Dr. De Luca of the Neurology Unit University of Pisa has no relevant financial relationships to disclose.)
Dr. Baldacci of the Neurology Unit University of Pisa has no relevant financial relationships to disclose.)
Dr. Wang of the Brain Research Center, National Yang-Ming University, and the Neurological Institute, Taipei Veterans General Hospital, received consulting fees from Eli Lilly, Daichi-Sankyo, and Novartis.)
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.
Migraine is the most common neurologic disorder in the younger population and is significantly associated life-long disability (GBD 2015 Neurological Disorders Collaborator Group 2017). Migraine occurrence decreases with age, but rare accompaniments experienced by some patients (especially elderly and not necessarily migraineurs) are migraine auras without associated headache. In this article, the authors review the clinical manifestations, prevalence, pathophysiology, therapeutic options, differential diagnosis, and prognosis for this selective group of patients. Latest developments in understanding the pathogenesis and clinical manifestations are highlighted.
• Typical aura without headache consists of visual, sensory, or speech symptoms with a mix of positive and negative features and complete reversibility.
• One characteristic, such as duration, could not be respected in most cases; in fact, it is not rare for aura to last more than 1 hour, but usually patients will show at least 2 other of the typical characteristics (ie, gradual spreading over 5 or more minutes, 2 or more aura symptoms in succession with at least 1 unilateral or positive aura symptom).
• Cortical spreading depression, glutamatergic neurotransmission, channelopathies, neuronal-glial gap-junction communications, endothelial disfunctions, and microembolization might be important players in the pathogenesis of migraine aura.
• Migraine with aura has been associated with higher risks of subclinical brain lesions, ischemic or hemorrhagic strokes, atrial fibrillation, and other causes of increased mortality; 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 reported 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.
Aura typically begins before headache but could also occur simultaneously or without headache. Lashley provided the first assessment of temporal spreading with quantitative recording of migrainous scotomas and fortification patterns in 1941 (Lashley 1941).
Fisher described a series of 120 patients: the majority had visual symptoms alone or associated with paresthesias or speech disturbances, followed by brainstem symptoms and few motor deficits (Fisher 1980). He proposed the late onset (for the first time after the age of 45 years) of ‘‘scintillating scotomas, numbness, aphasia, dysarthria, motor weakness, and brain stem symptoms.” These reports were enriched by the further description of 85 new cases with similar results in 1986. Their ages ranged from 40 to 73 years. In only 40% of cases headache occurred in association with the episodes. These episodes have been coined ‘‘late-life migraine accompaniments,” “migraine equivalents,” “acephalic migraine,” or “migraine aura without headache.” The International Headache Society classified typical aura occurring in the absence of any headache as “typical aura without headache.”
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