Cigarette smoking history (personal and secondary childhood exposure) in non-cluster headache trigeminal autonomic cephalalgias: A clinic based study.

OBJECTIVE: To look at cigarette smoking history (personal and secondary exposure as a child) in non-cluster headache trigeminal autonomic cephalalgias seen at a headache clinic and to determine smoking exposure prevalence utilizing previously published data. METHODS: Retrospective chart review and PubMed/Google Scholar search. RESULTS: Forty-eight clinic patients met ICHD-3 criteria for non-cluster headache trigeminal autonomic cephalalgias. Four had paroxysmal hemicrania, 75% were smokers and secondary exposure was noted in all. 16 patients had short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or short lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA), 12.5% were smokers and secondary exposure was noted in 91%. Twenty-eight patients had hemicrania continua, 21% were smokers and secondary exposure was found in 62.5%.Since 1974 there have been 88 paroxysmal hemicrania, 50 SUNCT or SUNA and 89 hemicrania continua patients with a documented smoking exposure history. From current data and previous studies, a smoking history was noted in 60% paroxysmal hemicrania, 18% SUNCT and SUNA and 21% hemicrania continua patients. CONCLUSION: A cigarette smoking history appears to be connected to paroxysmal hemicrania (personal and secondary exposure) and possibly to SUNCT/SUNA (secondary) and hemicrania continua (secondary).

Efficacy of Occipital Nerve Stimulation in Trigeminal Autonomic Cephalalgias: A Systematic Review.

BACKGROUND: Trigeminal autonomic cephalalgias (TACs) are a group of highly disabling primary headache disorders. Although pharmacological treatments exist, they are not always effective or well tolerated. Occipital nerve stimulation (ONS) is a potentially effective surgical treatment. OBJECTIVE: To perform a systematic review of the efficacy of ONS in treating TACs. METHODS: A systematic review was performed using Medline, Embase, and Cochrane databases. Primary outcomes were reduction in headache intensity, duration, and frequency. Secondary outcomes included adverse event rate and reduction in medication use. Because of large differences in outcome measures, data for patients suffering from short-lasting, unilateral, and neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and cranial autonomic symptoms (SUNA) were reported separately. Risk of bias was assessed using the NIH Quality Assessment Tools. RESULTS: A total of 417 patients from 14 published papers were included in the analysis, of which 15 patients were in the SUNCT/SUNA cohort. The mean reduction in headache intensity and duration was 26.2% and 31.4%, respectively. There was a mean reduction in headache frequency of 50%, as well as a 61.2% reduction in the use of abortive medications and a 31.1% reduction in the use of prophylactic medications. In the SUNCT/SUNA cohort, the mean decrease in headache intensity and duration was 56.8% and 42.8%. The overall responder rate, defined as a >50% reduction in attack frequency, was 60.8% for the non-SUNCT/non-SUNA cohort and 66.7% for the SUNCT/SUNA cohort. Adverse events requiring repeat surgery were reported in 33% of cases. Risk of bias assessment suggests that articles included in this review had reasonable internal validity. CONCLUSION: ONS may be an effective surgical treatment for approximately two thirds of patients with medically refractory TACs.

Status of diagnosis and preventative treatment for primary headache disorders: real-world data of unmet needs in China.

BACKGROUND: Headache disorders are widely prevalent and pose a considerable economic burden on individuals and society. Globally, misdiagnosis and inadequate treatment of primary headache disorders remain significant challenges, impeding the effective management of such conditions. Despite advancements in headache management over the last decade, a need for comprehensive evaluations of the status of primary headache disorders in China regarding diagnosis and preventative treatments persists. METHODS: In the present study, we analyzed the established queries in the Survey of Fibromyalgia Comorbidity with Headache (SEARCH), focusing on previous diagnoses and preventative treatment regimens for primary headache disorders. This cross-sectional study encompassed adults diagnosed with primary headache disorders who sought treatment at 23 hospitals across China between September 2020 to May 2021. RESULTS: The study comprised 2,868 participants who were systematically examined. Migraine and tension-type headaches (TTH) constituted a majority of the primary headache disorders, accounting for 74.1% (2,124/2,868) and 23.3% (668/2,868) of the participants, respectively. Medication overuse headache (MOH) affected 8.1% (231/2,868) of individuals with primary headache disorders. Over half of the individuals with primary headache disorders (56.6%, 1,624/2,868) remained undiagnosed. The previously correct diagnosis rates for migraine, TTH, TACs, and MOH were 27.3% (580/2,124), 8.1% (54/668), 23.2% (13/56), and 3.5% (8/231), respectively. The misdiagnosis of "Nervous headache" was found to be the most prevalent among individuals with migraine (9.9%, 211/2,124), TTH (10.0%, 67/668), trigeminal autonomic cephalalgias (TACs) (17.9%, 10/56), and other primary headache disorders (10.0%, 2/20) respectively. Only a minor proportion of individuals with migraine (16.5%, 77/468) and TTH (4.7%, 2/43) had received preventive medication before participating in the study. CONCLUSIONS: While there has been progress made in the rate of correct diagnosis of primary headache disorders in China compared to a decade ago, the prevalence of misdiagnosis and inadequate treatment of primary headaches remains a veritable issue. As such, focused efforts are essential to augment the diagnosis and preventive treatment measures related to primary headache disorders in the future.

