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Frontiers in Neurology 2024The Neuromyelitis Optica Spectrum Disorders (NMOSD) constitute a spectrum of rare autoimmune diseases of the central nervous system characterized by episodes of... (Review)
Review
The Neuromyelitis Optica Spectrum Disorders (NMOSD) constitute a spectrum of rare autoimmune diseases of the central nervous system characterized by episodes of transverse myelitis, optic neuritis, and other demyelinating attacks. Previously thought to be a subtype of multiple sclerosis, NMOSD is now known to be a distinct disease with unique pathophysiology, clinical course, and treatment options. Although there have been significant recent advances in the diagnosis and treatment of NMOSD, the field still lacks clinically validated biomarkers that can be used to stratify disease severity, monitor disease activity, and inform treatment decisions. Here we review many emerging NMOSD biomarkers including markers of cellular damage, neutrophil-to-lymphocyte ratio, complement, and cytokines, with a focus on how each biomarker can potentially be used for initial diagnosis, relapse surveillance, disability prediction, and treatment monitoring.
PubMed: 38817544
DOI: 10.3389/fneur.2024.1415535 -
Journal of Ophthalmic Inflammation and... May 2024To report a case of Pediatric-onset MS associated uveitis managed with local and systemic medications.
BACKGROUND
To report a case of Pediatric-onset MS associated uveitis managed with local and systemic medications.
CASE PRESENTATION
An 11-year-old boy who was diagnosed with Pediatric-onset MS (POMS) with the first presentation of left optic neuritis in another center, was referred to our clinic with the complaint of non-improved vision in the left eye despite receiving IV 5gr methylprednisolone. After the ophthalmologic examinations, the patient was diagnosed as bilateral POMS-associated intermediate uveitis, and local treatment with corticosteroid was administered to both eyes. He was continued on systemic therapy such as Rituximab and five sessions of plasmapheresis. After four months, the patient's vision improved from FC at 50cm to 9/10 in the left eye. The intensity of intraocular inflammation decreased in both eyes. In fluorescein angiography findings, the optic disc, as well as vascular leakage, subsided bilaterally.
CONCLUSION
Despite its rarity, POMS-associated uveitis presents a considerable challenge that necessitates the collaborative efforts of neurologists and ophthalmologists to achieve the most effective treatment outcomes.
PubMed: 38811495
DOI: 10.1186/s12348-024-00405-1 -
Current Therapeutic Research, Clinical... 2024Complementary and alternative medications (CAM) are common among patients with multiple sclerosis (MS) for physical and psychological support. However, there is...
Use of Complementary and Alternative Medicine in Patients With Idiopathic Inflammatory Demyelinating Diseases of the Central Nervous System: A Cross-Sectional Study in Thailand.
BACKGROUND
Complementary and alternative medications (CAM) are common among patients with multiple sclerosis (MS) for physical and psychological support. However, there is insufficient data regarding the application of CAM in the different cultures and beliefs of each community as well as patient's status.
OBJECTIVE
To evaluate the prevalence and modalities of the use of CAM among patients with central nervous system idiopathic inflammatory demyelinating diseases (CNS-IIDD) in a tertiary care hospital.
METHODS
A cross-sectional study was conducted at Siriraj Hospital from June to December 2021 involving patients with MS, neuromyelitis optic spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), idiopathic transverse myelitis (iTM), and optic neuritis (ON) to examine the prevalence and mode of CAM use and its correlation with patient characteristics.
RESULTS
There were 107 patients. The diagnoses were MS (38), NMOSD (55), MOGAD (5), iTM (7), and ON (2). Most of the patients were female (89.7%), and 61.7% were diagnosed over 5 years. The mean Expanded Disability Status Scale was 2.63 (S.D., 2.38), and the median ambulation index was 0 (range 0-8.5). There were 68 patients (63.6%) with a history of CAM use for at least 3 months, while those with current use decreased to 62 (58.5%). Vitamins and minerals were the most commonly used, particularly vitamin D (97.1%) and calcium (47.7%). Both treatments were primarily prescribed (95.3%) rather than self-administered (24.3%). The main reasons for the use of CAM were to strengthen their health (48.6%) and relieve existing symptoms (28.0%).
CONCLUSIONS
The use of CAM is common among patients with Thai CNS-IIDD. Further exploration of patient perspectives and preferences regarding CAM usage may contribute to a more comprehensive management approach for patients with CNS-IIDD.
