"Status trigeminal neuralgia": Analysis of 39 cases and proposal for diagnostic criteria.

OBJECTIVE: The aim of this descriptive study was to propose diagnostic criteria for acute exacerbation of trigeminal neuralgia (TN) based on the analysis of retrospective cases. BACKGROUND: TN is a rare and extremely painful condition whose evolution can be punctuated by major exacerbations, leading to significant functional impairment. Several denominations are used for these exacerbations: "acute exacerbation", "status of trigeminal neuralgia", and "status trigeminus". There is currently no clinical definition of this state. In this manuscript, we used the term "status trigeminal neuralgia" (STN). METHODS: We conducted a retrospective study, in a tertiary care specialist headache center, in France. Patients were selected from January 2015 to October 2022, with the French translation of the keyword "STN", in the medical records (outpatients) or the codage for trigeminal neuralgia (inpatients). Additional cases of STN were prospectively recruited from October 2022 to February 2023. We analyzed the clinical and paraclinical data of these patients. RESULTS: Thirty-nine patients presenting with STN were included. There was a preponderance of women (64%) with 24 cases of classic TN (62%) and 15 cases of secondary TN (38%). Concerning STN, 39 episodes were described. Pain was very severe in all patients. Cranial autonomic signs were present in 23% of cases. Pain extended beyond the usual territory in 44% of cases. A continuous pain background was present in 35% of cases. With regard to triggering factors, paroxysms of facial pain were triggered by eating (97% of patients), speaking (90%) or drinking (62% of patients). Repercussions on weight, hydration, or mood disorders were observed in 67%, 56% and 59% of the cases, respectively. CONCLUSION: STN is a rare clinical presentation of TN. We proposed criteria and a new denomination for this condition.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. METHODS: A retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. RESULTS: Among the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. CONCLUSIONS: This procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.

One-year prospective real-world assessment of effectiveness and safety of erenumab in migraine prevention: results of the French FHU INOVPAIN registry study.

BACKGROUND: Randomized clinical trials have demonstrated efficacy and safety of erenumab. The aim of this study is to evaluate the effectiveness and safety of erenumab in a real-world setting in French patients with migraine associated with extreme unmet needs. METHODS: This is a one year-prospective real-word study with enrolment of all consecutive adult patients included in the FHU InovPain registry who participated in a compassionate erenumab use program. RESULTS: Of 144 patients included, 140 patients (82.1% female / mean age of 50.9 ± 11.4) received at least one dose of erenumab and were concerned by effectiveness and safety assessment. All patients had failed 11 oral preventive treatments. Most of them suffered from chronic migraine (88.6%) and presented a medication overuse (90.7%) at baseline. Thirty-eight (27.1%) discontinued treatment during the 12-month follow-up, with 22 (15.7%), 11 (7.9%) and 5 (3.6%) patients before 3, 6 or 9 months of treatment. The proportion of ≥ 50% responders at M3, M6, M9 and M12 was 74/140 (52.9%), 69/118 (58.5%), 61/107 (57.0%) and 60/102 (58.8%) respectively. At M3, the rate of reversion from chronic migraine to episodic migraine was 57.3% and the rate of transition from medication overuse to non-overuse was 46.5%. For monthly migraine days, the median (IQR) was 18.0 (13.0-26.0), 9.0 (5.0-17.0), 7.5 (5.0-14.0), 8.0 (5.0-12.5) and 8.0 (5.0-12.0) at M0, M3, M6, M9 and M12 respectively. For HIT-6 score, the median (IQR) was 68.0 (63.8-73.3), 60.0 (54.0-65.0), 60.0 (50.3-53.0), 59.0 (50.0-63.0) and 58.0 (50.0-62.9) at M0, M3, M6, M9 and M12 respectively. Fifty-three (37.9%) patients reported at least one of the following adverse events: cutaneous erythema and/or pain at the injection site for 42 (30%) patients, constipation for 22 (15.7%) patients, muscle spasm for 2 (1.4%) patients, alopecia for one (0.7%) patient and blood pressure increase in one (0.7%) patient. There was no serious adverse event. One female patient became pregnant after 5 months of exposure to erenumab with a safe evolution after treatment discontinuation. CONCLUSION: This first French real-world study related to migraine prevention with CGRP-mAbs confirms effectiveness and safety of erenumab in patients with extreme unmet needs.

Study of severe migraine in general medicine.