Diagnosis and management of traumatically induced hemicrania continua and neuropathic pain secondary to facial gun shot wound.

Near fatal gunshot wound to the face results in lifesaving surgery and restorative procedures. Chronic pain followed. This is the probable first case report of posttraumatic hemicrania continua and its successful management.

Tolosa-Hunt syndrome presenting with features of a trigeminal autonomic cephalalgias and pituitary enlargement.

Tolosa-Hunt syndrome is understood as a steroid-responsive, relapsing-remitting, unilateral headache disorder associated with ipsilateral cranial neuropathies, of a probable granulomatous aetiology. The diagnosis is made clinically from the history and examination, supported by appropriate imaging. Here the authors report a case of Tolosa-Hunt syndrome with a headache phenotype mimicking a trigeminal autonomic cephalalgias (hemicrania continua), and serial MRI studies showing a stable enlarged pituitary. Due to her initial lack of clinical signs, she was diagnosed with chronic migraine, revised to hemicrania continua based on indomethacin response, then revised back to chronic migraine. Her final diagnosis was achieved after she developed a left cavernous sinus syndrome 4 years into her disease course. This case shows that Tolosa-Hunt syndrome may present with a non-side-locked headache and delayed development of clinical signs. Clinicians should also maintain a high degree of suspicion when faced with incidental MRI findings.

Hemicrania continua secondary to neurogenic paravertebral tumor- a case report.

OBJECTIVES: Hemicrania continua (HC) is one of the trigeminal autonomic cephalalgias (TAC), where sympathetic dysfunction and autonomic dysfunction resulting in parasympathetic over activation with some evidence of sympathetic inhibition have been suggested as probable causes. However, cases of hemicrania continua secondary to sympathetic dysfunction due to neurogenic paravertebral tumor impinging on the sympathetic chain has not been previously reported. In this case, the probability of the sympathetic dysfunction was more likely based on the clinical features and management. CASE PRESENTATION: A 23-year-old female presented with a chief complaint of right unilateral pain in the retro-bulbar, head and facial region for the past three years. An initial MRI of the brain was negative, whereas an MRI of the spine was advised to rule out a cervicogenic origin of the pain. The MRI revealed a well-defined mass lesion within right paravertebral region at T3 indicative of a neurogenic tumor. The patient was diagnosed with probable hemicrania continua secondary to neurogenic tumor impinging on adjacent sympathetic chain. A trial of indomethacin 75 mg/day was advised, which provided complete relief of the headache. The patient was referred to a neurologist for management of the neurogenic tumor. CONCLUSIONS: Headache disorders may be secondary to pathologies and comprehensive evaluation and accurate diagnosis are essential. Knowledge of neuroanatomy is paramount to understand and explain underlying pathophysiological mechanisms. Multidisciplinary management is essential in complex orofacial cases.

