10.1111/ene.13790Īrmangue T, Olivé-Cirera G, Martínez-Hernandez E, Sepulveda M, Ruiz-Garcia R, Muñoz-Batista M, et al. Encephalitis is an important clinical component of myelin oligodendrocyte glycoprotein antibody associated demyelination: a single-center cohort study in Shanghai, China. Wang L, ZhangBao J, Zhou L, Zhang Y, Li H, Li Y, et al. CLIPPERS mimickers: relapsing brainstem encephalitis associated with anti-MOG antibodies. 10.1016/j.msard.2020.102308īerzero G, Taieb G, Marignier R, Younan N, Savatovsky J, Leclercq D, et al.
Anti-myelin oligodendrocyte glycoprotein (MOG) associated disease masquerading as prolonged intractable nausea and vomiting. Myelin-oligodendrocyte glycoprotein antibody-associated disease.
Reindl M, Sato DK, Selmaj K, Siva A, Stankoff B, Tintore M, et al. (F–J) In the CLIPPERS-form case, extensive lesions were shown in the pons, pontibrachium, cerebellum, and hemispheres on T2-weighted images and after gadolinium-enhanced T1-weighted images, and a typical peppering sign was seen in both supratentorial and infratentorial. (E) A 44-year-old woman presented with numbness in her right upper extremity, and a hyperintense lesion in the cervical spinal cord was shown on sagittal T2-weighted images. (D) A 45-year-old woman presented with persistent nausea and vomiting, and MRI scans revealed a hyperintense lesion in the medulla on sagittal T2-weighted images. (B,C) A 26-year-old woman presented with acute vision loss of both eyes, and MRI scans revealed hyperintense lesions in the bilateral optic nerves on axial and coronal T2-weighted images. (A) A 40-year-old woman presented with acute vision loss of the right eye, and MRI scans revealed hyperintense lesion in the right optic nerve on T2-weighted images. MRI features of the representative patients. Further studies are warranted to determine the risk factors of relapse and identify the optimal steroid-sparing agents.ĬLIPPERS MOGAD area postrema syndrome case-series prognosis.Ĭopyright © 2022 Lei, Guo, Cui, Pu, Zhang and He. The long-term outcome of MOGAD seems benign. The clinical spectrum of MOGAD is heterogenous, wherein APS and CLIPPERS-form can occur. The median Expanded Disability Status Scale score at nadir was 3.5 (range 2-8) and was 0 (range 0-3) at the last follow-up.
At follow-up, twelve (44%) patients experienced a relapsing course, and the median time to the first relapse was 9.5 months (range 2-120). The median follow-up period was 20 months (range 6-127). Among them, six patients were treated with mycophenolate mofetil, three patients were treated with prednisone, rituximab, and teriflunomide, respectively. Moreover, nine patients received maintenance therapy. Intravenous methylprednisolone (IVMP) was administrated for 85% of the attacks while both IVMP and intravenous immunoglobulin were for 6% of the attacks. One patient was found to have anti-N-methyl-D-aspartate receptor antibodies both in his serum and cerebrospinal fluid. A total of 29 lumbar punctures were recorded, among which an elevated protein level was found in 34% of the samples, pleocytosis was found in 14% of the samples, and positive intrathecal oligoclonal bands were found in 19% of the patients. Another patient with RE presented with imaging characteristics of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). One patient presenting with RE also met the diagnostic criteria of area postrema syndrome (APS). Other presentations included rhombencephalitis (RE) (17%), limbic encephalitis (9%), simultaneous optic neuritis and myelitis (9%), acute disseminated encephalomyelitis (ADEM)-like presentation (6%), myelitis (4%), and ADEM (2%). A total of 47 episodes were observed, with optic neuritis (53%) being the most frequent presentation and 60% of them were unilateral. Clinical and para-clinical data, along with treatment outcomes of patients with MOGAD were analyzed.Ī total of 27 patients were identified, of which 19 (70%) patients were women, and the median age at disease onset was 40 years (range 20-67). This was a single-center case-series study. Therefore, this study aimed to report the clinical profiles and treatment outcomes of MOGAD in our center. However, the long-term management and prognosis of this disorder are still controversial. The clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is expanding over time.
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