Headaches Attributed to Disorders of Homeostasis.

Headaches attributed to disorders of homeostasis include those different headache types associated with metabolic and systemic diseases. These are headache disorders occurring in temporal relation to a disorder of homeostasis including hypoxia, high altitude, airplane travel, diving, sleep apnea, dialysis, autonomic dysreflexia, hypothyroidism, fasting, cardiac cephalalgia, hypertension and other hypertensive disorders like pheochromocytoma, hypertensive crisis, and encephalopathy, as well as preeclampsia or eclampsia. The proposed mechanism behind the causation of these headache subtypes including diagnostic criteria, evaluation, treatment, and overall management will be discussed.

The Toronto Concussion Study: a prospective investigation of characteristics in a cohort of adults from the general population seeking care following acute concussion, 2016-2020.

Purpose: There is limited research regarding the characteristics of those from the general population who seek care following acute concussion. Methods: To address this gap, a large cohort of 473 adults diagnosed with an acute concussion (female participants = 287; male participants = 186) was followed using objective measures prospectively over 16 weeks beginning at a mean of 5.1 days post-injury. Results: Falls were the most common mechanism of injury (MOI) (n = 137, 29.0%), followed by sports-related recreation (n = 119, 25.2%). Male participants were more likely to be injured playing recreational sports or in a violence-related incident; female participants were more likely to be injured by falling. Post-traumatic amnesia (PTA) was reported by 80 participants (16.9 %), and loss of consciousness (LOC) was reported by 110 (23.3%). In total, 54 participants (11.4%) reported both PTA and LOC. Male participants had significantly higher rates of PTA and LOC after their injury compared to their female counterparts. Higher initial symptom burden was associated with a longer duration of recovery for both male and female participants. Female participants had more symptoms and higher severity of symptoms at presentation compared to male participants. Female participants were identified to have a longer recovery duration, with a mean survival time of 6.50 weeks compared to 5.45 weeks in male participants (p < 0.0001). A relatively high proportion of female and male participants in this study reported premorbid diagnoses of depression and anxiety compared to general population characteristics. Conclusion: Although premorbid diagnoses of depression and/or anxiety were associated with higher symptom burden at the initial visit, the duration of symptoms was not directly associated with a pre-injury history of psychological/psychiatric disturbance. This cohort of adults, from the general population, seeking care for their acute concussion attained clinical and functional recovery over a period of 4-12 weeks.

Revisiting the ICHD-3 criteria for headache attributed to mild traumatic injury to the head: Insights from the Toronto Concussion Study Analysis of Acute Headaches Following Concussion.

BACKGROUND: There is limited prospective data on the prevalence, timing of onset, and characteristics of acute headache following concussion/mild traumatic brain injury. METHODS: Adults diagnosed with concussion (arising from injuries not related to work or motor vehicle accidents) were recruited from emergency departments and seen within one week post injury wherein they completed questionnaires assessing demographic variables, pre-injury headache history, post-injury headache history, and the Sport Concussion Assessment Tool (SCAT-3) symptom checklist, the Sleep and Concussion Questionnaire (SCQ) and mood/anxiety on the Brief Symptom Inventory (BSI). RESULTS: A total of 302 participants (59% female) were enrolled (mean age 33.6 years) and almost all (92%) endorsed post-traumatic headache (PTH) with 94% endorsing headache onset within 24 hours of injury. Headache location was not correlated with site of injury. Most participants (84%) experienced daily headache. Headache quality was pressure/squeezing in 69% and throbbing/pulsing type in 22%. Associated symptoms included: photophobia (74%), phonophobia (72%) and nausea (55%). SCAT-3 symptom scores, Brief Symptom Inventory and Sleep and Concussion Questionnaire scores were significantly higher in those endorsing acute PTH. No significant differences were found in week 1 acute PTH by sex, history of migraine, pre-injury headache frequency, anxiety, or depression, nor presence/absence of post-traumatic amnesia and self-reported loss of consciousness. CONCLUSIONS: This study highlights the very high incidence of acute PTH following concussion, the timing of onset and characteristics of acute PTH, the associated psychological and sleep disturbances and notes that the current ICHD-3 criteria for headaches attributed to mild traumatic injury to the head are reasonable, the interval between injury and headache onset should not be extended beyond seven days and could, potentially, be shorted to allow for greater diagnostic precision.

A real-world, observational study of erenumab for migraine prevention in Canadian patients.

