Bilateral symmetrical deep gray matter involvement and leptomeningeal enhancement in a child with MOG-IgG-associated encephalomyelitis

Abstract

Background Currently, myelin oligodendrocyte glycoprotein (MOG)-IgG-associated encephalomyelitis (MOG-EM) is regarded as an independent inflammatory demyelinating disease. Magnetic resonance imaging (MRI) abnormalities occur in 44.4% of patients with MOG-EM. However, symmetrical deep gray matter involvement with leptomeningeal enhancement is rarely described in the literature. Case presentation A 3-year-old boy was admitted to our hospital because of acute onset fever, headache, vomiting and disturbance of consciousness. Neurological examination showed somnolence, neck stiffness and positive Kernig’s sign. Brain MRI demonstrated bilateral symmetrical lesions in the basal ganglia and thalamus as well as diffuse leptomeningeal enhancement along the sulci of bilateral hemisphere. Cerebrospinal fluid analysis demonstrated increased cell count (7 cells/mm3, mononuclear cells dominant) and protein (1.17 g/L) without glucose and chloride abnormality. Work-up for infectious and autoimmune causes, serum MOG IgG was positive by cell based assay. Therefore, a diagnosis of MOG-EM was established according to the international recommendatory criteria in 2018. He was administrated with intravenous methylprednisolone followed by oral corticosteroids and had recovered completely within 1 week. Conclusions In the setting of meningoencephalitis-like clinical presentation with bilateral symmetrical deep gray matter involvement, MOG-EM should be distinguished from other infectious and autoimmune disorders, such as Epstein-Barr virus (EBV) encephalitis, Japanese encephalitis and Anti-NMDA receptor (NMDAR) encephalitis. Besides, aseptic meningitis associated with leptomeningeal enhancement may be an atypical phenotype of MOG-EM.

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-AD-associated meningitis, which were described to have fever, nausea, and nuchal rigidity, have been reported including our case ( Table ) ( 10 - 16 ). The mean age of these patients was 24.4 years (range: 3-55); four of these patients were male.…”

“…Meningeal involvement may possibly occur as sequelae associated encephalitis because MOG-AD frequently presents with encephalitis. However, one case ( 16 ) had only subcortical encephalitis, it possibly means that inflammation spread to the meninges. Another potential mechanism is the spread of inflammation from the meninges to the brain.…”

Leptomeningeal enhancement of myelin oligodendrocyte glycoprotein antibody-associated encephalitis: uncovering novel markers on contrast-enhanced fluid-attenuated inversion recovery images

“…Previously, LME was reported in only a small number of case reports, rarely described in children with MOG-E ( 22 – 24 ), and considered to be an atypical MRI finding of MOG-E ( 25 – 27 ). In the current study, we found that LME in MOG-E is common on CE-FLAIR.…”

Rare nervous system involvement in an anti-myelin oligodendrocyte-positive case: spinal leptomeningeal involvement

“…However, few anti-MOG-positive cases have been reported with leptomeningeal involvement as case reports in the literature. Reported rare cases with leptomeningeal involvement are associated with cranial leptomeningeal involvement [13][14][15].…”