Improved description of the migraine aura by a diagnostic aura diary

We present a diagnostic aura diary for prospective recordings of migraine with aura. Three questionnaires are supplemented with sheets for drawings and plottings of visual and sensory auras. Twenty patients recorded 54 attacks of migraine with aura and 2 attacks of migraine aura without headache. The visual and sensory aura were usually gradually progressive, reaching maximum development in 15 and 25 min (median) respectively and had a total duration of 20 and 55 min (median) respectively. Approximately 13% of the attacks had acute onset of visual aura associated with other features more typical of migraine. The visual and sensory auras always preceded typical migraine headache, and headache occurring before aura symptoms was always of the tension type, The migraine headache was milder than in attacks of migraine without aura and often did not have migraine characteristics. In attacks with unilateral head pain, headache and aura symptoms were contralateral in 90% and ipsilateral in 10%.     

Specific Headache Phenomena: Their Frequency and Coincidence

The correlation of specific headache attack characteristics derived from a standardized questionnaire was studied in a consecutive series of 392 patients attending a headache clinic. In patients reporting headache with aura such attacks tended to be infrequent, and the percentage of their headache attacks that were preceded by aura varied widely, many cases having very low percentages. Paresthesias as auras were uncommon and rarely occurred without visual aura. Unilateral headaches were associated with nausea to widely varying degrees. Most patients reported both unilateral headache and headache with tension characteristics. Definite history of hypertension was significantly correlated specifically with migraine with aura (classic migraine).     

Recurrent Spontaneous Paresthesia in the Upper Limb Could Be Due to Migraine: A Case Series

Transient neurologic dysfunction is a characteristic feature of migraine. About 20% of migraineurs may experience various symptoms in the absence of any headache at one time or another. Visual auras are the most common auras of migraine, and migraine is considered as the most common cause of transient vision loss in young patients. Sensory auras are the second most common migrainous auras. However, the literature is silent for isolated sensory aura as a migraine equivalent. Herein we report 14 patients with recurrent episodic paresthesia in the limbs and other body parts. All patients fulfilled the diagnostic criteria of “typical aura without headache” of ICHD‐3β. All patients were subjected to various investigations to rule out secondary causes. Ten patients received antimigraine drugs and all showed a positive response to therapy. Recurrent spontaneous paresthesia is quite common in the general population and many patients remain undiagnosed. We speculate that a subset of patients might be related to migrainous sensory auras.     

Migraine variants

The term "migrant variant" is not used in the headache classification of the International Headache Society (IHS), but it includes those forms of migraine that are not typical of migraine with or without aura. Headaches that do not quite fulfill all of the IHS criteria are termed "migrainous disorder." Migraine associated with auras arising from unusual sites includes basilar migraine, retinal migraine, and ophthalmoplegic migraine. Two of the chromosomal sites for hemiplegic migraine have been identified. Migraine aura may occur without headache and an aura may be prolonged. Migrainous infarct has occurred when the aura lasts more than 1 week or imaging studies are positive and other etiologies have been ruled out. If the migraine attack is prolonged beyond 3 days the term "status migrainousus" is applied.     

Are cortical spreading depression and headache in migraine causally linked?

During the past few decades, much controversy has surrounded the pathophysiology of migraine. Cortical spreading depression (CSD) is widely accepted as the neuronal process underlying visual auras. It has been proposed that CSD can also cause the headaches, at least in migraine with aura. We describe three patients, each fulfilling the International Headache Society criteria for migraine with aura, who suffered from headaches 6–10 days per month. Two patients were treated with flunarizine and the third patient with topiramate for the duration of 4 months. All patients reported that aura symptoms resolved completely, whereas the migraine headache attacks persisted or even increased. These observations question the theory that CSD (silent or not) is a prerequisite for migraine headaches.     

Diagnostic criteria for CADASIL in the International Classification of Headache Disorders (ICHD-II): are they appropriate?

