Table 2 Case vignettes of patients at risk of MOG-IgG seropositivity (examples)
From: MOG encephalomyelitis: international recommendations on diagnosis and antibody testing
Example 1: 35-year-old woman presenting with bilateral acute ON. Develops transient blindness; fundoscopy shows papilledema; lumbar puncture reveals lymphomonocytic pleocytosis with 10% neutrophils and negative OCBs; brain MRI shows perioptic Gd enhancement but is otherwise normal; flaring up of symptoms after tapering of oral steroids; later recurrent ON attacks, stabilization with rituximab. Example 2: 40-year-old woman with two attacks of acute, OCB-negative myelitis. Spine MRI shows an isolated short spinal cord lesion at first attack and a longitudinally extensive spinal cord lesion at relapse; brain MRI abnormal but no Dawson’s finger-type lesion, no juxtacortical U fibre lesion, and no lesion adjacent to a lateral ventricle that is ovoid or associated with an inferior temporal lobe lesion [36, 37, 50]; flaring up of myelitis symptoms after discontinuation of intravenous steroid treatment, good response to PEX. Example 3: Young man with a previous diagnosis of “OCB-negative RRMS”. Predominantly ON and myelitis attacks; conus lesion with severe erectile and sphincter disturbance after first myelitis; longitudinally extensive optic nerve lesion with involvement of the optic chiasm; increase in relapse rate under treatment with interferon-beta but stabilization with rituximab. Example 4: 42-year-old woman presenting with incomplete, painful tetraparesis. Previous diagnosis of RRMS with positive OCB; spinal cord MRI reveals a contiguous lesion extending from C3 to T1; negative serology for AQP4-IgG. Example 5: ADEM-like presentation with large white matter lesions and disturbance of consciousness, brainstem lesions, and involvement of the entire spinal cord in a 25-year-old woman; onset 3 weeks after vaccination. Example 6: Simultaneous unilateral ON and LETM extending into the brainstem in a 39-year-old man. CSF pleocytosis (90 white cells/μl) with 5% neutrophils; no CSF-restricted OCB; negative AQP4-IgG serostatus. Example 7: Young woman presenting with recurrent and steroid-dependent isolated ON, previously classified as CRION; normal brain MRI. Example 8: Young man with acute encephalitis and seizures. MRI reveals large cortical/subcortical white matter lesions not involving the inferior temporal lobe; good response to steroids; negative for typical viral and autoimmune causes of encephalitis. |