Orofacial Migraine and Other Idiopathic Non-Dental Facial Pain Syndromes: A Clinical Survey of a Social Orofacial Patient Group.

Background: Orofacial pain syndromes (OFPs) are a heterogeneous group of syndromes mainly characterized by painful attacks localized in facial and oral structures. According to the International Classification of Orofacial Pain (ICOP), the last three groups (non-dental facial pain, NDFP) are cranial neuralgias, facial pain syndromes resembling primary headache syndromes, and idiopathic orofacial pain. These are often clinical challenges because the symptoms may be similar or common among different disorders. The diagnostic efforts often induce a complex diagnostic algorithm and lead to several imaging studies or specialized tests, which are not always necessary. The aim of this study was to describe the encountered difficulties by these patients during the diagnostic-therapeutic course. Methods: This study was based on the responses to a survey questionnaire, administered to an Italian Facebook Orofacial Patient Group, searching for pain characteristics and diagnostic-therapeutic care courses. The questionnaire was filled out by patients affected by orofacial pain, who were 18 years and older, using a free online tool available on tablets, smartphones, and computers. Results: The sample was composed of 320 subjects (244F/76M), subdivided by age range (18-35 ys: 17.2%; 36-55 ys: 55.0%; >55 ys 27.8%). Most of the patients were affected by OFP for more than 3 years The sample presented one OFP diagnosis in 60% of cases, more than one in 36.2% of cases, and 3.8% not classified. Trigeminal neuralgia is more represented, followed by cluster headaches and migraines. About 70% had no pain remission, showing persisting background pain (VAS median = 7); autonomic cranial signs during a pain attack ranged between 45 and 65%. About 70% of the subjects consulted at least two different specialists. Almost all received drug treatment, about 25% received four to nine drug treatments, 40% remained unsatisfied, and almost 50% received no pharmacological treatment, together with drug therapy. Conclusion: To the authors' knowledge, this is the first study on an OFP population not selected by a third-level specialized center. The authors believe this represents a realistic perspective of what orofacial pain subjects suffer during their diagnostic-therapeutic course and the medical approach often results in unsatisfactory outcomes.

Differences in the Clinical Characteristics of Persistent Idiopathic Facial Pain (Atypical Odontalgia) Patients with or Without Neurovascular Compression of the Trigeminal Nerve.

BACKGROUND: Persistent idiopathic facial pain (PIFP) is the unexplained pain along the territory of the trigeminal nerve, including nonorganic tooth pain called atypical odontalgia (AO). Though PIFP is debilitating to patients' livelihood and well-being, its pathophysiology remains poorly understood. Although neurovascular compression (NVC) of the trigeminal nerve is known to be associated with trigeminal neuralgia (TN), the relationship between NVC and other orofacial pains has not been fully elucidated. METHODS: In this study, we investigated the differences in the characteristics of PIFP (primarily AO) patients in the presence or absence of NVC. A retrospective analysis was performed on data from 121 consecutive patients who had been diagnosed with unilateral PIFP according to the criteria of the International Classification of Headache Disorders (ICHD)-3 and underwent magnetic resonance imaging scans of the head. RESULTS: In the group without NVC, characteristic findings were significant for psychiatric morbidity, somatization, and pain disability, when compared with the group with NVC. Furthermore, the group without NVC exhibited significant headache, noncardiac chest pain, shortness of breath, and pain catastrophizing. CONCLUSIONS: These results suggest that PIFP patients can be divided into two groups: one consistent with a neuropathic pain phenotype when NVC is present and a functional somatic symptom phenotype when presenting without NVC. Our findings may enable a more precise understanding of pathophysiology of PIFP and lead to better treatment strategies.

Eagle Syndrome: An Unusual Cause Limited Mouth Opening and Surgical Management.

Eagle syndrome, defined "stylalgia," occurs when an elongated styloid process or calcified stylohyoid ligament causes dysphagia, facial pain recurrent throat pain or foreign body sensation, also associated symptoms such as neck or throat pain with radiation to the ear. The symptoms related to this condition can be confused or misdiagnosed to a wide variety of facial neuralgias. The incidence of Eagle syndrome varies among population. Usually asymptomatic, it occurs in adult patients, and can be diagnosed by physical examination and radiologically. A 30-year-old male patient presented to the maxillofacial unit of Sulaimaniyah Teaching Hospital with a complaint of pain in the right side of face interfering with mouth opening and causing deviation to the right side of mouth for 6 months duration. The elongated styloid process of the right side was resected surgically by the intra-oral approach. The patient was asymptomatic and comfortably followed up for 5 months.