SUNCT and SUNA: An Update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are rare and disabling primary headache disorders that are subtypes of Short-lasting unilateral neuralgiform headache attacks (SUNHA). AIM: The aim of this narrative review was to provide a comprehensive update on headache phenotype, pathophysiology, and various treatment options available for SUNCT and SUNA. METHODS: References for this review were identified by searches of articles published in the English language in PubMed between 1978 and October 2020 using "short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)", "short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA)", "short-lasting unilateral neuralgiform headache attacks (SUNHA)", "trigeminal autonomic cephalalgias" as keywords in various combinations. RESULTS: Of a potential 1103 articles, seven case series describing clinical characteristics of SUNCT/SUNA patients were identified for this review. For symptomatic/secondary SUNCT/SUNA, 53 individual case reports, and one case series were reviewed. One placebo-controlled trial and 11 open-label case series that evaluated various medical and surgical treatments in SUNCT/SUNA were also reviewed. Available literature suggests that SUNCT and SUNA are subtypes of the same disorder characterized by severe side locked short duration headache with ipsilateral prominent cranial autonomic symptoms and signs. Pathophysiology may involve both peripheral and central mechanisms. Lamotrigine is the most effective preventive therapy while intravenous lidocaine is the most efficacious drug as transitional therapy for severe disabling attacks. Surgical options including microvascular decompression in those having neurovascular conflict, occipital nerve stimulation, and hypothalamic deep brain stimulation can be alternative treatment options for medically refractory patients.

Is pituitary MRI screening necessary in cluster headache?

OBJECTIVE: To determine the prevalence and clinical predictors of pituitary adenomas in cluster headache patients, in order to determine the necessity of performing dedicated pituitary magnetic resonance imaging in patients with cluster headache. METHODS: A retrospective study was conducted of all consecutive patients diagnosed with cluster headache and with available brain magnetic resonance imaging between 2007 and 2017 in a tertiary headache center. Data including demographics, attack characteristics, response to treatments, results of neuroimaging, and routine pituitary function tests were recorded. RESULTS: Seven hundred and eighteen cluster headache patients attended the headache clinic; 643 underwent a standard magnetic resonance imaging scan, of whom 376 also underwent dedicated pituitary magnetic resonance imaging. Pituitary adenomas occurred in 17 of 376 patients (4.52%). Non-functioning microadenomas (n = 14) were the most common abnormality reported. Two patients, one of whom lacked the symptoms of pituitary disease, required treatment for their pituitary lesion. No clinical predictors of those adenomas were identified after multivariate analysis using random forests. Systematic pituitary magnetic resonance imaging scanning did not benefit even a single patient in the entire cohort. CONCLUSION: The prevalence of pituitary adenomas in cluster headache is similar to that reported in the general population, thereby precluding an over-representation of pituitary lesions in cluster headache. We conclude that the diagnostic assessment of cluster headache patients should not include specific pituitary screening. Only patients with standard brain magnetic resonance imaging findings or symptoms suggestive of a pituitary disorder require brain magnetic resonance imaging with dedicated pituitary views.

[Clinical Aspects of Short-Lasting Unilateral Neuralgiform Headache Attacks].

Short-lasting unilateral neuralgiform headache attacks (SUNHA), within the group of trigeminal autonomic cephalalgias, are characterized by repetitive, brief and severe attacks. The SUNHA includes short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). SUNCT is diagnosed if both conjunctival injection and tearing occur ipsilateral to the pain, whereas SUNA is diagnosed if only one, or neither, of conjunctival injection and tearing occurs. Previously considered to be rare and rather refractory to treatment, there is increasing awareness of this syndrome and the therapeutic possibilities. During the worst periods, intravenous lidocaine may decrease the intensity of SUNHA. Lamotrigine is the most-effective prophylactic treatment. For medically intractable SUNHA, several surgical approaches, i.e., microvascular decompression of the trigeminal nerve and neuromodulation, have been tried. There is considerable clinical, therapeutic and radiological overlap between SUNCT, SUNA, and trigeminal neuralgia. Despite being considered distinct conditions, the emerging evidence suggests a broader nosological concept of SUNCT, SUNA, and trigeminal neuralgia; these conditions may constitute a continuum of the same disorder, rather than separate clinical entities. This article discusses the clinical aspects of the syndrome, including pathophysiology, differential diagnosis, radiological imaging and treatment. (Received May 26, 2020; Accepted June 16, 2020; Published November 1, 2020).

LASH syndrome: New cases with a broadening clinical phenotype.

