Approach
Pain relief is the goal of therapy. Patients with trigeminal neuralgia (TN) should be offered psychologic and nursing support. Patients with secondary TN generally respond less well to medical therapy or surgical treatment. As no treatment has sufficient evidence to prove its specific efficacy in patients with secondary TN, they should be treated similarly to patients with primary TN.[20]
Medical therapy
Medical therapy is the initial treatment of choice. The mainstay of medical therapy consists of anticonvulsant medications, generally carbamazepine or oxcarbazepine.
Carbamazepine is the only anticonvulsant medication with efficacy proven in randomized controlled trials, and is typically first-line therapy.[20][22][26][27] [
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Almost 90% of patients achieve meaningful initial pain control with carbamazepine or oxcarbazepine. However, long-term use may be associated with decreased efficacy.[2]Oxcarbazepine, a derivative of carbamazepine with fewer drug-drug interactions and a lower adverse effect profile, appears equally efficacious. The individual response to both drugs varies significantly, therefore if one is not effective, the other can be trialled.[2][3]
If carbamazepine and oxcarbazepine are not tolerated or are ineffective, lamotrigine can be used as monotherapy or add-on therapy.[2][3][20] One small randomized cross-over trial (n=14) showed that lamotrigine was efficacious as an add-on therapy for patients with refractory TN.[28]
Development of allergic rash with use of carbamazepine or lamotrigine may be a precursor to the development of potentially serious reactions (e.g., Stevens-Johnson syndrome) and should prompt immediate discontinuation.
Gabapentin or pregabalin may also be used as monotherapy or add-on therapy.[2][3][4][20] Clinical experience indicates that these agents are less effective than carbamazepine or oxcarbazepine, but are associated with fewer adverse events.[4]
Patients whose TN is partially or completely refractory to anticonvulsant medications are often tried on other classes of pain medication, such as baclofen, although data supporting their use in TN are scant.[3][20] Baclofen may be helpful for TN in patients with multiple sclerosis who are using the drug for symptoms of spasticity.[2] Based on practitioner preference, it can be given sequentially as monotherapy or in combination until an effective regimen is established.
Surgical therapy
Considered when pharmacologic management fails. The choice of procedure is ultimately dependent upon patient preference but should only be undertaken after a complete discussion and understanding of the efficacy, recurrence rates, and potential complications of each method.
Microvascular decompression
Microvascular decompression is the first-line procedural treatment for classical TN, as it is targeted to the presumptive pathologic initiator of TN and has the least long-term neurologic sequelae, with rates and durability of symptom improvement that are equal to or better than other surgical/procedural treatments.[20][22][29][30] A pooled analysis including 5149 patients showed that microvascular decompression is efficacious, with 62% to 89% of patients pain-free at follow-up (after 3-11 years). Severe complications, such as death, stroke, or meningitis, are rare. Other possible complications include cranial nerve palsy (4%), hearing loss (1.8%), and facial hypoesthesia (3%).[20]
Ablative procedures
Neuroablative techniques should be strongly considered for idiopathic TN.[20] Patients who do not want open surgery or who carry an unacceptably high surgical risk may be offered radiosurgery as a first option, with percutaneous methods typically reserved for patients with V2 and V3 distribution symptoms, as they are less likely to develop postprocedural impairment of corneal sensation.[31][32] There are a number of ablative techniques available:
Stereotactic radiosurgery involves targeting the sensory root of the trigeminal nerve stereotactically to deliver high doses of radiation without significant spread to surrounding tissues. It is the least invasive surgical option, and pain relief typically occurs within weeks to months with maximum pain improvement at 1 month. Between 30% and 66% of people undergoing stereotactic radiosurgery achieve long-term remission.[20] The procedure should be carried out in specialist centers and patients should be selected by a multidisciplinary team experienced in the management of TN.[33]
Trigeminal gangliolysis can be thermal (radiofrequency) or chemical (glycerol). Between 26% and 82% of patients achieve remission after radiofrequency gangliolysis and 19% to 58% achieve remission after glycerol injection.[20]
Balloon compression of the trigeminal ganglion requires general anesthesia. It has similar efficacy and recurrence rates as gangliolysis but with decreased rates of severe dysesthesia and impaired corneal sensation. Pooled analyses reported that with a follow-up of 4-11 years, 55% to 80% of patients (n=755) with TN were pain-free after balloon compression.[20]
The most common complications for ablative procedures are facial hypoesthesia (19%), corneal hypoesthesia (5%), and trigeminal motor weakness (5%). Meningitis (0.7%) and anesthesia dolorosa (0.5%) are rare complications.[20]
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