BRIEF COMMUNICATION The pathological spectrum behind migraine aura status: a case series Alberto Terrin 1 & Federico Mainardi 2 & Ferdinando Maggioni 1 Received: 17 August 2018 /Accepted: 29 November 2018 # Fondazione Società Italiana di Neurologia 2018 Abstract Background The recently released International Classification of Headache Disorders—3rd edition (1) includes migraine aura status (MAS) among the complications of migraine (A1.4.5). It is defined as the recurrence of at least three auras over a period of 3 days, in a patient suffering from migraine fulfilling criteria for 1.2 Migraine with aura (MA) or one of its subtypes. Case series We describe three cases of MAS secondary to an organic brain lesion: a migrainous infarction, an acute ischemic stroke secondary to a vertebral artery dissection, and an inflammatory demyelinating disease of the central nervous system. Conclusions In front of a patient with a MAS, an organic lesion of the brain must be suspected, until a complete negative vascular and neuroradiological diagnostic workup has been performed. A spectrum of underlying pathologies (vascular or demyelinating diseases, epileptic or degenerative conditions) may cause a MAS-like clinical onset. The variability of aura symptoms may result in a real diagnostic challenge. Keywords Migrainous infarction . Ischemic stroke . Inflammatory diseases . Recurrent auras . Spectrum Abbreviations MAS Migraine aura status MA Migraine with aura CT Computed tomography MRI Magnetic resonance imaging MRA Magnetic resonance angiography CTA Computed tomography angiography Introduction Migraine Aura Status (MAS) has been included among the complications of migraine, reported in the Appendix (A1.4.5) of the recently released International Classification of Headache Disorders – 3rd edition (ICHD-III) [1]. This clinical entity, ranging from 1.7 to 4.2% of consecutive patients observed in a tertiary headache outpatient center [2], is now defined as the recurrence of at least three auras over a period of 3 days, in a patient suffering from migraine fulfilling criteria for 1.2 Migraine with aura (MA) or one of its subtypes. Differently, the previous diagnostic criteria referred to MAS as the recur- rence of at least two auras per day for 3 days or more [3]. Here, we report three cases, which are clinically adherent to the new definition of MAS, but concealing completely different etiolo- gies. On their path, we emphasize the need to consider MAS as a potentially threatening clinical condition. Case reports Case 1 A 56-year-old woman, with a past medical history of autoim- mune hypothyroidism and mood disorders, had suffered from MA during her fertile age. Her attacks were preceded by scin- tillating scotomas, with a homonymous distribution in her binocular visual field, gradually extending and disappearing along a 30-min duration. Several years before, a brain mag- netic resonance imaging resulted unremarkable. Recently, she experienced a 4-year attack-free period. In the last 2 weeks, the patient experienced the return of her well-known MA at- tacks: her 30-min usual visual auras were followed by a mi- grainous headache. This time, a few visual symptoms without subsequent head pain were reported. Her auras gradually be- came more frequent, up to six visual auras a day. She was evaluated at our Headache Centre: the examination revealed * Alberto Terrin alberto.terrin89@gmail.com 1 Department of Neuroscience, University of Padova, Via Giustiniani 5, 35128 Padova, Italy 2 Headache Centre, Hospital SS, Giovanni and Paolo, Venice, Italy Neurological Sciences https://doi.org/10.1007/s10072-018-3671-0