The changing face of trigeminal neuralgia-A narrative review.

OBJECTIVE: This narrative review aims to update the reader on the new classification of trigeminal neuralgia (TN), clinical signs, pathophysiologic evidence, and their implications on management. This review is based on the authors' collective experience and knowledge of the literature in addition to a literature search. BACKGROUND: In recent years, the phenotype of TN has been intensively studied leading to discrete groups of patients. These include patients with TN with additional continuous pain, and patients with and without neurovascular compression of the trigeminal dorsal root entry zone. A number of associated clinical signs such as tearing and sensory changes need further research. METHODS: The literature on TN was searched in PubMed with the aims of providing evidence for the recently published third edition of the International Classification of Headache Disorders (ICHD) and update the clinical phenotype and management of the TN subcategories. RESULTS: The ICHD's new classification for TN is based on reliable clinical data, imaging, and neurophysiologic studies. The TN classification reflects current knowledge and has improved the possibility for clinicians to choose adequate management options. However, there is a lack of effective, safe drugs for the management of TN and sparse, robust data on neurosurgical options. CONCLUSION: Research into all aspects of TN-diagnosis, pharmacotherapy, surgery, long-term management prognosis, and natural history-is needed. Research should adhere to the ICHD's schema for TN. Improved drugs are needed along with rigorous research into surgical options and their efficacy for different subtypes of TN.

Advances in diagnosis, classification, pathophysiology, and management of trigeminal neuralgia.

Trigeminal neuralgia is a very painful neurological condition with severe, stimulus-evoked, short-lasting stabbing pain attacks in the face. The past decade has offered new insights into trigeminal neuralgia symptomatology, pathophysiology, and treatment, leading to a change in the classification of the condition. An accurate diagnosis is crucial because neuroimaging interpretation and clinical management differ among the various forms of facial pain. MRI using specific sequences should be a part of the diagnostic workup to detect a possible neurovascular contact and exclude secondary causes. Demonstration of a neurovascular contact should not be used to confirm a diagnosis but rather to facilitate surgical decision making. Carbamazepine and oxcarbazepine are drugs of first choice for long-term treatment, whereas microvascular decompression is the first-line surgery in medically refractory patients. Advances in neuroimaging techniques and animal models will provide further insight into the causes of trigeminal neuralgia and its pathophysiology. Development of more efficacious treatment options is highly warranted.

Familial classic trigeminal neuralgia. / Neuralgia del trigémino clásica familiar.

INTRODUCTION: The classic form of trigeminal neuralgia is usually sporadic (no familial clustering). However, around 2% of all cases of trigeminal neuralgia may be familial. Describing this entity may be useful for diagnosing this process and may also be key to determining the underlying causes of sporadic classical trigeminal neuralgia. We report on cases in a series of 5 families with at least 2 members with classic trigeminal neuralgia, amounting to a total of 11 cases. MATERIAL AND METHODS: We recorded cases of familial classical trigeminal neuralgia between March 2014 and March 2015 by systematically interviewing all patients with a diagnosis of trigeminal neuralgia who visited the neurology department on an outpatient basis. RESULTS: In our sample, most patients with familial classic trigeminal neuralgia were women. Mean age at onset was 62.9±13.93 years, decreasing in subsequent generations. V2 was the most frequently affected branch. Most of our patients responded well to medical treatment, and surgery was not effective in all cases. CONCLUSIONS: These family clusters support the hypothesis that classic trigeminal neuralgia may have a genetic origin. Several causes have been suggested, including inherited anatomical changes affecting the base of the skull which would promote compression of the trigeminal nerve by vascular structures, familial AHT (resulting in tortuous vessels that would compress the trigeminal nerve), and mutations in the gene coding for calcium channels leading to hyperexcitability. Classic trigeminal neuralgia may be an autosomal dominant disorder displaying genetic anticipation.

Trigeminal neuralgia (TN): A descriptive literature analysis on the diagnosis and management modalities.

OBJECTIVE: The primary objective of this review is to explore the different aspects of the diagnosis and management of Trigeminal neuralgia (TN). We look at the role of radiological imaging in the work-up of this condition, and based on the findings in the literature, we report data on the medical and surgical management of TN. MATERIALS AND METHODS: A literature review was conducted using PubMed and Cochrane search engines in order to explore the data available on the diagnosis and management of TN. Clinical features and various treatment modalities were analyzed by the authors. The identified studies were evaluated and data was reported on the different aspects of the condition in order to provide an evidence-based update on the topic. DISCUSSION: The diagnosis of TN is based on the patient's clinical history and radiological imaging. The commonest cause of TN is a micro-vascular compression by a looping blood vessel. Radiological evaluation is critical in the work-up of the disorder and in order to eliminate other possible causes. Management of the disorder can be medical or surgical, with micro-vascular decompression having the highest remission rate. CONCLUSION: Patients with TN present paroxysmal pain attacks in the territories innervated by the trigeminal nerve. Diagnostic investigations must allow precise anatomical evaluation of the CPA, and MR imaging is the gold-standard radiological investigation for this purpose. Management of TN can be medical or surgical, with micro-vascular decompression having the highest success rate.

