A brief diagnostic screen for cluster headache: Creation and initial validation of the Erwin Test for Cluster Headache.

OBJECTIVE: To use 1) newly generated data, 2) existing evidence, and 3) expert opinion to create and validate a new cluster headache screening tool. METHODS: In phase 1 of the study, we performed a prospective study of an English translation of an Italian screen on 95 participants (45 with cluster headache, 17 with other trigeminal autonomic cephalalgias, 30 with migraine, and 3 with trigeminal neuralgia). In phase 2, we performed a systematic review in PubMed of all studies until September 2019 with diagnostic screening tools for cluster headache. In phase 3, a 6-person panel of cluster headache patients, research coordinators, and headache specialists analyzed the data from the first two phases to generate a new diagnostic screening tool. Finally, in phase 4 this new screen was validated on participants at a single headache center (all diagnoses) and through research recruitment (trigeminal autonomic cephalalgias only, as recruitment was essential but was otherwise low). RESULTS: In total, this study included 319 unique participants including 109 cluster headache participants (95 total participants/45 cluster headache participants in phase 1, and 224 total participants/64 cluster headache participants in phase 4). It also found 123 articles on potential screening tools in our systematic review. In phase 1, analysis of the English translation of an Italian screen generated 7 questions with high sensitivity and specificity against migraine, trigeminal neuralgia, and other trigeminal autonomic cephalalgias, but had grammatical and other limitations as a general screening tool. In phase 2, the systematic review revealed nine studies that met inclusion criteria as diagnostic screening tools for cluster headache, including four where sensitivity and specificity were available for individual questions or small groups of questions. In phase 3, this data was reviewed by the expert panel to generate a brief (6-item), binary (yes/no), written screening test. In phase 4, a total of 224 participants completed the new 6-item screening test (81 migraine, 64 cluster headache, 21 other trigeminal autonomic cephalalgias, 35 secondary headaches, 7 neuralgias, 5 probable migraine, and 11 other headache disorders). Answers to the 6 items were combined in a decision tree algorithm and three items had a sensitivity of 84% (confidence interval or 95% confidence interval 73-92%), specificity of 89% (95% confidence interval 84-94%), positive predictive value of 76% (95% confidence interval 64-85%), and negative predictive value of 93% (95% confidence interval 88-97%) for the diagnosis of cluster headache. These three items focused on headache intensity, duration, and autonomic features. CONCLUSION: The 3-item Erwin Test for Cluster Headache is a promising diagnostic screening tool for cluster headache.

Clinical features of new daily persistent headache: A retrospective chart review of 328 cases.

OBJECTIVE: To describe the clinical features of new daily persistent headache (NDPH) at an outpatient neurology clinic with a subspecialty interest in headache in Houston, Texas. METHODS: A retrospective chart review was performed by a neurologist subspecialty certified in headache medicine of all patients seen from September 1, 2011 through February 28, 2020 (8.5 years) with a provisional diagnosis of NDPH and abstracted charts meeting criteria for primary NDPH. RESULTS: A total of 328 patients met the International Classification of Headache Disorders, 3rd edition criteria including 215 (65.5%) female patients. The mean age of onset was 40.3 years (range 12-87 years) with a mean age at first consultation in the study clinic of 42.6 years (range 12-87 years). The median [25th, 75th] duration of NDPH at initial consultation was 0.7 [0.3, 2.0] years, and at last visit, it was 1.9 [0.7, 4.8] years. The headaches were side-locked unilateral in 28/328 (8.5%). 12/328 cases (3.6%) with a thunderclap onset are reported. There was no obvious seasonal or other cyclical variation. The pain was usually moderate to severe and more often severe for the migraine phenotype (MP; 260/328 [79.3%] of cases) than for the tension-type phenotype (TTP). Precipitating factors were the following: stressful life events, 67/328 (20.4%); upper respiratory infection or flu-like illness, 33/328 (10.1%); and extracranial surgery, 5/328 (1.5%). Exacerbating or aggravating factors similar to migraine triggers were more often reported with the MP than with the TTP in this sample. For the MP, vertigo or dizziness was reported by 19/260 (7.3%) and a visual aura by 21/260 (8.1%) with descriptions provided. The prognostic types were the following for all patients: persisting (refractory), 305/328 (93.0%); relapsing-remitting, 9/328 (2.7%); and remitting (self-limited), 14/328 (4.3%). CONCLUSION: NDPH is typically of moderate to severe intensity often with migraine features without obvious seasonal or other cyclical variation. Most cases are refractory. 3.6% have a thunderclap onset often with a good response to treatment.

