Pain is Temporary, EBV is Forever: Epstein-Barr Virus-Associated Lymphoproliferative Disorder Causing Trigeminal Neuropathy

Background: Compressive lesions of the trigeminal nerve may cause debilitating trigeminal neuropathy. In addition to classic vascular compression, common compressive lesions are schwannomas or meningiomas, although lymphoma and lymphoproliferative disorders (LPD) have been reported. Epistein–Barr virus (EBV) infects over 90% of the world population and is associated with the development of various lymphoproliferative disorders (LPDs). EBV-LPDs can occur from either a B cell or T cell line, typically presenting as a lymphoma with systemic symptoms, with B cell predominant disease being far more common and extra-nodal occurrence noted chiefly in immunosuppressed patients. We present a case of an immunocompetent woman who was found to have an extra-nodal EBV-associated T-cell LPD of Meckel's cave causing trigeminal neuropathy

Methods: Case Report.

Results: A 62-year-old immunocompetent woman initially presented with progressive left V2–3 numbness and burning face pain over 4 weeks. MRI showed enhancement within left Meckel's cave, with direct involvement of the trigeminal nerve root to the brainstem. Blood tests and CSF examination was unremarkable for an infectious or inflammatory process. A PET scan showed avid FDG in the Meckel's cave lesion, along with sub centimeter enhancement of the right lower lobe of the lung, and in the T12 vertebral body. The patient underwent a biopsy of the thoracic spine which was inconclusive. As a result, the patient was taken to the operating room for a left sided middle fossa craniotomy and exploration of the Meckel's cave lesion. The lesion appeared to directly involve the trigeminal ganglion and V2–3 fascicles; an aggressive subtotal resection was completed. In the postoperative period, the patient's pain improved, and she noted increased numbness along V2 and V3 distributions. Pathology confirmed Epstein–Barr virus-associated lymphoproliferative disorder with CD2+, CD3+, CD8+, and TCR βF1+, but CD5- and CD7-, T-cell infiltrate.

Conclusion: Trigeminal neuropathy can be caused by solid lesion, such as schwnanomas or meningiomas, with LPDs remaining a rare cause. Currently, primary EBV+ LPDs of the cavernous sinus are reported in patients with autoimmune disorders, or immunosuppression, and almost always of a B-cell origin. Lymphomatous involvement of the trigeminal nerve has also been documented as a metastatic complication of lymphoma, but remains rare in immunocompetent patients. We present the first reported case of T-cell EBV-LPD causing focal left trigeminal neuropathy in a healthy immunocompetent patient, which improved with surgical debulking, highlighting the role of operative intervention to not only obtain diagnostic tissue but also treat the neuropathy.

Fig. 1 Left: preoperative coronal MR with Gadolinium. Right: preoperative axial MR with gadolinium.