Elsevier

Survey of Ophthalmology

Volume 56, Issue 2, March–April 2011, Pages 135-161
Survey of Ophthalmology

Major Review
Migraine-like Visual Aura Due to Focal Cerebral Lesions: Case Series and Review

https://doi.org/10.1016/j.survophthal.2010.07.005Get rights and content

Abstract

Visual aura is a common presenting symptom of migraine to both neurologists and ophthalmologists. Features such as photopsia, fortification spectra, and the slow propagation of a scintillating scotoma across the visual field are usually considered diagnostic features of the visual aura of migraine. In the vast majority of cases, the diagnosis can be made without the need for further investigations. We present 9 patients and a further 31 cases from the literature who experienced visual aura fulfilling the diagnostic criteria for migraines but caused by focal occipital pathology. Key clinical features that could help to differentiate between the visual aura of migraine and those suggestive of a structural lesion are outlined. We review current scientific theories into the pathophysiology of visual aura, drawing on clinical and basic science research, including human functional imaging studies of migraine aura and advances in the genetic characterization of familial channelopathies, in order to explain the overlap which occurs in the clinical features of visual aura associated with migraine, cortical lesions, and epilepsy. We conclude that any disease process that is able to create a state of neuronal hyperexcitability can therefore increase an individual’s susceptibility to the development of cortical spreading depression, the electrophysiological correlate of the visual aura.

Introduction

Ten percent (10%) of the general population are active migraineurs,94 and a third of all migraine attacks are associated with aura.96 Positive, episodic visual phenomena are the most common form of aura in migraine (99%);94 may also be the first and only ictal manifestation of occipital lobe epilepsy75, 104 or seizures arising from any part of the visual pathway; and can occur in the context of focal cerebral pathology, in particular of the visual cortex. Notably, post-ictal headaches often indistinguishable from migraine occur in over 50% of patients, even after brief visual seizures;84, 85 following a similar “asymptomatic interval” as seen in migraine.11 Headache also occurs in more than 60% of patients with brain tumors over the course of their disease.100

Section snippets

The Classic Visual Aura of Migraine

Most patients with typical migrainous visual aura experience a biphasic aura preceding the onset of headache that consists of a homonymous scotoma of shimmering lights (positive visual phenomena), in most cases originating from the primary visual cortex, usually restricted to one half of the visual field. Typically the scotoma arises near to fixation and gradually enlarges in the form of an arc, with scintillating or sparkling zigzag lines at its leading edge. The arc gradually moves to the

Typical Migraine-like Visual Aura Not Due to Migraine

The diagnostic criteria for typical migraine visual aura as defined by the International Classification of Headache Disorders (ICHD)43 excludes any organic disease that may cause headache (Table 1). It is a commonly held view that the visual disturbances caused by cortical lesions do not conform to the time, pattern, appearance, or manner of spread of migraine scintillations.41 Migraine-like visual aura otherwise fulfilling the ICHD criteria, however, have been reported to occur with a variety

Migraine-like Visual Aura: Migraine or Structural Intracranial Pathology? The Evidence

A third of migraineurs, or 1–3% of the general population, will present to their general practitioner, neurologist, or ophthalmologist complaining of visual aura. The occurrence of typical migraine-like visual aura as the result of a structural lesion, in the absence of other neurological signs or symptoms, is rare, yet knowing which patient requires neuroimaging is a common clinical problem.

In order to critically examine the true nature of visual aura secondary to structural lesions and

1. Scintillating Scotoma or Teichopsia

The semiology of the visual aura of typical migraine varies considerably; however, the most common is the fortification spectrum—otherwise known as teichopsia—89 an expanding arc of zigzag lines orientated at 60° with respect to one another, generated from activation of the orientation specific ocular dominance columns in the striate cortex.38 All nine of our cases had been diagnosed as idiopathic migraine on the basis of the semiology of their visual aura and serve to illustrate the point that

Visual Aura: Migraine or Epilepsy?

The relationship between migraine and epilepsy has been debated for more than 100 years.1, 66 Both are chronic neurological disorders with episodic and overlapping clinical manifestations, characterized by recurrent attacks of central nervous system dysfunction. The biological abnormality in these disorders is sufficiently mild that patients are phenotypically normal between attacks, and factors such as fatigue, stress, exercise, and certain foods can precipitate attacks.90 Both conditions can

Cortical Hyperexcitability: A Shared Pathophysiology and Unifying Theory?

Alterations in cortical blood flow are no longer regarded as the primary etiology of migraine, but are secondary to changes in neuronal activity, so-called “vasoneuronal coupling.”56 Migraine, like epilepsy, is now considered to be a disorder of neuronal hyperexcitability118 or an expression of disturbed cortical activity.112 It is no surprise then that both inherited and acquired brain diseases are the most important risk factor for the comorbidity of epilepsy and migraine.82 Disorders which

VIII. Conclusion

We hypothesize that, when structural lesions produce visual symptoms with the semiology of migraine, they share a common pathophysiology: namely, a state of neuronal hyperexcitability. Whether due to a genetic predisposition, acquired through brain injury, ionic dysregulation, or a structural lesion, a state of neuronal hyperexcitability is thought to increase susceptibility to the generation of CSD, the electrophysiological correlate of the visual aura in migraine. The propagation of spreading

IX. Method of Literature Search

References for the literature review were identified by conducting a Medline search using the PubMed database (National Library of Medicine) and EMBASE search using OVID (Walters Kluwer) from 1950 until December 2009 with the terms migraine visual aura, cerebral lesions, visual hallucinations, occipital lobe epilepsy, and epileptic aura. Articles were also identified through citations in related articles and neuro-ophthalmic textbooks, in particular Walsh & Hoyt’s Clinical Neuro-Ophthalmology40

X. Disclosure

The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.

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    We wish to thank Mr. Nigel P Davies, Dr Randy H. Kardon and Mr. Timothy D. Matthews for their comments on this manuscript, and Ms. Sara Stock, Ms. Eileen Scrivens and Ms. Debbie Heatlie for their help in obtaining the articles for the review.

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