We reviewed the characteristics of headache in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), to verify the appropriateness of the International Classification of Headache Disorders, second edition (ICHD-II) criteria. Available data were found through Medline/PubMed using the keyword “cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)”. The search was restricted to studies published in English in the years between 1993 and 2008. We excluded studies that did not report original data on CADASIL and information regarding the presence of headache. We found 34 studies reporting data on 749 patients overall; 387 (51.7%) patients had headache. According to the authors’ definition, 356 (92%) patients were reported as having migraine and 31 (8%) as having headache. Of the 356 patients who were defined as migraineurs, 125 (35.1%) had migraine with aura, 7 (2%) migraine without aura, 156 (43.8%) unspecified migraine and 68 (19.1%) had more than one type of migraine. Among the 31 patients reported as suffering from headache, the headache was not further detailed in 18 (58.1%) patients; it was defined as chronic in 6 (19.3%), as resembling migraine with aura in 4 (12.9%), as resembling migraine without aura in 2 (6.5%) and as tension type in 1 (3.2%) patient. In patients with CADASIL, the headache was usually referred to as migraine and mostly as migraine with aura. However, this referral is formally incorrect since the diagnostic criteria for any type of migraine in the ICHD-II require that the disturbance is not attributed to another disorder. For this reason, we suggest updating the ICHD-II in relation to CADASIL. Our suggestion is to insert a new category referred to as Headache attributed to genetic disorder including Headache attributed to CADASIL.     

An association of migraine with hereditary haemorrhagic telangiectasia independently of pulmonary right-to-left shunts

Hereditary haemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia and visceral vascular manifestations. It is associated with migraine with aura due to pulmonary arteriovenous malformations (pAVMs). The aim of the study was to evaluate headache prevalence in 106 consecutive HHT patients (67 female, 39 male, age 53.5 ± 14.5 years) and age- and gender-matched controls. An extensive clinical work-up was performed and headache prevalence was determined. Lifetime prevalence of migraine was higher in HHT patients (39.6%) than in controls (19.8%) [ P  < 0.001, χ² = 12.17, odds ratio (OR) 3.0; 95% confidence interval 1.6 < OR < 5.7]. A positive association was confirmed between HHT patients with pAVMs and migraine with aura (38.5% vs. 10%). Furthermore, HHT patients without pAVMs had a higher prevalence of migraine without aura (11.5% vs. 26.3%; χ² = 11.85; d.f. = 2; P  = 0.003). We speculate that pathophysiological mechanisms, including dysfunction of the transforming growth factor-beta pathways and resulting vascular changes, contribute to the higher prevalence of migraine without aura in HHT patients without pAVMs.     

Preventing disturbing migraine aura with lamotrigine: an open study

BACKGROUND: Lamotrigine has been suggested as possibly effective for preventing migraine aura. OBJECTIVE: To describe our experience with a series of patients with disturbing migraine aura treated with lamotrigine. METHODS: The members of the Headache Group of the Spanish Society of Neurology were sent an ad hoc questionnaire to collect patients treated with lamotrigine due to disturbing migraine aura. The main outcome parameter ("response") was a >50% reduction in the mean frequency of migraine auras at 3 to 6 months of treatment. RESULTS: A total of 47 patients had been treated with lamotrigine due to severe migraine aura. Three could not complete the protocol as a result of developing skin rashes. Thirty (68%) patients responded. These were 21 females and 9 males whose ages ranged from 19 to 71 years. Eight suffered from migraine with "prolonged" aura, 8 typical aura with migraine headache (but had frequent episodes including speech symptoms), 6 basilar-type migraine, 6 typical aura without headache, and 2 hemiplegic migraine. Fifteen had been previously treated, without response, with other preventatives. The mean monthly frequency of migraine auras in these 30 patients changed from 4.2 (range: 1 to 15) to 0.7 (range: 0 to 6). Response was considered as excellent (>75% reduction) in 21 cases (70% of responders). Auras reappeared in 2 months in 9 out of 13 patients where lamotrigine was stopped, and ceased as soon as this drug was reintroduced. CONCLUSIONS: Lamotrigine should be considered in clinical practice for the preventive treatment of selected patients with disturbing migraine auras. Lamotrigine seems worthy of a controlled trial as prophylaxis of migraine aura.     

