Review Article TRIGEMINAL NEURALGIA : AN OVERVIEW

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1 Review Article TRIGEMINAL NEURALGIA : AN OVERVIEW A AGRAWAL*, R CINCU**, RM BORLE***, N BHOLA**** ABSTRACT Trigeminal neuralgia or tic douloureux is an idiopathic disorder and most common cause of unilateral facial pain. In this article we discuss the management of trigeminal neuralgia and review the literature. The list of differential diagnoses for trigeminal neuralgia is long and includes a number of pathological conditions. There is no specific test to make a diagnosis of trigeminal neuralgia and a clinical examination, including assessment of cranial nerve function is mandatory. Magnetic resonance imaging can be useful in examining patients with neurological abnormalities. Medical treatment (particularly carbamazepine) in these patients is very effective in controlling pain symptoms. For patients with continued pain inspite of adequate medical treatment surgical options can be considered. Key Words : Trigeminal neuralgia, tic douloureux, facial pain Introduction : Trigeminal neuralgia, or tic douloureux is an idiopathic disorder of unilateral facial pain and has an annual occurrence rate of 3 to 5 per people. 1,2 Trigeminal neuralgia is characterized by lancinating paroxysms of pain in the lips, gums, cheek, or chin and usually precipitated by physical triggers in the distribution of the trigeminal nerve. 3 In this article we discuss *Assoc. Prof. in Neurosurgery, Dept. of Surgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), **Consultant Neurosurgeon, Dept. of Neurosurgery, Miguel Servet University Hospital, Zaragoza, Spain, *** Prof. & Head, Dept. of Oral and Maxillofacial Surgery, Sharad Pawar Dental College, Sawangi (Meghe), ****Asst. Prof. in Oral & Maxillofacial Surgery, Dept. of Oral and Maxillofacial Surgery, Sharad Pawar Dental College, Sawangi (Meghe). Address for correspondence : Dr Amit Agrawal, Associate Professor (Neurosurgery), Clinical and Administrative Head, Division of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe) Wardha ,MH,India. the management of trigeminal neuralgia and review the literature. The International Association for the Study of Pain (IASP) and International Headache Society (IHS) has suggested a definition for trigeminal neuralgia. 4 The IHS definition is : Painful unilateral affliction of the face, characterized by brief electric shock like pain limited to the distribution of one or more divisions of the trigeminal nerve. Pain is commonly evoked by trivial stimuli including washing, shaving, smoking, talking, and brushing the teeth, but may also occur spontaneously. The pain is abrupt in onset and termination and may remit for varying periods. IASP definition is more simple and trigeminal neuralgia is defined as a sudden, usually unilateral, severe brief stabbing recurrent pains in the distribution of one or more branches of the V th cranial nerve. 40