OBJECTIVES: To assess real-world effectiveness, safety, and usage of erenumab in Canadian patients with episodic and chronic migraine with prior ineffective prophylactic treatments. BACKGROUND: In randomized controlled trials, erenumab demonstrated efficacy for migraine prevention in patients with ≤4 prior ineffective prophylactic migraine therapies. The "Migraine prevention with AimoviG: Informative Canadian real-world study" (MAGIC) assessed real-world effectiveness of erenumab in Canadian patients with migraine. METHODS: MAGIC was a prospective open-label, observational study conducted in Canadian patients with chronic migraine (CM) and episodic migraine (EM) with two to six categories of prior ineffective prophylactic therapies. Participants were administered 70 mg or 140 mg erenumab monthly based on physician's assessment. Migraine attacks were self-assessed using an electronic diary and patient-reported outcome questionnaires. The primary outcome was the proportion of subjects achieving ≥50% reduction in monthly migraine days (MMD) after the 3-month treatment period. RESULTS: Among the 95 participants who mostly experienced two (54.7%) or three (32.6%) prior categories of ineffective prophylactic therapies and who initiated erenumab, treatment was generally safe and well tolerated; 89/95 (93.7%) participants initiated treatment with 140 mg erenumab. At week 12, 32/95 (33.7%) participants including 17/64 (26.6%) CM and 15/32 (48.4%) EM achieved ≥50% reduction in MMD while 30/86 (34.9%) participants including 19/55 (34.5%) CM and 11/31 (35.5%) EM achieved ≥50% reduction in MMD at week 24. Through patient-reported outcome questionnaires, 62/95 (65.3%) and 45/86 (52.3%) participants reported improvement of their condition at weeks 12 and 24, respectively. Physicians observed improvement in the condition of 78/95 (82.1%) and 67/86 (77.9%) participants at weeks 12 and 24, respectively. CONCLUSION: One-third of patients with EM and CM achieved ≥50% MMD reduction after 3 months of erenumab treatment. This study provides real-world evidence of erenumab effectiveness, safety, and usage for migraine prevention in adult Canadian patients with multiple prior ineffective prophylactic treatments.

Real-world persistence of erenumab for preventive treatment of chronic and episodic migraine: Retrospective real-world study.

OBJECTIVE: To describe the real-world treatment persistence (defined as the continuation of medication for the prescribed treatment duration), demographics and clinical characteristics, and treatment patterns for patients prescribed erenumab for migraine prevention in Canada. BACKGROUND: The effectiveness of prophylactic migraine treatments is often undermined by poor treatment persistence. In clinical trials, erenumab has demonstrated efficacy and tolerability as a preventive treatment, but less is known about the longer term treatment persistence with erenumab. METHODS: This is a real-world retrospective cohort study where a descriptive analysis of secondary patient data was conducted. Enrollment and prescription data were extracted from a patient support program for a cohort of patients prescribed erenumab in Canada between September 2018 and December 2019 and analyzed for persistence, baseline demographics, clinical characteristics, and treatment patterns. Descriptive analyses and unadjusted Kaplan-Meier (KM) curves were used to summarize the persistence and dose escalation/de-escalation at different timepoints. RESULTS: Data were analyzed for 14,282 patients. Median patient age was 47 years, 11,852 (83.0%) of patients were female, and 9443 (66.1%) had chronic migraine at treatment initiation. Based on KM methods, 71.0% of patients overall were persistent to erenumab 360 days after treatment initiation. Within 360 days of treatment initiation, it is estimated that 59.3% (KM-derived) of patients who initiated erenumab at 70 mg escalated to 140 mg, and 4.4% (KM-derived) of patients who initiated at 140 mg de-escalated to 70 mg. CONCLUSIONS: The majority of patients prescribed erenumab remained persistent for at least a year after treatment initiation, and most patients initiated or escalated to a 140 mg dose. These results suggest that erenumab is well tolerated, and its uptake as a new class of prophylactic treatment for migraine in real-world clinical practice is not likely to be undermined by poor persistence when coverage for erenumab is easily available.

Headache Hygiene in Pediatrics: Brushing Up on the Basics.

