Cluster Headache, SUNCT, and SUNA.

OBJECTIVE: This article reviews the epidemiology, clinical features, differential diagnosis, pathophysiology, and management of three types of trigeminal autonomic cephalalgias: cluster headache (the most common), short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). LATEST DEVELOPMENTS: The first-line treatments for trigeminal autonomic cephalalgias have not changed in recent years: cluster headache is managed with oxygen, triptans, and verapamil, and SUNCT and SUNA are managed with lamotrigine. However, new successful clinical trials of high-dose prednisone, high-dose galcanezumab, and occipital nerve stimulation provide additional options for patients with cluster headache. Furthermore, new genetic and imaging tests in patients with cluster headache hold promise for a better understanding of its pathophysiology. ESSENTIAL POINTS: The trigeminal autonomic cephalalgias are a group of diseases that appear similar to each other and other headache disorders but have important differences. Proper diagnosis is crucial for proper treatment.

Surgical management of nervus intermedius neuralgia: A report of 4 cases and literature review.

BACKGROUND: Nervus intermedius neuralgia (NIN) is characterized by paroxysmal episodes of sharp, lancinating pain in the deep ear. Unfortunately, only a few studies exist in the literature on this pain syndrome, its pathology and postoperative outcomes. METHOD: We conducted a retrospective review of four cases diagnosed with NIN who underwent a neurosurgical intervention at our center from January 2015 to January 2023. Detailed information on their MRI examinations, intraoperative findings and other clinical presentations were obtained, and the glossopharyngeal and vagus nerves were isolated for immunohistochemistry examination. RESULTS: A total of 4 NIN patients who underwent a microsurgical intervention at our institution were included in this report. The NI was sectioned in all patients and 3 of them underwent a microvascular decompression. Of these 4 patients, 1 had a concomitant trigeminal neuralgia (TN), and 1 a concomitant glossopharyngeal neuralgia (GPN). Three patients underwent treatment for TN and 2 for GPN. Follow-up assessments ranged from 8 to 99 months. Three patients reported complete pain relief immediately after the surgery until last follow-up, while in the remaining patient the preoperative pain gradually resolved over the 3 month period. Immunohistochemistry revealed that a greater amount of CD4+ and CD8+ T cells had infiltrated the glossopharyngeal versus vagus nerve. CONCLUSIONS: NIN is an extremely rare condition showing a high degree of overlap with TN/GPN. An in depth neurosurgical intervention is effective to completely relieve NIN pain, without any serious complications. It appears that T cells may play regulatory role in the pathophysiology of CN neuralgia.

Trigeminal neuralgia and trigeminal neuropathic pain.

It is very important that the dental team are aware of the varied presentations of pain in the mouth, face and other parts of the trigeminal region which are not directly caused by teeth or oral structures. Our understanding of underlying causes in this complex area is evolving. Ultimately, patients who present with what may at first seem to be oral or dental problems will require specialist input in secondary care with potential for use of systemic medications. This article reviews the common non-dental pains encountered in the orofacial region related to dysfunction of the trigeminal nerve.

[Specificities of orofacial neuropathic pain]. / Spécificités de la douleur neuropathique oro-faciale.

Head pain and notably orofacial pain differs from spinal pain on pathophysiological, clinical, therapeutic and prognostic levels. Its high prevalence, important impact on quality of life and significant socio-economical burden justify specific study of such type of pain. Among them, neuropathic orofacial pain resulting from disease or trauma of the trigeminal nervous system is among the most difficult types of pain to diagnose and to treat. Deciphering of underlying peripheral and central mechanisms has allowed numerous conceptual, clinical and therapeutic advances, notably the role of neural and non neural cell types, such as glia, immunocytes, vascular endothelial cells or the role of trigeminal sensory complex neural circuitry reconfiguration in the development of post-traumatic trigeminal neuropathic pain. Cellular interactions within the trigeminal ganglion, allowing a better understanding of several painful dental, ocular or cephalalgic comorbidities, are also described.

