Migraine with Persistent Aura in a Mexican Patient: Case Report and Review of the Literature

Abstract

Persistent aura symptoms in patients with migraine are rare but well documented. The International Headache Society defines persistent aura without infarction as when the aura symptoms persist for >1 week without radiographic evidence of infarction. The visual aura of migraine attacks has been explained by cortical spreading depression. We describe a case of a 28-year-old Mexican woman, who presented with persistent aura symptoms, and a literature review. The patient had a 24-year history of migraine headache. In November 2005 the patient had an attack which started with scintillating scotomas bilaterally associated with photopsias and amaurosis followed by migraine headache. All imaging studies were negative. The episode lasted 35 days and probably resolved with nimodipine therapy. Persistent aura symptoms are rare entities. This is the first case documented of a Mexican patient with persistent aura without infarction and probably resolved with nimodipine therapy.

Introduction

The migraine aura may be defined as any neurological disturbance that appears shortly before or during the development of migraine headache. Migraine auras may have different features, suggesting involvement of different brain regions (1). The International Headache Society defines persistent aura without infarction as when the aura symptoms persist for >1 week without radiographic evidence of infarction. Although well defined, this presentation is uncommon (2).

We report the first Mexican case and review published cases and potential mechanisms.

Case report

We present a 28-year-old woman with a history of migraine in association with typical aura since the age of 4 (scintillating bilateral scotomas). These events recurred every 4 months. The patient was not taking prophylactic therapy. None of her first-degree relatives had a history of migraine. In September 2005 the frequency of attacks worsened and scotomas began to be persistent and accumulative. She started topiramate and sumatriptan therapy without any response. In November 2005 the patient had an attack which initiated with scintillating bilateral scotomas as well as photopsias and amaurosis followed by migraine headache. On admission she did not present with a history of drug or contraceptive use. Physical, neurological, psychiatric and ophthalmological examinations were normal, as were the basic laboratory work-up, prothrombotic profile, inmunological tests and computed tomography performed during the attack. Interictal studies of EEG, visual evoked responses, electroretinogram and cerebral magnetic resonhance imaging (MRI), perfusion- and diffusion-weighed images, magnetic resonance (MR) angiography and MR venography were also normal. The fMRI with visual paradigm en bloc showed left occipital activation and there was decreased transit mean time in the right frontal region on perfusion-weighed images. She started nimodipine therapy. The episode lasted 35 days and probably resolved with the current treatment.

Search strategy and selection criteria

References were identified by searches of PubMed from 1966 until August 2006 with the terms ‘migraine’, ‘headache’ and ‘migraine persistent aura’. Only papers published in English were reviewed.

Discussion

The persisting aura symptoms are rare but well documented. Haas reported two individuals with migraine and prolonged aura; the first was a 70-year-old man with a longstanding history of migraine headache who developed a constant ‘pinwheel of bright whirling colour, mainly yellow and red’ in the left homonymous hemifield accompanied by left-hand paraesthesias and clumsiness. The episode lasted 5 weeks and resolved with aspirin and cyproheptadine. The second patient was an 18-year-old man with a history of migraine with aura who experienced 7 months of concentric grey circles like ‘ripples in a pond’ and ‘clustered sets of concentric circles in the right visual field’, unaccompanied by headache (3). Luda et al. reported a 65-year-old woman with ‘sustained visual aura’ who had a 50-year history of migraine with aura and then developed 12 months of ‘scintillanting geometric figures (in the shape of either rings or chains)’ in the right visual hemifield. Single photon emission computed tomography demonstrated relative hypoperfusion of the left parieto-occipital and frontal areas (4). Liu et al. recently reported 10 patients with migraine who developed persistent positive visual phenomena lasting months to a year. The complaints were similar in their simplicity and involvement of the entire visual field and usually consisted of diffuse small particles such as TV static, snow, lines of ants, dots and rain (5). In all cases other causes of visual alterations were excluded. To the best of our knowledge, only 29 cases of such a complication have been reported as ‘sustained visual aura’ or ‘persistent positive visual phenomena’ (Table 1) (6–9). It is important to note that in only 16 of these cases was an MRI performed, eliminating the possibility of a vascular event causing the symptomatology.

Table 1

Characteristics of 29 patients with persistent aura reported in the literature since 1982