Headache hygiene refers to self-management behaviors and practices aimed at reducing headache-related disability and improving self-efficacy. Although self-management interventions have an established place in the management of a wide range of chronic conditions, there is still not a standardized approach to this in pediatric headache. In this article, we focus on headache hygiene approaches including education, lifestyle interventions, and psychologic interventions. We also present our center's resource compilation, made available to patients by quick response code technology, as an example of a structured approach to headache hygiene. Further work should explore a standardized approach to headache hygiene and strategies to support adherence, including the use of technology as an innovative health care delivery pathway.

The Non-Inferiority Margins in Migraine Research (NIMM) Survey.

OBJECTIVES: To survey experts in Headache Medicine on their opinions regarding appropriate non-inferiority margins for outcomes commonly used in migraine research. METHODS: Members of the American Headache Society and the Canadian Headache Society were invited to participate in the Non-Inferiority Margins in Migraine Research (NIMM) survey. Adult and child neurologists with expertise in Headache Medicine were eligible to participate. The survey had a multiple choice format and comprised questions on respondent characteristics, eligibility, as well as expert opinion on non-inferiority margins for outcomes commonly used in trials of both prophylactic and acute interventions for migraine. RESULTS: Ninety-nine eligible respondents completed the survey. Most respondents were adult neurologists (84.9%) and 74% reported practicing in the USA. The following were the most commonly selected non-inferiority margins: (1) change in monthly migraine attacks comparing baseline to the treatment period: 1 attack (39.4% selecting), (2) change in monthly migraine days comparing baseline to the treatment period: 1 day (44.4%), (3) change in average migraine intensity on a 4-point scale comparing baseline to the treatment period: 1.0 (31.3%), (4) percentage of participants who are pain-free 2 hours after the intervention: 5% (41.4%), (5) percentage of participants who have a migraine recurrence within 48 hours of treatment: 5% (42.4%), and (6) percentage of participants with sustained pain freedom: 5% (42.4%). CONCLUSIONS: The results of the NIMM survey describe the opinions of a group of experts on appropriate non-inferiority margins for outcomes commonly used in migraine clinical trials. There was significant variability in responses and lack of consensus on the choice of non-inferiority margins. The survey did not incorporate the patient perspective and was not validated prior to distribution. Further work in this area is required in order to explore how to incorporate clinical considerations into the selection of non-inferiority margins for migraine research.

Canadian Headache Society systematic review and recommendations on the treatment of migraine pain in emergency settings.

BACKGROUND: There is a considerable amount of practice variation in managing migraines in emergency settings, and evidence-based therapies are often not used first line. METHODS: A peer-reviewed search of databases (MEDLINE, Embase, CENTRAL) was carried out to identify randomized and quasi-randomized controlled trials of interventions for acute pain relief in adults presenting with migraine to emergency settings. Where possible, data were pooled into meta-analyses. RESULTS: Two independent reviewers screened 831 titles and abstracts for eligibility. Three independent reviewers subsequently evaluated 120 full text articles for inclusion, of which 44 were included. Individual studies were then assigned a US Preventive Services Task Force quality rating. The GRADE scheme was used to assign a level of evidence and recommendation strength for each intervention. INTERPRETATION: We strongly recommend the use of prochlorperazine based on a high level of evidence, lysine acetylsalicylic acid, metoclopramide and sumatriptan, based on a moderate level of evidence, and ketorolac, based on a low level of evidence. We weakly recommend the use of chlorpromazine based on a moderate level of evidence, and ergotamine, dihydroergotamine, lidocaine intranasal and meperidine, based on a low level of evidence. We found evidence to recommend strongly against the use of dexamethasone, based on a moderate level of evidence, and granisetron, haloperidol and trimethobenzamide based on a low level of evidence. Based on moderate-quality evidence, we recommend weakly against the use of acetaminophen and magnesium sulfate. Based on low-quality evidence, we recommend weakly against the use of diclofenac, droperidol, lidocaine intravenous, lysine clonixinate, morphine, propofol, sodium valproate and tramadol.

Metabolic headaches.

This article discusses headaches secondary to disorders of homeostasis, which include headaches attributed to (1) hypoxia and/or hypercapnia (high-altitude, diving, or sleep apnea), (2) dialysis, (3) arterial hypertension (pheochromocytoma, hypertensive crisis without hypertensive encephalopathy, hypertensive encephalopathy, preeclampsia or eclampsia, or autonomic dysreflexia), (4) hypothyroidism, (5) fasting, (6) cardiac cephalalgia, and (7) other disorder of homeostasis. Clinical features and diagnosis as well as therapeutic strategies are discussed for each headache type.

