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.

AAPT Diagnostic Criteria for Peripheral Neuropathic Pain: Focal and Segmental Disorders.

Peripheral neuropathic pain is among the most prevalent types of neuropathic pain. No comprehensive peripheral neuropathic pain classification system that incorporates contemporary clinical, diagnostic, biological, and psychological information exists. To address this need, this article covers the taxonomy for 4 focal or segmental peripheral neuropathic pain disorders, as part of the Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities, and Networks (ACTTION) public-private partnership and the American Pain Society (APS) collaborative to develop a standardized, evidence-based taxonomy initiative: the ACTTION-APS Pain Taxonomy (AAPT). The disorders-postherpetic neuralgia, persistent posttraumatic neuropathic pain, complex regional pain disorder, and trigeminal neuralgia-were selected because of their clinical and clinical research relevance. The multidimensional features of the taxonomy are suitable for clinical trials and can also facilitate hypothesis-driven case-control and cohort epidemiologic studies. PERSPECTIVE: The AAPT peripheral neuropathic pain taxonomy subdivides the peripheral neuropathic pain disorders into those that are generalized and symmetric and those that are focal or segmental and asymmetric. In this article, we cover the focal and segmental disorders: postherpetic neuralgia, persistent posttraumatic neuropathic pain, complex regional pain disorder, and trigeminal neuralgia. The taxonomy is evidence-based and multidimensional, with the following dimensions: 1) core diagnostic criteria; 2) common features; 3) common medical and psychiatric comorbidities; 4) neurobiological, psychosocial, and functional consequences; and 5) putative neurobiological and psychosocial mechanisms, risk factors, and protective factors.

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.

Neuralgia del trigémino: nueva clasificación y tipificación diagnóstica para la práctica clínica y la investigación / Trigeminal neuralgia: new classification and diagnosis grading for clinical practice and research

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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.

[Orofacial pain - Trigeminal neuralgia and posttraumatic trigeminal neuropathy: Common features and differences]. / Orofazialer Schmerz - Trigeminusneuralgie und posttraumatische Trigeminusneuropathie : Gemeinsamkeiten und Unterschiede.

Neuropathic pain is the result of a lesion or disease of the somatosensory system in the peripheral or central nervous system. Classical trigeminal neuralgia and posttraumatic trigeminal neuropathy are pain disorders which oral and maxillofacial surgeons and dentists are confronted with in the differential diagnostics in routine daily practice. The etiopathogenesis of classical trigeminal neuralgia is attributable to pathological blood vessel-nerve contact in the trigeminal nerve root entry zone to the brain stem. The typical pain symptoms are characterized by sudden stabbing pain attacks. The pharmaceutical prophylaxis is based on the individually titrated administration of anticonvulsant drugs. The indications for interventional treatment are dependent on the course, response to drug treatment, resilience and wishes of the patient. The neuropathic mechanism of posttraumatic trigeminal neuropathy originates from nerve damage, which leads to peripheral and central sensitization with lowering of the pain threshold and multiple somatosensory disorders. The prophylaxis consists of avoidance of excessive acute and long-lasting pain stimuli. Against the background of the biopsychosocial pain model, the treatment of posttraumatic trigeminal neuropathy necessitates a multimodal, interdisciplinary concept.

Pain paper 2b: classification of orofacial pain and an update on assessment and diagnosis.

The classification of chronic orofacial pain remains a contentious area. However, more recently, with the clarification of pain mechanisms and improved understanding of the underlying neurophysiology and modulation factors, there is more clarity of the possible division of pain conditions. Interestingly, the pathophysiology provides a basis for classification that has more clinical relevance. The principles of assessing and managing patients with pain have modified significantly, in line with recent improved understanding of the affective and emotional components in pain behaviour and suffering. Clinical Relevance: This paper aims to provide the dental and medical teams with a review of the classification of trigeminal pain with an overview of how to assess and diagnose patients with trigeminal pain.

Field-testing of the ICHD-3 beta diagnostic criteria for classical trigeminal neuralgia.

