A 3-year-old child with acute vision loss: Optic Neuritis in the setting of Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease

Case Presentation

A 3-year-old female patient presented to the pediatric emergency clinic with ‘worsening vision loss and imbalance’ in one day. The patient had been treated for urinary tract infection for the last 2 weeks with ceftriaxone and cephalexin. During the initial visit, the laboratory could not run the culture from the urine sample, and the post-antibiotic urine culture obtained later failed to demonstrate bacterial growth. Otherwise, the patient had no significant familial, past medical, or surgical history. Physical examination confirmed vision loss with ‘papilledema, sluggishly reactive dilated pupils, and limited upward gaze’.

Initially, a non-contrast head CT was requested after the initial evaluation with the suspicion of ‘increased intracranial pressure’ and to rule out acute intracranial abnormalities. The CT scan ([Fig. 1]) demonstrated no acute intracranial abnormality. However, the study revealed papilledema with swollen optic nerves and surrounding fat stranding. Follow-up MRI orbits ([Fig. 2]) confirmed papilledema with ‘heterogenous enhancement of bilateral swollen optic nerves back to the prechiasmatic segment and surrounding fat stranding’. Additionally, the obtained MRI venogram was unremarkable without dural venous sinus or cortical vein thrombosis and dural venous sinus stenosis.

Fig. 1 Noncontrast CT brain: Axial image demonstrates bilateral papilledema (blue arrows), optic nerve swelling (yellow dashed lines), and mild fat stranding (red arrow).

Fig. 2 MRI brain and orbits: Coronal T2 fat saturation, coronal postinfusion T1 fat saturation, and axial diffusion (b:1000) weighted images. Diffusely swollen anterior (prechiasmatic) optic nerves (green arrows) with diffuse enhancement (red arrows) and long segment increased diffusion signal (yellow arrows).

The patient was admitted to the pediatric ICU for close monitoring. The bedside lumbar puncture was significant, with elevated WBC and protein. However, opening pressure was normal, and no organisms were observed ([Table 1]).

Table 1 Lumbar Puncture Findings.

Variant	Result	Ref
Ref: Reference, neg: negative, pos: positive
Opening Pressure (cmH2O)	13.5	6–25
WBC (/uL)	34	neg
Protein (mg/dL)	46.2	15–45
Glucose (mg/dL)	55	45–70
HSV-1 DNA	neg	neg
HSV-2 DNA	neg	neg
VZV DNA	neg	neg
CMV ab	neg	neg
EBV ab	neg	neg
Cytology	neg	neg
Culture	neg	neg
Oligoclonal band	neg	neg
AQP4 ab	neg	neg

The patient was treated with acyclovir, methylprednisone, and IVIG. Acyclovir was discontinued as HSV and VZV tests were negative. The patient was treated with high-dose methylprednisolone for 5 days with a 3-month steroid taper and IVIG for 3 days. The patient’s vision significantly improved during the hospital admittance for 7 days. AQP4-Ig NMOSD, MOG antibody disease, and MS were considered underlying etiology for the bilateral optic neuritis. The serum MOG antibody test was positive and all the other serum and CSF markers including oligoclonal bands and AQP-4 antibodies were negative.

The interim 2-month follow-up MRI brain ([Fig. 3]) demonstrated near complete resolution of the bilateral orbital nerve swelling and enhancement. Serum anti-MOG antibody test 5 months after the initial diagnosis was negative.

Fig. 3 Two month follow up MRI brain: Axial T2/FLAIR and coronal post infusion non-fat saturation T1 weighted images. Near complete resolution of the optic nerve swelling and papilloma. No discrete enhancement was seen within the limitation of the non-fat saturated imaging.

Publikationsverlauf

Eingereicht: 28. Mai 2024

Angenommen nach Revision: 21. Januar 2025

Artikel online veröffentlicht:
18. Februar 2025