Smith, Jonathan H.

Episodic status migrainosus is a previously described migraine variant where the exclusive phenotype is that of relapsing status migrainosus. The majority of cases have been reported based on retrospective chart review. A prospectively identified case fulfilling previously published criteria for episodic status migrainosus is reported, with an enduring pattern of attacks meeting criteria for status migrainosus over a 35-year period. A family history in a maternal aunt of a similar phenotype was noted. This prospectively identified case further validates the existence of episodic status migrainosus as a novel nosologic entity. The family history in our case suggests the possibility of genetic susceptibility. Perspective regarding a potential temporal continuum between acute migraine, status migrainosus, and new daily persistent headache is described. Status migrainosus may occur both as a complication of migraine and in the pure form of episodic status migrainosus.

Objective To report a case series of a novel migraine subtype, which we term as episodic status migrainosus (ESM), characterized by attacks of migraine exclusively lasting more than 72 hours. We hypothesized that this would represent a novel nosologic entity, possibly an unstable migraine phenotype with a high conversion rate to chronic migraine (CM). Methods We conducted a retrospective review of patients diagnosed with status migrainosus at the Mayo Clinic, Rochester, between January 2005 and December 2015. All the records were then manually reviewed for patients with migraine headaches exclusively lasting more than 72 hours. Results We identified 18 patients with ESM, with a female predominance (15(83.3%)) and a median age of onset of 16.5 (IQR 13–19) years. The median monthly attack frequency was two (IQR 1–3), with each attack lasting a median duration of seven (IQR 4–12.5) days. Stress was the most commonly reported precipitant (11 (61.1%)). Migraine with aura was common (10 (55.6%)), as was comorbid depression (10 (55.6%)). Fifteen (83.3%) patients developed CM at a median of 7.8 (IQR 2.6–21.9) years from their first attack. There was no significant association between the time to the development of chronic migraine with either attack frequency or duration. Conclusions and relevance We report the existence of a novel migraine subtype, episodic status migrainosus. This migraine subtype appears to have similar clinical characteristics to episodic migraine with or without aura, except for a notably high tendency to progress to chronic migraine.

Giant cell arteritis ( GCA ) is a medium and large‐vessel vasculitis, which is an important cause of secondary headache in older adults. While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis. The topography of vascular involvement has implications for disease‐related complications, which can result in neurologic disease at multiple levels of the nervous system. The most feared complication, vision loss, fortunately becomes uncommon after initiation of corticosteroids. Corticosteroid treatment should not be withheld while waiting the results of a temporal artery biopsy ( TAB ), which remains the gold standard for GCA diagnosis. Newer diagnostic modalities, including ultrasound, magnetic resonance imaging, and positron emission tomography can play an important role in directing treatment in cases with negative TAB . After successful control of the disorder, patients should be gradually tapered off corticosteroids, with careful monitoring using both clinical and laboratory parameters to assess for relapse. Corticosteroid‐related treatment complications are not uncommon in GCA . There is mixed evidence for use of adjunct corticosteroid‐sparing agents (eg, methotrexate), although these should be initiated in the setting of corticosteroid‐related morbidity and/or cases with frequent relapse.

Background and purposeTrochlear headaches are a recently recognized cause of headache, of which both primary and inflammatory subtypes are recognized. The clinical features, long‐term prognosis and optimal treatment strategy have not been well defined.MethodsA cohort of 25 patients with trochlear headache seen at the Mayo Clinic between 10 July 2007 and 28 June 2012 were identified.ResultsThe diagnosis of trochlear headache was not recognized by the referring neurologist or ophthalmologist in any case. Patients most often presented with a new daily from onset headache (n = 22, 88%). The most characteristic headache syndrome was reported as continuous, achy, periorbital pain associated with photophobia and aggravation by eye movement, especially reading. Individuals with a prior history of migraine were likely to have associated nausea and experience trochlear migraine. Amongst individuals with trochleitis, 5/12 (41.6%) had an identified secondary mechanism. Treatment responses were generally, but not invariably, favorable to dexamethasone/lidocaine injections near the trochlea. At a median follow‐up of 34 months (range 0–68), 10/25 (40%) of the cohort had experienced complete remission.ConclusionsTrochlear headaches are poorly recognized, have characteristic clinical features, and often require serial injections to optimize the treatment outcome. The identification of trochleitis should prompt neuroimaging to look for a secondary cause.

Background Isolated neuralgic pain in the deep ear may arise from either nervus intermedius (NIN) or glossopharyngeal (GPN) neuralgias. Current International Headache Society (IHS) International Classification of Headache Disorders, second edition (ICHD-2) criteria for these cranial neuralgias require the presence of a characteristic trigger. Aim The aim of this article is to report cases of triggerless neuralgic otalgia to better understand a subset of patients for whom there may be diagnostic uncertainty. Methods Methods included an observational cohort series and systematic literature review. Results We identified five female patients with a median age at symptom onset of 58 (range: 47 to 73). Our patients generally experienced an excellent clinical response to carbamazepine. Patients were contacted by telephone at a median follow-up duration of seven years (range: four to 32) from symptom onset, at which time carbamazepine-free remissions were reported by five of five (100%) of the patients. A systematic review of the literature on neuralgic otalgia led us to conclude that NIN was most common among young women (age < 50), and GPN across a wider range of ages of either gender. Among surgically validated cases reported in the literature, triggers were frequently absent in NIN, and variably noted in GPN. Conclusions We conclude that the presence of a trigger is not fundamental, and may be impractical, to the diagnosis of neuralgic otalgia, but remains important for specificity between NIN and GPN.