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Trigeminal Neuralgia Review Article 04/25/2018 on mAXWo3ZnzwrcFjDdvMDuzVysskaX4mZb8eYMgWVSPGPJOZ9l+mqFwgfuplwVY+jMyQlPQmIFeWtrhxj7jpeO+505hdQh14PDzV4LwkY42MCrzQCKIlw0d1O4YvrWMUvvHuYO4RRbviuuWR5DqyTbTk/icsrdbT0HfRYk7+ZAGvALtKGnuDXDohHaxFFu/7KNo26hIfzU/+BCy16w7w1bDw== by https://journals.lww.com/continuum from Downloaded Downloaded Address correspondence to Giorgio Cruccu, Department of Neurology and Psychiatry, from Trigeminal Neuralgia Viale Universita´ 30, Rome, https://journals.lww.com/continuum Italy 00185, Giorgio Cruccu, MD [email protected]. Relationship Disclosure: Dr Cruccu has received personal compensation for ABSTRACT serving on the advisory board Purpose of Review: Although trigeminal neuralgia is well known to neurologists, of and as a consultant for by mAXWo3ZnzwrcFjDdvMDuzVysskaX4mZb8eYMgWVSPGPJOZ9l+mqFwgfuplwVY+jMyQlPQmIFeWtrhxj7jpeO+505hdQh14PDzV4LwkY42MCrzQCKIlw0d1O4YvrWMUvvHuYO4RRbviuuWR5DqyTbTk/icsrdbT0HfRYk7+ZAGvALtKGnuDXDohHaxFFu/7KNo26hIfzU/+BCy16w7w1bDw== Angelini and Biogen, recent developments in classification and clinical diagnosis, new MRI methods, and a Inc and has received personal debate about surgical options necessitate an update on the topic. compensation for serving on Recent Findings: Currently, a worldwide controversy exists regarding the classification, the advisory board of and as a speaker for Sigma Tau diagnostic process, and surgical treatment of trigeminal neuralgia. This controversy has Pharmaceuticals, Inc. been caused on one side by the recognition that some 50% of patients with trigeminal Dr Cruccu has received neuralgia, apart from characteristic paroxysmal attacks, also have continuous pain in research/grant support from Sapienza University of the same territory, which results in greater diagnostic difficulties and is associated with Rome and Sigma Tau a lower response to medical and surgical treatments. In contrast, recent developments Pharmaceuticals, Inc. in MRI methods allow differentiation between a mere neurovascular contact and an Unlabeled Use of effective compression of the trigeminal root by an anomalous vessel, which implies Products/Investigational Use Disclosure: more difficulties in the choice of surgical treatment, with the indication for Dr Cruccu discusses the microvascular decompression becoming more restricted. unlabeled/investigational use Summary: This article proposes that the diagnosis of trigeminal neuralgia, with or of BIIB074 for the treatment of elderly patients with without concomitant continuous pain, must rely on clinical grounds only. Diagnostic trigeminal neuralgia. tests are necessary to distinguish three etiologic categories: idiopathic trigeminal neu- ralgia (nothing is found), classic trigeminal neuralgia (an anomalous vessel produces morphologic changes of the trigeminal root near its entry into the pons), and secondary trigeminal neuralgia (due to major neurologic disease, such as multiple sclerosis or tumors at the cerebellopontine angle). Carbamazepine and ox- carbazepine (ie, voltage-gated, frequency-dependent sodium channel blockers) are still the first-choice medical treatment, although many patients experience significant side effects, and those with concomitant continuous pain respond less well to treatment. The development of sodium channel blockers that are selective for the sodium channel 1.7 (Nav1.7) receptor will hopefully help. Although all the surgical interventions (percutaneous ganglion lesions, gamma knife radiosurgery, and microvascular decompression) are very efficacious, precise MRI criteria for differentiating a real neurovascular compression from an irrelevant contact will be of benefit in better selecting patients for microvascular decompression. Continuum (Minneap Minn) 2017;23(2):396–420. INTRODUCTION known as tic douloureux)isfrequently Facial pain is easily misdiagnosed. mistaken for dental pain, leading to When the pain is intense and recurrent redundant diagnostic procedures such and the underlying etiology is elusive, as x-rays of the jaw and, in more than the condition is often labeled trigem- a few cases, unnecessary extractions inal neuralgia, although other condi- of teeth.3 tions are much more likely to be the Accurate diagnosis of trigeminal cause. The prevalence of trigeminal neuralgia depends critically on the Supplemental digital content: neuralgia in the population is 0.07%, patient’s description of its characteris- Direct URL citations appear in on the printed text and are in- compared to approximately 2% in tic features. Clarification of the char- 04/25/2018 cluded in the HTML, PDF, and patients with facial pain in general.1,2 acteristics of the pain is therefore app versions of this article. Conversely, trigeminal neuralgia (also necessary to guide