The temporal evolution of a facial pain syndrome associated with neurovascular contact: a case report.

BACKGROUND: Trigeminal autonomic cephalalgias are primary headaches characterized by unilateral pain and cranial autonomic symptoms. However, associated autonomic symptoms have also been reported in other headaches and facial pains, e.g. trigeminal neuralgia, with the clinical differentiation proving a complex task. CASE: A 54-year-old man presented with right-sided, sharp, intense facial pain in the distribution area of the trigeminal nerve. Pain duration was from seconds to a few minutes, and trigger factors included ipsilateral touching of the skin and hair. Over the next ten years, symptoms progressed and changed presentation, also displaying as right-sided, severe, orbital pain, lasting 60 to 90 minutes, with conjunctival injection and rhinorrhea. Neurological examination was normal. Numerous medications were tried with limited or no effect. In 2010, magnetic resonance imaging revealed a right-sided deviation of the basilar artery at the level of pons, creating neurovascular contact with the trigeminal nerve. Microvascular decompression was performed, and symptoms resolved within days. CONCLUSION: Differentiating between trigeminal autonomic cephalalgias and trigeminal neuralgia with autonomic symptoms can be challenging. The distinct change and evolution over time in the clinical presentation of the patient's head pain suggests a temporal plasticity of the pain in head and facial syndromes, irrespective of underlying pathoanatomic features.

The Utility of the Sino-Nasal Outcome Test (SNOT) as a Treatment Outcome Measure in Nonsinogenic Facial Pain Syndrome.

INTRODUCTION: The purpose of this study was to assess the strength of correlation between the Sino-Nasal Outcome Test (SNOT) subdomains to determine which bore the greatest disease burden so that it could be used as a suitable measure of clinical response. METHODS: Prospective clinical audit of patients attending the Liverpool Multi-Disciplinary Facial Pain Clinic. The Brief Pain Inventory-Facial (BPI-F) was used as a comparator and surrogate measure of quality of life. RESULTS: Fifty patients (38 female) were reviewed, of whom 66% had midfacial segment pain. The total SNOT score was highly correlated with the BPI-F. Both the sleep function and psychological issues were the most highly correlated SNOT subdomains with the BPI-F, followed by ear-facial symptoms. The rhinologic symptom subdomain had a weak correlation with the BPI-F. The total SNOT score was most significantly correlated with the sleep function and psychological issues subdomains. CONCLUSION: This study supports the utility of the SNOT questionnaire as a measure of clinical outcome for nonsinogenic facial pain. The total SNOT score is highly correlated with the BPI-F, which has been used as a surrogate measure of quality of life. In addition, the sleep function and psychological issues subdomains are suitable variables to measure response to treatment.

Introduction to neuropathic pain syndromes.

Chronic pain impairs the quality of life for millions of individuals and therefore presents a serious ongoing challenge to clinicians and researchers. Debilitating chronic pain syndromes cost the US economy more than $600 billion per year. This article provides an overview of the epidemiology, clinical presentation, and treatment outcomes for craniofacial, spinal, and peripheral neurologic pain syndromes. Although the authors recognize that the diagnosis and treatment of the chronic forms of neuropathic pain syndromes represent a clinical challenge, there is an urgent need for standardized classification systems, improved epidemiologic data, and reliable treatment outcomes data.

Hemifacial atrophy.

We report the case of a 44-year-old woman with a one-year history of en coup de sabre morphea and progressive hemifacial atrophy with ipsilateral hemifacial neuralgia, migraine, and contralateral neurologic abnormalities. While rare, Parry-Romberg syndrome typically presents in the first or second decade of life; this patient's case is unusual in that the onset of her disease is demonstrated at age 43. Common clinical features, laboratory findings, and pathogenetic theories are discussed.

Orofacial pain and sensory characteristics of chronic patients compared with controls.

OBJECTIVES: To investigate orofacial and sensorial characteristics of patients with orofacial pain and healthy controls. STUDY DESIGN: Case-control. Seventy-five patients (61 women) who had chronic orofacial pain for more than 6 months (i.e., idiopathic trigeminal neuralgia, burning mouth syndrome, persistent idiopathic facial pain, nonidiopathic neuropathic pain, fibromyalgia or temporomandibular disorders were compared with 41 healthy subjects (19 women). They were evaluated with a quantitative sensory testing protocol involving a combination of gustative, olfactory, thermal, mechanical and pain stimuli. Mean pain duration in this population was 6.27 ± 6.06 years. Orofacial characteristics (masticatory and articular abnormalities), oral health and sensitivity to muscular palpation were assessed. RESULTS: The majority of patients (73.3%) had pain upon craniofacial muscle palpation and 46.7% had numbness. High cold thresholds were observed with burning mouth syndrome and nonidiopathic neuropathic pain (P = .017), while high tactile thresholds were observed in persistent idiopathic facial pain patients (P = .048). Persistent idiopathic facial pain and temporomandibular disorders were associated with a low threshold for pain perception (P < .002). Several of these sensorial alterations were associated with positive musculoskeletal findings. CONCLUSIONS: Sensorial abnormalities were observed in neuropathic and somatic pain patients. The masticatory system is associated with and may be a secondary cause of pain in such patients.