Title: Spécificités de la douleur neuropathique oro-faciale. Abstract: Les douleurs de la région céphalique ­ et notamment les douleurs oro-faciales ­ diffèrent des douleurs spinales sur les plans physiopathologique, clinique, thérapeutique et pronostique. Leur prévalence élevée, leur fort retentissement sur la qualité de vie individuelle et leur impact économique et sociétal important justifient une étude spécifique. Parmi ces douleurs, les douleurs neuropathiques, résultant d'une maladie ou d'un traumatisme du système nerveux trigéminal, sont parmi les plus difficiles à diagnostiquer et à soigner. L'étude des mécanismes neurobiologiques, périphériques et centraux les sous-tendant a permis de nombreuses avancées conceptuelles, cliniques et thérapeutiques, avec, par exemple, la mise en évidence du rôle des cellules nerveuses et non nerveuses, telles que la glie, les immunocytes, les cellules endothéliales vasculaires ou le rôle de la reconfiguration de la circuiterie nerveuse au niveau du complexe sensitif trigéminal, dans la genèse des douleurs neuropathiques post-lésionnelles. Les interactions cellulaires au sein du ganglion trigéminal, susceptibles d'éclairer la compréhension de certaines comorbidités douloureuses dentaires, oculaires ou céphalalgiques, sont également décrites.

SUNCT, SUNA and short-lasting unilateral neuralgiform headache attacks: Debates and an update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA. METHODS: We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases. RESULTS: Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases. CONCLUSIONS: Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.

A rare case of vestibular schwannoma manifesting as trigeminal neuralgia.

BACKGROUND: In this case report, the authors reviewed a rare case of a vestibular schwannoma manifesting as trigeminal neuralgia (TN). Intracranial tumors can have a variety of orofacial pain symptoms. Among benign cerebellopontine angle tumors, vestibular schwannoma is the most common cause of a TN-like manifestation. Although the most common symptoms of a vestibular schwannoma are hearing loss and vestibulopathy, the unique feature of this case was the manifestation of symptoms consistent with TN. CASE DESCRIPTION: The patient had right-sided episodic facial pain that was short in duration and severe in intensity. The initial differential diagnoses included short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing and TN. As part of the routine evaluation, the patient was referred for brain magnetic resonance imaging, which revealed a right-sided vestibular schwannoma. The patient was prescribed 200 mg of gabapentin 3 times daily and was referred to neurosurgery for excision of the schwannoma. Surgical excision resulted in complete resolution of pain. PRACTICAL IMPLICATIONS: This case illustrates the importance of interdisciplinary treatment and how it can lead to an optimal outcome for a patient with complex orofacial pain symptoms.

Sixth cranial nerve palsy and trigeminal neuropathic pain due to a space-occupying lesion.

Various neuropathies of the cranil nerves can accompany trigeminal neuropathic pain attributed to space-occupying lesions. In this case report, the patient presented with persistent intraoral pain and numbness on the right side of the face. Cranial nerve examination revealed dysfunctional eye movements, diplopia, and mechanical hyposensitivity in the mandibular region. The patient was diagnosed with neuropathy due to intracranial lesions and referred to the Department of Neurosurgery and Otorhinolaryngology. The patient was suspected of having malignant lymphoma and is currently undergoing neurosurgical intervention. This article discusses the importance of the examination of the cranial nerve for patients with persistent pain in the trigeminal nerve distribution.

Trigeminal Lipomatosis: A Rare Cause of Intractable Neuralgia.

Lipomatosis of nerve, earlier known as fibrolipomatous hamartoma is a rare condition which predominantly affects peripheral nerves, cranial nerve involvement being extremely uncommon. Preoperative consideration of this entity is of paramount importance as its inadvertent complete surgical resection may inevitably result in significant neurological deficit. We report a case of trigeminal lipomatosis in a young patient with trigeminal neuralgia.

Trigeminal Autonomic Cephalalgias and Neuralgias in Children and Adolescents: a Narrative Review.

PURPOSE OF REVIEW: To summarize the available literature as well as the authors' experience on trigeminal autonomic cephalalgias (TACs) and cranial neuralgias in children and adolescents. RECENT FINDINGS: While TACs and cranial neuralgias are rare in children, several recent case series have been published. TACs in children share most of the clinical features of TACs in adults. However, there are many reported cases with clinical features which overlap more than one diagnosis, suggesting that TACs may be less differentiated in youth. Indomethacin-responsive cases of cluster headache and SUNCT/SUNA have been reported in children, whereas in adults indomethacin is usually reserved for paroxysmal hemicrania and hemicrania continua. Neuralgias appear to be rare in children. Clinical features are often similar to adult cases, though clinicians should maintain a high index of suspicion for underlying causes.

Orofacial pain for clinicians: A review of constant and attack-like facial pain syndromes.