OBJECTIVE: To evaluate the criteria of severe migraine in a general population consulting a general practitioner (GP) and to evaluate assessment of migraine severity in the migraine patient as well as treating physicians' knowledge of their patient's migraine and its severity. METHODS: We questioned voluntary headache patients who had an appointment with a GP about the severity of their migraine using recognized scores - HIT-6 and MIDAS - as well as with a specific questionnaire created for the study. We compared the criteria for severe migraine with the patient's description of their symptoms, their HIT-6 score, their MIDAS score, and the GP's opinion, analyzing collected data using means and standard deviations. RESULTS: We found that 152 out of 942 headache patients questioned in the general medicine setting met the criteria of "strict migraine", corresponding to 10.3% prevalence. Seventy-one out of 100 patients (71%) with migraine who filled out in the questionnaire completely had what is characterized as "severe migraine". Forty-one (57%) of the 71 severe migraine patients presented the strict criteria. Additionally, 21 of the 29 (72%) patients with a non-severe diagnosis agreed that their headache was non-severe. When the HIT-6 score was stratified above 65, correspondence between the questionnaire-derived diagnosis and patient perception of severity was observed in 36 of the 58 (62%) with severe migraine. Finally, participating GPs were aware of their patient's migraine for 60% of patients with migraine. GPs correctly classified the severity of their patient's migraine for 55 (36%) patients. CONCLUSION: GP education and training about migraine remain a public health issue. The diagnosis of severe migraine is necessary for proper patient management. Current criteria for severe migraine are not robust enough; we propose a modification of the criteria.

Revised guidelines of the French headache society for the diagnosis and management of migraine in adults. Part 3: Non-pharmacological treatment.

The French Headache Society proposes updated French guidelines for the management of migraine. This article presents the third part of the guidelines, which is focused on the non-pharmacological treatment of migraine, including physical exercise, dietary supplements and plants, diets, neuromodulation therapies, acupuncture, behavioral interventions and mindfulness therapy, patent foramen ovale closure and surgical nerve decompression.

Revised guidelines of the French headache society for the diagnosis and management of migraine in adults. Part 2: Pharmacological treatment.

The French Headache Society proposes updated French guidelines for the management of migraine. This article presents the second part of the guidelines, which is focused on the pharmacological treatment of migraine, including both the acute treatment of attacks and the prophylaxis of episodic migraine as well as chronic migraine with and without medication overuse. The specific situations that can be encountered in women with migraine are also discussed, including pregnancy, menstrual migraine, contraception and hormonal replacement therapy.

Revised guidelines of the French Headache Society for the diagnosis and management of migraine in adults. Part 1: Diagnosis and assessment.

The French Headache Society proposes updated French guidelines for the management of migraine. The first part of these recommendations is focused on the diagnosis and assessment of migraine. First, migraine needs to be precisely diagnosed according to the currently validated criteria of the International Classification of Headache Disorders, 3d version (ICHD-3). Migraine-related disability has to be assessed and we suggest to use the 6 questions of the headache impact test (HIT-6). Then, it is important to check for risk factors and comorbidities increasing the risk to develop chronic migraine, especially frequency of headaches, acute medication overuse and presence of depression. We suggest to use a migraine calendar and the Hospital Anxiety and Depression scale (HAD). It is also necessary to evaluate the efficacy and tolerability of current migraine treatments and we suggest to systematically use the self-administered Migraine Treatment Optimization Questionnaire (M-TOQ) for acute migraine treatment. Finally, a treatment strategy and a follow-up plan have to be proposed. Guidelines for pharmacological and non-pharmacological treatments are presented in the second and third part of the recommendations.

Headaches during pregnancy.

Headache during pregnancy is frequent and challenging. A secondary headache is diagnosed in one third of women, and most causes are vascular and infectious disorders. Anamnestic and clinical features must be investigated, and brain imaging is often required. Primary headaches are commonly modified during pregnancy. Migraine is mainly improved in this context, especially migraine without aura. Particular cautions about treatment must be applied, and non-pharmacological measures must be preferred. Tension-type headache and cluster headache seem to be less hormone-dependent.

European Academy of Neurology guideline on trigeminal neuralgia.

BACKGROUND AND PURPOSE: Trigeminal neuralgia (TN) is an extremely painful condition which can be difficult to diagnose and treat. In Europe, TN patients are managed by many different specialities. Therefore, there is a great need for comprehensive European guidelines for the management of TN. The European Academy of Neurology asked an expert panel to develop recommendations for a series of questions that are essential for daily clinical management of patients with TN. METHODS: A systematic review of the literature was performed and recommendations was developed based on GRADE, where feasible; if not, a good practice statement was given. RESULTS: The use of the most recent classification system is recommended, which diagnoses TN as primary TN, either classical or idiopathic depending on the degree of neurovascular contact, or as secondary TN caused by pathology other than neurovascular contact. Magnetic resonance imaging (MRI), using a combination of three high-resolution sequences, should be performed as part of the work-up in TN patients, because no clinical characteristics can exclude secondary TN. If MRI is not possible, trigeminal reflexes can be used. Neurovascular contact plays an important role in primary TN, but demonstration of a neurovascular contact should not be used to confirm the diagnosis of TN. Rather, it may help to decide if and when a patient should be referred for microvascular decompression. In acute exacerbations of pain, intravenous infusion of fosphenytoin or lidocaine can be used. For long-term treatment, carbamazepine or oxcarbazepine are recommended as drugs of first choice. Lamotrigine, gabapentin, botulinum toxin type A, pregabalin, baclofen and phenytoin may be used either alone or as add-on therapy. It is recommended that patients should be offered surgery if pain is not sufficiently controlled medically or if medical treatment is poorly tolerated. Microvascular decompression is recommended as first-line surgery in patients with classical TN. No recommendation can be given for choice between any neuroablative treatments or between them and microvascular decompression in patients with idiopathic TN. Neuroablative treatments should be the preferred choice if MRI does not demonstrate any neurovascular contact. Treatment for patients with secondary TN should in general follow the same principles as for primary TN. In addition to medical and surgical management, it is recommended that patients are offered psychological and nursing support. CONCLUSIONS: Compared with previous TN guidelines, there are important changes regarding diagnosis and imaging. These allow better characterization of patients and help in decision making regarding the planning of medical and surgical management. Recommendations on pharmacological and surgical management have been updated. There is a great need for future research on all aspects of TN, including pathophysiology and management.

