Cerebrospinal Fluid Oligoclonal Bands Research Articles

Cerebrospinal fluid findings in neurological diseases associated with COVID-19 and insights into mechanisms of disease development

ObjectivesTo analyze the cerebrospinal fluid (CSF) of patients with SARS-CoV-2 infection and neurological manifestations to provide evidence for the understanding of mechanisms associated with central nervous system (CNS) involvement in COVID-19. MethodsPatients (n = 58) were grouped according to their main neurological presentation: headache (n = 14); encephalopathy (n = 24); inflammatory neurological diseases, including meningoencephalitis (n = 4), acute myelitis (n = 3), meningitis (n = 2), acute disseminated encephalomyelitis (ADEM) (n = 2), encephalitis (n = 2), and neuromyelitis optica (n = 1); and Guillain-Barré syndrome (n = 6). Data regarding age, sex, cerebrovascular disease, and intracranial pressure were evaluated in combination with CSF profiles defined by cell counts, total protein and glucose levels, concentration of total Tau and neurofilament light chain (NfL) proteins, oligoclonal band patterns, and detection of SARS-CoV-2 RNA. ResultsCSF of patients with inflammatory neurological diseases was characterized by pleocytosis and elevated total protein and NfL levels. Patients with encephalopathy were mostly older men (mean age of 61.0 ± 17.6 years) with evidence of cerebrovascular disease. SARS-CoV-2 RNA in CSF was detected in 2 of 58 cases: a patient with refractory headache, and another patient who developed ADEM four days after onset of COVID-19 symptoms. Three patients presented intrathecal IgG synthesis, and four had identical oligoclonal bands in CSF and serum, indicating systemic inflammation. ConclusionPatients with neurological manifestations associated with COVID-19 had diverse CSF profiles, even within the same clinical condition. Our findings indicate a possible contribution of viral replication on triggering CNS infiltration by immune cells and the subsequent inflammation promoting neuronal injury.

Kappa Index Versus CSF Oligoclonal Bands in Predicting Multiple Sclerosis and Infectious/Inflammatory CNS Disorders.

Background: Cerebrospinal fluid (CSF) kappa free light chains (KFLC) are gaining increasing interest as markers of intrathecal immunoglobulin synthesis. The main aim of this study was to assess the diagnostic accuracy (AUC) of the kappa index (CSF/serum KFLC divided by the CSF/serum albumin ratio) compared to CSF oligoclonal IgG bands (OCB) in predicting Multiple Sclerosis (MS) or a central nervous system infectious/inflammatory disorder (CNSID). Methods: We enrolled patients who underwent a diagnostic spinal tap throughout two years. KFLC levels were determined using a Freelite assay (Binding Site) and the turbidimetric Optilite analyzer. Results: Of 540 included patients, 223 had a CNSID, and 84 had MS. The kappa index was more sensitive (0.89 versus 0.85) and less specific (0.84 versus 0.89), with the same AUC (0.87) as OCB for MS diagnosis (optimal cut-off: 6.2). Adding patients with a single CSF IgG band to the OCB-positive group slightly increased the AUC (0.88). Likewise, the kappa index (cut-off: 3.9) was more sensitive (0.67 versus 0.50) and less specific (0.81 versus 0.97), with the same AUC (0.74) as OCB, for a CNSID diagnosis. Conclusion: The kappa index and CSF OCB have comparable diagnostic accuracies for a MS or CNSID diagnosis and supply the clinician with useful, complementary information.

Semi-automated methodology for detection of IgM oligoclonal bands in cerebrospinal fluid and serum samples

Among the new biomarkers to propose therapeutic decisions in patients suffering from multiple sclerosis (MS) are the IgM oligoclonal bands (OCBs) in cerebrospinal fluid (CSF). At the current time, however, IgM OCBs are detected in laboratories at investigation level and not in the routine practice due to their complexity. For this, we have applied a semi-automated method based on an isoelectrofocusing platform from Sebia of wide availability in clinical laboratories. The IgM OCBs results were validated in paired samples of CSF and serum from patients with MS previously carried out in a reference laboratory. We found a sensitivity of 91.67%, in agreement with previous data obtained with the reference method for IgM OCBs.

Cerebrospinal fluid oligoclonal bands in Neuroborreliosis are specific for Borrelia burgdorferi.