The Very Important Person (VIP) and the Headache Medicine Physician.

Headache medicine specialists often treat VIPs but little is known about whether these encounters are different than with other patients. Questions considered include who is a VIP, should you treat a VIP like any other patient, and what are the challenges and advantages? A non-random sampling of comments is presented from headache medicine specialists. Eleven principles are presented to consider in these encounters.

Medico-Legal Aspects of Concussion.

Concussion and the sequelae have been controversial medico-legal issues for over 150 years. The following topics which are also important in clinical practice are discussed: definitions of concussion, neuroimaging, onset and prognosis of headaches, cognitive impairment, cognitive rehabilitation, post-traumatic stress disorder, and risk of later development of dementia.

Primary Empty Sella.

Primary empty sella (PES) syndrome is a misnomer as it is not a syndrome but a radiological finding with possible endocrine abnormalities. No specific headache type has been shown to be caused by PES. Endocrine screening may be considered for asymptomatic persons with PES.

Gluten-Free Diet and Migraine.

Migraine is common in celiac disease (CD) and usually improves on a gluten-free diet (GFD). The benefit for people impacted by migraine without CD is poorly evidenced. A GFD may have adverse health consequences and is expensive.

Neuroimaging for Migraine: The American Headache Society Systematic Review and Evidence-Based Guideline.

OBJECTIVE: To provide updated evidence-based recommendations about when to obtain neuroimaging in patients with migraine. METHODS: Articles were included in the systematic review if they studied adults 18 and over who were seeking outpatient treatment for any type of migraine and who underwent neuroimaging (MRI or CT). Medline, Web of Science, and Cochrane Clinical Trials were searched from 1973 to August 31, 2018. Reviewers identified studies, extracted data, and assessed the quality of the evidence in duplicate. We assessed study quality using the Newcastle-Ottawa Scale. RESULTS: The initial search yielded 2269 publications. Twenty three articles met inclusion criteria and were included in the final review. The majority of studies were retrospective cohort or cross-sectional studies. There were 4 prospective observational studies. Ten studies evaluated the utility of CT only, 9 MRI only, and 4 evaluated both. Common abnormalities included chronic ischemia or atrophy with CT and MRI scanning, and non-specific white matter lesions with MRI. Clinically meaningful abnormalities requiring intervention were relatively rare. Clinically significant neuroimaging abnormalities in patients with headaches consistent with migraine without atypical features or red flags appeared no more common than in the general population. RECOMMENDATIONS: There is no necessity to do neuroimaging in patients with headaches consistent with migraine who have a normal neurologic examination, and there are no atypical features or red flags present. Grade A Neuroimaging may be considered for presumed migraine for the following reasons: unusual, prolonged, or persistent aura; increasing frequency, severity, or change in clinical features, first or worst migraine, migraine with brainstem aura, migraine with confusion, migraine with motor manifestations (hemiplegic migraine), late-life migraine accompaniments, aura without headache, side-locked headache, and posttraumatic headache. Most of these are consensus based with little or no literature support. Grade C.

Raynaud's Phenomenon Associated With Calcitonin Gene-Related Peptide Monoclonal Antibody Antagonists.

Two cases are reported of migraineurs who reported Raynaud's phenomenon (RP) exacerbated while taking monoclonal antibodies to the calcitonin gene-related peptide (CGRP) ligand (fremanezumab and galcanezumab) and 1 case of new onset RP while taking the CGRP receptor antagonist (erenumab). The prevalence of primary and secondary RP, causes of secondary RP, co-morbidity with migraine, and medications which might induce or exacerbate RP are reviewed. The pathophysiology of how CGRP monoclonal antagonists might exacerbate or induce RP is discussed. The cases suggest but do not prove causation.