Trigeminal neuralgia: New classification and diagnostic grading for practice and research.

Trigeminal neuralgia (TN) is an exemplary condition of neuropathic facial pain. However, formally classifying TN as neuropathic pain based on the grading system of the International Association for the Study of Pain is complicated by the requirement of objective signs confirming an underlying lesion or disease of the somatosensory system. The latest version of the International Classification of Headache Disorders created similar difficulties by abandoning the term symptomatic TN for manifestations caused by major neurologic disease, such as tumors or multiple sclerosis. These diagnostic challenges hinder the triage of TN patients for therapy and clinical trials, and hamper the design of treatment guidelines. In response to these shortcomings, we have developed a classification of TN that aligns with the nosology of other neurologic disorders and neuropathic pain. We propose 3 diagnostic categories. Classical TN requires demonstration of morphologic changes in the trigeminal nerve root from vascular compression. Secondary TN is due to an identifiable underlying neurologic disease. TN of unknown etiology is labeled idiopathic. Diagnostic certainty is graded possible when pain paroxysms occur in the distribution of the trigeminal nerve branches. Triggered paroxysms permit the designation of clinically established TN and probable neuropathic pain. Imaging and neurophysiologic tests that establish the etiology of classical or secondary TN determine definite neuropathic pain.

Preoperative evaluation of the neurovascular compression using magnetic resonance tomographic angiography: our radiologic indications for microvascular decompression to treat trigeminal neuralgia.

PURPOSE: The aim of this study was to introduce our classification of the neurovascular compression (NVC) in trigeminal neuralgia and the radiologic indications for microvascular decompression (MVD) based on magnetic resonance tomographic angiography. METHODS: From 2003 to 2011, 322 patients with primary trigeminal neuralgia were treated with MVD. The score of NVC was from 0 to 3. Three scores, separately from axial, oblique sagittal, and coronal images, were added together. The degree of NVC was classified as follows: grade 0 (0-1), grade 1 (2-3), grade 2 (4-6), and grade 3 (7-9). RESULTS: In summary, 88.3% (182/206) patients with absolute indication, 78.3% (65/83) patients with relative indication, and 90.9% (30/33) without indication showed excellent results. Among the 27 patients with good result, 13 patients (48.1%) were in grade 1, and 3 (11.1%) were in grade 0. Among the 18 patients with poor result, 5 patients (27.8%) were in grade 1 preoperatively. Five patients with severe complications were all in grade 0 with vague NVC. CONCLUSION: The patients with grades 2 and 3 (absolute indications) NVC were recommended with MVD.

[Treatment of trigeminal neuralgia with radiosurgery]. / Traitement de la névralgie essentielle du trijumeau par radiochirurgie stéréotaxique.

Idiopathic trigeminal neuralgia is defined as brief paroxysms of pain limited to the facial distribution of the trigeminal nerve. Drug therapy is considered to be the first-line of treatment for trigeminal neuralgia. Unfortunately, medical treatment does not always provide satisfactory pain relief for 25% of the patients. Moreover, the relief provided by drug therapy generally decreases over time, and increased dosages of these medications are limited because of side effects. In this case, patients can be offered several surgical approaches, such as percutaneous techniques (thermocoagulation, microcompression, glycerol injection) or microvascular decompression in the cerebello-pontine angle (Gardner-Jannetta's technique). In this indication, stereotactic radiosurgery, driven by teams using Gamma Knife(®), has shown promising efficacy and tolerance to allow this treatment being truly part of trigeminal neuralgia treatment. Technological progresses now allow performing radiosurgery with ballistic and dosimetric processes optimized with stereotactic radiosurgery dedicated linear accelerators. This procedure supports frame implantation to guarantee targeting accuracy in accordance of elevated dose distribution. This article on trigeminal neuralgia treatment will review the different medical and surgical therapeutic options and specify the contemporary place of stereotactic radiosurgery in the light of its clinical results and tolerance aspects.

Idiopathic trigeminal neuropathies: a presentation of 15 cases.

