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Migraine Aura Without Headache by Shih-Pin Chen MD Phd (Dr Migraine aura without headache By Shih-Pin Chen MD PhD (Dr. Chen of the National Yang-Ming University School of Medicine has no relevant financial relationships to disclose.) Originally released December 30, 1993; last updated March 20, 2017; expires March 20, 2020 Introduction This article includes discussion of migraine aura without headache and acephalgic migraine. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations. Overview Migraine is a common neurologic disorder that is prevalent in the younger population. With age, migraine prevalence decreases, but some people continue to experience migraine auras without the subsequent or associated headache pain. In this article, the author reviews the clinical manifestations, prevalence, pathophysiology, therapeutic options, and prognosis for this selective group of patients. Breakthroughs in understanding the pathogenesis and clinical manifestations are highlighted. Key points • Typical aura is consisted of visual, sensory, or speech symptoms with a mix of positive and negative features and complete reversibility. • Migraine aura could initiate from multiple distinct sites, propagate nonconcentrically with a variable extent in the occipital cortex, and can sometimes be clinically “silent”. • Differential diagnoses, including transient ischemic attack, should be considered when aura is late-onset with predominant negative features or is prolonged or of very short duration. • Cortical spreading depression, glutamatergic neurotransmission, channelopathies, neuronal-glial gap-junction communications, and microembolization might be important players in the pathogenesis of migraine aura. • Migraine with aura is associated with higher risks of subclinical brain lesions, ischemic or hemorrhagic strokes, and all cause mortalities; whether this remains true for “migraine aura without headache” requires further studies. Historical note and terminology Migrainous aura has been used to explain unusual visions, experiences, and perceptions that have been experienced by well-known personages. Lewis Carroll's pictorial descriptions in Alice in Wonderland and Alice Through the Looking Glass have been ascribed to his migrainous auras. His depiction of Alice may be a manifestation of the micropsia, macropsia, or metamorphopsia seen in migrainous auras of childhood. There have also been suggestions that the painter Pablo Picasso may have had migrainous auras. His works feature illusory splitting in the vertical plane of his subjects' faces, and this has been compared to similar paintings by migraine patients depicting what they see during their aura phase (Podoll 2000). The absence of descriptions of the painter suffering from headaches may infer the presence of migraine aura without headache. Fisher described a series of 120 patients: 25 had only visual symptoms; 18 had visual symptoms and paresthesias; 7 had visual symptoms and speech disturbances; 14 had visual and brainstem symptoms; 7 had visual symptoms, paresthesias, and speech disturbances; 25 had visual symptoms, paresthesias, speech disturbances, and paresis; 9 had recurrence of old stroke deficit; and 8 had miscellaneous symptoms (Fisher 1980). In 1986, a study of 85 cases demonstrated that 21 patients had visual symptoms; 6 had visual symptoms and paresthesias; 2 had visual symptoms and speech disturbances; 3 had visual symptoms, paresthesias and speech disturbances; 20 had visual symptoms, paresthesias, speech disturbances, and weakness; 3 had visual and brainstem symptoms; and 32 were without visual symptoms. Their ages ranged from 40 to 73 years. In only 40% of cases did headache occur in association with the episodes. These episodes have been coined ‘‘late-life migraine accompaniments,” “migraine equivalents,” “acephalic migraine,” or “migraine aura without headache.” Clinical manifestations Presentation and course Historically, an aura without migraine headache was described as slowly evolving patterns of scintillating scotomas that have precisely the same range of patterns and duration of evolution. On reaching middle age, patients with typical migraine preceded by aura can continue to have auras without the succeeding headache. It can be preceded by hunger, tiredness, or increased micturition frequency, similar to the prodromes of migraine attacks, and it can be followed by lassitude and photophobia, which are frequent migraine postdromal symptoms. Precipitants of migraine aura without headache (ie, hunger, bright lights, insufficient sleep, and anoxia) are also triggers of migraine with and without aura. Fisher has published a personal account of life with migrainous auras without headache (Fisher 1999). He described 41 episodes of scintillating zigzags that