Article ID: WMC003984 ISSN 2046-1690

Migrainous Binocular Peripheral Oscillopsia: A typical Persistent Visual Aura Without Infarction

Corresponding Author: Dr. Daniel E Jacome, MD, Dartmouth Hitchcock Medical Center Department of Neurology, One Burnham Street, Suite 2, 01376 - United States of America

Submitting Author: Dr. Daniel E Jacome, MD, Dartmouth Hitchcock Medical Center Department of Neurology, One Burnham Street, Suite 2, 01376 - United States of America

Article ID: WMC003984 Article Type: Case Report Submitted on:01-Feb-2013, 04:39:44 AM GMT Published on: 01-Feb-2013, 06:27:17 AM GMT Article URL: http://www.webmedcentral.com/article_view/3984 Subject Categories:NEUROLOGY Keywords:Migraine, Migraine aura, Oscillopsia, Headache, Vertigo, Status epilepticus How to cite the article:Jacome DE. Migrainous Binocular Peripheral Oscillopsia: A typical Persistent Visual Aura Without Infarction . WebmedCentral NEUROLOGY 2013;4(2):WMC003984 Copyright: This is an open-access article distributed under the terms of the Creative Commons Attribution License(CC-BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Source(s) of Funding: Self funded

Competing Interests: None

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Migrainous Binocular Peripheral Oscillopsia: A typical Persistent Visual Aura Without Infarction

Author(s): Jacome DE

Abstract population of migraine individuals belonging to the study group. The scale fundamentals call for assessment of duration, rate of progression, lateralization of the aura, and presence of An 18 year old female with history of migraine without and scintillation, that allows defining with greater aura reported the abrupt onset of continuous, irregular, precision if the symptoms experienced by the patient rapid (“shaking”) movements of objects perceived on represent a true visual migraine aura or a the periphery of her visual fields over both eyes, when non-migranous visual disturbance (1). The greater the fixating on any given target. Symptoms were not score assigned on the VARS (i.e., > than 5) the suppressed by monocular eye closure. In some form, greater diagnostic weight for migraine visual she experienced incomplete tunnel vision phenomena. The scale also facilitates the distinction (“pseudo-tunnel”), since the periphery of her fields was between classic visual migraine aura, normally not totally dark, lost coloration or was populated by preceding the headache and of longer duration, and phosphenes. She had some inconsistent “transient visual disturbance”, prevalent in adolescents, lightheadedness but no vertigo, and reported a of shorter duration and occurring during the headache moderate continuous mid-facial headache. She denied phase (2). By applying the VARS, Wang, et al, were symptoms referable to any other cranial nerve able to discriminate between the two groups (migraine dysfunction. Her ophthalmologic and repeated versus non-migraine) in a total of 29 patients, six of neurological examinations were normal showing their own (3). Individuals with greater VARS scores symmetric eye movements, normal saccades and approximate migraine and have a better prognosis for smooth pursuit, no and no ataxia. Her short term resolution of their symptoms. Visual auras brain MRI and MRA of the extra and intracranial may occur in the absence of headache (i.e. vessels were normal. Her EEG showed a normal “acephalgic migraine”) or headache may occur within background in the absence of a focal slowing, epileptic the context of persistent visual aura with variable discharges or periodic complexes. Her visual evoked features and degree of intensity (2,3,4). responses were symmetric with normal latencies. Her testing revealed no deficits, yet, she had Persistent aura without infarction refers to focal initially persistent peripheral movement over neurological symptoms (i.e., of visual nature in this both eyes. Her ocular symptoms lasted for several discussion) persisting for more than 1 week, in the weeks progressively dissipating with the prophylactic absence of radiographic evidence of ischemic lesions administration of topiramate. I suggest that this on brain imaging studies (5). If auras are intermittent, patient’s binocular peripheral oscillopsia represented occurring twice a day for 5 or more consecutive days persistent cortical oscillopsia without nystagmus, as a as a minimum, the patient is classified as exhibiting very rare atypical variant of migraine persistent visual “aura status” (5). In my experience these two aura without infarction. Cortical oscillopsia without definitions overlap in clinical practice and commonly nystagmus constitutes a neuro-ophtalmological are used interchangeably, given the circadian syndrome, recently validated in a patient with fluctuations on symptoms intensity and the neuromyelitis optica (NMO) and visual pathways participations of multiple variables in the clinical demyelinating lesions. In my belief, this patient’s construct. These variables are among others: stress symptoms probably emanated from sustained cortical levels, effect of sleep, effect of medication and the occipital hyper-excitability and from reverberating menstrual cycle in women. The actual clinical features spreading cortical depression (CSD). of visual auras are variable and often extensively complex to the point of almost achieving individually Introduction created outstanding artistic schemes in a significant number of patients, when interrogated in the clinical encounter. In general terms, auras include scintillating Visual auras are the hallmark of migraine with aura. scotoma, bilateral central scotoma, tunnel vision, Patterns of visual disturbance can be assessed by temporal crescent involvement, dyschromatopsia, applying the Visual Aura Rating Scale (VARS) to a amaurosis fugax, altitudinal loss of vision, transient