Cerebrospinal fluid (CSF) oligoclonal bands (OCB) occur in chronic or post-acute phase of inflammatory diseases of the central nervous system. To determine whether CSF OCB in patients with neuroborreliosis (NB) are specific for borrelia burgdorferi senso lato. We performed isoelectric focusing followed by immunoblotting in CSF of 10 NB patients and 11 controls (7 patients with multiple sclerosis, 2 patients with neuromyelitis optica spectrum disease, 1 patient with dementia and 1 patient with monoclonal gammopathy). Immunoblotting was performed using an uncoated as well as a borrelia antigen pre-coated nitrocellulose membrane (NCM). OCB were counted by visual inspection and photometric analysis. OCB were compared between uncoated und pre-coated NCM both in the NB and control group. For validation purposes inter-assay precision was determined by calculating the coefficient of variation (CV). Borrelia-specific OCB were found in the CSF of 9 NB patients and in none of the control subjects resulting in a sensitivity of 90% and a specificity of 100%. Number of NB specific OCB were 11±7 bands by photometric analyses compared to 9±5 bands by visual inspection. Validation experiments revealed an inconsistent inter-assay precision between visual and photometric analyses (NB uncoated: visual 28% versus photometric 14%, control subject uncoated: visual 16% versus photometric 24%). In CSF samples with positive OCB, Borrelia-specific bands were detected in almost all NB patients and in none of the control subjects. Inconsistent inter-assay precision may be explained by a poor comparability of visual and photometric approach.

Advanced MRI features in relapsing multiple sclerosis patients with and without CSF oligoclonal IgG bands

Oligoclonal IgG bands (OCB) in cerebrospinal fluid (CSF) are important in diagnosis of multiple sclerosis (MS). We evaluated the MRI features of clinically definite MS subjects with and without CSF-OCB. Relapsing MS subjects were recruited from a prospective registry in a university center. CSF-OCB were detected using isoelectric focusing and lgG-specific immunofixation. MRI metrics including brain volumes, lesion volumes and microstructural measures, were analyzed by FMRIB Software Library (FSL) and Statistical Parametric Mapping (SPM). Seventy-five subjects with relapsing MS were analyzed. Forty-four (59%) subjects had an interval MRI at around 1 year. CSF-OCB were detected in 46 (61%) subjects. The OCB-positive group had a higher proportion of cerebellar lesions than the OCB-negative group (23.9% vs. 3.4%, p = 0.057). Except for amygdala volumes which were lower in the OCB-positive group (p = 0.034), other regional brain volumes including the subcortical deep gray matter and corpus callosum were similar. The two groups also showed comparable brain atrophy rate. For DTI, the OCB-positive group showed significantly higher mean diffusivity (MD) value in perilesional normal-appearing white matter (p = 0.043). Relapsing MS patients with and without CSF-OCB shared similar MRI features regarding volumetric analyses and DTI microstructural integrity.

Pediatric Inflammatory and Autoimmune Neurologic Disorders at a Tertiary Medical Center.

To describe the spectrum of pediatric inflammatory neurologic diseases and compare the sensitivity of ancillary testing for these diagnoses. We analyzed clinical features and outcomes of 98 children with an immune-mediated central nervous system disorder. We compared sensitivities of each diagnostic modality. We identified the following diagnoses: acute cerebellar ataxia (n = 14; 14.3%), acute demyelinating encephalomyelitis (n = 13; 13.3%), multiple sclerosis (MS) (n = 18; 18.4%), anti-N-methyl-d-aspartate receptor encephalitis (anti-NMDAR encephalitis) (n = 15; 15.3%), encephalitis not otherwise specified (n = 12; 12.2%), and "Other" (n = 26; 26.5%). "Other" included acute transverse myelitis, neuromyelitis optica, central nervous system lupus, primary central nervous system vasculitis, Rasmussen encephalitis, opsoclonus myoclonus ataxia syndrome, and clinically isolated syndrome. The mean age of onset of all diagnoses was 7.9 ± 5.5 years. The diagnostic sensitivity of magnetic resonance imaging (MRI) for acute demyelinating encephalomyelitis and multiple sclerosis was 92.3% and 94.4%, respectively. Cerebrospinal fluid was sensitive for multiple sclerosis in 92.3%, where 75% of patients had cerebrospinal fluid oligoclonal bands. Electroencephalogram (EEG) coupled with cerebrospinal fluid studies was highly sensitive for anti-NMDAR encephalitis (100%). EEG was sensitive for acute demyelinating encephalomyelitis and encephalitis not otherwise specified (77.8% and 80%). No diagnostic studies were sensitive for acute cerebellar ataxia. Seventy-three percent of patients with multiple sclerosis had residual deficits. Thirty-six percent of anti-NMDAR encephalitis patients were nonverbal and wheel-chair bound. We found that MRI is useful for detecting multiple sclerosis and acute demyelinating encephalomyelitis, cerebrospinal fluid is helpful in diagnosing multiple sclerosis and anti-NMDAR encephalitis, and EEG is often abnormal in suspected anti-NMDAR encephalitis, acute demyelinating encephalomyelitis, and encephalitis not otherwise specified. Neurologic outcome at follow-up was unfavorable in patients with multiple sclerosis and anti-NMDAR encephalitis.

