Delayed Tumefactive Demyelination in a Patient with Meningioma and Optic Neuritis: Case Report and Review of the Literature

Background: Brain meningiomas (MGM) can be associated with visual disturbances. However, acute optic neuritis (ON) is rarely encountered. Radiotherapy is frequently used as a treatment modality. Cases of de-novo multiple sclerosis (MS) or exacerbation of an existing disease have been previously described following brain irradiation. The mechanism is thought to be related to localized or diffuse blood brain barrier disruption and inflammation. 

Objectives: To present a case of a woman who initially presented with ON, found to have a MGM and later developed tumefactive demyelination (TD) following radiotherapy. A review of relevant literature will be provided. 

Methods: Case report and literature review.

Results: A 54-year-old right handed woman presented with painful right eye and blurry vision. She was diagnosed with right ON and treated with IV steroids with good response. Magnetic resonance imaging (MRI) showed an incidental right cavernous sinus MGM (separate from the optic nerve). Fractional radiotherapy was performed (total dose of 50.4.Gy). Overall, patient improved and the size of her MGM remained stable. However, 30 months later, she presented with blurry vision, binocular diplopia, and gait imbalance. At that time, brain MRI showed numerous areas of abnormal FLAIR/T2 signal, involving white matter structures within the corona radiata, centrum semiovale and subcortical regions. The largest area of abnormal signal was demonstrated in the right parieto-occipital region and measured 1.8 x 2.3 x 2.7 cm. T1 enhancement was evident in multiple lesions and incomplete ring enhancement was identified. CSF analysis showed 6 unmatched oligoclonal bands, elevated immunoglobulin G synthesis rate and unremarkable cytology. A diagnosis of TD was established and patient was treated with steroids.  Clinical follow up data for 2 years will be revealed at the CMSC conference. Review of literature shows a few cases where MS was triggered or worsened following brain radiotherapy. Also, experimental studies support the notion that brain radiation can have either early and/or delayed adverse effects on brain tissue such as localized or disseminated demyelination and/or brain necrosis.

Conclusions: Our patient represents an atypical case of quiescent MS that was unmasked later in life by localized disruption imposed by the brain MGM and/or brain radiotherapy.  Further research is needed to look for similar associations and perhaps confirm such potential relationships.