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hemianopia, inability to focus the eyes, and she had no fever. Her neurological was normal while (3,4). experiencing her visual symptoms and included a Variants on the theme are recognized as “visual normal eye examination. Her eye movements were full snow”, “primary persistent visual disturbance”, and and symmetric without nystagmus. Her ocular “persistent positive visual phenomena” (4,6,7). In saccades and her smooth pursuit were normal. Formal addition to imaging studies, electroencephalography ophtalmological examination was normal including slit (EEG), is indicated to discard “status epilepticus lamp examination, automated visual perimetry and migrainosus” (8). Of pertinent relevance, exceptional pattern shift visual evoked reponses. Her patients with occipital lobe epilepsy manifested by ictal electroencephalogram (EEG), brain MRI and MRI of symptoms simulating typical lateralized visual aura, the intra and extra-cranial circulation were normal. Her may also experience epileptic oscillopsia. Furthermore, peripheral binocular visual oscillations persisted for as illustrated by Perucca, et al, in a 56 year woman several weeks, eventually dissipating following the with occipital post-hypoxic perinatal porencephaly, administration of topiramate orally on incremental these ictal manifestations may associate with doses up a final dose of 50 mg twice a day. persistent headache on basis of secondary Eventually, when headache improved topiramate was “non-convulsive status epilepticus”, establishing the discontinued without relapse on her oscillopsia. occasional link between the epileptic process and Discussion cortical spreading depression (8). “Status epileptic migrainosus” can be equally observed in patients with “posterior reversible encephalopathy syndrome” Oscillopsia refers to a visual percept movement (PRES), as described by Palma, et al, in a patient with disorder in where the objects in the environment move PRES secondary to cetuximab (9). Finally, patients back and forth, up and down or side to side, in a with persistent visual aura without infarction may show disconcerting oscillation (11). Transient environmental focal cerebral hypo-perfusion solely detected by oscillopsia is common as a compensatory corrective perfusion MRI, or by single photon emission computed counter movement sensation, as when coming down tomography (SPECT), yet, in the absence of clinical from an elevator ride for instance. Typical recurrent or deficits or delayed radiographic evidence of cerebral non-physiological oscillopsia, is a cardinal sign of infarction (10). superior oblique myokimia (SOM) among other Case Report conditions (12). Symptoms in SOM arise from irregular involuntary spontaneous contractions of the superior oblique muscle pulling the eye “down and in”. An 18 year old female college freshman was seen in Individuals suffering with this condition describe neurological consultation because of the rapid monocular, intermittent, spontaneous oscillation of development of “shaky vision”. She described that perceived targets that is not gaze dependant. The objects or any background on the periphery of her oscillation occupies the entire visual field, rather than visual fields were trembling of moving in irregular only the periphery. Nystagmus is present but maybe fashion without specific direction, and that her subtle, intermittent, or both, therefore it may not be symptoms were initiated by attempting central fixation apparent to the clinical observer, unless on any given target. Closing one eye did not suppress electro-oculography (EOG) or orbital ultrasound is the movement on the open eye. She never had performed. Patients with SOM have no headache or experienced this before. Her visual symptoms were additional visual symptoms other than periodic diplopia associated initially with lightheadedness, or polyopia, while they respond readily to and mild postural imbalance. In addition she reported anti-epileptics agents and to surgical microvascular a moderately intense mid-facial that soon became decompression of the homolateral trochlear nerve intermittent and controlled by rescue non-prescription (12). Oscillopsia and tunnel vision are in the other analgesics. Her previous medical history included hand, defining symptoms in patients with migraine without aura, mild asthma and occasional hindbrain-related syringomyelia (13). Although visual dizziness. She used albuterol inhalations rarely for may be a presenting sign of occipital strokes, asthma. Her mother had migraine and one aunt died the brain imaging studies in this patient did not from a ruptured intracranial aneurysm. Her paternal demonstrate ischemic or hemorrhagic lesions, grandfather had Alzheimer’s disease. General physical evidence of reversible cerebral vasoconstriction examination was unremarkable. Her blood pressure syndrome (RCVS) or of syringomyelia. Oscillopsia was 130/91 mm Hg. Her pulse was 71, regular and needs to be distinguished from “fixation switch diplopia”

