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Show Reversible Prolonged Bilateral Inferior Altitudinal Visual Field Defects Associated With Migraine Hartej S. Sethi, MD, Byron L. Lam, MD, Jose G. Romano, MD Abstract: A 14-year-old girl with a history of migraine head-aches and methylphenidate use presented with 2 episodes of prolonged but completely reversible inferior altitudinal homonymous visual field loss, lasting 5 days and 4.5 weeks, respectively. Neuroimaging studies were unremarkable. We discuss the potential causes of our patient's visual symp-toms and speculate and hypothesize that it may represent an atypical manifestation of migraine, perhaps related to a channelopathy. Journal of Neuro-Ophthalmology 2012;32:252-255 doi: 10.1097/WNO.0b013e31824f3a1c © 2012 by North American Neuro-Ophthalmology Society Transient visual field defects are a relatively common accompaniment of migraine headaches. These usually last a few minutes to an hour, but prolonged visual field loss has rarely been described (1). Visual field defects that persist are usually caused by permanent visual pathway damage. We present a patient with reversible, recurrent, prolonged, bilat-eral, inferior altitudinal visual field defect that initially was thought to represent cerebral ischemia and discuss potential mechanisms causing this rare manifestation of migraine. CASE REPORT A 14 year-old girl experienced sudden onset of change in color perception described "as if seeing through a dark red lens" in the lower visual fields of both eyes. The patient had no associated symptoms and had no headaches. Medical history was remarkable for a 5-year history of throbbing headaches and associated with photophobia and phonopho-bia. The headaches usually lasted a few hours, were often preceded by 30 minutes of nausea and scintillating scotomas, and were triggered by stress and the smell of chocolate. They were relieved by ibuprofen and sleep, and there was a strong paternal family history of migraine. The patient had been diagnosed with attention-deficit hyperactivity disorder and for which she was taking methylphenidate. One day after the onset of symptoms, the patient's visual acuity was 20/20 in each eye with no relative afferent pap-illary defect. Ocular motility and anterior segment exami-nations were normal as were intraocular pressures (16 mm Hg, right eye; 15 mm Hg, left eye). Automated perimetry (24-2 threshold, SITA-standard strategy; Zeiss-Humphrey Division, Dublin, CA) showed bilateral homonymous infe-rior visual field defects (Fig. 1). The fundi were normal. Two days after the onset of symptoms, MRI with FLAIR, diffusion weighted and T1 with and without contrast seq-uences of the brain, and fat saturation of orbits were unremarkable. The visual field defects resolved over the next 5 days. Five and a half months later, while in school, the patient developed sudden painless loss of the lower half of the visual field in each eye. She had no other symptoms, including headache, nausea, or vomiting. The patient was still on methylphenidate for attention-deficit disorder. Three days later, the patient's visual acuity was 20/20 bilaterally, with a normal ophthalmic examination. Automated perimetry (30-2 SITA standard) showed dense bilateral inferior field defects (Fig. 2A). General physical and neurologic exami-nations were normal. Repeat brain and orbital MRI with and without contrast was normal. Magnetic resonance angiogram of the neck, transthoracic echocardiogram, transcranial electroencephalo-gram, and a shunt detection study with transcranial Doppler failed to show any abnormality. Extensive laboratory testing, including complete blood count, serum chemistries, a hyper-coagulable and rheumatologic panels, serum lactic acid and pyruvic acid levels, was normal. Three weeks after the onset of symptoms, the patient retained 20/20 acuity in each eye, and the visual field defects were smaller (Fig. 2B). Over the following week, her visual field defects resolved. Department of Neurology (HSS, JGR) and Bascom Palmer Eye Institute (BLL), University of Miami Miller School of Medicine, Miami, Florida. The authors report no conflicts of interest. Address correspondence to: Jose G. Romano, MD, 1120 NW 14th Street, Suite 1357, Miami, FL 33136; E-mail: jromano@med.miami.edu 252 Sethi et al: J Neuro-Ophthalmol 2012; 32: 252-255 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. The patient's methylphenidate was discontinued, and she was placed on aspirin and topiramate. She developed vomiting, and a metabolic panel showed slight hypoglyce-mia and metabolic acidosis. This resolved after topiramate was stopped, and she has not had any visual episodes over the ensuing 16 months. DISCUSSION In our patient, the history of headaches and the recurrent nature of the events suggested a migrainous etiology of the prolonged bilateral episodes of inferior visual field loss. Other diagnostic considerations included stroke, occipital seizures, glaucoma, optic neuropathy, metabolic encepha-lopathy, lactic