BACKGROUND: Primary headache syndromes such as migraine are among the most common neurological syndromes. Chronic facial pain syndromes of non-odontogenic cause are less well known to neurologists despite being highly disabling. Given the pain localization, these patients often consult dentists first who may conduct unnecessary dental interventions even if a dental cause is not identified. Once it becomes clear that dental modalities have no effect on the pain, patients may be referred to another dentist or orofacial pain specialist, and later to a neurologist. Unfortunately, neurologists are also often not familiar with chronic orofacial pain syndromes although they share the neural system, i.e., trigeminal nerve and central processing areas for headache disorders. CONCLUSION: In essence, three broad groups of orofacial pain patients are important for clinicians: (i) Attack-like orofacial pain conditions, which encompass neuralgias of the cranial nerves and less well-known facial variants of primary headache syndromes; (ii) persistent orofacial pain disorders, including neuropathic pain and persistent idiopathic facial/dentoalveolar pain; and (iii) other differential diagnostically relevant orofacial pain conditions encountered by clinicians such as painful temporomandibular disorders, bruxism, sinus pain, dental pain, and others which may interfere (trigger) and overlap with headache. It is rewarding to know and recognize the clinical picture of these facial pain syndromes, given that, just like for headache, an internationally accepted classification system has been published and many of these syndromes can be treated with medications generally used by neurologists for other pain syndromes.

Joint European Academy of Neurology-European Pain Federation-Neuropathic Pain Special Interest Group of the International Association for the Study of Pain guidelines on neuropathic pain assessment.

BACKGROUND AND PURPOSE: In these guidelines, we aimed to develop evidence-based recommendations for the use of screening questionnaires and diagnostic tests in patients with neuropathic pain (NeP). METHODS: We systematically reviewed studies providing information on the sensitivity and specificity of screening questionnaires, and quantitative sensory testing, neurophysiology, skin biopsy, and corneal confocal microscopy. We also analysed how functional neuroimaging, peripheral nerve blocks, and genetic testing might provide useful information in diagnosing NeP. RESULTS: Of the screening questionnaires, Douleur Neuropathique en 4 Questions (DN4), I-DN4 (self-administered DN4), and Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) received a strong recommendation, and S-LANSS (self-administered LANSS) and PainDETECT weak recommendations for their use in the diagnostic pathway for patients with possible NeP. We devised a strong recommendation for the use of skin biopsy and a weak recommendation for quantitative sensory testing and nociceptive evoked potentials in the NeP diagnosis. Trigeminal reflex testing received a strong recommendation in diagnosing secondary trigeminal neuralgia. Although many studies support the usefulness of corneal confocal microscopy in diagnosing peripheral neuropathy, no study specifically investigated the diagnostic accuracy of this technique in patients with NeP. Functional neuroimaging and peripheral nerve blocks are helpful in disclosing pathophysiology and/or predicting outcomes, but current literature does not support their use for diagnosing NeP. Genetic testing may be considered at specialist centres, in selected cases. CONCLUSIONS: These recommendations provide evidence-based clinical practice guidelines for NeP diagnosis. Due to the poor-to-moderate quality of evidence identified by this review, future large-scale, well-designed, multicentre studies assessing the accuracy of diagnostic tests for NeP are needed.

Abnormal activation of brain regions in idiopathic trigeminal neuralgia patients by fMRI: An activation likelihood estimation meta-analysis.

BACKGROUND: Idiopathic trigeminal neuralgia (ITN) is one of the most common types of neuropathic pain, severely affecting the physiological and psychological wellbeing of patients. Recently, fMRI has been used to examine abnormal activation of brain regions in patients with ITN. However, sample sizes have been small in these few studies, and the abnormally activated brain regions remain unclear. Therefore, in the present study, we retrieved and analyzed literature on the brain areas with abnormal or reduced activation in ITN patients, with the aim of providing insight into the neuropathological basis of the disease and to provide new targets for treatment. METHODS: We retrieved resting state fMRI studies on trigeminal neuralgia patients from PubMed, the Web of Science and Scopus databases until November 2022, and we extracted the coordinates of the sites with increased or decreased activation. We used activation likelihood estimation (ALE) meta-analysis to identify regions of abnormal activation in ITN patients. RESULTS: ALE meta-analysis revealed that the left caudate nucleus and right anterior ventral nucleus of the thalamus are abnormally hyperactivated in ITN patients. Moreover, ITN patients showed reduced activation in the left precuneus, middle temporal gyrus, lingual gyrus, and medial frontal gyrus. CONCLUSION: ALE meta-analysis identified several brain regions with abnormally high or decreased activation in ITN patients. Sites with altered activation may be potential targets for non-invasive brain stimulation as adjunct therapy for ITN.