Searching for autoimmune encephalitis: Beware of normal CSF

ObjectiveTo determine the prevalence of cerebrospinal fluid (CSF) markers associated with inflammation (i.e., elevated white blood cell count, protein concentration, and CSF-specific oligoclonal bands) in patients with early active autoimmune encephalitis (AE). MethodsCSF characteristics, including WBC count, protein concentration, and oligoclonal banding, were analyzed in patients diagnosed with AE at two tertiary care centers. ResultsNinety-five patients were included in the study. CSF white blood cell counts and protein levels were within normal limits for 27% (CI95%: 19–37) of patients with AE. When results of oligoclonal banding were added, 14% (CI95%: 6–16) of patients with AE had “normal” CSF. The median CSF white blood cell count was 8 cells/mm3 (range: 0–544) and the median CSF protein concentration was 0.42 g/L (range: 0.15–3.92). ConclusionsWhite blood cell counts and protein levels were within normal limits in the CSF of a substantial proportion of patients with early active AE. Inclusion of CSF oligoclonal banding identified a higher proportion of patients with an inflammatory CSF profile, especially when CSF was sampled early in the disease process.

Neurological Spectrum Disorders Associated with Anti–MOG Antibody

Optic neuritis (ON) is an inflammatory disease of the optic nerve characterized by pain and visual lossand often associated with multiple sclerosis (MS) or neuromyelitis optica spectrum disorders (NMOSD).Autoantibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been reported in patientswith inflammatory central nervous system disorders including isolated optic neuritis (ON).Objective: To investigate the differences of clinical features, cerebrospinal fluid (CSF), MRI findings andresponse to steroid therapies between patients with optic neuritis (ON) who have myelin oligodendrocyteglycoprotein (MOG) antibodies and seronegative group. This study was done in a period between June2015 and July 2018, 65 patients were included in this study with ON who ophthalmologists had diagnosedas having or suspected to have ON with acute visual impairment and declined critical flicker frequency,abnormal findings of brain MRI, optical coherence tomography and fluorescein fundus angiography at theironset or recurrence. After exclusion of all patients who fulfilled the diagnostic criteria of neuromyelitisoptica (NMO)/NMO spectrum disorders (NMOSD), MS McDonald’s criteria, we defined 40 patients withidiopathic ON (12 males, 28 females, age range 15-60 years). Sera from patients were tested for antibodiesto MOG and aquaporin-4 (AQP4) with a cell-based assay.Results: 37.5% (15/40) were positive for MOG antibodies, 2.5% (1/40) were positive for AQP4 and 25(62.5%) were seronegative. Among the 15 patients with MOG antibodies, four had optic pain (p=0.007) andfive had prodromal infection (p=0.05). Two of the 15 MOG-positive patients showed significantly high CSFlevels of myelin basic protein (p=0.05) and none were positive for oligoclonal band in CSF. On MRIs, fiveMOG-positive patients showed high signal intensity on optic nerve, four had a cerebral lesion and two had aspinal cord lesion. Six of the eight MOG-positive patients had a good response to steroid therapy.Conclusions: The present results indicate that Patients with NMOSD and MOG positive antibodies havedistinct clinical features, fewer attacks and better recovery than seronegative patients.

Possible clinical role of MOG antibody testing in children presenting with acute neurological symptoms.

