A young Caucasian male (7y) with normal BMI was atypical for his provisional diagnosis of Idiopathic Intracranial Hypertension (IIH), that resolved following a Lumbar Puncture (LP). At 8y he presented with a 2-week history of headaches and vomiting that started some weeks after flu vaccination and an upper respiratory infection.
Visual Acuity (VA) and colour vision were normal. Ocular motility was full. Fundoscopy and OCT showed recurrence of papilloedema, with enlarged blind spots on Kinetic perimetry.
LP opening pressure was 30cm H2O and CSF white cells were elevated (23). Repeat brain and spine imaging showed new white matter signal changes in keeping with neuroinflammation, as well as enhancement of the left optic nerve extending to the chiasm and optic tract. VA, colour vision and pupillary reactions remained normal.
Pattern VEP peak times were prolonged from the left eye compared to right eye to small check widths, consistent with relative macular-cortex pathway dysfunction. Hemifield PVEPs were slightly prolonged and reduced from the bitemporal fields indicating chiasmal dysfunction. Normal PERGs excluded PVEP delay associated with primary RGC disease.
Further investigations showed oligoclonal band and serum-MOG antibody positivity.
Management: Initial treatment with Acetazolamide 125mg bd for a week, following LP, was changed to IV methylprednisolone followed by oral prednisolone.
Symptoms improved significantly following LP and steroid treatment. He will be followed in a Demyelination Clinic.
MOG-associated disease has been reported with raised intracranial pressure and should be considered especially in children with atypical clinical phenotype for IIH.
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