INTRODUCTION: We aimed to field-test the beta version of the third edition of the International Classification of Headache Disorders (ICHD-3 beta) diagnostic criteria for classical trigeminal neuralgia (TN). The proposed beta draft of the 11th version of the International Classification of Diseases (ICD-11 beta) is almost exclusively based on the ICHD-3 beta classification structure although slightly abbreviated. We compared sensitivity and specificity to ICHD-2 criteria, and evaluated the needs for revision. METHODS: Clinical characteristics were systematically and prospectively collected from 206 consecutive TN patients and from 37 consecutive patients with persistent idiopathic facial pain in a cross-sectional study design. RESULTS: The specificity of ICHD-3 beta was similar to ICHD-2 (97.3% vs. 89.2%, p = 0.248) and the sensitivity was unchanged (76.2% vs. 74.3%, p = 0.134). The majority of false-negative diagnoses in TN patients were due to sensory abnormalities at clinical examination. With a proposed modified version of ICHD-3 beta it was possible to increase sensitivity to 96.1% (p < 0.001 compared to ICHD-3 beta) while maintaining specificity at 83.8% (p = 0.074 compared to ICHD-3 beta). CONCLUSION: ICHD-3 beta was not significantly different from ICHD-2 and both lacked sensitivity. A modification of the criteria improved the sensitivity greatly and is proposed for inclusion in the forthcoming ICHD-3.

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.

Oral pre-trigeminal neuralgia pain: clinical differential diagnosis and descriptive study results.

AIMS: To better quantify oral pre-trigeminal neuralgia (PTN) symptoms, attempt to identify PTN symptoms that could reliably differentiate between PTN and odontogenic tooth pain, and determine whether an anesthetic test would reliably differentiate these disorders. METHODOLOGY: This was accomplished through a survey of symptom recall for 49 trigeminal neuralgia patients who had PTN tooth and/or gum pain. RESULTS: The variability of oral PTN symptoms, factors that worsened or improved them, and how dental anesthesia affected them, explain the reason for variations found in the literature. A throbbing pain quality is not in the literature, but present for 63% of respondents. CONCLUSIONS: No specific PTN symptom would reliably differentiate PTN from odontogenic tooth pain. The results also suggest that an anesthetic test would not be totally reliable for differentiating these disorders. A protocol is provided that should help practitioners identify the tooth pain source when there is no dental pathology.

Concomitant persistent pain in classical trigeminal neuralgia--evidence for different subtypes.

OBJECTIVE: To describe the clinical characteristics in classical trigeminal neuralgia (TN) with concomitant persistent pain and to investigate whether TN with concomitant persistent pain represents a distinct phenotype. BACKGROUND: There has been much debate about the possible pathophysiological and clinical importance of concomitant persistent pain in TN. This has led to subgrouping of TN into forms with and without concomitant persistent pain in the recent 3rd International Classification of Headache Disorders beta classification. METHODS: In this cross-sectional study, data on the clinical characteristics were systematically and prospectively collected from consecutive TN patients. RESULTS: A total of 158 consecutive TN patients were included. Concomitant persistent pain was present in 78 patients (49%). The average intensity of concomitant persistent pain was 4.6 (verbal numerical rating scale). The concomitant persistent pain was present at onset or early in the disease course. Patients with concomitant persistent pain were on average 6.2 (P = .008) years younger at onset, but the 2 groups had the same duration of disease (P = .174). There was a preponderance of women in TN with (P < .001) but not in TN without concomitant persistent pain (P = .820). Right-sided pain was more prevalent than left-sided in TN without (P = .007) but not in TN with concomitant persistent pain (P = .907). TN with concomitant persistent pain more frequently had sensory abnormalities (P < .001) and less frequently responded to sodium channel blockers (P = .001). There were no significant differences in other clinical characteristics. CONCLUSIONS: Concomitant persistent pain is very prevalent in TN and is not a consequence of paroxysmal pain. Findings support that the 3rd International Classification of Headache Disorders beta division of TN with and without concomitant persistent pain is clinically and scientifically important.