OBJECTIVE: To describe three new cases of the headache syndrome of long-lasting autonomic symptoms with hemicrania (LASH), and to establish a clinical phenotype utilizing all LASH cases noted in the medical literature. METHODS: A case series of patients was evaluated in an academic headache clinic over a two-year time period. LASH syndrome was defined by episodic headache attacks with associated cranial autonomic symptoms that start before headache onset, last the entire duration of the headache and continue on for a period of time after the headache ceases. RESULTS: Three patients were noted to have LASH syndrome in a two-year time period (2017-2018). One patient was diagnosed with primary LASH, while two others had probable secondary LASH from a secretory pituitary tumor. The primary LASH patient was female. She had on average one headache per week lasting 1-3 days in duration. She experienced migrainous associated symptoms along with their cranial autonomic symptoms. She also developed a fixed Horner's syndrome along with a typical headache attack, which was present for 6 months at the time of consultation. She had complete headache relief with indomethacin and her miosis and ptosis also resolved with treatment. Secondary LASH was diagnosed in two patients (one male, one female) both with prolactin secreting pituitary microadenomas. One of the patients had his headaches abolish with dopamine agonist therapy while the other patient did not respond to hormonal modulation but became pain free on indomethacin. Secondary LASH patients had less frequent headache episodes and lacked any migrainous associated features, but exhibited agitation with headache. CONCLUSION: LASH syndrome may be rare, but more reported cases are entering the headache literature. The temporal profile of onset and offset of cranial autonomic symptoms is key to making the diagnosis. Primary and secondary LASH may present differently based on gender predominance, the presence of migrainous associated features, and attack frequency. Secondary LASH appears to be indomethacin responsive, suggesting that medication effectiveness should not obviate the need to do testing for secondary etiologies.

The neurosurgical treatment of craniofacial pain syndromes: current surgical indications and techniques.

Craniofacial pain syndromes are comprised of multiple pathological entities resulting in pain referred to the scalp, face, or deeper cranial structures. In a small subset of patients affected by those syndromes, pharmacological and physical therapies fail in alleviating pain. In some of those refractory patients surgical procedures aimed at relieving pain are indicated and have been adopted with variable results and safety profiles. In this review, the authors describe craniofacial pain syndromes that most commonly fail to respond to pharmacological therapies and may be amenable to tailored surgical procedures. In particular, trigeminal, glossopharyngeal, and occipital neuralgias are considered, as well as some primary headache syndromes such as cluster headache, short unilateral neuralgiform headache with conjunctival injection and tearing/short unilateral neuralgiform headache with autonomic symptoms, and migraine. Surgical techniques, including the implantation of deep brain or peripheral nerve electrodes with subsequent chronic stimulation, microvascular decompression of neurovascular conflicts, and percutaneous lesioning of neural structures are described. Finally, surgical indications, outcomes, and safety of these procedures are presented.

Hints on Diagnosing and Treating Headache.

BACKGROUND: Headache, like dizziness, is one of the more common presenting complaints in outpatient care and in the emergency room. More than 200 varieties of headache have been described, and the false impression may arise that the diagnosis and treatment of these syndromes is a highly challenging task. METHODS: This review is based on pertinent articles retrieved by a selective search in PubMed. RESULTS: In primary headache, the headache is not a symptom but a disease in its own right. There are four types of primary headache: migraine, tension headache, trigeminal autonomic cephalalgia, and other primary headache disorders. By definition, the physical examination is normal, including the neurological examination. Secondary headache, in contrast, is a symptom of another disease (e.g., a tumor or cerebral hemorrhage). Triptans and nonsteroidal anti-inflammatory drugs (NSAID) are the drugs usually given for the acute treatment and prophylaxis of migraine. In tension headache, NSAID are given acutely, and tricyclic drugs for prophylaxis. There are various options for the treatment of trigeminal autonomic cephalalgia syndromes such as cluster headache and paroxysmal hemicrania. For group 4 headaches (other primary headache disorders), the treatment must be chosen on an individual basis; indomethacin is often effective. CONCLUSION: If the patient is clearly suffering from none of the four types of primary headache, the problem must be a headache of a secondary nature, potentially reflecting a dangerous underlying disease. The treatment of headache is usually successful and thus highly rewarding for physicians of all medical specialties.

Therapeutic Approaches for the Management of Trigeminal Autonomic Cephalalgias.

