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  • Updated 12.02.2023
  • Released 05.08.2001
  • Expires For CME 12.02.2026

Trigeminal neuralgia



This article delves into the complexities of trigeminal neuralgia, a disorder characterized by severe neuralgiform pain within the trigeminal distribution. The intensity of this pain is so profound that it has earned the condition a grim moniker, “the suicide disease.” The authors provide an in-depth exploration of its epidemiology, diagnosis, and evidence-based treatments. The diagnostic section outlines the categorization per the third edition of The International Classification of Headache Disorders (ICHD-3), shedding light on both primary and secondary etiologies. The section on treatment discusses pharmacological agents, including vixotrigine and basimglurant, which are under clinical trials, along with an overview of contemporary surgical approaches.

Key points

• Trigeminal neuralgia presents as recurrent, unilateral, brief electric shock-like pains, abrupt in onset and termination, limited to the distribution of the trigeminal nerve

• Trigeminal neuralgia can be categorized as classical, idiopathic, or secondary. Painful trigeminal neuropathy instead presents with burning pain, numbness, or tingling with causes including zoster, postherpetic neuralgia, and trauma.

• Classical trigeminal neuralgia is attributed to demyelination of the dorsal root entry zone, leading to spontaneous or triggered discharges of the nerve, whereas idiopathic trigeminal neuralgia may be due to molecular changes, channelopathies, or electrophysiological abnormalities.

• Brain MRI is recommended in all patients without contraindications.

• First-line pharmacotherapy includes carbamazepine and oxcarbazepine; second-line pharmacotherapy includes lamotrigine, gabapentin, pregabalin, baclofen, phenytoin, and botulinum toxin type A.

• Microvascular decompression is the gold standard surgical treatment for classical trigeminal neuralgia.

Historical note and terminology

Trigeminal neuralgia has been described throughout history. Early descriptions have been noted in writings dating back to the second century AD by Galen and Artaeus of Cappadocia, who is also credited with one of the earliest descriptions of migraine (101). Avicenna, a Persian physician, is credited by some with the first description of trigeminal neuralgia, “tortura oris,” in the 11th century (06). A German physician, Johannes Laurentius Bausch, wrote of lightning-like pain in the right face in the 1600s. Nicolas André coined the term “tic doloureaux” in his 1756 work “Observations pratiques sur les maladies de l’urèthre et sur plusiers faits convulsifs,” postulating that the facial pain was due to nerve compression from early wound closure (23). Physician and well-known philosopher John Locke described a case in the Countess of Northumberland in 1677 (31).

John Fothergill, a Quaker physician in London, is credited with the first full account of trigeminal neuralgia (Fothergill disease) in a letter to the Medical Society of London titled “On a Painful Affliction of the Face” in 1773 (35; 40; 101), describing 14 cases of paroxysmal unilateral facial pain, commonly triggered by light touch or eating and starting and ending abruptly (95). In the 18th and 19th centuries, observations from Pujol, Chapman, and Tiffany helped to distinguish trigeminal neuralgia from other causes of facial pain (90). Charles Bell established the different functions of the trigeminal and facial nerves in the 1820s, further supporting the differentiation of trigeminal neuralgia from other facial pain syndromes (31). In the 1850s, Trousseau likened the paroxysmal pain to a seizure, calling trigeminal neuralgia an “epileptiform neuralgia” and attributing pain to paroxysmal activity of the trigeminal system (120; 50). Kugelberg and Lindblom discovered a latent period between stimulus and onset of pain; once this pain started, it was self-sustained, followed by a period of latency (75). In the 20th century, Oppenheim suggested a connection between multiple sclerosis and trigeminal neuralgia (90).

Pharmacotherapy was not successful until the mid-20th century. Early treatment included quinine, blistering, purging, mercury, opium, and arsenic, up to the use of trichloroethylene and stilbamidine in the early 1900s. These treatments caused a wide array of side effects, including ventricular arrhythmias and cardiac arrest (31). Based on Trousseau’s “epileptiform neuralgia” theory, trigeminal neuralgia was first treated with phenytoin in 1942 by Bergouignan, and soon after, it was first used to treat epileptic seizures (16; 50). In 1963, Blom successfully treated trigeminal neuralgia with carbamazepine (20). Oxcarbazepine, a derivative of carbamazepine, was subsequently found also to be effective in controlling trigeminal neuralgia pain (31).

Early surgical treatments include chemoneurolysis in the late 1800s and destruction of the gasserian ganglion in the early 1900s (31). The microvascular compression theory also arose in the 20th century (37). The concept of microvascular decompression was introduced in 1925, from which modern neurosurgical treatment can be traced (34). Dandy’s posterior fossa approach to the trigeminal nerve revealed vascular loops impinging on the root entry zone (31). Microvascular decompression did not gain widespread acceptance until almost half a century later (1950s to 1960s) when Gardner and Miklos promoted the theory and modified the technique (52). A shift in neurosurgical practice toward microvascular decompression occurred in the 1970s after Janetta published a large case series (66). Radiofrequency ablation was introduced in 1974 by Sweet and Wepsic, leading to precise partial destruction within the gasserian ganglion and sensory root (114).

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