Interaction of IL-1ß and P2X(3) receptor in pathologic masseter muscle pain.

The exact mechanism underlying chronic masseter muscle pain, a conspicuous symptom in temporomandibular disorder, remains unclear. We investigated whether expression of P2X3 receptor (P2X3R) is involved in mechanical hyperalgesia after contraction of masseter muscle (CMM). As compared with sham rats, the head-withdrawal threshold (HWT) to mechanical pressure stimulation of masseter muscle (MM) (but not after similar stimulation of facial skin) was significantly lower, and IL-1ß level was significantly higher, in CMM rats on day 7 after CMM. The mean percentage of FG-labeled P2X3R-positive neurons was significantly increased in TG following successive IL-1ß injections into the MM for 7 days. Successive administration of an IL-1ß receptor-antagonist into the MM attenuated the increase of P2X3-IR cells in the TG. ATP release from MM after 300-g pressure stimulation of MM was also significantly enhanced after CMM. Administration into MM of the selective P2X3,2/3 receptor antagonist A-317491 attenuated the decrement of HWT in CMM rats. A significant increase in HWT was also observed at 30 min after A-317491 (60 µg) injection in IL-1ß-injected rats. These findings suggest that P2X3R expression associated with enhanced IL-1ß expression and ATP release in MM has a possible important role in MM mechanical hyperalgesia after excessive muscular contraction.

The relationship of temporomandibular disorders with headaches: a retrospective analysis.

OBJECTIVES: The objective of this study was to retrospectively analyze the incidence of the concurrent existence of temporomandibular disorders (TMD) and headaches. METHODS: Forty patients (36 female, 4 male, mean age: 29.9±9.6 years) clinically diagnosed with TMD were screened. Patient records were analyzed regarding: range of mouth opening, temporomandibular joint (TMJ) noises, pain on palpation of the TMJ and masticatory muscles and neck and upper back muscles, and magnetic resonance imaging of the TMJ. RESULTS: According to patient records, a total of 40 (66.6%) patients were diagnosed with TMD among 60 patients with headache. Thirty-two (53%) patients had TMJ internal derangement (ID), 8 (13%) patients had only myofascial pain dysfunction (MPD) and 25 (41.6%) patients had concurrent TMJ ID/MPD. There were statistically significant relationships between the number of tender masseter muscles and MPD patients (p=0.04) and between the number of tender medial pterygoid muscles and patients with reducing disc displacement (RDD) (p=0.03). CONCLUSION: The TMJ and associated orofacial structures should be considered as possible triggering or perpetuating factors for headaches, especially tension-type. There might be a significant connection between TMD and headache. However, most medical and dental practitioners are unaware of this relationship. Therefore, a careful evaluation of the TMJ and associated orofacial structures is required for a correct interpretation of the craniofacial pain in headache patients, and these patients should be managed with a multidisciplinary approach.

Otalgia.

Otalgia, pain in the ear, can be a consequence of otologic disease (primary or otogenic otalgia), or can arise from pathologic processes and structures other than the ear (secondary or referred otalgia). In children, ear disease is far and away the most common cause of otalgia, but in adults, secondary or referred otalgia is more common. Otalgia can be the only presenting symptom of several serious conditions, and its etiology should be fully explored. Unfortunately, its workup is complex and no simple algorithm exists. This article outlines the common causes, presentation, and treatment options for primary and secondary otalgia.

Dysphagia and neuropathic facial pain treated with motor cortex stimulation: case report.