occurred when he was between 59 and 85 years of age. The spells occurred irregularly and were unrelated to season of the year, time of day, activity at onset, diet, or temperamental state. The characteristic appearance was a flickering zigzag line that began centrally and migrated to the periphery. The display, which was stereotyped, was achromatic. The average duration was 15 minutes. Both visual fields were equally involved, though never at the same time. Other migrainous auras unaccompanied by headache include paresthesias, speech disturbances, motor weakness, and brainstem symptoms, as depicted in Fisher's report (Fisher 1980). A multicenter study in patients with migraine with aura showed that visual aura symptoms were variable and often overlapping, and approximately half of the patients reported nonvisual aura symptoms, with sensory and speech symptoms being the most common (Hansen et al 2016). Of note, prospective recordings for 861 aura attacks in this study showed that 27% of the aura attacks were not followed by headache, in contrast to 4% to 10% in previous studies. The International Classification of Headache Disorders, 3rd edition (beta version), proposed the diagnostic criteria for typical aura without headache (code 1.2.1.2), which allows the aura symptoms to be one or more of the followings: visual, sensory, speech and/or language, motor, brainstem, and retinal (Headache Classification Committee of the International Headache Society 2013). These aura symptoms should fulfill at least 2 of the following characteristics: 1.) At least 1 aura symptom spreads gradually over 5 minutes, or 2 or more symptoms occur in succession; 2.) Each individual aura symptom lasts 5 to 60 minutes; and 3.) At least 1 aura symptom is unilateral. In a systemic review, however, it was found that a nonhemiplegic migraine aura could last longer than 1 hour in a significant proportion of migraineurs, especially in patients with nonvisual aura symptoms (Viana et al 2016). A prospective study also showed that 14% of visual aura symptoms, 21% of sensory symptoms, and 17% of dysphasic symptoms last for more than 1 hour (Viana et al 2013). The term “prolonged aura” might be clinically useful for atypical cases. Prognosis and complications Unlike migraine headache, which frequently lessens or even disappears after 55 years of age, migraine aura without headache often persists without permanent deficit into the 70s and 80s. Female migraineurs without aura who develop an aura while on combined oral contraceptives must stop this mode of contraception, as they are at risk for developing strokes (MacGregor and Guillebaud 1998). A few cases of patients with migraine headache without aura developing a retinal infarct have been recorded. A report suggested that patients suffering from migraine with aura and having more than 1 migraine attack a month have a greater risk of having a subclinical infarct in the cerebellar area as seen on MRI (Kruit et al 2004). Subsequent studies also demonstrated increased risks of transient ischemic attacks (Rist et al 2010), ischemic infarcts (Kurth et al 2011), hemorrhagic strokes (Kurth et al 2010), cardiovascular events (Gudmundsson et al 2010), and all-cause mortalities (Gudmundsson et al 2010) in patients with migraine with aura. This has implications for patients having migraine aura without headache, as they tend to be older with more risk factors for having stroke and, therefore, an argument may be made to treat these patients with preventive therapy. Although solid data are still lacking, controlling traditional vascular risk factors is believed to be the mainstay in preventing these complications. Clinical vignette A 69-year-old man reported that his first attack of migraine aura without headache had appeared 4 years earlier. While reading the morning paper, the patient noticed that the print looked a little unclear in the center of his vision. A bright dot appeared 2 to 3 minutes later; this gradually spread into his upper left visual field, making an arc-shaped zigzag. It took about 5 minutes to develop to its full extent; it lasted another 10 minutes and then disappeared suddenly. There was no ensuing headache. The man had 3 more attacks in the next 2 years without any trigger being identified. He emphasized that he was retired and under no stress. He had never experienced any migrainous symptoms prior to the first attack. Biological basis Etiology and pathogenesis Migraine aura without headache is considered a migraine-associated phenomenon. Both vascular and neurogenic causes of migraine have been proposed, but it now appears that the 2 causes are tightly intertwined. Following are some of the prevailing theories: Cortical spreading depression. The spreading cortical depression of Leao, a primary brain
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