The differential diagnosis between acquired inflammatory demyelinating syndromes of the central nervous system (CNS), such as multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and acute disseminated encephalomyelitis (ADEM) can be very challenging at onset. Apart from cerebrospinal fluid oligoclonal bands and anti-aquaporin-4 antibodies (AQP4-Ab), definite diagnostic biomarkers are lacking. Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) have been increasingly described in children with AQP4-seronegative NMOSD, ADEM and other inflammatory demyelinating CND syndromes; despite partial overlaps with AQP4-Ab disease, a novel "MOG-Ab-disorder" phenotype has been suggested. In this study, we tested the presence of MOG-Ab and AQP4-Ab in 57 children at first onset of acute neurological symptoms; three clinical subgroups were identified: 12 patients had acquired inflammatory demyelinating CNS syndromes, 11 had other autoimmune/immune-mediated disorders of the central and peripheral nervous system and 34 had non-immune-mediated CNS disorders. MOG-Abs were found positive only in a subset of cases in the subgroup with acquired inflammatory demyelinating CNS syndromes (in 2/12 patients, both with non-MS phenotype) and in none of the patients with other autoimmune and immune-mediated disorders of the central and peripheral nervous system or with non-immune-mediated disorders of the CNS.Data from the literature review support clinical and analytical observations.

Novel Surrogate Markers of CNS Inflammation in CSF in the Diagnosis of Autoimmune Encephalitis.

Background: Autoimmune encephalitis (AE) is an important cause of refractory epilepsy, rapidly progressive cognitive decline, and unexplained movement disorders in adults. Whilst there is identification of an increasing number of associated autoantibodies, patients remain with a high clinical probability of autoimmune encephalitis but no associated characterized autoantibody. These patients represent a diagnostic and treatment dilemma.Objective: To evaluate routine and novel diagnostic tests of cerebrospinal fluid (CSF) in patients with a high probability of AE to attempt to identify better biomarkers of neuroinflammation.Methods: Over 18 months (2016–2018), adult patients with a high clinical probability of AE were recruited for a pilot cross-sectional explorative study. We also included viral polymerase-chain-reaction (PCR) positive CSF samples and CSF from neurology patients with “non-inflammatory” (NI) diagnoses for comparison. CSF was examined with standard investigations for encephalitis and novel markers (CSF light chains, and cytokines).Results and Conclusions: Thirty-two AE patients were recruited over 18 months. Twenty-one viral controls, 10 NI controls, and five other autoimmune neurological disease controls (AOND) were also included in the analysis. Our study found that conventional markers: presence of CSF monocytosis, oligoclonal bands, anti-neuronal immunofluorescence, and magnetic resonance imaging (MRI) changes could be suggestive of AE, but these investigations were neither sensitive nor specific. Promising novel makers of autoimmune encephalitis were the CSF cytokines IL-21 and IP10 which may provide better delineation between viral infections and autoimmune encephalitis than conventional markers, potentially leading to more immediate diagnosis and management of these patients.

Sporadic hereditary neuropathies misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy: Pitfalls and red flags.

Hereditary neuropathies may be misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). A correct diagnosis is crucial for avoiding unnecessary therapies and access genetic counseling. We report on nine patients (seven men, mean age 49.2 ± 16.1) diagnosed with and treated as CIDP, in whom mutations or variants of unknown significance (VUS) in genes associated with hereditary neuropathies were reported. All underwent neurological and neurophysiological examination, eight also cerebrospinal fluid (CSF) analysis. In 4/9, nerve ultrasound and/or MR-neurography were performed. All the patients complained of progressive upper or lower limbs sensory-motor symptoms, with heterogeneous disease duration (1-34 years, mean 8.6 ± 10.8). Neurophysiology showed demyelinating signs in seven patients, mixed findings with predominant axonal damage in two patients. Neuroimaging disclosed diffuse abnormalities at proximal and distal segments. Molecular screening showed PMP22 duplication in two patients, mutations in the MPZ, EGR2, and GJB1 genes were reported in each of the remaining patients. The two patients with mixed neurophysiological findings had p.Val30Met mutation in the transthyretin gene. Two patients had VUS in the MARS and HSPB1 genes. Four patients had partial response to immunomodulant therapies, and CSF and neurophysiological features suggesting an inflammatory condition concomitant with the hereditary neuropathy. Hereditary neuropathy may be misdiagnosed with CIDP. The most common pitfalls are CSF (high protein levels and oligoclonal bands), incorrect interpretation of neurophysiology, and transient benefit from therapies. Neuroimaging may be helpful in cases with atypical presentations or when severe axonal damage complicate the neurophysiological interpretation.

