Pediatric SARS-CoV-2–Related Diplopia and Mesencephalic Abnormalities

Case

A previously healthy, not vaccinated against SARS-CoV-2, 14-year-old adolescent boy presented with a 2-day history of binocular diplopia, particularly evident for distant vision, without any other neurologic symptom. At admission, a nasopharyngeal swab real-time PCR (RT-PCR) for detection of SARS-CoV-2 was negative. The boy reported persisting binocular diplopia at a distance primary position and lateral gaze bilaterally. Spontaneous nystagmus more right beating than left beating was present, whereas no ocular movement abnormality or ocular alignment defect was noticed. Pupils were isocyclic, isochoric, and normally reactive to light; no photophobia was recorded. Physical examination was otherwise normal. The boy was alert and oriented, denying weakness, headache, vomiting, or dizziness. Fundus examination was normal. Brain MRI revealed hyperintensity of the mesencephalic tegmentum and periaqueductal region on fluid attenuated inversion recovery and T2-weighted images, with no diffusion restriction or contrast enhancement (Figure, A.a. and A.b.).

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Figure Brain MRI Findings at the Onset and Follow-up

Sagittal (A.a) and axial (A.b) fluid attenuated inversion recovery (FLAIR) images at admission showing hyperintensity of the midbrain tegmentum (arrows) consistent with an inflammatory lesion. Sagittal (B.a) and axial (B.b) FLAIR images after 1 month showed a reduction of midbrain lesions. Sagittal (C.a) and axial (C.b) FLAIR images acquired after 4 months showed complete resolution of signal changes.

Spinal MRI was normal. Routine blood tests were normal, including inflammatory markers (erythrocyte sedimentation rate, c-reactive protein), thyroid function, and autoantibodies (antinuclear antibodies—ANA, anti-cardiolipin and anti–B2-glycoprotein antibodies). The results of infectious diseases detection tests are reported in Table 1.

CSF analysis showed increased glycorrhachia and pleocytosis; 9 cells were present, mostly mononucleated. No malignant cell was detected (Table 2).

CSF viral and bacterial molecular screening, as well as isoelectric focusing assay, anti–myelin oligodendrocyte glycoprotein, anti–aquaporine-4 and anti–N-methyl-d-aspartate antibodies, were negative. SARS-CoV-2 PCR experimentally tested on CSF was not detected (Table 1).

SARS-CoV-2 serology, using Snibe 2019-Novel Coronavirus (nCoV) Kit Ab, on MAGLUMI 800 (Snibe Co), a fully automated chemiluminescence immunoassay analyzer, showed immunoglobulin M 1.834 AU/mL (positive if ≥1) and immunoglobulin G 31.9 AU/mL (positive if ≥1,1).

Then, methylprednisolone pulses (30 mg/kg/d) were started and administered for 3 days, with a subsequent shift to oral prednisone (1 mg/kg/d) on the fourth day together with a course of intravenous immunoglobulin (400 mg/kg/d) for 5 days. Gradual improvement of nystagmus and diplopia with complete resolution in a few days was observed. MRI follow-up after 1 month showed a significant reduction of signal changes in the midbrain (Figure, B.a. and B.b.). Steroid therapy was progressively tapered and finally stopped after further 2 months. At the 4-month follow-up, neurologic examination and neuroimaging were normal (Figure, C.a. and C.b.).