Canadian Headache Society Guideline: acute drug therapy for migraine headache.

OBJECTIVES: The primary objective of this guideline is to assist the practitioner in choosing an appropriate acute medication for an individual with migraine, based on current evidence in the medical literature and expert consensus. It is focused on patients with episodic migraine ( headache on ≤ 14 days a month). METHODS: A detailed search strategy was used to find a relevant meta-analyses, systematic reviews and randomized double-blind controlled trials. Recommendations were graded with the Grading of Recommendations Assessment, Development and Evaluation (GRADE) Working Group, using a consensus group. In addition, a general literature review and expert consensus were used for aspects of acute therapy for which randomized controlled trials were not available. RESULTS: Twelve acute medications received a strong recommendation for use in acute migraine therapy (almotriptan, eletriptan, frovatriptan, naratriptan, rizatriptan, sumatriptan, zomitriptan, ASA, ibuprofen, naproxen sodium, diclofenac potassium, and acetaminophen). Four received a weak recommendation for use (dihydroergotamine, ergotamine, codeine-containing combination analgesics, and tramadol- containing medications). Three of these were NOT recommended for routine use (ergotamine and codeine- and tramadol- containing medications). Strong recommendations were made to avoid use of butorphanol and butalbital- containing medications. Metoclopramide and domperidone were strongly recommended for use when necessary. Our analysis also resulted in the formulation of eight general acute migraine management strategies. These were grouped into: 1) two mild-moderate attack strategies, 2) two moderate-severe attack or NSAID failure strategies, 3) three refractory migraine strategies, and 4) a vasoconstrictor unresponsive-contraindicated strategy. Additional were developed for menstrual migraine during pregnancy, and migraine during lactation. CONCLUSION: This guideline provides evidence-based advice on acute pharmacological migraine therapy, and should be helpful to both health professionals and patients, The available medications have been organized into a series of strategies based on patient clinical features. These strategies may help practitioners make appropriate acute medication choices for patients with migraine.

Canadian Headache Society guideline for migraine prophylaxis.

OBJECTIVES: The primary objective of this guideline is to assist the practitioner in choosing an appropriate prophylactic medication for an individual with migraine, based on current evidence in the medical literature and expert consensus. This guideline is focused on patients with episodic migraine (headache on ≤ 14 days a month). METHODS: Through a comprehensive search strategy, randomized, double blind, controlled trials of drug treatments for migraine prophylaxis and relevant Cochrane reviews were identified. Studies were graded according to criteria developed by the US Preventive Services Task Force. Recommendations were graded according to the principles of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) Working Group. In addition, a general literature review and expert consensus were used for aspects of prophylactic therapy for which randomized controlled trials are not available. RESULTS: Prophylactic drug choice should be based on evidence for efficacy, side-effect profile, migraine clinical features, and co-existing disorders. Based on our review, 11 prophylactic drugs received a strong recommendation for use (topiramate, propranolol, nadolol, metoprolol, amitriptyline, gabapentin, candesartan, butterbur, riboflavin, coenzyme Q10, and magnesium citrate) and 6 received a weak recommendation (divalproex sodium, flunarizine, pizotifen, venlafaxine, verapamil, and lisinopril). Quality of evidence for different medications varied from high to low. Prophylactic treatment strategies were developed to assist the practitioner in selecting a prophylactic drug for specific clinical situations. These strategies included: first time strategies for patients who have not had prophylaxis before (a beta-blocker and a tricyclic strategy), low side effect strategies (including both drug and herbal/vitamin/mineral strategies), a strategy for patients with high body mass index, strategies for patients with co-existent hypertension or with co-existent depression and /or anxiety, and additional monotherapy drug strategies for patients who have failed previous prophylactic trials. Further strategies included a refractory migraine strategy and strategies for prophylaxis during pregnancy and lactation. CONCLUSIONS: There is good evidence from randomized controlled trials for use of a number of different prophylactic medications in patients with migraine. Medication choice for an individual patient requires careful consideration of patient clinical features.

Cost of health care among patients with chronic and episodic migraine in Canada and the USA: results from the International Burden of Migraine Study (IBMS).