Neuralgia del trigémino: comportamiento en pacientes atendidos en el hospital Abel Santamaría Cuadrado, años 2006-2011 / Trigeminal neuralgia: its behavior in patients attended at Abel Santamaria Cuadrado” General University Hospital, 2006-2011

La neuralgia del trigémino constituye una enfermedad que aunque no es de elevada incidencia reviste un gran interés por su repercusión en la calidad de vida de los pacientes que la padecen. Son escasos los estudios que muestren el comportamiento de la misma en nuestra provincia. El objetivo fue analizar el comportamiento de la neuralgia del trigémino en los pacientes atendidos en el hospital Abel Santamaría Cuadrado de la ciudad de Pinar del Río. Se realizó una investigación observacional, descriptiva, de corte transversal en dicho centro, en el período comprendido entre los años 2006 al 2011. El método utilizado fue la revisión documental, a partir de la información recogida en las historias clínicas del total de pacientes atendidos en la sala T Maxilofacial de dicho hospital con el diagnóstico de neuralgia del trigémino en dicho período. Los datos fueron procesados utilizando la estadística descriptiva y la prueba Chi cuadrado, con un nivel de confianza del 95 por ciento. El grupo más afectado fue el de 51 años y más, con predominio en el sexo femenino, y a su vez las ramas II y III de dicho nervio, siendo el tratamiento más utilizado el medicamentoso. Se concluye que el comportamiento de la neuralgia del trigémino en la muestra estudiada, desde el punto de vista epidemiológico, es similar al de otros estudios, no sucediendo así con su tratamiento(AU)

Trigeminal neuralgia constitutes a disease, although its incidence is not high, this entity is of great interest due to its repercussion on the quality of life of patients suffering from this condition. Not many studies show its behavior in our province. The objective was to analyze the behavior of trigeminal neuralgia in patients attending to Abel Santamaria Cuadrado General University Hospital in Pinar del Rio city. An observational, descriptive, cross-sectional research was carried during 2006-2011. The method used was the documentary review, collecting the information from the clinical histories of patients who attended to the maxillofacial ward (T) having the diagnosis trigeminal neuralgia during the period under study. Data were processed using descriptive statistics and chi-square test with a level of confidence of 95 per cent. 51 years old and older were the most affected ages, female sex prevailed, at the same time the II and III rami of this nerve were more affected, using above all pharmacological therapy. The behavior of trigeminal neuralgia from the epidemiological view point is similar to other studies, but differing from its treatment(AU)

[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.

Evidence of descending inhibition deficits in atypical but not classical trigeminal neuralgia.

Trigeminal neuralgia (TN) is a rare neuropathic facial pain disorder. Two forms of TN, classical TN (CTN) and atypical TN (ATN), are reported and probably have different aetiologies. The aim of the present study was to evaluate the functional integrity of the diffuse noxious inhibitory controls (DNIC) in (1) a group of patients with classical trigeminal neuralgia (CTN), (2) a group of patients with atypical trigeminal neuralgia (ATN), and (3) a group of healthy controls in order to determine if a descending pain modulation deficit could participate in the pathophysiology of TN pain. DNIC responses of 14 CTN patients, 14 ATN patients and 14 healthy controls were obtained by comparing thermode-induced facial heat pain scores before and after activating DNIC. DNIC was triggered using a standard counter-irritation paradigm (i.e., immersion of the arm in painfully cold water). General sensitivity to pain was also evaluated by measuring mechanical pain thresholds over 18 points located outside the trigeminal territory. Healthy participants and CTN patients showed a 21% and 16% reduction in thermode-induced pain following the immersion, respectively (all p-values <.01), whereas ATN patients experienced no change (p=.57). ATN patients also had more tender points (mechanical pain thresholds<4.0kg) than CTN and healthy controls (all p-values <.05). Taken together, these results suggest that the underlying physiopathology differs between CTN and ATN and that a deficit in descending inhibition may further contribute to the pain experienced by patients with ATN.

Redefining primary headaches.

In the light of the pathophysiologic knowledge acquired in the recent years, a tentative redefinition is now possible of some types of headache until now defined as idiopathic, and indistinctly described as primary headaches. Cluster headache and trigeminal neuralgia are known examples of diseases classified as primary, which are, in contrast, well-defined diseases to be distinguished from headaches without any recognized anatomic site of lesion or pathogenesis. Another still debated condition, chronic migraine, is proposed here as the consequence of "processes" to be ascribed to mechanisms activated by other comorbid conditions. The observations supporting the possibility that allodynia represents the implicit process leading to pain progression, which occurs in some migraineurs, are discussed.

[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.