Elevated intracranial pressure (ICP) has been described in patients with demyelinating diseases of the central nervous system (CNS). Elevated ICP is classified into idiopathic intracranial hypertension (IIH), where a clear precipitant is not found; and secondary intracranial hypertension, in which elevated ICP is attributed to an underlying condition. This case illustrates a patient with markedly elevated ICP who was found to have myelin oligodendrocyte glycoprotein (MOG) antibody associated disease after being initial diagnosed with IIH and with an incidental finding of a malignant thyroid nodule.
A 17 year old previously healthy male transferred from an outside hospital due to concern for idiopathic intracranial hypertension (IIH).
Two months prior, he had a generalized tonic-clonic seizure lasting 10 minutes, with subsequent left-sided weakness, confusion and difficulty speaking lasting 5 days. EEG and MRI were normal. Six weeks later, he developed left eye pain and blurry vision. Ophthalmologic evaluation showed bilateral optic nerve swelling and he was prescribed acetazolamide. Brain MRI/MRA suggested increased intracranial pressure. Lumbar puncture (LP) opening pressure was 28 cmH2O and CSF was negative for infection. Post-LP he developed severe headaches and his neurologist stopped the acetazolamide due to concern for a CSF leak.
The day of presentation, he developed right eye pressure and blurry vision and was admitted due to concern for IIH. He reported no numbness, tingling, fever, mental status changes, or decreased appetite.
The patient was afebrile, in no acute distress. Neurological examination showed pain with extraocular movements, bilateral 5 mm dilated pupils with adequate response to light, decreased visual acuity 20/50 OD and 20/40 OS. Fundoscop revealed bilateral mild optic nerve swelling.
Differential diagnosis included optic neuritis, IIH, multiple sclerosis, or an intracranial mass. Brain and optic nerve MRI showed bilateral optic neuritis with scattered white matter hyperintensities. MRI total spine was normal. Repeat LP opening pressure was 40cmH2O with 24 WBC, normal protein, and glucose. Oligoclonal band profile was negative. Autoimmune demyelinating antibody panel in the serum was positive for MOG IgG at a titer of 1:40.
He was diagnosed with Myelin Oligodendrocyte Glycoprotein Antibody-Associated disease (MOGAD) due to symptoms consistent with optic neuritis, MRI with characteristic pattern of demyelination and positive serum MOG IgG.
MOGAD is a demyelinating disease whose most common manifestation is optic neuritis. This can present with various degrees of vision loss and is almost always associated with eye pain. IIH can mimic the clinical features of MOGAD and other demyelinating diseases of the CNS, which can also present with increased CSF opening pressures.
This case underscores the importance of a complete work-up for demyelinating diseases of the CNS in patients presenting with bilateral optic disc edema and increased intracranial pressure, especially when presenting with other associated neurologic symptoms like seizure or central vision changes.
What will audience learn from your presentation? ???????
- This case provides a clinical example of how IIH can be misdiagnosed in patients with demyelinating CNS disorders, the audience will recognize the importance of broadening their differential diagnosis for patients presenting with increased ICP.
- Learners will review the clinical clues that should raise your suspicion for a demyelinating CNS disorder in patients presenting with increased ICP, such as co-existing seizures, central vision changes, and increased CSF pleocytosis or increase protein.
- Further research such as prospective cohort studies in patients presenting with symptoms of increased ICP, examining the frequency of a misdiagnosis of IIH are needed to determine how common this scenario is in Pediatric Neurology and Hospital Medicine