Trigeminal autonomic cephalalgia (TAC) encompasses 4 unique primary headache types: cluster headache, paroxysmal hemicrania, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms. They are grouped on the basis of their shared clinical features of unilateral headache of varying durations and ipsilateral cranial autonomic symptoms. The shared clinical features reflect the underlying activation of the trigeminal-autonomic reflex. The treatment for TACs has been limited and not specific to the underlying pathogenesis. There is a proportion of patients who are refractory or intolerant to the current standard medical treatment. From instrumental bench work research and neuroimaging studies, there are new therapeutic targets identified in TACs. Treatment has become more targeted and aimed towards the pathogenesis of the conditions. The therapeutic targets range from the macroscopic and structural level down to the molecular and receptor level. The structural targets for surgical and noninvasive neuromodulation include central neuromodulation targets: posterior hypothalamus and, high cervical nerves, and peripheral neuromodulation targets: occipital nerves, sphenopalatine ganglion, and vagus nerve. In this review, we will also discuss the neuropeptide and molecular targets, in particular, calcitonin gene-related peptide, somatostatin, transient receptor potential vanilloid-1 receptor, nitric oxide, melatonin, orexin, pituitary adenylate cyclase-activating polypeptide, and glutamate.

Cefaleia Trigêmino-autonômica e Neuromielite Óptica / Trigemino-autonomic Headache and Neuromyelitis Optica

As cefaleias trigêmino-autonômicas compartilham os aspectos clínicos da cefaleia, além de proeminentes sintomas disautonômicos crânio faciais. A Neuromielite Óptica (NMO) ou Doença de Devic é uma doença inflamatória grave, desmielinizante e auto-imune do sistema nervoso central que acomete, preferencialmente, os nervos ópticos e a medula espinhal, causando neurite óptica aguda, uni ou bilateral, e mielite transversa. O objetivo desse trabalho é relatar um caso clínico de NMO, cuja manifestação inicial é atípica. Uma revisão de literatura com as palavras-chaves Neuromielite Óptica e Cefaleia Trigêminoautonômica foi realizada no PubMed e foram selecionados os artigos e relatos de casos mais relevantes sobre o assunto. Conclui-se que estas duas doenças podem ter em comum uma alteração hipotalâmica e uma doença desmielinizante grave pode se iniciar com uma cefaleia trigêmino-autonômica. (AU)

The Trigeminal-autonomic headaches share the clinical features of headache, as well as prominent facial skull disautonomic symptoms. The Neuromyelitis Optica (NMO) or Devic's disease is a severe inflammatory disease, demyelinating and autoimmune of the central nervous system that affects mainly the optic nerves and spinal cord, causing acute optic neuritis, unilateral or bilateral, and transverse myelitis. The objective of this study is to report a case of NMO, whose initial manifestation is atypical. A literature review with keywords Neuromyelitis Optica and Trigeminal-autonomic Headache has conducted in PubMed and we have selected the most relevant articles and case reports on the subject. In conclusion, these two diseases may have a common hypothalamic disturbance and a severe demyelinating disease can start with a trigeminal-autonomic headache. (AU)

Trigeminal autonomic cephalalgias: beyond the conventional treatments.

The trigeminal autonomic cephalalgias include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua. While the majority responds to conventional pharmacological treatments, a small but significant proportion of patients are intractable to these treatments. In these cases, alternative choices for these patients include oral and injectable drugs, lesional or resectional surgery, and neurostimulation. The evidence base for conventional treatments is limited, and the evidence for those used beyond convention is more so. At present, the most evidence exists for nerve blocks, deep brain stimulation, occipital nerve stimulation, sphenopalatine ganglion stimulation in chronic cluster headache, and microvascular decompression of the trigeminal nerve in short-lasting unilateral neuralgiform headache attacks.

SUNCT/SUNA and neurovascular compression: new cases and critical literature review.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA) are primary headache syndromes. A growing body of literature has focused on brain magnetic resonance imaging (MRI) evidence of neurovascular compression in these syndromes. OBJECTIVE: The objective of this article is to assess whether SUNCT is a subset of SUNA or whether the two are separate syndromes and clarify the role of neurovascular compression. METHOD: We describe three new SUNCT cases with MRI findings of neurovascular compression and critically review published SUNCT/SUNA cases. RESULTS: We identified 222 published SUNCT/SUNA cases. Our three patients with neurovascular compression added to the 34 cases previously described (16.9%). SUNCT and SUNA share the same clinical features and therapeutic options. At present, there is no available abortive treatment for attacks. Lamotrigine was effective in 64% of patients; topiramate and gabapentin in about one-third of cases. Of the 34 cases with neurovascular compression, seven responded to drug therapies, 16 patients underwent microvascular decompression of the trigeminal nerve (MVD) with effectiveness in 75%. CONCLUSIONS: We suggest that SUNCT and SUNA should be considered clinical phenotypes of the same syndrome. Brain MRI should always be performed with a dedicated view to exclude neurovascular compression. The high percentage of remission after MVD supports the pathogenetic role of neurovascular compression.