Effects of visual pathway lesions on the visual aura of migraine

The aim was to to determine if the visual aura of migraine is altered by disease of the afferent visual pathways and if visual aura changes are associated with pre- or postgeniculate lesions. Functional neuroimaging during migraine demonstrates primary visual/extrastriate cortex as an anatomical substrate of visual aura. Neuro-ophthalmological records (including kinetic and static perimetry) of 25 patients with visual loss and typical aura with or without migraine headache were reviewed. Twenty-five patients (16 women, nine men) (mean age 59.8 years) with typical aura had visual loss from pregeniculate (72%) or postgeniculate lesions (28%). Eight patients (four postgeniculate cerebrovascular accidents or arteriovenous malformations, two lifelong optic neuropathy/retinopathy, one childhood ocular trauma, one anisometropic amblyopia) reported absence or alteration of visual aura. Postgeniculate lesions were significantly associated ( P  = 0.017) with visual aura changes. The association of postgeniculate lesions with altered auras points to a postgeniculate effect on aura appearance (consistent with functional neuroimaging findings). Although statistically significant, this series' association of postgeniculate disease and aura changes is even more robust ( P  = 0.0002) when structural changes of ocular dominance columns are posited in three patients with optic neuropathy, retinopathy and keratopathy of congenital or childhood origin.     

Orientation discrimination thresholds in migraine: a measure of visual cortical inhibition

Orientation discrimination is a visual task dependent on inhibitory mechanisms in the visual cortex. In this study, orientation discrimination thresholds for bar and grating patterns were measured at two visual field locations in subjects with migraine with (n = 20) and without aura (n = 20) and in migraine-free control subjects (n = 20). No statistically significant differences were found between migraine groups and the control group on either task at foveal or peripheral visual field locations. No significant correlations were found between psychophysical thresholds and age, total lifetime auras or total lifetime migraine episodes. However, a trend was seen toward slightly impaired performance on the two foveal tasks in a subgroup of subjects with the highest total lifetime aura count. Thus we have found no convincing evidence that impaired cortical inhibitory mechanisms are a predisposing characteristic in migraine, but cannot rule out the possibility that cortical inhibitory mechanisms may be adversely affected by repeated visual auras.     

Side-shifting hemicrania continua with aura (migraine with aura with autonomic symptoms responsive to indomethacin?)

Atypical features of hemicrania continua (HC), including both visual aura and side shifting, have been reported previously. However, auras and variable unilaterality have never been reported together in HC. We report two patients with side-shifting HC with aura. These patients' symptoms are unilateral headaches, visual aura, autonomic features, throbbing pain, nausea and photo/phonophobia. One could speculate that the unilaterality and/or the autonomic symptom modules are indomethacin responsive. The patients can also be classified as chronic migraine with aura, with autonomic symptoms, responsive to indomethacin. Neither migraine subtype nor side-shifting HC with aura is included in the current International Headache Society (IHS) classification, so these patients are not classifiable. Side-shifting HC with aura implies the need to revisit the traditional IHS categorization of headaches into unique diagnostic groups. The modular headache theory may be a tool for the understanding of these rare and complex cases.     

Characteristics of Migraine Visual Aura

Visual auras (VAs) of 100 patients with migraine with aura were studied by questionnaire. Visual auras accompanied the patients' first headache (HA) in 39% of patients. Only 19% had VAs with every attack. Patients with VAs over the entire HA history had a high frequency (greater than 50%) of attacks with VA; patients with VA during only part of the HA history had a low frequency (less than 50%} of attacks with VA. The auras occurred exclusively prior to the HA in 57%. The free interval between the end of the VA and the start of the HA was usually (75%) shorter than 30 minutes. Most (59%) patients had VAs that lasted from 1 to 30 minutes. They started in the periphery of the visual fields in 56%. The most common phenomena described were: small bright dots (42%), flashes of light (39%), "blind spots" (32%), and "foggy vision" (27%). Fortification spectra was reported by only 20%. Although most (65%) patients had a combination of phenomena, the majority (72%) had only one uniform constellation of manifestations. There was no clear-cut relationship between side of VA and side of HA.
Migraine VA is a pleomorphic and complex symptom. Many patients not qualifying for the diagnostic criteria of migraine with aura, as proposed by the International Headache Society (IHS) unequivocally present with visual phenomena that strongly suggest this diagnosis.     