Management of patients presenting with generalized musculoskeletal pain and a suspicion of Lyme disease.

Lyme disease is caused by bacteria of the B. burgdorferi sensu lato complex, and can give polymorphic clinical manifestations that can affect several organs such as the skin, the central nervous system, or the joints. In recent years, patients' associations and physicians have been supporting the hypothesis that this infection would manifest as chronic generalized musculoskeletal pain symptoms, named "chronic Lyme disease". Fibromyalgia is a clinical presentation characterized by chronic generalized musculoskeletal pain with a major impact on quality of life and social and psychological functioning. We analyzed existing literature data on pain syndromes associated with Lyme disease (post-treatment Lyme disease syndrome) or tick bites (polymorphic symptoms after a tick bite). We also analyzed existing data on the diagnosis, pathophysiology, and treatment of fibromyalgia. Our review shows that post-treatment Lyme disease syndrome has characteristics very close to post-infectious fibromyalgia. On the other hand, patients presenting for Lyme disease screening because of chronic generalized musculoskeletal pain symptoms after a tick bite should also be screened for fibromyalgia to allow appropriate management. Antibiotics are not recommended here.

Ergot Use and Overuse: A Pharmacoepidemiology Retrospective Cohort Study.

OBJECTIVE: The objective of this study was to estimate and to characterize the actual patterns of ergot use and overuse in France using a drug reimbursement database. METHODS: We included all people covered by the French General Health Insurance System (GHIS) from the Provence-Alpes-Côte-d'Azur (PACA) and Corsica administrative areas who had at least one prescription of ergot between May 2010 and December 2011. All prescriptions of ergots, migraine prophylactic treatment, and psychotropic medications were extracted from the GHIS database. We defined occasional ergot users (<3 months of prescription) and regular ergot users (>3 months of prescription). Among regular ergot users, we identified overusers and nonoverusers. RESULTS: We included 4358 patients who had at least one prescription of ergots (oral ergotamine tartrate, dihydroergotamine mesilate nasal spray, intravenous dihydroergotamine mesilate). Among ergot overusers, a large majority of patients had ergotamine tartrate overuse. The proportion of ergotamine tartrate overusers is maximum after 55 years. Compared with regular users, overusers use more frequently a prophylactic treatment (93/165 [56.4%] versus 398/1057, OR = 2.15, P < .001), antidepressants (72/165 [43.6%] versus 326/1057 [30.8%] OR = 1.79, P < .001), benzodiazepines (111/165 [67.3%] versus 613/1057 [58.0%], OR = 1.50, P < .001), weak opioids (95/165 [57.6%] versus 463/1057 [43.8], OR = 1.77, P < .001) and strong opioids (13/165 [7.9%] versus 24/1057 [2.3%], OR = 3.86, P < .001). The coexistence of ergot consumption and triptan overuse, and the possibility of both triptan and ergot overuse was described; triptan overusers were more described in ergotamine overusers than in nonoverusers. CONCLUSIONS: This work outlines a high prevalence of ergotamine tartrate overuse (11.1%). As ergotamine tartrate users are mostly aged more than 55 years, an evaluation of ergotamine cardiovascular risk profile is necessary in the elderly population.

[Migraine and hypothalamus]. / Migraine et hypothalamus.

Migraine is a complex brain disease. The "generator" of the migrainous attacks remains a subject of debate, but the hypothalamus, with its multiple connections with the other parts of the central nervous system and its controls on the pituitary gland and the autonomic nervous system, is a very serious candidate. Many of the premonitory symptoms of migraine attacks find their origin in the hypothalamus. The hormonal changes which occur during feminine genital life and which impact on the life of the migrainous women have their origin in the hypothalamus. The hypothalamus exerts control over the balance between the parasympathetic and orthosympathetic systems. Orexine, hormones originating in the hypothalamic, are involved in sleep regulation, thermoregulation and neuroendocrine and nociceptive functions. They could play a crucial role in the origin of the migrainous attack and might explain the influence of sleep, eating habits and excessive weight in the occurrence of attacks. Hypothalamic cerebral activation via H2 15OPET activity, suspected by clinical and experimental arguments as a possible trigger for migraine, has been demonstrated during spontaneous attacks. However, no conclusion can be made however as to whether this activation is the cause or the consequence of the migrainous pain.