BACKGROUND: We recently described magnetic resonance imaging (MRI) features of children with transverse myelitis (TM) at first event with important and unique differences depending on the underlying disease entity. OBJECTIVE: To study the resolution of lesions over time in children with TM due to MOG-antibody associated disorders (MOGAD), multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) or double seronegative TM. PATIENTS AND METHODS: In this prospective study, 78 children from 29 different medical centres with TM as part of MOGAD (n = 34), MS (n = 20), NMOSD (n = 5) and in double seronegative children (n = 19) were included. A grading system consisting of 4 grades (grade 0 = complete resolution; grade 3 = no resolution at all) was used to compare the degree of lesion resolution over time in the different disease entities. Time to lesion resolution was evaluated by Kaplan-Meier statistics and log-rank test. RESULTS: Significant differences of the interval between first MRI until resolution of lesions were observed between the four disease entities. The most rapid and complete resolution was seen in MOGAD, followed by double seronegative, MS and NMOSD. Median periods until total resolution (grade 0) were 191 days (MOGAD), 750 days (double seronegative), 1117 days (MS), while none of the patients with NMOSD reached a complete resolution during the observation period. The better prognosis of MOGAD compared to MS was independent of sex, age, oligoclonal bands and cell count in the multivariate Cox analysis (P < 0.001). CONCLUSION: Children with TM and antibodies to MOG show a faster resolution of radiological lesions compared to children with MS and NMOSD.

• Klíčová slova
• Acquired demyelinating syndrome, MOG, MRI, Neuroinflammation, Pediatric, Radiologic, Transverse myelitis,
• MeSH
• autoprotilátky krev   MeSH
• dítě MeSH
• glykoprotein v myelinu oligodendrocytů imunologie   MeSH
• lidé MeSH
• magnetická rezonanční tomografie * MeSH
• mícha diagnostické zobrazování   patologie   MeSH
• mladiství MeSH
• následné studie MeSH
• neuromyelitis optica * diagnostické zobrazování   MeSH
• předškolní dítě MeSH
• prospektivní studie MeSH
• roztroušená skleróza * diagnostické zobrazování   patologie   MeSH
• transverzální myelitida * diagnostické zobrazování   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Názvy látek
• autoprotilátky MeSH
• glykoprotein v myelinu oligodendrocytů MeSH
• MOG protein, human MeSH Prohlížeč

BACKGROUND: Serum antibodies to myelin-oligodendrocyte glycoprotein (MOG) are biomarkers of MOG-IgG-associated disorder (MOGAD), a demyelinating disease distinct from both multiple sclerosis and aquaporin-4-IgG neuromyelitis optica spectrum disorder. The phenotype of MOGAD is broad and includes optic neuritis, transverse myelitis, and acute demyelinating encephalomyelitis. Myelitis is common with MOGAD and typically results in acute and severe disability, although prospects for recovery are often favorable with prompt immunotherapy. CASE PRESENTATION: This contribution presents a unique case report of a young male patient exhibiting relapsing myelitis with normal spinal cord and brain magnetic resonance imaging. Comprehensive diagnostic assessment revealed myelin-oligodendrocyte glycoprotein-IgG-associated disorder. CONCLUSION: MOGAD is one of the conditions which should be considered in MRI-negative myelitis. The diagnosis, however, may prove difficult, especially if the patient is not exhibiting other neurological symptoms of MOGAD. Conus or epiconus involvement is common in MOGAD; the patient reported herein exhibited incomplete rostral epiconus symptoms which, together with somatosensory evoked potential abnormalities, led to the diagnosis.