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(FSD) in where the patient experiences brief double displacements (11,19). This principle requires a vision when refocusing on new targets, but without the mechanism of high order visual afferent suppression illusion of movement of the background. FSD is an with cancellation of irrelevant cognitive peripheral infrequent condition seen in patients with history of elements, during selective attention (11,18,19). early and loss of ocular dominance Unfortunately this patient impossibility to access secondary to a change in their , or to promptly sophisticated testing in a major tertiary the use of glasses encouraging fixation with the institution, did not permit the completion of functional non-dominant eye (14). MRI, PET scan and transcranial magnetic stimulation The vestibular apparatus and its pathways may be (TMS) studies. In particular, TMS and compromised by the migraine process as often magnetoencephalography (MEEG) could have patients report dizziness, lightheadedness, vertigo, determined if she had hyper-excitability, nausea and vomiting during the acute attacks. When as it was demonstrated on six patients with migraine the vestibular involvement becomes the sole with persistent visual aura without infarction, by Chen, expression of migraine, the symptoms are recognized et al. (20). These authors advanced the notion that under the rubber of vestibular or vertiginous migraine patients with the latter migraine variant, had (15). In fact, individuals with migraine are susceptible reverberating cortical spreading depression (CSD) to motion sickness, and vice versa, with preferential resulting in sustained excitatory effects. It is vulnerability to either movement-induced, or to appropriate to suspect however, that the underlying visual-induced triggers (16). The latter association occipital cortical hyper-excitability spared this patient’s underscores the anatomic and physiological link occipital poles, since she had no central oscillopsia. between the visual and the vestibular systems, via “The rotating snakes illusion” is elicited by looking to brain stem trigemino-vestibular connectivity, and to its an arrangement of colored patches of four different tendency to periodically become hyperexcitable in luminance, periodically placed along the migraine sufferers. Of interest, facial pain induced circumference of concentric circles (21). This layout experimentally by applying ice to the temples, allows the of a spatiotemporal illusory aggravate nausea and headache during rotational motion triggered by micro-saccades and optokinetic-induced motion sickness in migraine blinks. A related false percept is the “The pursuit patients (17). The latter finding reflects that –pursuing illusion” (22). The latter illusion is created by accentuating cranial neurovascular reflexes by pain pursuing a circularly traveling small target located in mechanisms or stress, heightens symptoms of motion the center of a display containing several peripherally sickness and increases susceptibility to migraine (17). located color disks placed in the center of a radial These investigational subjects however, did not arrangement of sectors resembling sun flowers. The experience oscillopsia or visual disturbance in the form eye pursuit of the circular travel of the central target of tunnel vision. The patient herein described reported while exposed to the experimental background visual lightheadedness and vertigo at the onset of her display creates the illusion of movement of the symptoms of binocular persistent peripheral oscillopsia, peripheral color disks in the direction of the moving as well as mid-facial headache. central target (22). It appears that the peripheral disks I suggest that this patient had cortical oscillopsia in the radial arrangements are “carried over” with the without nystagmus. This rare, or at least poorly pursuing eye movements of the rotating central recognized disorder, is precisely defined by its name: fixation target, as the brain attempts to average or to these patients experiencing oscillopsia have no synthesize the percept, in order to “make ” of a nystagmus on their examination, but evidence of discordant or illogical happenstance, given its occipital cortical dysfunction detected by functional evolutional design to provide fast and useful MRI or PET scan (18). One single patient with NMO responses (21 ). “The pursuit-pursuing illusion” was reported very recently with the syndrome in illustrates the existence of a selective supersensitive question, and was found to harbor demyelinating visual sense of peripheral object motion, especially lesions over the occipital lobes, corpus callosum and when following a moving central target. If this patient cortical lesions involving parietal area V5 (19). It is yet migrainous spontaneous visual phenomenon in a way to be elucidated if co-activation of the vestibular cortex simply represents repeats or the unmasking of constitutes a pre-requisite for the development of atavistic pre-conscious abilities of the brain oscillopsia, and if it indeed violates the visual interpretative peripheral perceptual mechanisms, physiological principle of “space constancy” that remains totally speculative without further maintains perceptual stability despite gaze experimental advanced psycho-physiological visual

