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European Journal of Paediatric... May 2024Patients with myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) clinically present e.g. with acute disseminated encephalomyelitis (ADEM), optic...
BACKGROUND AND OBJECTIVES
Patients with myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) clinically present e.g. with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), transverse myelitis (TM) or aquaporin-4-IgG (AQP4-IgG) negative neuromyelitis optica spectrum disorders (NMOSD)-like phenotypes. We aimed to analyze and compare blood parameters in children with MOGAD, AQP4-IgG-positive NMOSD (hence NMOSD), multiple sclerosis (MS) and healthy controls (HC).
METHODS
We evaluated differences in complete blood counts (CBC), neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR) and C-reactive protein (CRP) between these four groups and within the groups between clinical attack, acute treatment and remission.
RESULTS
Our cohort consisted of 174 children and adolescents with a total of 550 timepoints: 66 patients had MOGAD (202 timepoints), 11 NMOSD (76 timepoints), 58 MS (219 timepoints) and 39 were HC (53 timepoints). At clinical attack, leukocyte counts were elevated in MOGAD compared to remission (p < 0.001) and compared to all other groups (p < 0.001). NLR was high in MOGAD and NMOSD, and PLR was high in NMOSD, however, after correction for multiple testing these findings did not remain significant. While glucocorticoids caused an increase of leukocyte counts and NLR in NMOSD and MS, these values remained stable during acute treatment in MOGAD. In remission, NLR normalized in MOGAD, while it stayed high in NMOSD. PLR increased in NMOSD and was significantly higher compared to all other groups.
DISCUSSION
Some blood parameters, mainly leukocyte and differential counts, might help clinicians to evaluate disease activity, differentiate relapses from pseudo-relapses and even distinguish between different disease entities.
Topics: Humans; Child; Myelin-Oligodendrocyte Glycoprotein; Female; Male; Adolescent; Neuromyelitis Optica; Autoantibodies; Multiple Sclerosis; Child, Preschool; Aquaporin 4; C-Reactive Protein; Encephalomyelitis, Acute Disseminated
PubMed: 38705015
DOI: 10.1016/j.ejpn.2024.04.011 -
Heliyon Apr 2024Central nervous system (CNS) tuberculosis is a post-primary form of tuberculosis. It has high mortality and morbidity rates despite early diagnosis and treatment. CNS... (Review)
Review
Central nervous system (CNS) tuberculosis is a post-primary form of tuberculosis. It has high mortality and morbidity rates despite early diagnosis and treatment. CNS tuberculosis can manifest as subacute/chronic meningitis, parenchymal tuberculous lesions, and spinal tuberculosis. Hematogenous spread of tuberculous bacilli to the brain results in the development of so called "rich foci" on the pial surface, ependyma, and grey-white matter junction. Rupture of these "rich foci" into the subarachnoid space triggers an intense granulomatous inflammatory reaction. Tuberculous meningitis can manifest as leptomeningitis or pachymeningitis. Intracranial parenchymal tuberculous lesions may present as tuberculoma, tuberculous abscess, cerebritis, rhombencephalitis, and encephalopathy, with atypical presentations not uncommon. Complications of CNS tuberculosis encompass hydrocephalus, syrinx formation, vasculitis, infarcts, neuritis, and enduring neurological deficits. Post-contrast 3D fluid-attenuated inversion recovery (FLAIR) and post-contrast T1 spin-echo sequences excel in detecting tuberculous meningitis compared to other conventional magnetic resonance imaging (MRI) sequences. In proton magnetic resonance spectroscopy (PMRS), the presence of a lipid peak at 1.3 ppm is indicative of tuberculous lesions. Magnetization transfer (MT) imaging enhances the detection of tuberculous lesions, as the magnetization transfer ratio (MTR) of tuberculous pathologies, owing to their high lipid content, is lower than that in bacterial or fungal pathologies and higher than that in viral pathologies. This review article delves into the various typical and atypical imaging presentations of CNS tuberculosis in MRI, along with recent advances in imaging techniques.
PubMed: 38699716
DOI: 10.1016/j.heliyon.2024.e29779 -
Cureus Apr 2024Background and objective The COVID-19 pandemic and mucormycosis epidemic in India made research on the radiological findings of COVID-19-associated mucormycosis...
COVID-19-Associated Rhino-Orbito-Cerebral Mucormycosis: A Single Tertiary Care Center Experience of Imaging Findings With a Special Focus on Intracranial Manifestations and Pathways of Intracranial Spread.