Negative Online Patient Reviews in Headache Medicine.

Patients commonly use online reviews in selecting a new physician. There have been no studies of online reviews of headache medicine physicians. To better understand headache medicine physicians' attitudes and opinions about negative reviews, 2 negative patient reviews were posted to the Southern Headache Society online discussion group and comments were requested. Twenty-two anonymous comments are provided. The strengths and inadequacies of online physician reviews in other specialties and the possible actions headache medicine physicians should take for negative reviews are discussed. A nationwide survey of online reviews of headache medicine specialists would be of interest.

Sports and Headaches.

In the United States, up to 3.8 million people per year have sports-related mild traumatic brain injury frequently followed by a variety of headaches. Headaches associated with sports (exertional, weightlifter's, and external compression headache) are also reviewed.

A Survey of Neurologists on Postconcussion Syndrome.

BACKGROUND AND OBJECTIVES: Postconcussion syndrome (PCS) has been controversial for more than 150 years. As there have not been any surveys of PCS among neurologists in the United States since 1992, another was performed using most of the prior items to assess current opinions and practices and whether there have been any changes since 1992. METHODS: Two hundred and eighty-nine neurologists attending the Texas Neurological Society 20th Annual Winter Conference continuing medical education meeting in 2017 were supplied the survey instrument with registration materials. The 25 item instrument (including 7 new items) contained items on demographics, definitions, causation, prognosis, medicolegal aspects, testing, and treatment. Forty percent of attendees completed the survey. RESULTS: The majority of respondents agree with the following: PCS is a clearly defined syndrome with a solid basis for determining prognosis with an organic basis; accept the authenticity of patients' reports of symptoms; effective treatment is available for headaches lasting 3 months or more; headaches persist in over 20% 1 year after injury; and cognitive rehabilitation is effective. The majority of the respondents do not agree with the following: symptoms improve in a relatively short period of time and quickly resolve once litigation is settled; effective treatment is available for PCS; and return to play guidelines are strongly evidence based. 68.4% disagree with the following: I would support my son or grandson (or if you do not have one, relative's or friend's) playing football. [Correction added on February 9, 2018, after first online publication: "One year after injury" deleted.] CONCLUSIONS: There has been growing acceptance of the organicity of PCS among neurologists in the last 25 years. There is significant concern over the long-term sequelae of concussion as most respondents would not recommend that their son or grandson play football.

Incidental Findings and Normal Anatomical Variants on MRI of the Brain in Adults for Primary Headaches.

When MRI scans of the brain are obtained for evaluation of primary headaches in adults, incidental findings are commonly present. After a review of the prevalence of incidental findings and normal anatomical variants, 21 types are presented.

Pourfour du Petit Syndrome Associated With Right Eye Pressure.

Pourfour du Petit (PDP) syndrome is a rare disorder characterized by ipsilateral mydriasis, eyelid retraction, and hemifacial hyperhidrosis caused by hyperactivity of the ipsilateral oculosympathetic pathway. A case is presented of PDP syndrome associated with likely ipsilateral occipital neuralgia. We review the causes and co-morbidities and the clinical features of PDP.

New Daily Persistent Headache Caused by a Multinodular Goiter and Headaches Associated With Thyroid Disease.

A 33-year-old female is presented with the first case to our knowledge of new daily persistent headache (NDPH) with a large right benign non-toxic multinodular goiter causing carotid and vertebral compression with complete resolution of the headache immediately after thyroidectomy. Although this may be quite rare, hypothyroidism or hyperthyroidism causing NDPH, migraine, or an exacerbation of pre-existing migraine is not. Clinicians should consider routinely obtaining serum thyroid-stimulating hormone (TSH) and free T4 in patients with new onset frequent headaches or an exacerbation of prior primary headaches.

Incidental Findings and Normal Anatomical Variants on Brain MRI in Children for Primary Headaches.

When MRI scans of the brain are obtained for evaluation of primary headaches in children, incidental findings and anatomical variants are commonly present. After a review of the prevalence, 11 types are presented.