OBJECTIVE: To evaluate and compare healthcare resource use and related costs in chronic migraine and episodic migraine in the USA and Canada. BACKGROUND: Migraine is a common neurological disorder that produces substantial disability for sufferers around the world. Several studies have quantified overall costs associated with migraine in general, with recent estimates ranging from $581 to $7089 per year. Although prior studies have characterized the clinical and humanistic burden of chronic migraine relative to episodic migraine, to the best of our knowledge only 1 previous study has compared chronic migraine and episodic migraine healthcare costs. The purpose of this study was to quantify and compare the direct medical costs of chronic migraine and episodic migraine using medical resource use data collected as part of the International Burden of Migraine Study. METHODS: Cross-sectional data were collected from respondents in 10 countries via a Web-based survey. Respondents were classified as chronic migraine (≥15 headache days/month) or episodic migraine (<15 headache days/month). Data collection included socio-demographic and clinical characteristics and medical resource use for headache (clinician and emergency department visits and hospitalizations over the preceding 3 months and medications over the preceding 4 weeks). Unit cost data were collected outside of the Web-based survey using publicly available sources and then applied to resource use profiles. Cost estimates are presented in 2010 US and Canadian dollars. RESULTS: In this manuscript, the analysis included data from respondents with migraine in the USA (N=1204) and Canada (N=681). The most common medical services utilized by all respondents included headache-specific medication, healthcare provider visits, emergency department visits, and diagnostic testing. In the USA, approximately one-quarter (26.2%) of chronic migraine participants vs 13.9% of episodic migraine participants reported visiting a primary care physician in the preceding 3 months (P<.001). In Canada, one-half (48.2%) of chronic migraine participants had a primary care physician visit, compared with 12.3% of episodic migraine subjects (P< .0001). Total mean headache-related costs for participants with chronic migraine in the USA were $1036 (±$1334) over 3 months compared to $383 (±807, P< .001) for persons with episodic migraine. In Canada, total mean headache-related costs among chronic migraine subjects were $471 (±1022) compared to $172 (±920, P< .001) for episodic migraine subjects. CONCLUSIONS: Chronic migraine was associated with higher medical resource use and total costs compared to episodic migraine. Therapies that reduce headache frequency could become important approaches for containing or reducing headache-related medical costs.

Patterns of care and outcomes after computed tomography scans for headache.

BACKGROUND: concerns exist about potential overuse of computed tomography (CT) scans for headache in ambulatory care. METHODS: we sought to examine health services use, brain tumor diagnosis, and death during the year after CT scanning for headache by linking records of an audit of 3930 outpatient CT brain scans performed in 2005 in Ontario, Canada, to administrative databases. RESULTS: of 623 patients receiving CT scans for a sole indication of headache, few (2.1%) scans contained findings potentially causing their headache. For most patients, the index CT scan was the only one received over an 11-year period. However, 28.4% of patients received 1 or more CT brain scans during the preceding decade and 6.7% received 1 or more CT brain scans during the subsequent year. Of the 473 patients (75.9%) whose index scan was ordered by a primary care physician, most (80.3%) did not see a specialist during follow-up. One patient with an indeterminate finding on the index scan was diagnosed with a malignant brain tumor (0.2%), and 6 patients (1.0%) died during follow-up. Among the 4 deaths in which the cause could be determined, none were due to central nervous system causes. CONCLUSION: because of the potential risk of cancer from exposure to ionizing radiation, efforts should be made to avoid CT scanning for headache when the likelihood of serious illness is low. Evidence-based decision rules that identify which patients with headache do not require neuroimaging may decrease the use of CT scans in situations of little benefit.

Botulinum toxin type A and acute drug costs in migraine with triptan overuse.

BACKGROUND: Patients with chronic migraine and medication overuse are significant consumers of health care resources. OBJECTIVE: To determine whether botulinum toxin type A prophylaxis reduces the cost of acute migraine medications in patients with chronic migraine and triptan overuse. METHODS: In this multicenter, open-label study, patients with chronic migraine (≥ 15 headache days/month) who were triptan overusers (triptan intake ≥ 10 days/month for ≥ 3 months) received botulinum toxin type A (95-130 U) at baseline and month three. Headache (HA) frequency and medication use were assessed with patient diaries, and headache-related disability by means of the MIDAS and Headache Impact Test-6 questionnaires. RESULTS: Of 53 patients enrolled (mean age ± standard deviation, 46.5 years ± 8.4; 47 [88.7%] females), 48 (90.6%) completed the study at month six. Based on headache diaries, significant (P ≤ 0.0002) decreases from baseline were observed for days per month with headache/migraine, days with any acute headache medication use, days with triptan use, and triptan doses taken per month. A significant (P < 0.0001) increase from baseline in headache-free days per month was also observed. Prescription medication costs for acute headache medications decreased significantly, including significant reductions in triptan costs (mean reduction of -C$106.32 ± 122.87/month during botulinum toxin type A prophylaxis; P < 0.0001). At baseline, 78% of patients had severe disability (MIDAS score) and 86.8% had severe impact due to headache (HIT-6 scores); at month six, this decreased to 60% and 68%, respectively. CONCLUSIONS: Botulinum toxin type A prophylactic therapy markedly decreased costs related to acute headache medication use in patients with chronic migraine and triptan overuse.