Background and objective The COVID-19 pandemic and mucormycosis epidemic in India made research on the radiological findings of COVID-19-associated mucormycosis imperative. This study aims to describe the imaging findings in COVID-19-associated mucormycosis, with a special focus on the intracranial manifestations. Methodology Magnetic resonance imaging (MRI) scans of all patients with laboratory-proven mucormycosis and post-COVID-19 status, for two months, at an Indian Tertiary Care Referral Centre, were retrospectively reviewed, and descriptive statistical analysis was carried out. Results A total of 58 patients (47 men, 81%, and 11 women, 19%) were evaluated. Deranged blood glucose levels were observed in 47 (81%) cases. The intracranial invasion was detected in 31 (53.4%) patients. The most common finding in cases with intracranial invasion was pachymeningeal enhancement (28/31, 90.3%). This was followed by infarcts (17/31, 55%), cavernous sinus thrombosis (11/58, 18.9%), fungal abscesses (11/31, 35.4%), and intracranial hemorrhage (5/31, 16.1% cases). The perineural spread was observed in 21.6% (11/51) cases. Orbital findings included extraconal fat and muscle involvement, intraconal involvement, orbital apicitis, optic neuritis, panophthalmitis, and orbital abscess formation in decreasing order of frequency. Cohen's kappa coefficient of interrater reliability for optic nerve involvement and cavernous sinus thrombosis was 0.7. Cohen's coefficient for all other findings was 0.8-0.9. Conclusions COVID-19-associated rhino-orbito-cerebral mucormycosis has a plethora of orbital and intracranial manifestations. MRI, with its superior soft-tissue resolution and high interrater reliability, as elucidated in this study, is the imaging modality of choice for expediting the initial diagnosis, accurately mapping out disease extent, and promptly identifying and scrupulously managing its complications.
PubMed: 38699084
DOI: 10.7759/cureus.57441 -
Frontiers in Neurology 2024Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is...
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is characterized by optic neuritis and meningoencephalomyelitis. We report the case of a 55-year-old man, otherwise healthy, who presented with isolated headaches for three months, without other features of meningoencephalitis or myelitis. His neurological examination and fundoscopy were unremarkable. Gadolinium-enhanced brain MRI demonstrated increased T2 hyperintensity within the right sub-lenticular basal ganglia, with additional leptomeningeal enhancement along the bilateral perisylvian regions and mesial temporal lobes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, elevated protein, matching oligoclonal bands, and a negative infectious and cytological workup. Cell-based assays for anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, autoimmune encephalitis panel, and vasculitis workup were all negative, except for CSF positivity for GFAP α antibody. Oncological screening, including CT of the chest, abdomen, pelvis, and scrotal US, was unremarkable. Immunotherapy with high-dose intravenous steroids for five days and subsequent single four-weekly doses resulted in the resolution of both clinical and radiographic features, with a maintained status 24 months after onset. This case highlights isolated headache and basal ganglia, mesial temporal lobe involvement as a rare presentation of autoimmune GFAP astrocytopathy.
PubMed: 38699059
DOI: 10.3389/fneur.2024.1366263 -
Neurology(R) Neuroimmunology &... Jul 2024Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating disease rarely associated with malignancy. We report the clinical,...
OBJECTIVES
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating disease rarely associated with malignancy. We report the clinical, MRI, immunopathology, and treatment response in a person with MOGAD and melanoma.
METHODS
This is a case report of a person with a multidisciplinary evaluation at a tertiary referral center.
RESULTS
A 52-year-old man presented with progressive encephalomyelitis that led to identification of metastatic melanoma. Investigations revealed positive MOG-IgG at high titers in serum (1:1,000; normal, <1:20) and CSF (1:4,096; normal, <1:2). MRI demonstrated multifocal T2 lesions with enhancement in the brain and spine. Brain biopsy showed demyelination and inflammation. MOG immunostaining was not present in the tumor tissue. He initially improved with methylprednisolone, plasmapheresis, prolonged oral steroid taper, and cancer-directed treatment with BRAF and MEK 1/2 inhibitors, but then developed bilateral optic neuritis. IV immunoglobulin (IVIG) was initiated. Five months later, he developed metastases and immune checkpoint inhibitor (ICI) treatment was started, which precipitated optic neuritis and myelitis despite IVIG and prednisone. Tocilizumab, an interleukin-6 receptor blocker, was started with excellent and sustained clinical and radiologic response.
DISCUSSION
This case revealed a presentation of MOGAD concurrent with melanoma without tumor MOG immunostaining. We highlight tocilizumab as a dual-purpose treatment of MOGAD and the neurologic immune-related adverse effect of ICI.
Topics: Humans; Male; Melanoma; Middle Aged; Myelin-Oligodendrocyte Glycoprotein; Immune Checkpoint Inhibitors; Autoantibodies; Demyelinating Autoimmune Diseases, CNS
PubMed: 38696737
DOI: 10.1212/NXI.0000000000200249 -
Frontiers in Neurology 2024Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system characterized by recurrent, disabling attacks that affect the...