A Survey of Headache Medicine Physicians on the Likeability of Headaches and Their Personal Headache History.

BACKGROUND: Two prior studies have shown an increased prevalence of migraine among physicians who are headache medicine specialists (HMS). There have been no studies of the prevalence of other headache disorders among HMS. A prior survey showed that neurologists like to treat some headaches more than others but there has not been a similar survey of HMS. OBJECTIVES: The aim of the survey was to learn more about the prevalence of headaches among HMS and which headache disorders they like to treat. METHODS: An email survey was sent to 749 physician members of the American Headache Society who were asked to respond to the following statement using a 5-point Likert scale (from 1, strongly disagree to 5, strongly agree): "I like to treat patients with the following types of headaches or syndromes." They were asked, "Have you personally suffered from any of the following at any time during your life: episodic migraine (EM), chronic migraine (CM), refractory migraine (RM), episodic cluster (EC), chronic cluster (CC), new daily persistent headache (NDPH), and postconcussion syndrome (PCS)." RESULTS: The response rate was 15.8% (n = 118) with a mean age of 51.4 years, 64.4% males, and 85.6% neurologists. HMS reported likeability for treating disorders in rank order as follows: EM (mean = 4.69, SD = 0.61); CM (mean = 4.20, SD = 0.94); RM (mean = 3.62, SD = 1.17); EC (mean = 4.37, SD = 0.80); CC (mean = 3.68, SD = 1.10); NDPH (mean = 3.52, SD = 1.21); and PCS (mean = 3.66, SD = 1.18). The lifetime prevalence of disorders was as follows: EM, 69.5% (85.7% in females and 60.5% in males); CM, 13.6% (19% in females and 10.5% in males); RM, .9% (2.4% females and 0% males); EC, 1.7% (0% females and 2.6% males); CC, 0%; NDPH, 0%, and PCS, 4.2% (7.1% females and 2.6% males). HMS with a personal history of EM (mean = 4.73, SD = 0.51) showed a significant preference (t130 = 7.30, P < .001) to treat episodic migraine more than other headaches (mean = 3.90, SD = 0.77). CONCLUSIONS: HMS preferred to treat some disorders more than others, with EM most liked and NDPH least preferred, which may reflect how well patients with those disorders respond to treatment. The lifetime prevalence of EM among HMS is significantly greater than among neurologists, both significantly greater than EM in the general population. An explanation for the higher prevalence among neurologists is not certain but perhaps migraine is associated with a choice to become an HMS during or after neurology residency. The lifetime prevalence among HMS of CM may be and EC is much greater than among the general population for uncertain reasons.

Migraine and the Risk of Suicide.

Rarely and tragically, migraineurs commit suicide or overdose on medications we prescribe.

Movement Disorders From the Use of Metoclopramide and Other Antiemetics in the Treatment of Migraine.

Nausea and vomiting are a frequent accompaniment of migraine and anti-nausea medications are frequently used in its management. The majority of anti-nausea medications that are used in migraine are dopamine receptor blocking agents and therefore have the potential to cause drug-induced movement disorders. This article explores the risk of such drug-induced movement disorders in migraineurs who were treated with these medications.

A Motion Simulator Ride Associated With Headache and Subdural Hematoma: First Case Report.

We report the first case report of symptomatic bilateral subdural hematomas (SDH) associated with riding a centrifugal motion simulator ride. A previously healthy 55-year-old male developed new onset daily headaches 1 week after going on the ride that were due to symptomatic bilateral SDH requiring operative intervention with a full recovery. There was no history of other trauma or other systemic or intracranial abnormality to account for the development of the SDH. We review the headaches and other clinical features associated with chronic SDH. Twelve cases of roller coaster headaches due to SDH associated with riding roller coasters have been reported. The pathophysiology is reviewed, which we believe is the same mechanism that may be responsible in this case. Although it is possible that this neurovascular injury is truly rare, it is also possible that this injury is underreported as patients and physicians may not make the association or physicians have not reported additional cases. The risk of this injury likely increases with age, as the size of the subdural space increases, and may support the maxim that "roller coasters and simulators are for kids."