PURPOSE: The aim of this study is to present a clinical series of patients with trigeminal neuropathy and their treatment. PATIENTS AND METHODS: We present a retrospective study of 15 cases of idiopathic trigeminal neuropathies, with unilateral involvement of 1 or more divisions of the trigeminal nerve. The clinical, radiologic, and laboratory data of the patients, in addition to the treatment and clinical evolution, were reviewed. The patients were followed up for a mean of 34.4 months (range, 12-120 months). RESULTS: The study consisted of 11 women and 4 men. The numbness was predominantly located in the innervated mental area and extended in some cases to the first and second trigeminal divisions. Seven patients had slight continuous discomfort in association with the numbness, one of whom had added bouts of typical neurogenic pain. Of the 15 cases, 8 (53%) had acute idiopathic trigeminal neuropathies and fully recovered within 3 months and 7 (47%) were chronic cases, without full recovery after 3 months. Mild pain was felt by 57% of the chronic patients and 37% of the acute patients; treatment with amitriptyline achieved complete or partial improvement in over half of these patients. CONCLUSION: Of the idiopathic trigeminal neuropathies, half were acute and half were chronic. Mild pain presented more frequently in the chronic patients and was relieved with amitriptyline.

[Radiosurgery in trigeminal neuralgia: long-term results and influence of operative nuances]. / La radiochirurgie dans le traitement de la névralgie trigéminale : résultats à long terme et influence des nuances techniques.

Stereotactic radiosurgery is an alternative to conventional surgery for the treatment of trigeminal neuralgia. To better define the safety of radiosurgery and optimal technical choices, we reviewed our patient records and the literature. A total of 334 patients presenting with trigeminal neuralgia were treated between December 1992 and September 2005. A minimum of 1 year of follow-up was available for 262 patients. The mean age was 68 years (range: 30-90); 128 patients were male and 134 female. A neurovascular conflict was clearly visualized on MRI in 167 patients. Twenty-one had a past history of multiple sclerosis and 110 had already received conventional surgical treatment for trigeminal neuralgia. The intervention consisted of gamma knife radiosurgery (GKS) to the retrogasserian cisternal portion of the Vth cranial nerve. The median maximal dose used was 85Gy (range: 70-90). Actuarial curves show a plateau at 5 years for both the risk of hypoesthesia and recurrence. At 5 years, 58% of the patients remain pain-free and 83% have no trigeminal nerve disturbance. The median delay for pain cessation was 15 days. The initial pain-relief rate was 89%. None of the complications reported for the other techniques were observed. Patient selection (typical versus atypical, age, past surgery, multiple sclerosis) and details of operative technique (maximum dose, volume of nerve treated, target location, etc.) had a major influence on the probability of pain relief and toxicity risk. The details of operative technique are turning out to have a major influence on the clinical results. In our experience, high-dose (80-90Gy) retrogasserian (7-8mm from the brainstem) GKS provides the patient with a better chance of long-term pain relief and a lower risk of trigeminal nerve functional disturbance. GKS applied to the cisternal anterior trigeminal nerve using high doses provided safe and effective treatment for trigeminal neuralgia over the long term.

Endoscopic neurovascular perspective in microvascular decompression of trigeminal neuralgia.

PURPOSE: To evaluate the advantages of endoscopic assistance in microvascular decompression (MVD) for the treatment of trigeminal neuralgia (TGN). METHODS: One hundred and sixty-seven patients suffering from TGN were treated by MVD from October 2003 to December 2006. The operating microscope was used in conjunction with a 30 degrees effect endoscope to diagnose neurovascular compression (NVC). RESULTS: The type and grade of NVC were determined in all cases. 85.26% of neurovascular conflicts were diagnosed with the microscope alone. An additional 14.76% could be diagnosed with the assistance of the endoscope. 96.79% of patients experienced immediate relief of pain. CONCLUSIONS: MVD is a highly effective treatment for TGN. Endoscopes are very useful during the procedures. They increase the clarity of the surgical field and they enable panoramic visualization in areas that are blind to the direct field of view of the operating microscope. A significant percentage of neurovascular conflicts would be missed at surgery without endoscopic assistance.

Trigeminal neuralgia.

Trigeminal Neuralgia (TN) is a well recognized syndrome characterized by lancinating attacks of severe facial pain. The diagnosis of TN is based on a history of characteristic pain attacks that are consistent with specific widely accepted criteria for the diagnosis. TN pain attacks may result from physiologic changes induced by a chronic partial injury to the brainstem trigeminal nerve root from a variety of causes. An early and accurate diagnosis of TN is important, because therapeutic interventions can reduce or eliminate pain attacks in the large majority of TN patients.

Trigeminal neuralgia: definition and classification.

