European Academy of Neurology guideline on trigeminal neuralgia

Abstract

Background and purpose Trigeminal neuralgia (TN) is an extremely painful condition which can be difficult to diagnose and treat. In Europe, TN patients are managed by many different specialities. Therefore, there is a great need for comprehensive European guidelines for the management of TN. The European Academy of Neurology asked an expert panel to develop recommendations for a series of questions that are essential for daily clinical management of patients with TN. Methods A systematic review of the literature was performed and recommendations was developed based on GRADE, where feasible; if not, a good practice statement was given. Results The use of the most recent classification system is recommended, which diagnoses TN as primary TN, either classical or idiopathic depending on the degree of neurovascular contact, or as secondary TN caused by pathology other than neurovascular contact. Magnetic resonance imaging (MRI), using a combination of three high‐resolution sequences, should be performed as part of the work‐up in TN patients, because no clinical characteristics can exclude secondary TN. If MRI is not possible, trigeminal reflexes can be used. Neurovascular contact plays an important role in primary TN, but demonstration of a neurovascular contact should not be used to confirm the diagnosis of TN. Rather, it may help to decide if and when a patient should be referred for microvascular decompression. In acute exacerbations of pain, intravenous infusion of fosphenytoin or lidocaine can be used. For long‐term treatment, carbamazepine or oxcarbazepine are recommended as drugs of first choice. Lamotrigine, gabapentin, botulinum toxin type A, pregabalin, baclofen and phenytoin may be used either alone or as add‐on therapy. It is recommended that patients should be offered surgery if pain is not sufficiently controlled medically or if medical treatment is poorly tolerated. Microvascular decompression is recommended as first‐line surgery in patients with classical TN. No recommendation can be given for choice between any neuroablative treatments or between them and microvascular decompression in patients with idiopathic TN. Neuroablative treatments should be the preferred choice if MRI does not demonstrate any neurovascular contact. Treatment for patients with secondary TN should in general follow the same principles as for primary TN. In addition to medical and surgical management, it is recommended that patients are offered psychological and nursing support. Conclusions Compared with previous TN guidelines, there are important changes regarding diagnosis and imaging. These allow better characterization of patients and help in decision making regarding the planning of medical and surgical management. Recommendations on pharmacological and surgical management have been updated. There is a great need for future research on all aspects of TN, including pathophysiology and management.

Trigeminal neuralgia: An overview from pathophysiology to pharmacological treatments

“…80,81 Moreover, for long-term treatment, CBZ and OXC are recommended as the first choice. 79 CBZ blocks VGSCs resulting in inhibition of action potentials, reduction of synaptic transmission, and stabilization of the membrane potential in hyperexcitable neurons. 77,82,83 This effect is achieved in a use-and voltage-dependent manner because CBZ binds to the inactivated channels with higher affinity than to channels in the open or resting state.…”

“…126,127 While CBZ and OXC are the first-line drugs recommended for TN, there is not much known about their comparative efficacy in TN. 79 Furthermore, both drugs are associated with frequent and/or severe side effects, but the latter is suggested to have greater tolerability. [128][129][130][131][132][133] A systematic review of the effectiveness and safety of CBZ in different pain conditions concluded that 40% to 60% of the patients would exhibit adverse events, mainly impaired cognition, somnolence, dizziness, gastrointestinal symptoms, headaches, dry mouth or taste change, and mood changes.…”

“…So far, the quality of evidence for their use in TN management is low to very low. 79 Gabapentinoids (gabapentin and pregabalin) have become the mainstay of treatment for various pain syndromes, including fibromyalgia, diabetic neuropathy, and postherpetic neuralgia. 136 These anticonvulsants have been consistently shown to induce analgesia by targeting the a2d auxiliary subunit of VGCCs, [137][138][139] which results in impaired trafficking of VGCCs to the plasma membrane and subsequent reduction of neurotransmitter release and neuronal excitability.…”

“…Its use, as well of its prodrug fosphenytoin, is still recommended in the treatment of refractory TN or in acute exacerbations of pain. 79,150 Phenytoin is considered a weak blocker of VGSCs at hyperpolarized membrane potentials, but its inhibitory action is greatly enhanced by sustained membrane depolarization and during high-frequency channel activity. Compared with CBZ, it has three-fold higher affinity for depolarized channels, but CBZ binds to them at a five-fold faster rate (for review, see Mantegazza et al 151 ).…”

Facial Pain: A Comprehensive Review and Proposal for a Pragmatic Diagnostic Approach

“…Success rate is lower and the recurrence rate higher than those of microvascular decompression [29]. For a treatment algorithm, we further refer to a recently published review article and an even more recent guideline of the European Academy of Neurology [30,31]. PTN is clinically characterized by a more constant burning sensation in the trigeminal area and may be accompanied by sensory changes in this area or even weakness in the masticatory muscles.…”