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analysis. cases. J Fr Ophtalmol 35: 284 The fact that this patient could not suppress her 13. Rowlands A, Sgouros S, Williams B (2000) Ocular peripheral oscillopsia by monocular eye closure seems manifestations of hindbrain-related syringomyelia and to indicate that the simultaneous engagement of both outcome following craniovertebral decompression. Eye occipital cortices by visual stimuli is not essential for (Lond) 14: 884-888. the clinical phenomenon to surface, even if sparing the 14. Kushner BJ (1995) Fixation switch diplopia. Arch occipital poles, the allocated areas for central vision. Ophtalmol 113: 896-899 15. Thakar A, Anjaneyulu C, Deka RC (2001) Vertigo References syndromes and mechanisms in migraine. J Laryngol Otol 115: 782-787 16. Drummond PD (2005) Triggers of motion sickness 1. Eriksen MK, Thomsen LL, Olesen J (2005) The in migraine sufferers. Headache 45: 653-656. visual aura rating scale (VARS) for migraine aura 17. Drummond PD, Granston A (2004) Facial pain diagnosis. Cephalalgia 25:801-810. increases nausea and headache during motion 2. Liu H-Y, Fuh J-L, Lu S-R, Chen S-P, Chou C-H, sickness in migraine sufferers. Brain 127: 526-534 Wang Y-F, Wang S-J (2012) Transient visual 18. Suzuki Y, Kiyosawa M, Mochizuki M, Wakakura M, disturbances in adolescents: Migrainous features or Ishii K, Senda M (2004) Oscillopsia associated with headache-accompanied phenomenon? Cephalalgia dysfunction of visual cortex. Jpn J Ophtalmol 48: 32; 1109-1105. 128-132. 3. Wang Y-F, Fuh J-L, Chen W-T, Wang S-J (2008) 19. Kim S-M, Kim J-S, Heo YE, Yang H-R, Park KS The visual aura rating scale as an outcome predictor (2012) Cortical oscillopsia without nystagmus, an for persistent visual aura without infarction. isolated symptom of neuromyelitis optica spectrum Cephalalgia 28:1298-1304. disorder with anti-aquaporin 4 antibody. Multiple 4. O’Connor PS, Tredici TJ (1981) Acephalgic Sclerosis Journal 18: 244-247. migraine: Fifteen years experience. Ophtalmology 88: 20. Chen W-T, Lin Y-Y, Fuh J-L, Hamalainen MS, Ko 999-1003. Y-C, Wang S-J (2011) Sustained visual cortex 5. Headache Classification Subcommittee of the hyperexcitability in migraine with persistent visual aura. International Headache Society. International Brain 134: 2387-2395. Classification of Headache Disorders. 2nd edition 21. Otero-Millan J, Macknik SL, Martinez-Conde S (2004) Cephalalgia 24 (Suppl 1) 9-160. (2012) Microsaccades and blinks trigger illusory 6. Liu GT, Schatz NJ, Galleta SL, Volpe NJ, rotation in the “Rotating Snakes” illusion. The Journal Skobieranda F, Komorsky GS (1995) Persistent visual of Neuroscience 32: 6043-6051. phenomena in migraine. Neurology 45:664-668. 22. Ito H (2012) Illusory object motion in the centre of 7. Jager HR, Griffin NJ, Goadsby PJ (2004) a radial pattern: The pursuit-pursuing illusion. Diffusion-and perfusion-weighted MR imaging in i-Perception 3: 59-87. persistent migrainous visual disturbance. Cephalalgia 25:323-332. 8. Perucca P, Terzaghi M, Manni R (2010) Status epilepticus migrainousus: clinical, electrophysiological and imaging characteristics. Neurology 75:373-374. 9. Palma JA, Gomez-Ibanez A, Martin B, Urrestarazu E, Gil-Bazo I, Pastor MA (2011) Nonconvulsive status epilepticus related to posterior reversible leukoencephalopathy syndrome induced by cetuximab. Neurologist 17; 273-275. 10. Relja G, Granato A, Ukmar M, Ferretti G, Antonello RM, Zorzon M (2005) Persistent aura without infarction: description of the first case studied with both brain SPECT and perfusion MRI. Cephalalgia 25:56-59. 11. Tilikete C, Vighetto A (2011) Oscillopsia: causes and management. Curr Opin Neurol 24: 38-43. 12. Thoorens V, Signolles C, Defoort-Dhellemmes S (2012) Superior oblique myokymia: a report of three

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