Persistent Migraine Aura: New Cases, a Literature Review, and Ideas About Pathophysiology

Sam Thissen, MD; Iris G. Vos, MD; Tobien H. Schreuder, MD; Wendy M.J. Schreurs, MD; Linda A. Postma, PhD; Peter J. Koehler, PhD

Disclosures

Headache. 2014;54(8):1290-1309. 

In This Article

Abstract and Introduction

Abstract

Background.—Persistent migraine aura without infarction (PMA) is a rare condition that is defined as an aura that lasts longer than 1 week in absence of infarction. Two types of PMA have been distinguished, notably persistent primary visual disturbance (PPVD) and typical aura (TA).

Objectives.—This case-based review article describes four new cases of PMA as well as reviews all cases reported, trying to identify relevant associations, in particular with respect to functional investigations.

Methods.—We performed a systematic literature search, extending from the period when it was first described (1991) to March 2014. We included all case descriptions of which criteria for PMA formulated in the International Classification of Headache Disorders, second edition, were met. In addition, we described four new cases.

Results.—We identified 47 cases of PMA, 27 PMA-PPVD and 19 PMA-TA. In one case, there was not enough information to define the type of PMA. The mean age of onset was 30 years, varying from 7 to 74 years. The duration of symptoms varied from 9 days to 28 years. Besides a longer duration in symptoms in the PMA-PPVD group, we could not identify any differences between these groups. Some authors report occipital hypoactivity on Tc99m-hexamethylpropylene amine oxime -single-photon emission computed tomography (Tc99m-HMPAO-SPECT) or fluorodeoxyglucose-positron emission tomography (FDG-PET) in PMA cases, but data are inconsistent. Multiple drugs have been used for the treatment of PMA, usually with little effect. Lamotrigine seems to be the most effective drug.

Conclusion.—Despite the fact that 47 cases of PMA have been reviewed in this paper, many questions remain. The cases that have been described so far show inconsistent data with respect to the results of functional studies as well as treatment effects. The pathophysiology of PMA is still largely a matter of conjecture.

Introduction

Migraine is a common and disabling disorder. In migraine with aura (MA), the aura is a most interesting phenomenon that is thought to be associated with Leao's cortical spreading depression. About 20% of migraineurs experience MA, of which 99% is visual.[1] Migraine auras are recurrent attacks of reversible focal neurological symptoms that typically spread in 5–20 minutes, and each symptom does not last more than 60 minutes. If these symptoms last longer, between 60 minutes and 1 week, they are called prolonged aura. MA is a risk factor for cardiovascular disease.[2] Persistent migraine aura without infarction (PMA) is a condition in which auras last longer than 1 week, in absence of radiological evidence of infarction.[3]

Luda et al were the first to report PMA in, a 65-year-old patient with a history of migraine. She experienced a sustained visual aura for over 12 months, following a typical MA attack.[4] Cases of PMA seem to be an extreme of the spectrum. Lance and Goadsby distinguished two types of PMA. First is PMA with typical aura (PMA-TA), in which patients experience a persistent typical migraine aura with oscillation, scotoma, and fortification in one hemifield. The second type is called persistent primary visual disturbance (PMA-PPVD). These patients describe "visual snow" or "television static" in the whole visual field of both eyes, in addition to intermittent scotoma or oscillating lights.[5] A number of PMA cases have been reported previously with inconsistent data with respect to diagnostic tools and treatment.

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