• Klíčová slova
• Case report, Demyelinating diseases, Evoked potentials, Magnetic resonance imaging, Myelin-oligodendrocyte glycoprotein,
• MeSH
• akvaporin 4 MeSH
• autoprotilátky MeSH
• glykoprotein v myelinu oligodendrocytů MeSH
• imunoglobulin G MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• magnetická rezonanční tomografie MeSH
• neuromyelitis optica * MeSH
• transverzální myelitida * diagnostické zobrazování   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• akvaporin 4 MeSH
• autoprotilátky MeSH
• glykoprotein v myelinu oligodendrocytů MeSH
• imunoglobulin G MeSH

Experimental autoimmune encephalomyelitis (EAE) represents the mouse model of multiple sclerosis, a devastating neurological disorder. EAE development and progression involves the infiltration of different immune cells into the brain and spinal cord. However, less is known about a potential role of eosinophil granulocytes for EAE disease pathogenesis. In the present study, we found enhanced eosinophil abundance accompanied by increased concentration of the eosinophil chemoattractant eotaxin-1 in the spinal cord in the course of EAE induced in C57BL/6 mice by immunization with MOG35-55 peptide. However, the absence of eosinophils did not affect neuroinflammation, demyelination and clinical development or severity of EAE, as assessed in ∆dblGATA1 eosinophil-deficient mice. Taken together, despite their enhanced abundance in the inflamed spinal cord during disease progression, eosinophils were dispensable for EAE development.

• Klíčová slova
• Autoimmunity, Demyelination, EAE, Eosinophils, MOG,
• MeSH
• chemokin CCL11 metabolismus   MeSH
• encefalomyelitida autoimunitní experimentální krev   diagnóza   imunologie   patologie   MeSH
• eozinofily imunologie   metabolismus   MeSH
• glykoprotein v myelinu oligodendrocytů aplikace a dávkování   imunologie   MeSH
• lidé MeSH
• mícha imunologie   patologie   MeSH
• myši transgenní MeSH
• myši MeSH
• peptidové fragmenty aplikace a dávkování   imunologie   MeSH
• roztroušená skleróza krev   diagnóza   imunologie   patologie   MeSH
• stupeň závažnosti nemoci MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• myši MeSH
• ženské pohlaví MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• Ccl11 protein, mouse MeSH Prohlížeč
• chemokin CCL11 MeSH
• glykoprotein v myelinu oligodendrocytů MeSH
• peptidové fragmenty MeSH

PURPOSE OF REVIEW: The purpose of this review is to describe the new 2017 revisions of the McDonald diagnostic criteria for multiple sclerosis and review first experiences in their application to different patient populations. RECENT FINDINGS: The 2017 revisions agreed on by an international expert panel, as the precursors, define criteria needed to fulfill dissemination in time and space in the clinically isolated syndrome after exclusion of alternative diagnoses. One major change is the inclusion of cerebrospinal fluid (CSF) oligoclonal bands as evidence of dissemination in time in a patient with dissemination in space gathered by clinical or magnetic resonance examination. The distinction between asymptomatic and symptomatic lesions in counting for evidence of dissemination in space or time in supra, infratentorial, and spinal cord syndrome has been abandoned. Finally, cortical lesions can be used to demonstrate dissemination in space. Major differential diagnoses, in particular, the still-evolving concept of neuromyelitis optica spectrum disorders and the myelin oligodendrocyte glycoprotein-IgG-related demyelinating central nervous system disorders. SUMMARY: The new 2017 revisions will simplify the application of the MRI criteria for dissemination in space and include CSF findings as evidence for dissemination in time in clinically isolated syndrome.

• MeSH
• glykoprotein v myelinu oligodendrocytů MeSH
• lidé MeSH
• magnetická rezonanční tomografie * MeSH
• neuromyelitis optica diagnóza   imunologie   MeSH
• roztroušená skleróza diagnóza   imunologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• glykoprotein v myelinu oligodendrocytů MeSH

Antibodies against myelin oligodendrocyte glycoprotein cause inflammatory lesions of central myelin - in optic nerves, of the brainstem, and spinal cord. There are characteristic changes of CNS white matter, protein-cytological association in cerebrospinal fluid, MOG IgG antibodies, a very important differential diagnosis and a relatively mild course.

