To describe a phenotype of Myelin oligodendrocyte glycoprotein antibody associated disease(MOGAD) presenting with aseptic meningitis, elevated intracranial pressure(ICP) requiring cerebral spinal fluid(CSF) diversion via external ventricular drain(EVD), seizures, unilateral optic neuritis, multifocal transverse myelitis and acute respiratory failure.

MOGAD is an immune mediated inflammatory process of the CNS causing demyelination of optic nerves, brain and spinal cord.  MOG is a component of myelin and found on the surface of oligodendrocytes.  MOG antibody associated disease has been associated with multiple phenotypes including optic neuritis, transverse myelitis, seizures, elevated intracranial pressure, acute disseminated encephalomyelitis (ADEM) and aseptic meningitis.

Case Presentation:

18 year old female presents with five days of headache and bilateral eye pain progressing to nausea, dizziness, blurry vision and fever. Exam positive for photophobia and nuchal rigidity. CSF analysis with neutrophilic predominant pleocytosis. Symptoms resolved after lumbar puncture.  The following day she had multiple seizures requiring intubation for airway protection.  Repeat CSF sampling with a high opening pressure of 55mmH20 necessitated EVD for CSF diversion and ICP monitoring. MRI Brain revealed diffuse leptomeningeal enhancement consistent with meningitis. Patient was extubated but experienced rapid progression quadriparesis requiring reintubation. MRI of the neuroaxis demonstrated T2 signal intensity of C3 to T1 and within the conus medullaris suggestive of transverse myelitis. MRI orbits revealed left sided optic neuritis.  She was treated with pulse dose steroids, acetazolamide and plasma exchange.  

CSF studies were positive for MOGAD. As treatment continued, the patient's strength rapidly improved, she was successfully extubated and EVD was weaned.

MOGAD is a demyelinating syndrome that can present as many different phenotypes and patients who present with elevated intracranial pressure may benefit from early CSF diversion and ICP monitoring.