Headaches attributable to infectious diseases.

Headache is the most common symptom that humans experience. While the vast majority of headaches are due to benign primary headache disorders, a small but important minority of headaches are due to secondary causes. Whereas significant emphasis is placed on educating physicians regarding prompt recognition of subarachnoid hemorrhage and headaches secondary to brain tumors, attention toward headaches secondary to infectious causes is often neglected. Unfortunately, a missed or delayed diagnosis of a headache secondary to meningitis, encephalitis, brain abscess, subdural empyema, or other infectious etiologies can lead to dire consequences for both the patient and physician. Accordingly, this article provides an overview of headaches attributed to systemic and intracranial infectious causes.

From psychoneurosis to ICHD-2: an overview of the state of the art in post-traumatic headache.

Post-traumatic headache (PTH) is an important public health issue - head injuries are common, headache is the most common sequelae of head injuries, and PTH can be particularly disabling. Fortunately, for most individuals with PTH, the headache gradually dissipates over a period of several days, weeks, or months either spontaneously or aided by non-pharmacologic and/or pharmacologic management. Regrettably, for a minority of head-injured individuals, the PTH is intractable and disabling despite aggressive and comprehensive treatment. Unfortunately, there are many prejudices against individuals with PTH. Frequently, the presence or absence of litigation and/or the mechanism of head injury (sports-related trauma, slip-and-fall injury, motor vehicle accident, or military service-related injury) biases physicians' views on the legitimacy of the patient's PTH. Accordingly, this review attempts to summarize the state of the art of our understanding of PTH. This clinical review highlights: (a) views on PTH throughout the last few centuries, (b) the ICHD-2 classification of PTH, (c) the epidemiology of head injuries and PTH, (d) the clinical characteristics of PTH, (e) PTH related postconcussive symptoms, (f) pathophysiology of PTH, (g) evaluation of PTH, and (h) management of PTH.

The metabolic headaches.

This review discusses headaches secondary to disorders of homeostasis, formerly known as "headaches associated with metabolic or systemic diseases." They include the headaches attributed to 1) hypoxia and/or hypercapnia (high altitude, diving, sleep apnea); 2) dialysis; 3) arterial hypertension; 4) hypothyroidism; 5) fasting; and 6) cardiac cephalalgia. For each headache type, we discuss the clinical features and diagnosis, as well as therapeutic strategies.

Migraine prevalence, diagnosis, and disability.

The goal of the Canadian Migraine Forum was to work towards improving the lives of Canadians with migraine by reducing their migraine-related disability. This paper reviews the epidemiology and diagnosis of migraine, and the effects of migraine on health related quality of life. Many patients with migraine do not consult a physician for their headaches, and when they do they often do not receive a correct diagnosis. The discussion at the Forum concluded that better education, both for physicians and the public, on issues relating to migraine was a necessary step in improving migraine diagnosis. The degree of disability caused by migraine is often not recognized by society, and can be substantial for individuals with migraine. Once again, education of the public and of the health professionals who see these patients is key, so that the best migraine management can be instituted to minimize the impact of migraine on the individual, the family, and society at large.

An approach to the patient with painful ophthalmoplegia, with a focus on Tolosa-Hunt syndrome.

The differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies, including neoplasms (ie, primary intracranial tumors, local or distant metastases), vascular (eg, aneurysm, carotid dissection, and carotid-cavernous fistula), inflammatory (ie, orbital pseudotumor, giant cell arteritis, sarcoidosis, and Tolosa-Hunt syndrome), and infectious etiologies (ie, fungal and mycobacterial), as well as other miscellaneous conditions (ie, ophthalmoplegic migraine and microvascular infarcts secondary to diabetes). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that can be associated with significant morbidity or mortality if left untreated. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids but should be diagnoses of exclusion.