Abstract
Objectives
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.
Methods
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
Results
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.
Conclusion
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.
Keywords
NF2 - schwannomatosis - trigeminal neuralgia - neurofibromatosis type 2