Idiopathic Intracranial Hypertension Manifested with Combined Trigeminal and Glossopharyngeal Neuralgia

Introduction: Idiopathic intracranial hypertension (IIH), known for the combination of headache and progressive vision loss, affects young, usually obese White women. The definitive treatment is surgery.

Methods: We presented a case of IIH manifested as simultaneous glossopharyngeal and trigeminal neuralgia in a 28 year old Caucasian female.
She reported a 3-year history of facial, mouth, throat and ear pain. Conservative treatment was ineffective.
Her comorbidities included obesity class III (BMI 42). Neuro exam revealed dysesthesia in left V1 and V3 distribution. MVD of the left CN V,VII,VIII and IX was performed; notably CSF opening pressure was high during placement of the lumbar drain.
We found that the superior surface of CN V was compressed by SCA at the root entry zone, the CN VII-VIII complex by the loop of AICA, and CN IX by the loop of PICA. All nerves were decompressed and padded with teflon. On the 4th day after surgery the existing lumbar drain, which had been draining 10 cc/hr, was closed prior to removal. Six hours later, the trigeminal and glossopharyngeal neuralgia recurred. Release of approximately 15 ml of CSF dropped pressure to 150-190 mm H2O, reduced pain to the minimum, and was consistent after several trials. Pain recurrence at all times was accompanied by elevation of the CSF pressure above the 330mm H2O and occurred roughly six hours after lumbar drain closure. Real time pressure measurements were consistent with pain intensity. VP shunt (Codman Certas valve set at 4) led to complete resolution of the symptoms.

Results: Comprehensive evaluation did not reveal any cause for elevated intracranial pressure. Nonetheless, the altered CSF dynamics justified the shunting procedure. The patient was discharged home on the 9th day after surgery. Two weeks later, she remains stable and free of any pain.

Conclusion : Presented case remains unique in the literature and provides valuable information about the diverse presentation of IIH. Even with the unusual and vague clinical manifestations the definitive treatment must not be delayed so dramatic complications can be prevented.