Diagnostic and therapeutic errors in trigeminal autonomic cephalalgias and hemicrania continua: a systematic review.

Trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) are relatively rare but clinically rather well-defined primary headaches. Despite the existence of clear-cut diagnostic criteria (The International Classification of Headache Disorders, 2nd edition - ICHD-II) and several therapeutic guidelines, errors in workup and treatment of these conditions are frequent in clinical practice. We set out to review all available published data on mismanagement of TACs and HC patients in order to understand and avoid its causes. The search strategy identified 22 published studies. The most frequent errors described in the management of patients with TACs and HC are: referral to wrong type of specialist, diagnostic delay, misdiagnosis, and the use of treatments without overt indication. Migraine with and without aura, trigeminal neuralgia, sinus infection, dental pain and temporomandibular dysfunction are the disorders most frequently overdiagnosed. Even when the clinical picture is clear-cut, TACs and HC are frequently not recognized and/or mistaken for other disorders, not only by general physicians, dentists and ENT surgeons, but also by neurologists and headache specialists. This seems to be due to limited knowledge of the specific characteristics and variants of these disorders, and it results in the unnecessary prescription of ineffective and sometimes invasive treatments which may have negative consequences for patients. Greater knowledge of and education about these disorders, among both primary care physicians and headache specialists, might contribute to improving the quality of life of TACs and HC patients.

Sistema trigémino vascular y cefalea / Trigeminovascular System and Primary Headache

La fisiopatología de las cefaleas primarias es compleja e incluye un sinnúmerode interacciones que regulan el proceso nociceptivo. Dentro de los principalesresponsables de generar el dolor se encuentra el sistema trigémino vascular, que esun conjunto de estructuras que integran vías tanto centrales corticosubcorticales comoperiféricas, que desempeñan un papel activo no solo en la génesis del dolor, sino enlas manifestaciones autonómicas y visuales que acompañan la cefalea. Así mismo, estesistema es el responsable de los mecanismos de sensibilización central característicosdel dolor. En el artículo se desarrollan brevemente las principales estructuras queparticipan en la génesis de las cefaleas primarias y sus interacciones en las diferentespartes del sistema nervioso...

The pathophysiology of primary headache iscomplex and it includes several interactionsthat regulate the nociceptive process. The trigeminal-vascular system is perhaps one of theprincipal structures that generate pain due tothe integration of several pathways both centraland peripheral. In addition to this, the trigeminalvascular system also plays a central role inthe autonomic and visual symptoms that affectindividuals with headache and in the centralsensitization process. In this article we brieflydiscuss the main structures that participate in thepathophysiology of primary headaches and theirinteractions in the different levels of the centralnervous system...

The treatment of medically intractable trigeminal autonomic cephalalgia with supraorbital/supratrochlear stimulation: a retrospective case series.

INTRODUCTION: This is a retrospective case series of five patients with intractable trigeminal autonomic cephalalgia (TAC) who were implanted with a supraorbital/supratrochlear neuromodulation system. OBJECTIVES: The aim of this Institutional Review Board-approved study was to investigate the percentage of pain relief, treatment response, pain level, work status, medication intake, implantation technique, lead placement, programming information, and device use. RESULTS: Trial stimulation led to implantation of all five patients. All patients reported improvement in their functional status in regard to activities of daily living. The device was revised in two patients due to skin erosion. It was later reimplanted in both patients due to worsening of symptoms, again with good pain relief. The device was explanted in two other patients because of the need to perform a magnetic resonance imaging or implant an automatic implantable cardioverter defibrillator. The follow-up of the patients ranged between 18 months and 36 months, with a mean of 25.2 months. There was no change in work status. Following the implant, the Visual Analog Scale score was reduced to a mean of 1.6 from an initial mean score of 8.9. Three patients were completely weaned off opioid medications, while two patients continued to take opioid at a lower dosage. All patients experienced a decrease of the adjuvant neuropathic drugs. CONCLUSION: Supraorbital/supratrochlear nerve stimulation appears to be a promising modality for the treatment of patients with intractable TAC.