PubMed: 38808155
DOI: 10.1016/j.curtheres.2024.100749 -
Frontiers in Immunology 2024Myelin oligodendrocyte glycoprotein (MOG) IgG is frequently elevated in pediatric patients with acquired demyelinating syndrome (ADS). However, no specific biomarkers...
BACKGROUND AND PURPOSE
Myelin oligodendrocyte glycoprotein (MOG) IgG is frequently elevated in pediatric patients with acquired demyelinating syndrome (ADS). However, no specific biomarkers exist for phenotype classification, symptom severity, prognosis, and treatment guidance of MOG-IgG-associated disease (MOGAD). This study evaluated neurofilament light chain (NfL) and endothelial growth factor receptor (EGFR) mRNA expression levels in serum and cerebrospinal fluid (CSF) as potential biomarkers for MOGAD in Chinese children.
METHODS
This was a cross-sectional and single-center study. We enrolled 22 consecutive pediatric patients hospitalized with MOGAD and 20 control pediatric patients hospitalized for noninflammatory neurological diseases in Hebei Children's Hospital. Serum and CSF were collected from MOGAD patients within 3 days before immunotherapy. The mRNA levels of NfL and EGFR in serum and CSF were measured by real-time polymerase chain reaction (qPCR), and the EGFR/NfL ratio mRNA was calculated. These measurement values were then compared between disease groups and among MOGAD phenotypes. In addition, the correlations between the mRNAs of three markers (NfL, EGFR, EGFR/NfL ratio), extended disability status scale (EDSS) scores, and clinical phenotypes were analyzed.
RESULTS
Serum and CSF NfL mRNA levels were significantly higher of acute-stage MOGAD patients than those of control patients ( 0.05 and 0.01, respectively), while the mRNA levels of serum EGFR and EGFR/NfL ratio were significantly lower of MOGAD patients than those of controls ( 0.05, 0.0001). Serum NfL mRNA was significantly correlated with mRNA of serum EGFR ( =0.480, < 0.05). Serum and CSF NfL mRNA levels in MOGAD patients with the ADEM-like phenotype were also significantly higher than those in control patients ( < 0.01, < 0.01) and optic neuritis (ON) phenotype ( < 0.05, < 0.05). Both mRNAs of NfL in CSF and EGFR/NfL ratio in serum were correlated with EDSS scores ( < 0.05, = 0.424; < 0.05, = -0.521).
CONCLUSION
The mRNA levels of elevated NfL in serum and CSF as well as lower EGFR and EGFR/NfL ratio in serum could help distinguish acute-phase MOGAD. Higher mRNA levels of NfL in serum and CSF of MOGAD patients help distinguish ADEM-like phenotype. In addition, serum EGFR/NfL mRNA ratio is indicative of disease severity in pediatric patients with MOGAD. Further investigations are warranted to elucidate the pathological mechanisms underlying these associations.
Topics: Humans; Male; Female; Biomarkers; Child; Neurofilament Proteins; Myelin-Oligodendrocyte Glycoprotein; RNA, Messenger; ErbB Receptors; Cross-Sectional Studies; Child, Preschool; Severity of Illness Index; Phenotype; Immunoglobulin G; Adolescent; Autoantibodies
PubMed: 38807603
DOI: 10.3389/fimmu.2024.1388734 -
Vestnik Otorinolaringologii 2024Vestibular neuritis occupies the third place in terms of prevalence in the structure of peripheral vestibulopathies, therefore, the choice of optimal diagnostic and...
UNLABELLED
Vestibular neuritis occupies the third place in terms of prevalence in the structure of peripheral vestibulopathies, therefore, the choice of optimal diagnostic and differential diagnostic tactics at different stages of the disease is an urgent task.
OBJECTIVE
To optimize the diagnostic algorithm for vestibular neuritis based on an assessment of the sensitivity of clinical methods for studying vestibular function in the recovery period of the disease.
MATERIAL AND METHODS
A comprehensive assessment of the sensitivity of clinical methods for the study of vestibular function in the acute (up to 14 days: at the time of initial treatment, on the 7th and 14th day) and subacute (up to 3 months: on the 28th and 90th day) periods of the disease in 52 patients with upper vestibular neuritis was carried out.
