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Trigeminal neuralgia

Abstract

Trigeminal neuralgia (TN) is a facial pain disorder characterized by intense and paroxysmal pain that profoundly affects quality of life and presents complex challenges in diagnosis and treatment. TN can be categorized as classical, secondary and idiopathic. Epidemiological studies show variable incidence rates and an increased prevalence in women and in the elderly, with familial cases suggesting genetic factors. The pathophysiology of TN is multifactorial and involves genetic predisposition, anatomical changes, and neurophysiological factors, leading to hyperexcitable neuronal states, central sensitization and widespread neural plasticity changes. Neurovascular compression of the trigeminal root, which undergoes major morphological changes, and focal demyelination of primary trigeminal afferents are key aetiological factors in TN. Structural and functional brain imaging studies in patients with TN demonstrated abnormalities in brain regions responsible for pain modulation and emotional processing of pain. Treatment of TN involves a multifaceted approach that considers patient-specific factors, including the type of TN, with initial pharmacotherapy followed by surgical options if necessary. First-line pharmacological treatments include carbamazepine and oxcarbazepine. Surgical interventions, including microvascular decompression and percutaneous neuroablative procedures, can be considered at an early stage if pharmacotherapy is not sufficient for pain control or has intolerable adverse effects or contraindications.

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Fig. 1: Proposed mechanisms of TN.
Fig. 2: Pathophysiology of TN.
Fig. 3: Proposed molecular mechanisms of TN.
Fig. 4: Anatomical localization of pain in TN.
Fig. 5: Neuroimaging in TN.
Fig. 6: Treatment of TN.

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Contributions

Introduction (S.A. and L.B.); Epidemiology (S.A., G.D.-S. and G.C.); Mechanisms/pathophysiology (S.A., G.D.-S., M.O., A.D., Y.S.P., C.E.R., L.B., M.R.-R. and A.S.); Diagnosis, screening and prevention (S.A., G.D.-S., M.H., M.O., A.D., C.E.R., L.B. and M.R.-R.); Management (S.A., M.H., M.O., G.C., Y.S.P., C.E.R., L.B. and M.R.-R.); Quality of life (S.A., G.D.-S., M.H. and A.D.); Outlook (S.A., M.H., M.O., L.B. and A.S.); overview of the Primer (S.A.).

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Correspondence to Sait Ashina.

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Competing interests

S.A. received honoraria for consulting or teaching from Allergan/AbbVie, Eli Lilly, Impel NeuroPharma, Linpharma, Lundbeck, Pfizer, Satsuma, Teva, and Theranica. C.E.R. has served on advisory boards for Lundbeck, Biohaven, Impel, Linpharma, and Satsuma, and has received research support from Teva, Pfizer, Lundbeck, with funds paid to her institution, and she also receives royalties as author and section editor for UpToDate. M.O. has received scientific support, travel support and/or honoraria from Biogen, Novartis, Sanofi, Pfizer, and Teva, Lilly, and Heel, and he received research grants from Allergan, Electrocore, Heel, and the German Ministry for Education and Research (BMBF). M.R.-R. received grant support from Amgen and honoraria for consulting from Pfizer. L.B. has given lectures and served on the scientific advisory board for Abbvie, Eli Lilly, Lundbeck, Novartis, Pfizer and Teva. All other authors declare no competing interests.

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Ashina, S., Robertson, C.E., Srikiatkhachorn, A. et al. Trigeminal neuralgia. Nat Rev Dis Primers 10, 39 (2024). https://doi.org/10.1038/s41572-024-00523-z

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