Bullous pemphigoid as an injection site reaction of glatiramer acetate

Glatiramer acetate (GA) is one of the well-tolerated disease-modifying therapeutic options, which is commonly administered subcutaneously in patients with multiple sclerosis (MS). The current study aimed at defining a bullous pemphigoid (BP) skin reaction in a patient with MS receiving treatment with GA. A 29-year-old women with MS, receiving GA treatment within the past nine months, November 11, 2018, was admitted to our MS clinic due to itching skin eruptions at the site of injection. Her disease was started at February 12, 2017 with left optic neuritis; and because of six periventricular and eleven juxtacortical brain magnetic resonance imaging (MRI) lesions without any enhancement, lumbar puncture was done for her. Due to positive cerebrospinal fluid (CSF) oligoclonal band, MS was diagnosed for her. At that time, she refused to start disease modifying treatment. GA was started 9 months before admission for her. After dermatologic consultation, the dermatologist defined the lesions as fluid-filled and blistering at the site of injection without mucosal involvement (Figure 1). Figure 1. Large, fluid-filled blisters Timeline: Skin lesions appeared exactly at the GA injection site.

Clinically isolated syndrome and multiple sclerosis in children: a single center study

This study was conducted to determine the differences in clinical and radiological features at the first demyelinating event in children with clinically isolated syndrome (CIS) and multiple sclerosis (MS). This was a single center retrospective cohort study of the children with CIS followed-up at Istanbul University Faculty of Medicine, Department of Pediatric Neurology, between 2010 and 2018. Children with CIS who were assessed at 3, 6, 12 and 24 months following their first identified demyelinating event were included. Demographic data, mode of presentation and the presence of the oligoclonal band in the cerebrospinal fluid (CSF) were abstracted from the medical records. Magnetic resonance imaging of the brain and spinal cord was analyzed for the location, number, size and gadolinium enhancement of the lesions. A total of 51 patients` data was assessed, 38 patients at a mean age of 12.3 years were enrolled in the study. Twenty-seven children (71%) evolved into clinically definite MS after a mean follow-up of 11 months. Older age at first demyelinating event and the presence of the oligoclonal band in CSF were tended to be more common in patients with MS than patients with CIS (p < 0.05). The increased number of T2-hyperintense lesion and the presence of the lesion in periventricular, infratentorial and corpus callosum were associated with a tendency for development of MS (p < 0.05). Older age at first demyelinating event, the presence of the oligoclonal band in CSF, the number and localization of T2-hyperintense lesion were associated with a tendency for development of MS.

The diagnostic value of IgG index versus oligoclonal bands in cerebrospinal fluid of patients with multiple sclerosis.

BackgroundDiagnostic criteria for multiple sclerosis have been developed to guide the diagnostic process. In the latest revision of the McDonald criteria, the presence of oligoclonal bands may replace the need for dissemination in time. The aim of this study is to investigate if the less time-consuming analysis of immunoglobulin G index in cerebrospinal fluid can safely predict the findings of oligoclonal bands.MethodsThis is a retrospective study of patients with multiple sclerosis at three hospitals in South-East Norway where lumbar puncture is performed routinely. We included patients diagnosed with multiple sclerosis after 2005 with known oligoclonal band status and an immunoglobulin G index score.ResultsOf 1295 patients diagnosed during or after 2005, 93.8% were oligoclonal band positive at diagnosis. Of 842 multiple sclerosis patients with known immunoglobulin G index and oligoclonal band status, 93.3% were oligoclonal band positive and 76.7% had an elevated immunoglobulin G index. The positive predictive value of a high immunoglobulin G index when oligoclonal bands are positive was 99.4% (95% confidence interval 98.4–99.8%). The negative predictive value of a normal immunoglobulin G index when oligoclonal bands are negative was 26.5% (95% confidence interval 23.5–29.9%).ConclusionAn immunoglobulin G index >0.7 has a positive predictive value >99% for oligoclonal bands. An elevated immunoglobulin G index adds diagnostic value versus oligoclonal bands and saves time in the diagnostic process.