OBJECTIVE: We report on a patient with a neuropathic facial pain syndrome, including elements of trigeminal neuralgia, glossopharyngeal neuralgia, and dysphagia. After failing medical and surgical decompressive treatments, the patient underwent implantation of a motor cortex stimulation (MCS) system. CLINICAL PRESENTATION: A 54-year-old woman presented with a 14-year history of left-sided facial pain, throat pain, and associated nausea and vomiting. The patient failed several open surgical and percutaneous procedures for her facial pain syndrome. Additionally, several medication trial attempts were unsuccessful. Imaging studies were normal. INTERVENTION: The patient underwent placement of a right-sided MCS system for treatment of her neuropathic facial pain syndrome. The procedure was tolerated well, and the trial stimulator provided promising results. The permanent MCS generator needed to be reprogrammed at the time of the 5-week follow-up visit to optimize symptom relief. The patient demonstrated dramatic improvements in her neuropathic facial and oral pain, including improvements in swallowing toleration, after the 5-week follow-up examination with subthreshold MCS. A decline in treatment efficacy also occurred 2 years after implantation due to generator depletion. Symptom improvement returned with stimulation after the generator was replaced. CONCLUSION: A novel implantable MCS system was used to treat this patient's neuropathic facial pain. Durable improvements were noted not only in her facial pain, but also in swallowing toleration. The ultimate role of MCS in the treatment of pain conditions is still not well-defined but might play a part in refractory cases and, as in this case, might improve other functional issues, including dysphagia.

A pilot study investigating the effects of fast left prefrontal rTMS on chronic neuropathic pain.

OBJECTIVE: Stimulating the human cortex using transcranial magnetic stimulation (TMS) temporarily reduces clinical and experimental pain; however, it is unclear which cortical targets are the most effective. The motor cortex has been a popular target for managing neuropathic pain, while the prefrontal cortex has been investigated for an array of nociceptive pain conditions. It is unclear whether the motor cortex is the only effective cortical target for managing neuropathic pain, and no published studies to date have investigated the effects of prefrontal stimulation on neuropathic pain. DESIGN: This preliminary pilot trial employed a sham-controlled, within-subject, crossover design to evaluate clinical pain as well as laboratory pain thresholds among four patients with chronic neuropathic pain. Each participant underwent three real and three sham 20-minute sessions of 10 Hz left prefrontal repetitive TMS. Daily pain diaries were collected for 3 weeks before and after each treatment phase along with a battery of self-report pain and mood questionnaires. RESULTS: Time-series analysis at the individual patient level indicated that real TMS was associated with significant improvements in average daily pain in 3 of the 4 participants. These effects were independent of changes in mood in two of the participants. At the group level, a decrease of 19% in daily pain on average, pain at its worst, and pain at its least was observed while controlling for changes in mood, activity level and sleep. The effects of real TMS were significantly greater than sham. Real TMS was associated with increases in thermal and mechanical pain thresholds, whereas sham was not. No statistically significant effects were observed across the questionnaire data. CONCLUSIONS: The prefrontal cortex may be an important TMS cortical target for managing certain types of pain, including certain neuropathic pain syndromes.

Pain relief after cervical ganglionectomy (C2 and C3) for the treatment of medically intractable occipital neuralgia.

Occipital neuralgia (ON) presents a diagnostic challenge because of the wide variety of symptoms, surgical findings, and postsurgical outcomes. Surgical removal of the second (C2) or third (C3) cervical sensory dorsal root ganglion is an option to treat ON. The goal of this study was to evaluate the short-term and the long-term efficacy of these procedures for management of cervical and occipital neuropathic pain. Twenty patients (mean age 48.7 years) were identified who had undergone C2 and/or C3 ganglionectomies for intractable occipital pain and a retrospective chart review undertaken. Patients were interviewed regarding pain relief, pain relief duration, functional status, medication usage and procedure satisfaction, preoperatively, immediately postoperative, and at follow-up (mean 42.5 months). C2, C3 and consecutive ganglionectomies at both levels were performed on 4, 5, and 11 patients, respectively. All patients reported preoperative pain relief following cervical nerve blocks. Average visual analog scale scores were 9.4 preoperatively and 2.6 immediately after procedure. Ninety-five percent of patients reported short-term pain relief (<3 months). In 13 patients (65%), pain returned after an average of 12 months (C2 ganglionectomy) and 8.4 months (C3 ganglionectomy). Long-term results were excellent, moderate and poor in 20, 40 and 40% of patients, respectively. Cervical ganglionectomy offers relief to a majority of patients, immediately after procedure, but the effect is short lived. Nerve blocks are helpful in predicting short-term success, but a positive block result does not necessarily predict long-term benefit and therefore cannot justify surgery by itself. However, since 60% of patients report excellent-moderate results, cervical ganglionectomy continues to have a role in the treatment of intractable ON.

[Arterial blood gas abnormalities during attacks of dizziness]. / Bas dönmesi ataklari sirasinda arteryel kan gazi anormallikleri.

