Relapsing optic neuritis and meningoencephalitis in a child: case report of delayed diagnosis of MOG-IgG syndrome

Abstract

Background Recurrent optic neuritis (ON) was previously thought to be associated with multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD). Meningoencephalitis has recently been suggested to be a clinical finding typical of myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis. We report a Chinese patient with recurrent ON at disease initiation, who had a delayed diagnosis of MOG-IgG syndrome, until recurrent meningoencephalitis appeared and serum MOG-IgG was detected. Case presentation From the age of 7 years, an AQP4-IgG negative female patient had 10 disease recurrences, including 4 episodes of recurrent ON, 4 episodes of fever and meningoencephalitis, and 2 episodes of ON as well as meningoencephalitis. She was initially diagnosed as recurrent ON and treated with glucocorticoids followed by gradual tapering when ON reoccurred. Later, she was diagnosed as central nervous system infection when fever and meningoencephalitis appeared, and antiviral drugs and glucocorticoids were used. However, when she returned to our department for follow-up on July 2017, the results of serum demyelinating autoimmune antibody revealed positive MOG-IgG (titer 1:320 by an in-house, cell-based assay using live cells transfected with full-length human MOG). A diagnosis of MOG-IgG syndrome was established. Conclusions Testing for MOG-IgG in atypical MS and NMOSD patients, and patients with meningoencephalitis with a history of relapsing demyelinating symptoms is warranted. Electronic supplementary material The online version of this article (10.1186/s12883-019-1324-4) contains supplementary material, which is available to authorized users.

Clinical and imaging features of children with autoimmune encephalitis and MOG antibodies

“…15 Two further single cases were recently published, describing children with symptoms suggestive of encephalitis associated with MOG abs but with MRI features more reminiscent of ADEM underscoring the need for precise terminology and diagnostic standards for children with MOG-SD. 25,26 Recently, Armangue et al showed that the spectrum of MOG-SD is wider than previously reported including children with encephalitis. 27 In their large cohort of children with ADS and encephalitis, they found that 22/296 children with definite or possible AE harbored MOG abs.…”

Myelin Oligodendrocyte Glycoprotein-antibody-associated Disorder Presenting with Corticomeningeal Encephalitis Prior to the Onset of Optic Neuritis

“…To the best of our knowledge, 7 cases of MOG-ADassociated meningitis, which were described to have fever, nausea, and nuchal rigidity, have been reported including our case (Table) (10)(11)(12)(13)(14)(15)(16). The mean age of these patients was 24.4 years (range: 3-55); four of these patients were male.…”