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.

Abstract

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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