• Klíčová slova
• antibodies, inflammation, myelin oligodendrocyte glycoprotein, spinal cord,
• MeSH
• autoprotilátky krev   MeSH
• dospělí MeSH
• glykoprotein v myelinu oligodendrocytů imunologie   MeSH
• lidé MeSH
• transverzální myelitida krev   diagnostické zobrazování   imunologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• autoprotilátky MeSH
• glykoprotein v myelinu oligodendrocytů MeSH

STUDY OBJECTIVES: To identify rare allelic variants and HLA alleles in narcolepsy patients with hypocretin (orexin, HCRT) deficiency but lacking DQB1*06:02. SETTINGS: China (Peking University People's Hospital), Czech Republic (Charles University), Denmark (Golstrup Hospital), Italy (University of Bologna), Korea (Catholic University), and USA (Stanford University). DESIGN: CSF hypocretin-1, DQB1*06:02, clinical and polysomnographic data were collected in narcolepsy patients (552 with and 144 without cataplexy) from 6 sites. Numbers of cases with and without DQB1*06:02 and low CSF hypocretin-1 were compiled. HLA class I (A, B, C), class II (DRBs, DQA1, DQB1, DPA1, and DPB1), and whole exome sequencing were conducted in 9 DQB1*06:02 negative cases with low CSF hypocretin-1. Sanger sequencing of selected exons in DNMT1, HCRT, and MOG was performed to exclude mutations in known narcolepsy-associated genes. MEASUREMENTS AND RESULTS: Classic narcolepsy markers DQB1*06:02 and low CSF hypocretin-1 were found in 87.4% of cases with cataplexy, and in 20.0% without cataplexy. Nine cases (all with cataplexy) were DQB1*06:02 negative with low CSF hypocretin-1, constituting 1.7% [0.8%-3.4%] of all cases with cataplexy and 1.8% [0.8%-3.4%] of cases with low CSF hypocretin independent of cataplexy across sites. Five HLA negative subjects had severe cataplexy, often occurring without clear triggers. Subjects had diverse ethnic backgrounds and HLA alleles at all loci, suggesting no single secondary HLA association. The rare subtype DPB1*0901, and homologous DPB1*10:01 subtype, were present in 5 subjects, suggesting a secondary association with HLA-DP. Preprohypocretin sequencing revealed no mutations beyond one previously reported in a very early onset case. No new MOG or DNMT1 mutations were found, nor were suspicious or private variants in novel genes identified through exome sequencing. CONCLUSIONS: Hypocretin, MOG, or DNMT1 mutations are exceptional findings in DQB1*06:02 negative cases with hypocretin deficiency. A secondary HLA-DP association may be present in these cases. These represent particularly difficult diagnostic challenges.

• Klíčová slova
• DNMT1, HLA, MHC, MOG, cataplexy, exome sequencing, hypocretin, narcolepsy, orexin,
• MeSH
• alely MeSH
• glykoprotein v myelinu oligodendrocytů genetika   MeSH
• HLA-DQ beta řetězec genetika   MeSH
• internacionalita MeSH
• intracelulární signální peptidy a proteiny mozkomíšní mok   nedostatek   genetika   MeSH
• kataplexie genetika   MeSH
• kohortové studie MeSH
• lidé MeSH
• mutace genetika   MeSH
• mutační analýza DNA MeSH
• narkolepsie genetika   MeSH
• neuropeptidy mozkomíšní mok   nedostatek   genetika   MeSH
• orexiny MeSH
• represorové proteiny genetika   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Research Support, N.I.H., Extramural MeSH
• Názvy látek
• DMAP1 protein, human MeSH Prohlížeč
• glykoprotein v myelinu oligodendrocytů MeSH
• HCRT protein, human MeSH Prohlížeč
• HLA-DQ beta řetězec MeSH
• HLA-DQB1 antigen MeSH Prohlížeč
• intracelulární signální peptidy a proteiny MeSH
• MOG protein, human MeSH Prohlížeč
• neuropeptidy MeSH
• orexiny MeSH
• represorové proteiny MeSH