OBJECTIVES: We investigated arterial blood gas abnormalities in patients presenting with dizziness. PATIENTS AND METHODS: The study included 58 patients (39 females, 19 males; mean age 46 years; range 22 to 74 years) who presented during attacks of dizziness. The duration of vertigo complaints ranged from one day to 30 years. Arterial gas measurements were performed at presentation and one month after treatment. The patients were examined in five groups according to the diagnoses: Meniere's disease (n=14), benign paroxysmal positional vertigo (n=13), vertebrobasilar insufficiency (n=12), vestibular neuritis (n=4), and craniocervical myofascial syndromes (n=15). RESULTS: At presentation, pH was low (acidosis) in two patients (3.5%), and high (alkalosis) in 15 patients (25.9%). After treatment, all abnormal pH values returned to normal. Twenty-four patients had high or low HCO3- values. High HCO3- values persisted in three patients together with dizziness. Patients with vestibular neuritis had significantly higher PO2 values compared to those with craniocervical myofascial syndrome and vertebrobasilar insufficiency (p<0.05). However, one month after treatment, there were no significant differences between five groups with respect to PO2 levels (p>0.05). Consecutive blood gas measurements did not differ significantly within each diagnosis group (p>0.05). CONCLUSION: Our results suggest that arterial blood gas abnormalities may be related to vertigo attacks.

[Atypical neuroleptics in the treatment of atypical facial neuralgia. Two case reports]. / Kasuistik: Atypische Neuroleptika in der Therapie des idiopathischen Gesichtsschmerzes--Zwei Fallberichte.

The criteria for the diagnosis of the atypical facial neuralgia make it is possible to classify under this neurological diagnosis also psychiatric disorders as conasthetic depression. In two case reports it is told about patients with both diagnosis. Due to this they were treated with an atypical neurolepticum (Olanzapine) to reach three targets: Therapy of the depression, pain i.e. atypical facial neuralgia and mood stabilizing. Within the last four years there were no side-effects as EPMS to observe.

[Atypical facial pains--sluder's neuralgia--local treatment of the sphenopalatine ganglion with phenol--case report]. / Nietypowe bóle twarzy--neuralgia sludera-- leczenie miejscowe--przyieganie zwoju skrzydlowo-- podniebiennego fenolem--opis przypadków.

AIM: Chronic reccuring head and facial pain can be very difficult for successful treatment. Such a pain can be in some rare cases Sluder's sphenopalatine ganglion neuralgia. The aim of the study was to obtain the pain relief by local treatment in patients with Sluder's sphenopalatine ganglion neuralgia. METHODS: We described three cases of Sluder's neuralgia among all the seventeen patients with reccuring head and face pain that were seen in our department. In all these cases 4% Xylocaine was applied intranasally, into the region of shenopalatine ganglion, behind the posterior tip of the middle turbinate four times for ten minutes. According to Kern, the diagnosis of Sluder's neuralgia was confirmed only in cases where local anesthetic block of the sphenopaltine ganglion was successful. It means the patients were pain-free for at least an hour after application of Xylocaine, so they were qualified for phenolization and 88% phenol was applied on the cotton carriers (number of the applications depended on the patient). RESULTS: The total relief of pain of different duration was obtained in all the presented cases. CONCLUSION: The relief of pain obtained by intranasal phenolization of sphenopalatine ganglion in three patients shows it could be the effective treatment of Sluder's neuralgia. The patients were totally free from the pain and accompanying symptoms like nasal obstruction, rhinorrhea, epiphora or conjunctivitis. The relief period was different but the patients were satisfied with the effectiveness and simplicity of the treatment. They did not need to take the additional medications for months and were able to continue work.

Occipital neuralgia evoked by facial herpes zoster infection.

Occipital neuralgia is a pain syndrome which may usually be induced by spasms of the cervical muscles or trauma to the greater or lesser occipital nerves. We report a patient with occipital neuralgia followed by facial herpes lesion. A 74-year-old male experienced sudden-onset severe headache in the occipital area. The pain was localized to the distribution of the right side of the greater occipital nerve, and palpation of the right greater occipital nerve reproduces the pain. He was diagnosed with occipital neuralgia according to ICHD-II criteria. A few days later, the occipital pain was followed by reddening of the skin and the appearance, of varying size, of vesicles on the right side of his face (the maxillary nerve and the mandibular nerve region). This was diagnosed as herpes zoster. This case represents a combination of facial herpes lesions and pain in the C2 and C3 regions. The pain syndromes can be confusing, and the classic herpes zoster infection should be considered even when no skin lesions are established.