Trigeminal Neuralgia in NF2-Related Schwannomatosis

Abstract NF2-related schwannomatosis (NF2) is an autosomal dominant syndrome resulting in a predisposition for benign tumors of the nervous system, including schwannomas, meningiomas, and ependymomas. There is a lack of data on the prevalence of trigeminal neuralgia (TN) in NF2 and the relationship between trigeminal schwannomas (TS) and TN in NF2 is also unclear. We sought to investigate the factors predisposing NF2 patients to TN. We performed a retrospective review of case notes and magnetic resonance imaging (MRI) in our cohort of adult NF2 patients over the age of 16 years meeting the international diagnostic criteria for NF2. We assessed presence and size of TS, size of vestibular schwannoma, clinical findings of neuropathy/pain, history of headache disorder, and intervention. We then looked at the subset of patients with TS and our radiologist reviewed their MRI brain imaging Fifteen of 189 NF2 patients had TN. Only six had ipsilateral TS. Ipsilateral vestibular schwannomas were significantly larger in the TN subgroup, as was history of ipsilateral radiation and migraine. The majority of TN patients did not have an ipsilateral trigeminal schwannoma. Our data suggest factors associated with TN include size of vestibular schwannoma, history of previous radiotherapy treatment, and migraine.