acidosis, and stroke-like episodes (MELAS). All of these were unlikely in the setting of normal MRI studies, laboratory testing, ophthalmologic examination, and electroencephalogram. Visual symptoms are a frequent manifestation of mi-graine with aura (classic migraine). Visual accompaniments of migraine with aura include unformed flashes of lights (photopsias), enlarging blind spots with shimmering edges (scintillating scotomas), formations of dazzling zigzag lines (fortification spectra or teichopsia), and blurred, shimmer-ing, or cloudy vision. These visual phenomena spread or migrate across the visual field over several minutes. Less commonly, there are more complex visual disturbances such as metamorphopsia and palinopsia (2,3). Negative visual field defects, seen less often, are usually of short duration and frequently homonymous, indicating a cortical origin. They are followed by the typical migraine headache, but occasionally, the headache may be mild or absent, and the episode is termed a migraine aura without headache. This can present a diagnostic dilemma, as seen in our patient. Persistent aura without infarction is a rare but recognized entity. It is described in the International Classification of Headache Disorders, 2nd edition (ICHD-II) (1) as typical aura symptoms in patients with migraine with aura lasting more than 7 days in the absence of neuroimaging evidence of infarction. We are aware of 28 cases of prolonged visual phenomena probably related to migraine (4-15). However, in most described cases, the visual phenomena had "posi-tive" characteristics of scintillation, photopsias, or scintilla-tion. Only 4 of these had persistent "negative" symptoms of visual field loss (12-15), and altitudinal defects were described in 2 (14,15). One of these patients had persistent visual field defects at 6-month follow-up, and the other had complete resolution at 4 months. Our understanding of the pathophysiology of the visual aura of migraine is incomplete. Alteration in neuronal or glial ion channels lowers the threshold for depolarization in certain cerebral cortical locations. This results in a wave of depolarization followed by inhibition (cortical spreading FIG. 2. A. Recurrent bilateral inferior visual field loss. B. Three weeks later, the visual field defects are less pronounced. FIG. 1. Bilateral inferior visual field defects are seen 1 day after the onset of visual symptoms. Sethi et al: J Neuro-Ophthalmol 2012; 32: 252-255 253 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. depression). Imaging studies have demonstrated changes consistent with cortical spreading depression that correlate with migraine aura (16). There is consensus that during the migraine aura, brief transient cerebral hyperemia occurs, followed promptly by a decrease in regional blood flow lasting up to a few hours (17-19). These findings suggest that vascular mechanisms do not play a central role in pro-longed migrainous aura. It is more likely the result of neu-ronal depression in contiguous cortical areas, although the responsible mechanisms for this phenomenon are not well understood (20,21). The best-studied model for migrainous aura is familial hemiplegic migraine (FHM). In this condition, hemiplegia associated with migraine can last from minutes to hours, but symptoms lasting 4 weeks have been reported (22). The molecular correlates of FHM have been elucidated; 3 genes that transcribe membrane ion channel proteins have been implicated: CACNA1A (FHM type 1), ATP1A2 (FHM type 2), and SCN1A (22). In a mouse model of FHM type 1, subcortical spread of cortical depression to the striatum with reverberation of the spreading depression waves has been demonstrated (23). Such reverberation of spreading depression between cortical and subcortical structures could explain the prolonged nature of this depression in certain cases (24). The ATP1A2 mutation has a greater association with prolonged aura in hemiplegic migraine compared to other mutations (25). It is possible that such ion channel abnormalities that result in prolonged neuronal suppression play an important role in migrainous defects. Our patient was taking methylphenidate during both episodes of prolonged visual field loss. Cerebral vasculitis and stroke associated with methylphenidate use have been reported anecdotally in children (26) and adults (27,28), but no strokes occurred among 128,000 children taking meth-ylphenidate in a large cohort study using administrative databases (29). As methylphenidate is related to amphet-amines and stroke is associated with adrenergic agents, the suggestion that methylphenidate can cause stroke is not surprising. However, this is unlikely to be the cause of the prolonged visual field defects in our patient given their repetitive nature and normal neuroimaging studies. In fact, a pilot study of the use of amphetamines in migraine patients showed the use to be of benefit without serious adverse effects (30). 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