Based on specific, objective, and reproducible criteria, a classification scheme for trigeminal neuralgia (TN) and related facial pain syndromes is proposed. Such a classification scheme is based on information provided in the patient's history and incorporates seven diagnostic criteria, as follows. 1) and 2) Trigeminal neuralgia Types 1 and 2 (TN1 and TN2) refer to idiopathic, spontaneous facial pain that is either predominantly episodic (as in TN1) or constant (as in TN2) in nature. 3) Trigeminal neuropathic pain results from unintentional injury to the trigeminal nerve from trauma or surgery. 4) Trigeminal deafferentation pain results from intentional injury to the nerve by peripheral nerve ablation, gangliolysis, or rhizotomy in an attempt to treat either TN or other related facial pain. 5) Symptomatic TN results from multiple sclerosis. 6) Postherpetic TN follows a cutaneous herpes zoster outbreak in the trigeminal distribution. 7) The category of atypical facial pain is reserved for facial pain secondary to a somatoform pain disorder and requires psychological testing for diagnostic confirmation. The purpose of a classification scheme like this is to advocate a more rigorous, standardized natural history and outcome studies for TN and related facial pain syndromes.

Trigeminalgias autonômicas / Trigeminal Authonomic

Os autores fazem uma revisão bibliográfica sobre as Trigeminalgias Autonômicas, grupo de cefaléias de curta duração que apresentam manifestações autonômicas associadas. Essas síndromes são pouco conhecidas pela maioria dos médicos atualmente. Suas manifestações clínicas, diagnóstico, tratamento e classificação são abordadas no presente texto

Neuralgía esencial del trigémino, estudio retrospectivo de 64 casos / Esential trigeminal neuralgia: restrospective study of 64 cases

The Trigeminal Neuralgia, which is a non odontogenic pain, that takes place in the cranial-maxillofacial area, is a situation that the general dentist and tha maxillofacial surgeon, in particular, has to reconize. This study analizes 64 cases treated in period of 6 year. The epidemiologic results show several coincidences with international studies. They also show some characteristics not mentioned on the universal literature revised. The unique objective of this document is to determine the epidemiologics aspects of this desease, with respect to patients that attend spontaneously and/ or are derived from the others Services

Radiosurgery for trigeminal neuralgia and epilepsy.

The role of Gamma Knife surgery in the field of functional surgery recently has evolved dramatically. For treatment of trigeminal neuralgia, Gamma Knife surgery is the least invasive procedure, with a low rate of hypesthesia. If a rate of complete relief similar to that of other surgical techniques could be achieved, this approach will become one of the main techniques used to treat the disease initially. The authors present their experience with a group of 16 patients with mesial temporal lobe epilepsy who have been treated successfully (15 completely seizure-free and 1 with rare, nondisabling seizures) without significant complication. After additional follow-up to demonstrate the absence of long-term consequences, this fascinating new approach could change epilepsy surgery practice dramatically.

Percutaneous stereotactic differential radiofrequency thermal rhizotomy for the treatment of trigeminal neuralgia.

PURPOSE: The purpose of this study was to evaluate the effectiveness of radiofrequency thermal rhizotomy (RTR) for trigeminal neuralgia, after failure of pharmacological management. PATIENTS AND METHODS: Two hundred fifteen patients underwent RTR from 1991 to 1996 and were prospectively evaluated. These patients were characterized by age, sex, side of the face, and division(s) involved. Patients were evaluated for pain relief, recurrence requiring or not requiring reoperation, and the type and rate of complications. They were followed-up by serial clinical evaluation and telephone interview. Patients were categorized into groups: 1) Successful result: excellent, good pain relief; and 2) Unsuccessful result: fair, poor, or no pain relief. The RTR group was compared with historical controls. Follow-up ranged from 9 to 68 months (mean, 32 months) and results were evaluated at early and long-term follow-up. RESULTS: At early follow-up (defined as immediately postoperatively to 6 months), pain relief of excellent or good quality (successful result) occurred in 198 of 215 patients (92%). Fair or poor or no pain relief (unsuccessful result) occurred in 17 (8%) patients. At long-term follow-up (>6 months to 68 months), recurrence of pain that required reoperation occurred in 24 patients (11%) and recurrence of pain that did not require reoperation (medically managed) occurred in 34 patients (16%). Dysesthesia developed in 18 patients (8%); seven patients (3%) had dysesthesia alone (medically managed) and 11 patients (5%) had dysesthesia with recurrence of pain (medically or surgically managed). "Anesthesia/analgesia dolorosa" developed in four patients (1.8%) and was medically managed. At long-term follow-up, 83% of patients had good to excellent pain relief (successful result). There were no mortalities, no significant morbidity, and a low rate of minor complications. CONCLUSION: With the use of this specific diagnostic approach and management algorithm, patients with trigeminal neuralgia can be successfully managed.