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Published on: April 14, 2014
Diagnostic delay in patients with myelin oligodendrocyte antibody-associated disease presenting with elevated
Taylor M Lakusta-Wong1, Kenrick E Wysong2, Andrew J Solomon1
1Larner College of Medicine at the University of Vermont, Department of Neurological Sciences, 111 Colchester Ave, Burlington, VT, 0540, USA.
Background:
Elevated intracranial pressure (ICP) is a recently recognized complication of myelin oligodendrocyte antibody associated disease (MOGAD).
Objective:
We describe 5 patients with MOGAD presenting with elevated ICP.
Methods:
Serum MOG antibody testing completed at the University of Vermont Medical Center between January 1, 2017 to May 1, 2025 was reviewed. Patients with MOGAD were included. Elevated ICP was defined as opening pressure > 25 cm H2O, or opening pressure >20 cm H2O with an initial diagnosis of idiopathic intracranial hypertension (IIH).
Results:
Of 9 patients with MOGAD who had opening pressures measured, 5 patients (56%) had elevated ICP. Corticosteroid treatment was delayed due to higher clinical suspicion for alternate diagnoses, with idiopathic intracranial hypertension being the most common misdiagnosis. One patient underwent brain biopsy given concern for malignancy.
Conclusion:
Red flags including rapidly progressive vision loss, lack of improvement on acetazolamide, CSF pleocytosis, and enhancement on neuroimaging may help differentiate MOGAD from IIH. Increased recognition of elevated ICP as a complication of MOGAD is needed to prevent potential morbidity associated with unnecessary diagnostic evaluation and treatment delay.
Insights
Elevated intracranial pressure (ICP) is a complication of myelin oligodendrocyte antibody-associated disease (MOGAD). Early recognition of MOGAD-related ICP is crucial to avoid misdiagnosis and treatment delays.
Area of Science:
- Neuroimmunology
- Neurology
- Ophthalmology
Background:
- Elevated intracranial pressure (ICP) is an emerging complication of myelin oligodendrocyte antibody-associated disease (MOGAD).
- This study investigates the presentation and diagnosis of ICP in MOGAD patients.
Purpose of the Study:
- To describe 5 patients with MOGAD who presented with elevated ICP.
- To highlight diagnostic challenges and potential red flags differentiating MOGAD-associated ICP from idiopathic intracranial hypertension (IIH).
Main Methods:
- Retrospective review of serum MOG antibody testing at a single center (2017-2025).
- Inclusion of patients diagnosed with MOGAD and measured opening pressures.
- Definition of elevated ICP based on cerebrospinal fluid (CSF) opening pressure thresholds and/or IIH diagnosis.
Main Results:
- 56% (5/9) of MOGAD patients with measured opening pressures had elevated ICP.
- Treatment delays occurred due to misdiagnosis, most commonly as IIH.
- One patient underwent unnecessary brain biopsy due to concern for malignancy.
Conclusions:
- Red flags such as rapid vision loss, lack of response to acetazolamide, CSF pleocytosis, and neuroimaging enhancement may help distinguish MOGAD from IIH.
- Increased awareness of elevated ICP as a MOGAD complication is vital to prevent diagnostic delays and associated morbidity.
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