Glossopharyngeal neuralgia as initial symptom in combined hyperactive dysfunction syndrome: case report.

OBJECTIVES: Hyperactive dysfunction syndrome (HDS) is defined as symptoms arising from overactivities in cranial nerves, like trigeminal neuralgia (TN), hemifacial spasm (HFS), and glossopharyngeal neuralgia (GPN). A combination of these cranial nerve neuralgias, that might or might not occur in one or both sides, either synchronously, or metachronously is called combined hyperactive dysfunction syndrome (CHDS). CASE PRESENTATION: We presented a 73 years-old male patient with CHDS presenting with GPN as the initial symptom, with total relief from GPN, TN, and HFS after microvascular decompression. Up to date, only nine patients have been reported in the literature with symptomatic. CONCLUSIONS: TN-HFS-GPN. Our case is the first case with GPN as the initial symptom. The combination of arterial and venous origin of the offending vessels makes the case picturesage.

Posttraumatic Trigeminal Neuropathic Pain in Association with Dental Implant Surgery.

Posttraumatic trigeminal neuropathy in association with dental implant surgery is preventable, and this should be the emphasis for all clinicians considering this treatment for a patient. Once the nerve injury and posttraumatic neuropathy with or without pain ensues, there is very little the clinician can do to reverse it and the high pain and permanency of the neuropathy will have a significant functional and psychological impact on the patient. Immediate implant removal is required, and home check should be routine for all cases. International diagnostic criteria are available and should be implemented in everyday practice.

Trigeminal Neuralgia.

Trigeminal neuralgia (TN) is a rare neuropathic pain disorder characterized by recurrent, paroxysmal episodes of short-lasting severe electric shock-like pain along the sensory distribution of the trigeminal nerve. Recent classification systems group TN into 3 main categories depending on the underlying pathophysiology. This article will present a case history and review the epidemiology, diagnostic criteria, classification, clinical features, diagnostic investigations, pathophysiology, and management of TN.

Recent Advances and Updates in Trigeminal Autonomic Cephalalgias.

Trigeminal autonomic cephalalgias (TACs) are discrete primary headache disorders, characterized by severe unilateral head pain, typically trigeminal distribution, with ipsilateral cranial autonomic symptoms. The conditions within this group are hemicrania continua, cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing and short-lasting unilateral neuralgiform headache with autonomic symptoms. Several advances have been made in understanding the pathogenesis and evolving treatment options in TACs. This review will outline the advances and updates in each TAC.

Imaging of Cranial Neuralgias.

Cranial neuralgia (CN) can cause significant debilitating pain within a nerve dermatome. Accurate diagnosis requires detailed clinical history and examination, understanding of pathophysiology and appropriate neuroimaging to develop an optimal treatment plan. The objective of this article is to review and discuss some of the more common CNs including trigeminal neuralgia and its associated painful neuropathies, occipital neuralgia, and less common glossopharyngeal neuralgia (GPN). The neuroanatomy, pathophysiology, diagnostic imaging, and treatment of each of these pathologies are reviewed with emphasis on the role of CT and MR imaging findings in guiding diagnosis. Although CT is often used to initially identify an underlying cause such as neoplasm, infection, or vascular malformation, MRI is optimal. Clinical history and examination findings along with MRI constructive interference steady state/fast imaging employing steady-state acquisition sequences and MRA of the brain can be used to distinguish between primary and secondary cranial neuropathies and to discern the best treatment option. Pharmacologic and noninvasive therapy is the first-line of treatment of these cranial and cervical neuralgias. If symptoms persist, stereotactic radiosurgery is an option for some patients, although microvascular decompression surgery is the most curative option for both trigeminal and GPN. Refractory occipital neuralgia can be treated with a nerve block, an ablative procedure such as neurectomy or ganglionectomy, or more recently occipital nerve stimulation.

Comorbidity between hypothyroidism and headache disorders in a Mexican population. / Comorbilidad entre hipotiroidismo y cefalea en la población mexicana.