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system characterized by recurrent, disabling attacks that affect the optic nerve, spinal cord, and brain/brainstem. While rituximab, targeting CD20-positive B-cells, is used as an off-label therapy for NMOSD, some patients continue to exhibit breakthrough attacks and/or adverse reactions. Inebilizumab, a humanized and glycoengineered monoclonal antibody targeting CD19-positive B-cells, has been FDA approved for the treatment of NMOSD in adult patients who are anti-aquaporin-4 (AQP4) antibody positive. Given the limited real-world data on the efficacy and safety of inebilizumab, especially in those transitioning from rituximab, a retrospective chart review was conducted on 14 NMOSD patients from seven centers. Of these, 71.4% ( = 10) experienced a combined 17 attacks during rituximab treatment, attributed to either breakthrough disease ( = 10) or treatment delay ( = 7). The mean duration of rituximab treatment was 38.4 months (3.2 years). Notably, no subsequent attacks were observed during inebilizumab treatment [mean duration of inebilizumab treatment was 19.3 months (1.6 years)], underscoring its potential as an effective treatment for NMOSD. Our data suggest that inebilizumab provides clinical benefit with effective disease control and a favorable safety profile for patients transitioning from rituximab.
PubMed: 38689876
DOI: 10.3389/fneur.2024.1352779 -
Oncology Research 2024Bortezomib results in peripheral neuropathy (PN) in approximately 50% of patients, during multiple myeloma (MM) treatment, a complication known as Bortezomib-induced...
BACKGROUND
Bortezomib results in peripheral neuropathy (PN) in approximately 50% of patients, during multiple myeloma (MM) treatment, a complication known as Bortezomib-induced peripheral neuropathy (BIPN). The drug response varies among individuals. Genetic factor may play an important role in BIPN.
METHODS
A next-generation sequencing (NGS) panel containing 1659 targets from 233 genes was used to identify risk variants for developing BIPN in 204 MM patients who received bortezomib therapy. mRNA expression of and in 62 peripheral blood samples was detected by real-time quantitative PCR (RT-qPCR). Serum homocysteine (Hcy) levels were detected in 40 samples by chemiluminescent microparticle immunoassay (CMIA).
RESULTS
Compared with the non-BIPN group (n = 89), a total of 8 significantly associated single nucleotide polymorphisms (SNPs) were identified in the BIPN group (n = 115): (rs1801131, rs1801133, rs17421511), (rs1051740), (rs2016848), (rs6151031), (rs1935349) and (rs8192720). The mRNA expression level of in newly diagnosed patients with peripheral neuritis after treatment (NP group) was lower than that of newly diagnosed patients without peripheral neuritis after treatment (NnP group) (1.70 ± 0.77 . 2.81 ± 0.97, = 0.009). Serum Hcy levels were significantly higher in BIPN group than in non-BIPN group (11.66 ± 1.79 μmol/L . 8.52 ± 3.29 μmol/L, = 0.016) and healthy controls (11.66 ± 1.79 μmol/L . 8.55 ± 2.13 μmol/L, ≤ 0.001).
CONCLUSION
and BIPN are linked in Chinese MM patients. BIPN is more likely to occur in patients with lower mRNA expression, which might result in higher serum Hcy levels.
Topics: Humans; Bortezomib; Peripheral Nervous System Diseases; Male; Female; Middle Aged; Polymorphism, Single Nucleotide; Multiple Myeloma; Aged; Methylenetetrahydrofolate Reductase (NADPH2); Asian People; Aldehyde Dehydrogenase 1 Family; Antineoplastic Agents; Retinal Dehydrogenase; Genetic Predisposition to Disease; Adult; China; High-Throughput Nucleotide Sequencing; East Asian People
PubMed: 38686049
DOI: 10.32604/or.2023.043922 -
Medicina 2024
Topics: Humans; Hypesthesia; Lower Extremity; Magnetic Resonance Imaging; Optic Neuritis; Spasm; Aged
PubMed: 38683534
DOI: No ID Found -
Journal of Integrative Neuroscience Apr 2024Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system characterized by severe attacks of optic neuritis, myelitis,... (Review)
Review
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system characterized by severe attacks of optic neuritis, myelitis, and/or area postrema. Advances in understanding the pathophysiology of NMOSD have led to improved diagnostic and therapeutic approaches. There has been a notable increase in research efforts worldwide, including in Latin America (LATAM). In recent years, LATAM has witnessed a surge in research on NMOSD, resulting in a growing body of evidence on various aspects such as epidemiology, clinical manifestations, paraclinical features (including AQP4-IgG [Aquaporin-4-immunoglobulin G] and imaging), acute and long-term treatment strategies, as well as accessibility to diagnostic tests. This narrative review aims to present the most relevant findings from different NMOSD cohorts in LATAM, providing a comprehensive overview of the current understanding of the disease in the region, while considering its unique characteristics and challenges. LATAM-focused evidence is crucial for adding valuable information to the international dataset and is therefore summarized in this review.
Topics: Humans; Latin America; Neuromyelitis Optica; Cohort Studies; Biomedical Research
PubMed: 38682226
DOI: 10.31083/j.jin2304074