2 A Agrawal, et al Clinical features : Trigeminal neuralgia is more common in females than males with a female to male ratio of 1.74:1 and the most common from age 50 to 69 years with preponderance for right side of face. 1,5 The attacks can occur during the day or night but rarely during sleep. 6 The pain is described as sudden, sharp, shock like, or burning. 1,5,7 Attacks are usually triggered by non-painful stimuli such as touch, movement, wind exposure, eating, brushing teeth, shaving, washing, talking, or swallowing. 5,6,7 Trigeminal neuralgia is most commonly found in the maxillary and mandibular division or the maxillary branch alone. A small percentage of cases affect the ophthalmic division alone. 1,5,7 Patients may have multiple clusters of pain that last from a few seconds to several minutes. 7 Diagnosis : There is no specific test to make a diagnosis of trigeminal neuralgia and a clinical examination, including assessment of cranial nerve function is mandatory. 8 Magnetic resonance imaging can be useful in examining patients with neurological abnormalities. 3 The resolution of MRI is superior to that of CT for visualizing soft-tissue lesions. Magnetic resonance angiography (MRA) allows visualization of the vascular anatomy of the relevant region without the use of contrast media. 9,10 Ideally all patients with the diagnostic criteria for trigeminal neuralgia should undergo MRI before they begin a trial of pharmacological therapy. 11 However, many clinicians recommend the use of MRI only in patients in whom a trial of standard medications has been unsuccessful or in those who have atypical symptoms should undergo imaging. 12 3D CISS MR imaging is further useful in the detection of neurovascular compression due to veins as 3D CISS images can clearly demonstrate the veins that are responsible for the neurovascular compression in trigeminal neuralgia. 13,14 Differential diagnosis : The list of differential diagnoses for trigeminal neuralgia is long and includes a number of pathological conditions and includes pathological conditions affecting the sinuses, teeth, temporomandibular joints, eyes, nose, and the neck, other cranial neuralgias (glossopharyngeal neuralgia, neuralgia of nervus intermedius, neuralgia of the superior laryngeal nerve, and occipital neuralgia). However the diagnosis of trigeminal neuralgia is based on clinical findings and a careful history and examination can distinguish between trigeminal neuralgia and other disorders that cause facial pain. 8,15 Local examination findings may indicate otitis media, sinusitis, temporomandibular joint disease, herpes zoster, dental caries, or eye disease. Associated neurological deficits, especially those involving the eye, are useful in diagnosis. 16 If there is any neurological deficit, there should be a suspicion of a structural lesion, including aneurysm, neurofibroma, meningioma, or any other structural intracranial lesion. 3 Treatment options : As the exact cause of trigeminal neuralgia is not known, various types of treatment modalities for this modality are available (Table-1). 8,17,18,19 Table - 1 : Treatment options for trigeminal neuralgia Medical management 1. Carbamazepine 2. Baclofen 3. Clonazepam 4. Phenytoin 5. Pimozide 6. Valproic acid 41

3 Trigeminal neuralgia : an overview Surgical management 1. MVD 2. Radiofrequency gangliolysis 3. Glycerol gangliolysis 4. Stereotactic radiosurgery 5. Peripheral neurectomy 6. Cryotherapy 7. Alcohol block Medical treatment Medical management remains the mainstay of treatment for trigeminal neuralgia. 20 The treatment of choice for trigeminal neuralgia is carbamazepine; however most of the evidence for medical treatment of trigeminal neuralgia is based on non-controlled studies from the 1960s. 21,22,23 Carbamazepine is an effective and well-tolerated treatment. Initial dose of carbamazepine is 100 mg twice daily, then increased to 3 times per day. The dose may then be increased by 100 mg/d (on a 3-times-daily schedule) until pain relief is achieved or 1200 mg/d is reached. 5,24 A complete blood cell count and liver function tests should be done periodically on patients treated for longer periods. After the pain has been controlled for 6 to 8 weeks, the dosage should be decreased to the lowest level that maintains pain control or withdrawn completely. 5,24 Lamotrigine is an anticonvulsant with an action similar to that of carbamazepine, but with fewer side effects. A small noncontrolled study of patients who could not tolerate carbamazepine showed response without the side effects associated with carbamazepine. 8,25 Surgical decompression For patients who do not respond to pharmacological therapy or have worsening symptoms or more frequent recurrence, a surgical procedure may be appropriate. 5,6 Among the available surgical methods microvascular decompression appears to be more physiological and does not result in facial sensory loss. 17 Postoperatively, immediate relief ranges from 91% to 97% and long term efficacy from 53% to 70%, with an estimated annual recurrence rate of 3.5%. 26 Complications of MVD include hearing loss, permanent facial anesthesia, brainstem infarction, cerebellar injury, ataxia, meningitis, headaches, and death. 27,28 However it is argued by many to be the treatment of choice, even in the elderly because of its high rate efficacy maintained in the long term. 8,14,29 Percutaneous microballoon compression Percutaneous microballoon compression (PMC) was first reported by Mullan. 30 Because of micro invasion, the technique is more physiological without leading to a loss of facial sensory. 31 Among the procedures, PMC is the best choice for elderly frail patients, because it had a very low associated morbidity and does not injure the gasserian ganglion. 32 The complications of PMC though rare but include hypesthesia, dysesthesia, masseter muscle weakness, anesthesia dolorosa, corneal anesthesia and absent corneal reflex, aseptic meningitis, transient sixth nerve palsy, otalgia, trochlear nerve palsy, and increased olfactory threshold. 3,34,35 The PMC technique seems to be better than injection of alcohol, glycerol or radio frequency lesion and had been used widely in recent years. 17,34,36 Gamma knife radiosurgery Radiosurgery is the least invasive surgical procedure for trigeminal neuralgia. It is associated with a low risk of facial paresthesias, an approximate 80% rate of significant pain relief, and a low recurrence rate in patients who initially attain complete relief. Longer-term evaluations are 42

