduce cell numbers, such as modulators of leukotriene B4 signaling, may be required in future studies. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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7 pages, 7289 KiB  
Case Report
Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
by Fernando Freua, João Vitor Mahler, Pedro Lucas Grangeiro de Sá Barreto Lima, Iuri Santana Neville, Leonardo Barreira Portella, Victor Hugo Rocha Marussi, Carmen Lucia Penteado Lancellotti, Paulo Ribeiro Nobrega and Guilherme Diogo Silva
Brain Sci. 2023, 13(8), 1191; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci13081191 - 11 Aug 2023
Cited by 1 | Viewed by 1397
Abstract
Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just [...] Read more.
Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or SLIPPERS. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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11 pages, 5035 KiB  
Case Report
A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
by Pei Liu, Xuemei Lin, Shenghua Zong, Yan Yan, Zhongzhong Liu, Qingli Lu, Qiaoqiao Chang and Songdi Wu
Brain Sci. 2023, 13(1), 17; https://0-doi-org.brum.beds.ac.uk/10.3390/brainsci13010017 - 22 Dec 2022
Cited by 3 | Viewed by 1633
Abstract
A nine-year-old boy manifested with headache, progressive mild cognitive decline and hemiparesis, but without clinical epileptic seizures (with abnormal EEG waves). Brain magnetic resonance imaging (MRI) showed bilateral cortical lesions mainly on the right hemisphere, and new lesions developed in frontal, parietal, occipital [...] Read more.
A nine-year-old boy manifested with headache, progressive mild cognitive decline and hemiparesis, but without clinical epileptic seizures (with abnormal EEG waves). Brain magnetic resonance imaging (MRI) showed bilateral cortical lesions mainly on the right hemisphere, and new lesions developed in frontal, parietal, occipital and temporal lobes around the old lesions presenting as a lace-like or ring-like enhancement in T1 with contrast over a disease course of five years. A suspected diagnosis of primary angiitis of the central nervous system was initially considered. Treated with high-dose corticosteroids, intravenous immunoglobulins and monthly pulse cyclophosphamide, his symptoms worsened with the intracranial lesion progression. Brain biopsy of the right frontal lobe was performed nearly five years after onset; prominent neuronal loss, a microglial nodule, as well as parenchymal and perivascular lymphocytic infiltrate within the cortex were found, which coincided with RE pathology changes. Encouragingly, after a regimen of rituximab, lesions on the follow-up brain MRI tended to be stable. Apparently, it was immune-mediated, but did not strictly fit any known disease entity, although it was similar to RE. We summarize this unique case, including clinical characteristics, imaging and pathology findings. We also discuss the diagnosis and treatment, focusing on comparison to RE as well as other possible neurological diseases. Full article
(This article belongs to the Special Issue Neuroinflammation in Neurological Diseases)
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