The Patterns of Clinical Presentation of Multiple Sclerosis in Patients Admitted to the National Center of Neurological Sciences, Khartoum, Sudan 2018

Multiple sclerosis (MS) is an immune- mediated inflammatory disease that attacks myelinated axons in the central nervous system leading to significant disability. This study is a descriptive cross-sectional study conducted at the national center for neurological sciences (NCNS), Khartoum Sudan, over 3 years period from August 2015 to April 2018. The diagnosis was performed based on Poser & Mackdonalds criteria. Data was collected using Questionnaire. The diagnosis was confirmed by brain and cervical spine magnetic resonance imaging (MRI) with sagittal FLAIR, serum and cerebrospinal fluid (CSF) oligoclonal band. Sixty-five patients were enrolled. The majority (90.8%) of them were females. 38.4% were within the age group from 21-30 years and 95.4% had no family history of MS. 18 patients (27.6%) had decreased visual acuity, 13 patients (20%) had ataxia, and 33 patients (50.7%) had a past history of similar condition. 64.6% out of 65 patients had more than two lesions detectable in their brain MRI. Only 18.4% of the patients were examined for CSF analysis. Oligoclonal band was found positive in all of them. Females predominated in 91% of the patients. The most affected age group was ranging between 21-30 years. Relapsing-remitting type was the most common disease course. Oligoclonal band was detected in all of the patients. Azathioprine was found to be very effective.

Atypical Multiple Sclerosis with Antibody to MOG

We report a case of a young female who had magnetic resonance imaging (MRI) brain lesions typical of multiple sclerosis (MS) with cerebrospinal fluid (CSF) oligoclonal bands (OCBs) and definite multiple sclerosis based on revised McDonald criteria; however, she also had atypical features of mild pleocytosis, brainstem and cerebellar peduncle involvement apart from opticospinal (OS) involvement. She also turned out to be positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody; hence, she was diagnosed with atypical multiple sclerosis. This case highlights when to suspect atypical MS and its management approach. Typical MS cases are largely anti-MOG-negative. In a study of 50 Japanese cases, with anti-aquaporin 4 (AQP4)-immunoglobulin (IgG)-negative OSMS, just 2 were MOG-IgG-positive, but they had some features atypical for MS, such as bilateral optic neuritis, longitudinally extensive transverse myelitis, or moderate pleocytosis. In another study, antibodies to MOG were found in about 5% (5/104) of preselected adult patients with MS. Patients with MS with antibodies to MOG showed typical MS lesions on brain MRI with concomitant severe brainstem and spinal cord involvement and had a severe disease course with high relapse rates. In conclusion, any patient showing typical MS lesions on brain MRI with OCB present in CSF but has atypical features like mild pleocytosis with brainstem, cerebellar, or OS involvement should also be tested for autoantibodies to MOG, and if positive, then he/she would require aggressive treatment approach in the form of plasma exchange, if resistant to pulse steroid therapy, followed by either rituximab or natalizumab rather than trying other disease modifying therapies (DMTs).

Oligoclonal bands and levels of interleukin 4, interleukin 10, and tumor necrosis factor alpha in idiopathic intracranial hypertension Egyptian patients

BackgroundIdiopathic intracranial hypertension (IIH) is a neurological disorder of unknown pathophysiology with many proposed theories that involve CSF dynamics but recently, involvement of inflammatory and autoimmune processes has been postulated.ObjectivesTo investigate presence of oligoclonal bands (OCB) in cerebrospinal fluid (CSF) and serum cytokine level in patients with IIH.MethodsThis study was conducted on 27 IIH female patients and 21 age- and sex-matched control groups. Patient and control groups were subjected to measurement of interleukin-4 (IL-4), IL-10, and tumor necrosis factor α (TNF-α) levels in serum, and CSF oligoclonal bands was measured in the IIH patient group. Body mass index (BMI) was measured to both patients and control.ResultsSerum levels of IL-4, IL-10, and TNF alpha were significantly higher in IIH patients than controls (p < 0.001); 22% of IIH patients had positive OCB in CSF. There was a statistically significant difference regarding TNF-α level in OCB-positive and OCB-negative patients being higher in positive patients. No statistically significant correlation was found between serum levels of IL-4, IL-10, TNF-α, and BMI.ConclusionAutoimmune inflammatory process may play a role in pathophysiology of IIH.