Glyceryl trinitrate triggers premonitory symptoms in migraineurs

Studying attacks of migraine is considerably hampered by its fundamentally episodic nature. Developing approaches to triggering migraine reliably is important for advancing understanding of the disorder by facilitating its study. Based on the work of the Copenhagen Group we administered an intravenous infusion of 0.5 μg/kg/min glyceryl trinitrate (GTN) to 44 migraineurs, 23 migraine without aura, 21 migraine with aura, and to 12 healthy controls. We sought to characterise the GTN-induced migraine in terms of the clinical features of the attacks and reproducibility of triggering, and included a non-migraine control group for the purpose of comparing any effects to exclude an ordering effect. Of the 44 patients administered GTN, 33 had a migraine attack fulfilling International Headache Society criteria. Thirty-two attacks were of migraine without aura and one of migraine with aura. Twelve patients described typical premonitory symptoms, which have not been previously documented with GTN-induced migraine. A repeat attack was triggered in all subjects but one. In one case a visual aura was also triggered both times. Our study shows that GTN-induced triggering is common in our patients, and remarkably reproducible. The data will facilitate the use of the GTN model in studies requiring extensive planning, such as brain imaging, or where preventive questions are at issue. We also report the first patient with a reproducible GTN-triggered migraine with aura.     

Hungarian migraine epidemiology

OBJECTIVE: A population-based epidemiological survey of migraine without aura and migraine with aura completed by mailed questionnaire in Hungary. The diagnosis and classification of migraine was according to the criteria of the International Headache Society. METHODS: The validity of the self-administered headache questionnaire was evaluated by a neurologist using clinical interviews. The study population was a random sample of 2000 men and women aged between 15 and 80 years. The questionnaire was completed by 813 of 1910 people (392 men and 421 women), providing a 42.6% participation rate. RESULTS: Sixty-seven percent of these people experienced some kind of headache during their lives. The 1-year prevalence of migraine without aura was 7.6% (the female/male ratio was 3:1), while the 1-year prevalence of migraine with aura was 2% (female/male ratio was 2:1). Most migraineurs experienced headache attacks 1 to 4 times monthly, which lasted 24 hours. The most characteristic accompanying signs were nausea and phonophobia. Stress, sleep deprivation, hot weather, and fasting were the most common precipitating factors of a migraine attack. In the migraine with aura group, the most common aura was a visual disturbance. Only 43% of migraineurs had ever consulted a physician for headache, and only 15% of patients had missed work or school because of a migraine attack in the previous year. Most of the patients suffering from migraine without aura were between 20 and 40 years old, while migraineurs with aura were over 40 years old. According to this investigation, the prevalence of migraine was not lower than in Western countries.     

Perceived scintillation rate of migraine aura

OBJECTIVE: To measure the perceived rate of flicker (temporal frequency) observed during visual auras. BACKGROUND: The flickering or scintillating quality of aura elements is a commonly described characteristic of visual migraine auras. Hypotheses about the neural mechanisms involved in aura have rarely taken this feature into account, perhaps because of a lack of quantitative data on this aspect of the aura. While a rate of 10 Hertz had been suggested in the literature, estimates have been speculative due to the difficulty of judging temporal frequencies subjectively. METHODS: Eleven participants were given portable devices that contained an adjustable light-emitting diode with which to match the flickering of their auras. Observers were asked to make flicker matches at two time points so that rate change during aura progression could be analyzed. RESULTS: Data were obtained for 36 aura episodes. The mean rate of flicker across individuals was 17.8 Hertz. Rates varied widely between individuals, but were more consistent across multiple episodes in the same observer. Rate of flicker did not appear to relate to aura side or type, or to individual characteristics such as migraine history. When episodes were analyzed for change in flicker rate over time, patterns of increase (n = 7), decrease (n = 4), and no change (n = 22) were all observed. CONCLUSIONS: When measured with an objective task, aura scintillation rates were found to be somewhat higher than previous anecdotal observations had suggested. These data are discussed in the context of two competing hypotheses concerning the neural mechanism underlying the flicker percept during migraine aura.     

Posterior Ischemic Optic Neuropathy Associated With Migraine

Anterior ischemic optic neuropathy is a well-recognized clinical syndrome that has been described in patients after an episode of migraine with visual aura (classic migraine) and, less commonly, after an episode of visual aura without headache (acephalgic migraine). Little emphasis, however, has been placed on migraine associated retrobulbar or posterior ischemic optic neuropathy. We report two cases of visual loss presumed to be due to posterior ischemic optic neuropathy that occurred In the setting of otherwise typical migraine episodes. We review the English language literature on Ischemic optic neuropathy associated with migraine. Although most cases of ischemic optic neuropathy associated with migraine are of the anterior variety, posterior ischemic optic neuropathy should be considered in the differential diagnosis of any patient with acute loss of vision and evidence for a retrobulbar optic neuropathy, during or after an attack of migraine headache or following an otherwise typical episode of visual aure without headache (acephalgic migraine).     