References	Age/sex	Duration	Aura	Therapy (aura)		MRI
				Unresponsiveness	Responsiveness
Haas et al. 1982 (3)	70/M	5 weeks	‘Pinwheel of bright whirling colour, mainly yellow and red’, in the left homonymous hemifield accompanied by left-hand paraesthesias and clumsiness	ASA and	cyproheptadine	–
	18/M	7 months	Concentric grey circles like ‘ripples in a pond’ and ‘clustered sets of concentric circles in the right visual field’	Absent		–
Luda et al. 1991 (4)	65/F	12 months	‘Scintillanting geometric figures (in the shape of either rings or chains)’ in the right visual hemifield	CBZ, DZP, flunarizine, nimodipine, citicoline	Absent	Normal
Liu et al. 1995 (5)	32/F	4 years	Photopsias as floating in space, with flickering quality	Verapamil, ASA, fluoxetin, baclofen, CBZ	Absent	Normal
	26/F	2 months	A spiral, with spokes	Amitriptyline	Absent	–
	29/F	5 months	Flashing lights and circles	PHT, CBZ, verapamil, nifedipine, PB, amitriptyline	Absent	–
	9/F	2 months	Constant blob of white and grey, squiggles, clouds, comets, bubles, ‘lines of ants’, ‘a million dots’	Ibuprofen, amitriptyline	Absent	Abnormal
	67/F	4 months	‘Black cracks and lines’	ASA and amitriptyline	Absent	–
	37/M	6 months	Blurry and ‘grey’ vision in the left lower quadrant of both eyes	Nortryptiline and CBZ, nifedipine, sertraline	Absent	–
	20/F	2 years	White and black dots, ‘snow’, and ‘TV static’ over her entire visual field and palinopsia	Verapamil, CLZ and ASA	Absent	Abnormal
	30/M	8 months	‘Snow’ and ‘flickering’	Absent	Absent	–
	23/F	3.5 years	‘Heat waves’ with flickering lights		Absent	–
	36/M	5 years	‘Bright white snowflake spinning in front of his eyes’
Rothrock 1997 (6)	61/F	2 months	Scotomas just to the left of centre in both eyes with scintillations	Propranolol, sertraline	Divalproex sodium	Normal
	53/F	4 years	‘Bright flashing dots of lights, zig-zags, blind spots, sparkles’ in all visual field	Propranolol, metoprolol, naproxen, amitriptyline, verapamil and sumatriptan	Divalproex sodium	Normal
Mathew 1998 (15)∗	?	?	?		Dihydroergotamine	?
Evans 2000 (8)	33/F	11 days	Numbness of her left cheek, mouth and ar		Furosemide Verapamil	Normal
Rozen 2000 (9)	34/F	11 days	Loss of vision in her outer visual field. Numbness in her right face, arm and leg	Methylprednisolone Divalproex sodium	Furosemide	Normal
	37/F	5 days?	Small bright flashes of light, zigzag lines, and fortification spectra in both visual fields	Methylprednisolone Magnesium	Furosemide	Normal
Chen et al. 2001 (7)	45/F	3 months	Coin-sized white spot with occasional flickering in the left hemifield		Lamotrigine	Normal
	24/F	3 years	Numerous stars persistently flickering in her right hemifield	Flunarizine, atenolol, propranolol	Lamotrigine	Normal
Relja et al. 2004 (13)	43/F	18 months	Bright halo on the right edge of the objects and a scintillanting scotoma with grey and white squares in the right hemifield	Lamotrigine	Absent	Normal
Jager et al. 2004 (17)	39/F	28 years	Small yellow/white dots, blurring left hemifield	Acetazolamide, amitriptyline, pizotifen and propranolol	Absent	Normal
	46/F	4 years	Coloured blotches	Pizotifen, VPA, flunarizine, TMP, dothiepin	Absent	Normal
	27/F	5 years	Shimmering pin points of lights		Absent	Normal
	33/F	3 years	Like looking at bright light flashes of lights		Absent	Normal

∗

Included four patients without information.

Visual migrainous aura consists of visual symptoms including positive features and/or negative features, much rarely metamorphopsias or palinopsia. The visual aura of the migraine attack has been explained by cortical spreading depression (CSD), a neuroelectric event beginning in the occipital cortex and propagated into contiguous brain regions (10), and studies with blood oxygen level-dependent fMRI of visually triggered headache in patients with migraine have shown spreading suppression of initial neuronal activation and increased occipital cortex oxygenation (11). Studies showed, during the attacks, alterations in relative cerebral blood flow, cerebral blood volume and tissue mean transit time in the grey matter of occipital cortex contralateral to the affected visual hemifield, and, more rarely, hypoperfusion of the whole hemisphere (12, 13). This finding was confirmed by positron emission tomography (14, 15). In our patient all the functional studies done after the attack were normal.

The pathophysiology of sustained visual aura is not yet established. Pathophysiological mechanisms suggested in magnetic resonance spectroscopy studies in migraineurs with typical aura, i.e. abnormal energy metabolism in the brain, brain magnesium levels significantly lower than those of non-migraineurs, greater reactivity of N-methyl-D aspartate receptors to glutamate, a lowered threshold to CSD, and loss of inhibitory gabaergic interneurons, may combine to cause sustained visual aura. It has also been hypothesized that sustained reverberating waves of CSD may be implicated (1, 4, 5, 7, 13, 15).

Nimodipine therapy decreased the frequency of headaches, whilst the visual phenomena persisted and resolved spontaneously 1 month later. The mechanism of action of calcium antagonists in the prophylaxis of migraine is still not completely understood. One experimental study has shown that nimodipine (l-type voltage-gated calcium channels) inhibits repetitive CSD (16). The mechanism is unclear, but as l-type channels control excitability, changes may affect the release of transmitters (16). The outcome with other therapies is also controversial (3, 6–9, 15).

Competing interests

None declared.

Acknowledgements

We thank B. Vanesa Alatriste for her excellent technical assistance with the translation.