RESULTS
The timing of the processes of restoration of vestibular function after a transferred vestibular neuritis is individual: after 14 days, restoration of vestibular function was recorded in 52% (=27) patients, after 1 month - in 62% (=32), after 3 months - in 71% (=37) patients with upper vestibular neuritis. Statocoordination, statokinetic, oculomotor tests under visual control have the highest sensitivity in the acute period of vestibular neuritis, within up to 7 days from the onset of symptoms. In the subacute period of vestibular neuritis, the study of spontaneous nystagmus and nystagmus in the head shaking test retains high sensitivity only when using special tools (Frenzel goggles or videonystagmography). A decrease in the sensitivity of the head rotation test and the dynamic visual acuity test in the subacute period of vestibular neuritis is associated with the processes of central compensation and the formation of a latent saccade.
CONCLUSION
The sensitivity of clinical tests in patients with vestibular neuritis depends on the timing of the examination.
Topics: Humans; Vestibular Neuronitis; Vestibular Function Tests; Adult; Female; Male; Middle Aged; Vestibule, Labyrinth; Diagnosis, Differential; Recovery of Function
PubMed: 38805456
DOI: 10.17116/otorino2024890214 -
Current Neurology and Neuroscience... May 2024Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a distinct neuroinflammatory condition characterized by attacks of optic neuritis, transverse myelitis,... (Review)
Review
PURPOSE OF REVIEW
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a distinct neuroinflammatory condition characterized by attacks of optic neuritis, transverse myelitis, and other demyelinating events. Though it can mimic multiple sclerosis and neuromyelitis optica spectrum disorder, distinct clinical and radiologic features which can discriminate these conditions are now recognized. This review highlights recent advances in our understanding of clinical manifestations, diagnosis, and treatment of MOGAD.
RECENT FINDINGS
Studies have identified subtleties of common clinical attacks and identified more rare phenotypes, including cerebral cortical encephalitis, which have broadened our understanding of the clinicoradiologic spectrum of MOGAD and culminated in the recent publication of proposed diagnostic criteria with a familiar construction to those diagnosing other neuroinflammatory conditions. These criteria, in combination with advances in antibody testing, should simultaneously lead to wider recognition and reduced incidence of misdiagnosis. In addition, recent observational studies have raised new questions about when to treat MOGAD chronically, and with which agent. MOGAD pathophysiology informs some of the relatively unique clinical and radiologic features which have come to define this condition, and similarly has implications for diagnosis and management. Further prospective studies and the first clinical trials of therapeutic options will answer several remaining questions about the peculiarities of this condition.
PubMed: 38805147
DOI: 10.1007/s11910-024-01344-z -
Audiology Research May 2024Hyperventilation induces metabolic changes that can elicit nystagmus (hyperventilation-induced nystagmus, HVIN) in various vestibular disorders, revealing vestibular...
Hyperventilation induces metabolic changes that can elicit nystagmus (hyperventilation-induced nystagmus, HVIN) in various vestibular disorders, revealing vestibular imbalance and bringing out central or peripheral asymmetries. In acute unilateral vestibulopathy (AUVP, namely vestibular neuritis), hyperventilation can induce different patterns of nystagmus (excitatory, inhibitory, or negative), disclosing or modifying existing static vestibular asymmetries through its ability to invalidate compensation or increase peripheral excitability. In this context, we followed the evolutionary stages of HVIN in AUVP across 35 consecutive patients, with the goal of assessing alterations in the oculomotor pattern caused by hyperventilation over time. In the acute phase, the incidence of the excitatory pattern (and the strongly excitatory one, consisting of a reversal nystagmus evoked by hyperventilation) was significantly higher compared to the inhibitory pattern; then, a progressive reduction in the incidence of the excitatory pattern and a concomitant gradual increase in the incidence of the inhibitory one were observed in the follow-up period. Assuming the role of the ephaptic effect and the transient loss of vestibular compensation as opposing mechanisms, i.e., excitatory and inhibitory, respectively, the oculomotor pattern evoked by hyperventilation is the result of the interaction of these two factors. The data obtained allowed us to hypothesize an interpretative model regarding the pathogenetic aspects of responses evoked by hyperventilation and the etiologies of the disease: according to our hypotheses, the excitatory pattern implies a neuritic (viral) form of AUVP; instead, the inhibitory (and negative) one can be an expression of both the neuritic (viral) and vascular forms of the disease.