Migraine Among Norwegian Neurologists

Background.— Previous studies have shown a high prevalence of migraine among neurologists. The main objective of this study was to assess the prevalence of migraine and its subtypes among neurologists in Norway. Method.— Questionnaire‐based cross‐sectional study among every Norwegian neurologist registered on March 19, 2010. Results.— Among the 384 neurologists, 245 (64%) participated. Of these, 95 (39%) reported having experienced migraine aura, and 86 having experienced migraine headache (35%). By employing the International Headache Society criteria for migraine with regard to the number of attacks, the gender‐adjusted lifetime and 1‐year prevalence was 38.7% (95% CI 30.3‐47.7) and 33.8% (95% CI 25.9‐47.2), respectively. Age‐adjusted 1‐year prevalence of migraine headache (not including subjects experiencing visual aura only) for men was 15.9% and for women 36.7%, which gives an overall age and gender‐adjusted prevalence of 26.3% (95% CI 18.5‐34.2). Solitary auras were experienced by 83 (34%), of which 73 (30%) had experienced this twice or more frequently. The majority of the neurologists thought that migraine was underdiagnosed and undertreated, 70% and 68%, respectively. Conclusion.— The study confirms the results of previous studies, indicating that migraine, including visual aura, is more common among neurologists than what would be expected from population‐based studies. Because this group, through professional experience with the condition, can make accurate diagnoses in themselves, and will have fewer problems with recalling headache episodes, the prevalence figures obtained may give the most precise estimate of the true population prevalence.     

灵通胶囊治疗头痛的临床研究

目的:观察灵通胶囊对无先兆偏头痛(Migraine without aura)和高频发作性紧张型头痛(Fre-quent episodic tension-type headache,FETTH)的即时止痛疗效。方法:头痛患者共95例随机分配到灵通组和元胡组(对照组)。其中无先兆偏头痛入灵通组25例服药51例次,入元胡组28例服药53例次;FETTH入灵通组25例服药39例次,入元胡组17例服药27例次。分别于疼痛时即时服用灵通胶囊2粒和元胡止痛颗粒2包。将疼痛程度分级量化,由患者记录服药前和服药后2小时内每30分钟时的疼痛程度。以服药后2小时疼痛减轻程度≥50%作为有效。结果:无先兆偏头痛有效率在灵通组为68.6%,在元胡组为32.1%(P<0.001);FETTH有效率在灵通组为76.9%,在元胡组为59.3%(P>0.05);两种头痛的综合有效率在灵通组为72.2%,在元胡组为41.3%(P<0.001)。灵通组在服药30分钟后各时点的疼痛评分显著低于元胡组(P<0.001)。结论:灵通胶囊对两种头痛有明显的即时止痛疗效。     

Persistent and Repetitive Visual Disturbances in Migraine: A Review

Visual disturbances in migraineurs, such as visual aura, are typically episodic, that is, associated with the headache attack, and overlaid by head pain and other symptoms that impact the patient. In some patients, however, visual symptoms are dominant due to frequency (migraine aura status), duration (persistent migraine aura and other persistent positive visual phenomena), or complexity (visual snow syndrome). These syndromes are more rare and challenging to classify in clinical practice resulting in a lack of systematic studies on pathophysiology and treatment. We aim at describing clinical features and pathophysiological concepts of typical migraine aura with a focus on cortical spreading depression and differentiation from non‐typical migraine aura. Additionally, we discuss nomenclature and the specifics of migraine aura status, persistent migraine aura, persistent positive visual phenomena, visual snow, and other migrainous visual disturbances. The term migraine with prolonged aura might be a useful bridge between typical aura and persistent aura. Further studies would be necessary to assess whether a return of the classification category eventually helps diagnosing or treating patients more effectively. A practical approach is presented to help the treating physician to assign the correct diagnosis and to choose a medication for treatment that has been successful in case reports of these rare but disabling conditions.