4 A Agrawal, et al warranted. 37,38,39 No patient sustained any form of neurological morbidity other than a low risk for facial numbness. 38,39 The absence of infection, cerebrospinal fluid leakage, anesthesia complications, hearing loss, facial hematoma, facial weakness, or brainstem injury has established radiosurgery as an attractive surgical alternative for many patients. 39 Conclusion Trigeminal neuralgia is the most common neurological cause of facial pain. Spontaneous remission of trigeminal neuralgia is common and these remissions may last for months or even years 7 but in some case the disorder can be progressive. 5 Attacks may come in clusters and can completely disrupt activities of daily living if left untreated. 5,7 Medical treatment (particularly carbamazepine) in these patients is very effective in controlling pain symptoms. If patients fail to respond to first-line agents, there are promising newer agents that may be more effective. For patients with continued pain inspite of adequate medical treatment surgical options can be considered. References 1. Yoshimasu F, Kurland LT, Elveback LR. Tic douloureux in Rochester, Minnesota, Neurology. 1972;22: Katusic S, Beard CM, Bergstralh E, Kurland LT. Incidence and clinical features of trigeminal neuralgia, Rochester, Minnesota, Ann Neurol 1990;27: Delzell JE Jr, Grelle AR. Trigeminal Neuralgia New Treatment Options for a Well-known Cause of Facial Pain. Arch Fam Med. 1999;8: Merskey H, Bogduk N. Classification of chronic pain. Descriptions of Chronic Pain Syndromes and Definitions of Pain Terms. Seattle: IASP Press, 1994; Cheshire WP. Trigeminal neuralgia: a guide to drug choice. CNS Drugs. 1997;7: Turp JC, Gobetti JP. Trigeminal neuralgia versus atypical facial pain: a review of the literature and case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996;81: Green MW, Selman JE. Review article : the medical management of trigeminal neuralgia. Headache. 1991;31: Nurmikko TJ, Eldridge PR. Trigeminal neuralgiapathophysiology, diagnosis and current treatment. Br J Anaesth 2001; 87: Kumon Y, Sasaki S, Kohno K, Ohta S, Ohue S, Miki H. Three-dimensional imaging for presentation of the causative vessels in patients with hemifacial spasm and trigeminal neuralgia. Surg Neurol 1997; 47: Boecher-Schwarz HG, Bruehl K, Kessel G, Guenthner M, Perneczky A, Stoeter P. Sensitivity and specificity of MRA in the diagnosis of neurovascular compression in patients with trigeminal neuralgia: a correlation of MRA and surgical findings. Neuroradiology 1998; 40: Scrivani SJ, Keith DA, Mathews ES, Kaban LB. Percutaneousstereotactic differential radiofrequency thermal rhizotomy for the treatment of trigeminal neuralgia. J Oral Maxillofac Surg 1999; 57: Darlow LA, Brooks ML, Quinn PD. Magnetic resonance imaging in the diagnosis of trigeminal neuralgia. J Oral Maxillofac Surg 1992; 50: Trigeminal Neuralgia: Evaluation of Neuralgic Manifestation and Site of Neurovascular Compression with 3D CISS MR Imaging and MR Angiography 14. Barker FG, Janetta PJ, Bissonette DJ, Larkins MV, Jho HD. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med 1996; 334: Solomon S, Lipton RB. Facial pain. Neurol Clin. 1990; 8: Hooge JP, Redekop WK. Trigeminal neuralgia in multiple sclerosis. Neurology. 1995;45:

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