PubMed: 38804461
DOI: 10.3390/audiolres14030037 -
Antibodies (Basel, Switzerland) May 2024Clinical syndromes associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) are now recognized as a distinct neurological disease entity, and are... (Review)
Review
Clinical syndromes associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) are now recognized as a distinct neurological disease entity, and are gaining increasing attention. The pathogenic mechanisms underlying MOG-antibody disease (MOGAD) remain incompletely understood. Case series, facilitated by registries, and observational studies over the past few years have shed increasing light on the clinical aspects and therapeutic approaches of MOGAD. MOGAD may manifest with a variety of clinical syndromes, including acute disseminated encephalomyelitis (ADEM), autoimmune encephalitis, optic neuritis (ON) and transverse myelitis (TM). MOGAD can be either monophasic or relapsing. This review aims to provide a comprehensive updated description of the clinical spectrum, paraclinical features, and prognosis of MOG-antibody disease, as well as summarize its therapeutic considerations. Randomized clinical trials, standardized diagnostic criteria and treatment guidelines are the steps forward.
PubMed: 38804311
DOI: 10.3390/antib13020043 -
Veterinary Ophthalmology May 2024Nodular granulomatous episcleritis (NGE) typically presents as an elevated mass or elevated masses at the limbus and often infiltrates the cornea (episclerokeratitis)....
Case report: Medical and surgical management of presumed canine nodular granulomatous episcleritis with progression to posterior nodular scleritis, choroiditis, and optic neuritis.
Nodular granulomatous episcleritis (NGE) typically presents as an elevated mass or elevated masses at the limbus and often infiltrates the cornea (episclerokeratitis). In the current report, a granulomatous lesion was observed subretinally in the right eye (OD) of a 5-year-old male castrated American Staffordshire Terrier dog. There was concurrent retinal hemorrhage and detachment OD; the right eye was not visual. Due to poor prognosis for vision and potential for a neoplastic etiology of the mass, staging with higher imaging was recommended but declined by the owner. Therefore, an enucleation was performed. Histopathology of the globe identified a subretinal mass, marked histiocytic and lesser lymphoplasmacytic choroiditis, posterior episcleritis, and optic neuritis with retinal detachment. The subretinal mass was composed of densely packed, large, spindle histiocytes mixed with occasional lymphocytes, plasma cells, and only rare neutrophils. Regions of the mass showed lymphocytes aggregate to form nodules. This histological presentation was a type of proliferative histiocytic disease with similarities to nodular granulomatous episcleritis or granulomatous/necrotizing scleritis. This is a novel presentation of NGE-like progression to subretinal scleral, choroidal, and retinal involvement and provides a new differential possibility for posterior segment masses observed on fundic examination.
PubMed: 38803082
DOI: 10.1111/vop.13230 -
Scientific Reports May 2024Optical coherence tomography (OCT) displays the retinal nerve fiber layer (RNFL) or macular ganglion cell and inner plexiform layer (GCIPL) thickness below 1st...
Optical coherence tomography (OCT) displays the retinal nerve fiber layer (RNFL) or macular ganglion cell and inner plexiform layer (GCIPL) thickness below 1st percentile in red color. This finding generally indicates severe inner-retinal structural changes and suggests poor visual function. Nevertheless, some individuals show preserved visual function despite these circumstances. This study aimed to identify the correlation between best-corrected visual acuity (BCVA) and inner-retinal thickness based on OCT parameters in various optic neuropathy patients with extremely low RNFL/GCIPL thickness, and determine the limitation of OCT for predicting visual function in these patients. 131 patients were included in the study. The mean BCVA in logMAR was 0.55 ± 0.70 with a broad range from - 0.18 to 3.00. Among the OCT parameters, temporal GCIPL (r = - 0.412) and average GCIPL (r = - 0.366) exhibited the higher correlations with BCVA. Etiological comparisons of optic neuropathies revealed significantly lower BCVA in LHON (all p < 0.05). Idiopathic optic neuritis (ON) and MOGAD exhibited better and narrower BCVA distributions compared to the other optic neuropathies. OCT had limited utility in reflecting BCVA, notwithstanding significant inner-retinal thinning after optic nerve injuries. Caution is needed in interpreting OCT findings, especially as they relate to the etiology of optic neuropathy.
Topics: Humans; Male; Female; Tomography, Optical Coherence; Adult; Middle Aged; Optic Nerve Diseases; Visual Acuity; Retina; Young Adult; Adolescent; Retinal Ganglion Cells; Aged; Nerve Fibers; Child
PubMed: 38802443
DOI: 10.1038/s41598-024-62704-w