Cerebrospinal fluid free light chains compared to oligoclonal bands as biomarkers in multiple sclerosis

Cerebrospinal fluid (CSF) free light chains (FLC) may be an alternative biomarker to oligoclonal bands (OCB) in multiple sclerosis (MS). Herein, we compared the diagnostic accuracy of CSF OCB and FLC and we tested the prognostic value of FLC in a cohort of 64 MS patients and 106 controls. A κ-index >7.83 was more sensitive but less specific than OCB in discriminating MS patients from controls. Additionally, a κ-index >10.61 performed better than OCB in the discrimination between MS and controls with inflammatory neurological diseases (p < .001). In clinically isolated syndrome (CIS) patients, a κ-index >10.61 significantly predicted time to conversion to MS (p = .020). κ-index might be a valid alternative to OCB as a diagnostic biomarker for MS and might also be a prognostic marker in CIS.

Multiple sclerosis with adrenal insufficiency mimicking adrenoleucodystrophy

The diagnosis of multiple sclerosis (MS) is based on clinical, radiological and biological findings. A misinterpretation of non specific clinical and radiological findings may contribute to misdiagnosing MS mimickers such as inherited leucoencephalopathies. Mrs NS, a 28 year-old woman was referred to our unit for vertigos and gait disturbances evolving since 15 days. Neurological assessment showed central vestibular syndrome and deep sensory alterations. Brain and spinal cord MRI showed non enhancing T2 and Flair hyperintensities in the parieto-occipital periventricular white matter, cerebellar pedoncules and the cervical portion of the spinal cord. Lumbar puncture showed oligoclonal bands in cerebrospinal fluid. The visual evoked potentials showed bilateral optic demyelinating neuropathy. Biochemistry revealed adrenal insufficiency. The diagnosis of adrenoleucodystrophy in a carrier woman was plausible all the more we noted high Lactate-to-pyruvate ratio. However the dosage of Very-Long-Chain-Fatty-Acids was normal. Hence, the diagnosis of MS was made. The patient received five days pulsed-methylprednisolone and experienced rapid improvement. Many patients are misdiagnosed with multiple sclerosis, and might be given disease modifying therapies while they suffer from metabolic or systemic disorders mimicking inflammatory demyelinating diseases. There are many overlapping factors mainly: inflammation which is the underlying mechanism of X-linked adrenoleucodystrophy (X-ALD), demyelinating optic neuritis, the presence of oligoclonal bands in CSF which were reported in X-ALD patients. In our case we ruled out the diagnosis of X-ALD but this should be considered in patients with atypical MRI features or family history of adrenomyelopathy mainly in males.

Atypical multiple sclerosis with antibody to MOG

Case Presentation: We report a case of a young female who had magnetic resonance imaging (MRI) brain lesions typical of multiple sclerosis (MS) with cerebrospinal fluid (CSF) oligoclonal bands (OCBs) and definite multiple sclerosis based on revised McDonald criteria; however, she also had atypical features of mild pleocytosis, brainstem and cerebellar peduncle involvement apart from opticospinal (OS) involvement. She also turned out to be positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody; hence, she was diagnosed with atypical multiple sclerosis. This case highlights when to suspect atypical MS and its management approach. Discussion: Typical MS cases are largely anti-MOG-negative. In a study of 50 Japanese cases, with anti-aquaporin 4 (AQP4)-immunoglobulin (IgG)-negative OSMS, just 2 were MOG-IgG-positive, but they had some features atypical for MS, such as bilateral optic neuritis, longitudinally extensive transverse myelitis, or moderate pleocytosis. In another study, antibodies to MOG were found in about 5% (5/104) of preselected adult patients with MS. Patients with MS with antibodies to MOG showed typical MS lesions on brain MRI with concomitant severe brainstem and spinal cord involvement and had a severe disease course with high relapse rates. Conclusion: In conclusion, any patient showing typical MS lesions on brain MRI with OCB present in CSF but has atypical features like mild pleocytosis with brainstem, cerebellar, or OS involvement should also be tested for autoantibodies to MOG, and if positive, then he/she would require aggressive treatment approach in the form of plasma exchange, if resistant to pulse steroid therapy, followed by either rituximab or natalizumab rather than trying other disease modifying therapies (DMTs).