INTRODUCTION: Headache and hypothyroidism are common comorbidities. This is a cross-sectional study of the prevalence of hypothyroidism in headache patients in the largest Mexican headache registry. PATIENTS AND METHODS: PREMECEF is an e-database for patients with headaches. Data was recollected from July 2017-April 2019 in three centers of Monterrey, Mexico. RESULTS: Of 869 patients, 35 (4%) had hypothyroidism. Four had two different headache diagnoses; of the 39 individual diagnoses, 23 were primary, 1 secondary, 13 cranial neuralgias, and 2 unspecified headaches. Hypothyroidism prevalence: 8.3% in unspecified, 6.5% in cranial neuralgias, 3.4% in primary, and 1.9% in secondary headaches; in tension-type headache (TTH) was 3.9%, in migraines 3.2%, in trigeminal neuralgia 6.1%, and in occipital neuralgia 6.3%. CONCLUSION: This is the first report on the prevalence of hypothyroidism in occipital and trigeminal neuralgia. The prevalence of hypothyroidism in migraine and TTH is higher than the general population.

TITLE: Comorbilidad entre hipotiroidismo y cefalea en la población mexicana.Introducción. Cefalea e hipotiroidismo son comorbilidades comunes. Éste es un estudio transversal de la prevalencia del hipotiroidismo en pacientes con cefalea en el registro de cefalea más grande de México. Pacientes y métodos. PREMECEF es una base de datos electrónica para pacientes con cefalea. La información se recolectó de julio de 2017 a abril de 2019 en tres centros médicos de Monterrey, México. Resultados. De 869 pacientes, 35 (4%) tenían hipotiroidismo y cuatro tenían diagnósticos de dos diferentes cefaleas. De los 39 diagnósticos individuales, 23 fueron primarias; una, secundaria; 13, neuralgias craneales; y dos, cefaleas no especificadas. La prevalencia de hipotiroidismo fue del 8,3% en las no especificadas, del 6,5% en las neuralgias craneales, del 3,4% en las cefaleas primarias y del 1,9% en las secundarias. En la cefalea de tipo tensional fueron del 3,9%; en las migrañas, del 3,2%; en la neuralgia trigeminal, del 6,1%; y en la neuralgia occipital, del 6,3%. Conclusión. Éste es el primer informe de la prevalencia de hipotiroidismo en la neuralgia occipital y la trigeminal. La prevalencia de hipotiroidismo en la migraña y la cefalea de tipo tensional es mayor que en la población general.

Neural activity in trigeminal neuralgia patients with sensory and motor stimulations: A pilot functional MRI study.

OBJECTIVE: Trigeminal neuralgia (TN) is a neuropathic pain syndrome that typically exhibits paroxysmal pain. However, the true mechanism of pain processing is unclear. We aim to evaluate the neural activity changes, before and after radiofrequency rhizotomy, in TN patients using functional MRI (fMRI) with sensory and motor stimulations. METHODS: Six patients with classical TN participated in the study. Each patient underwent two boxcar paradigms of fMRI tasks: air-sensation and jaw-clenching around 1-3 weeks before and after the surgical intervention. McGill Pain Questionnaire (MPQ) was used to evaluate the pain intensity prior to fMRI study. RESULTS: Before rhizotomy, the jaw-clenching stimulation yielded reduced brain activation in primary motor (M1) and primary (SI) and secondary somatosensory (SII) cortices. Following intervention, activation in those regions returned to near normal levels observed in healthy subjects. For air-sensation stimulation, several pain and pain modulation regions such as right thalamus, right putamen, insula, and brainstem, were activated before the intervention, but subsided after the intervention. This correlated well with the change of MPQ scores (p < 0.01). CONCLUSIONS: In our study, we observed significant pain reduction accompanied by increased motor activities after rhizotomy in patients with TN. We hypothesize that the reduced motor activities identified in fMRI may be reversed after the treatment with radiofrequency rhizotomy. More research is warranted.

Neuralgia and Atypical Facial, Ear, and Head Pain.

Though there have been considerable strides in the diagnosis and care of orofacial pain disorders, facial neuralgias, and myofascial pain dysfunction syndrome remain incredibly cumbersome for patients and difficult to manage for providers. Cranial neuralgias, myofascial pain syndromes, temporomandibular dysfunction (TMD), dental pain, tumors, neurovascular pain, and psychiatric diseases can all present with similar symptoms. As a result, a patient's quest for the treatment of their orofacial pain often begins on the wrong foot, with a misdiagnosis or unnecessary procedure, which makes it all the more frustrating for them. Understanding the natural history, clinical presentation, and management of facial neuralgias and myofascial pain dysfunction syndrome can help clinicians better recognize and treat these conditions. In this article, we review updated knowledge on the pathophysiology, incidence, clinical features, diagnostic criteria, and medical management of TN, GPN, GN, and MPDS.