(E) Nine weeks later, the pontine lesion no longer demonstrates contrast enhancement and there was a reduction in the extent of abnormal high T2 signal in the pons and middle cerebellar peduncles. There was no restricted diffusion and no supratentorial lesions. (C, D) T1-weighted MRI shows curvilinear, punctate, and nodular gadolinium contrast enhancement most severe adjacent to the surface but extending into the center of the pons (C, white arrow) and cerebellar peduncles (D, white arrow). There is diffuse but patchy signal change in pons (A, dark arrow), extending into the cerebellar peduncles (B, white arrow) with some associated swelling but without substantial mass effect or hydrocephalus. (A, B) T2-weighted MRI shows axial slices through pons. Angiotensin converting enzyme levels were normal, and antinuclear antibody screening was negative. Flow cytometry identified reactive T cells (CD4:CD8 ratio 3:1). Microscopy, culture, and viral PCR were negative. CSF showed 2 lymphocytes/µL and elevated protein (686 mg/L). 1 CT of the chest/abdomen/pelvis was normal. Her gait was ataxic.īrain MRI showed patchy pontocerebellar signal change ( figure, A–D), consistent with CLIPPERS. Limb reflexes were brisk, without clonus. Visual acuities and fundal appearances were normal, as were tone, power, sensation, and sphincter function. There were no constitutional symptoms suggesting systemic illness.Įxamination revealed diplopia, horizontal nystagmus on left gaze, dysarthria, and left-sided facial weakness. Shortly before admission, she developed slurred speech, gait ataxia, and double vision. Although neuromyelitis optica spectrum disorders (NMOSD) with brainstem involvement may feature in the broad differential diagnosis of CLIPPERS, this is the first report describing an overlap with the anti-MOG phenotype of NMOSD, and highlights that CLIPPERS may not be a distinct nosologic entity.Case report.Ī 36-year-old woman presented with a 2-week history of dizziness, left facial paresthesia, allodynia, and altered intraoral sensation. Five months later, she developed a longitudinally extensive spinal cord inflammatory lesion affecting mainly the conus, and had antibodies to myelin-oligodendrocyte glycoprotein (MOG). Our patient initially presented with classical clinicoradiologic features of CLIPPERS. 1 Autoimmunity has been postulated, although specific CNS antibodies have not been reported. eCollection 2018.Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory brainstem syndrome of uncertain etiology, with distinct radiologic features. Defining distinct features of anti-MOG antibody associated central nervous system demyelination. (4) Weber MS, Derfuss T, Metz I, Brück W. MOG-IgG-Associated Optic Neuritis, Encephalitis, and Myelitis: Lessons Learned From Neuromyelitis Optica Spectrum Disorder. (3) Dos Passos GR, Oliveira LM, da Costa BK, et al. Diagnosis and treatment of anti-myelin oligodendrocyte glycoprotein antibody positive optic neuritis. Comparison of myelin oligodendrocyte glycoprotein (MOG)-antibody disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) when they co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis. 4 Furthermore, those with MOG antibody disease seem to be less likely to have other autoimmune disorders (such as rheumatoid arthritis, Hashimoto’s thyroiditis, etc.) than those with AQP-4 positive NMOSD. MOG antibody disease and AQP-4 positive NMOSD are thought to have distinct immunological mechanisms. Those with MOG antibody disease do not test positive for the NMO antibody called aquaporin 4 (AQP-4). Patients with persistently positive antibodies are at risk for recurrent events. Those with MOG Antibody Disease may previously have been diagnosed with Neuromyelitis Optica Spectrum Disorder (NMOSD), Transverse Myelitis (TM), Acute Disseminated Encephalomyelitis (ADEM), Optic Neuritis (ON), or multiple sclerosis (MS) because of the pattern of inflammation it causes including brain, spinal cord and optic nerve damage. 3 The diagnosis is confirmed when MOG antibodies in the blood are found in patients who have repeated inflammatory attacks of the central nervous system. 1,2 While the function of this glycoprotein is not exactly known, MOG is a target of the immune system in this disease. Myelin oligodendrocyte glycoprotein (MOG) is a protein that is located on the surface of myelin sheaths in the central nervous system. MOG antibody disease (MOGAD) is a recently coined neuro-inflammatory condition that preferentially causes inflammation in the optic nerve but can also cause inflammation in the spinal cord and brain. This information sheet has been reviewed and approved by members of SRNA’s Medical and Scientific Council.
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