clinical diagnosis 396 ContinuumJournal.com April 2017 Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited. KEY POINTS and management. Successful diagnostic and trigeminal neuralgia secondary h Although trigeminal criteria must account for established to neurovascular contact. To solve neuralgia is variants of the phenotype (eg, typical this problem, the International Clas- still frequently versus atypical trigeminal neuralgia), sification of Headache Disorders misdiagnosed, its incorporate symptoms or signs that (ICHD) endorsed the term classic, peculiar clinical features correlate with different etiologies (pri- specifying that classic trigeminal neu- are unmistakable. mary trigeminal neuralgia versus trigem- ralgia should be diagnosed when no h A neurovascular inal neuralgia secondary to a major cause other than neurovascular contact compression is 8 neurologic disease), and identify pain is apparent. acknowledged as features that indicate underlying patho- The author agrees with this solu- the most frequent physiologic mechanisms (peripheral ver- tion because it avoids confusion. cause of trigeminal sus central), as they are relevant to direct However, the author favors additional neuralgia (classic further investigations or treatment de- differentiation of idiopathic trigeminal trigeminal neuralgia). cisions (pharmacologic therapy versus neuralgia from classic trigeminal neu- h Notwithstanding the surgery). Physicians should be aware ralgia. Even after surgical exploration most accurate and that the literature on trigeminal neural- of the posterior fossa at the base of advanced investigations, gia has been hampered by a terminolog- the skull for microvascular decom- no cause can be found ical dishomogeneity, which must be pression, approximately 11% of pa- in about 11% of solved to the benefit of researchers, tients with trigeminal neuralgia patients with trigeminal neuralgia (ie, idiopathic clinicians, and, ultimately, patients. remain without diagnosis of an appar- trigeminal neuralgia). Although tic douloureux is fre- ent cause.7,9 The frequency of cases quently used as a synonym for trigem- without an etiology justifies their inal neuralgia, the term was originally designation as idiopathic. For trigem- introduced to describe the involuntary inal neuralgia caused by a neurologic wincing associated with the occur- disease other than neurovascular com- rence of pain. Other diagnostic labels pression, the author prefers the term have been proposed to indicate differ- secondary rather than symptomatic ences in the etiology or clinical pre- because it is less ambiguous. The term sentation of trigeminal neuralgia. symptomatic may also indicate the Variable use of seemingly inter- painful side of the face or the affected changeable labels, such as classic and trigeminal root on MRI. idiopathic or secondary and symp- tomatic, has been a major source of CLINICAL DIAGNOSIS OF confusion. To differentiate idiopathic TRIGEMINAL NEURALGIA trigeminal neuralgia from manifesta- The International Association for the tions of neuralgia that are secondary Study of Pain (IASP) defines trigeminal to an identified disease, in 1973 neuralgia as ‘‘sudden, usually unila- Strandjord4 established the term pri- teral, severe, brief, stabbing, recurrent mary trigeminal neuralgia,which episodes of pain in the distribution of contradicts the notion of trigeminal one or more branches of the trigem- neuralgia as a condition of neuropathic inal nerve.’’10 The ICHD, Third Edi- pain. MRI shows neurovascular contact tion, beta version (ICHD-3 beta) in 70% to 83% of patients with typical describes trigeminal neuralgia in sim- trigeminal neuralgia.5,6 In neurosurgical ilar terms, ‘‘as a disorder characterized case series, this frequency increases by recurrent unilateral brief electric to 89%.7 Hence, primary trigeminal shocklike pains, abrupt in onset and neuralgia was loosely used by many termination, limited to the distribu- authors to describe both trigeminal tion of one or more divisions of the neuralgia without identifiable cause trigeminal nerve and triggered by Continuum (Minneap Minn) 2017;23(2):396–420 ContinuumJournal.com 397 Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Trigeminal Neuralgia KEY POINT h innocuous stimuli.’’ According to the their recent meta-analysis, Cruccu Paroxysmal pain in 14 trigeminal neuralgia is International Headache Society (IHS) and colleagues reviewed studies of a very abrupt and definition, trigeminal neuralgia ‘‘may MS associated with trigeminal neural- short-lasting pain develop without apparent cause or gia and identified a total of 24 cases of usually described as be a result of another diagnosed dis- bilateral trigeminal neuralgia out of stabbing
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