| Identifier |
939-4 |
| Title |
Migraine Visual Aura |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Contributors |
Steve Smith, Videographer |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital, Boston, Massachusetts |
| Subject |
Migraine Visual Aura; Occipital Lobe; Visual Phenomena |
| History |
The patient is a 73 year old retired teacher who was referred in 1993 for a second opinion regarding treatment of episodic visual hallucinations. As a school boy in junior school, he began to experience transient episodes of a spot appearing in the right lower homonymous quadrant of his field of vision, edged by a zig-zag line which would shimmer. The spot would gradually enlarge to produce a blind spot obliterating what he was reading and continue to expand covering the entire visual field and blur his vision. He could see the zigzags with his eyes closed and with each eye separately confirming that it was binocular. He had no headache, nausea, vomiting, photophobia, phonophobia or other associated symptoms. Between attacks of visual aura he was also headache free. Initially, as a boy, he was scared that the visual disturbance would lead to blindness. But, as the episodes were so frequent, and his vision always fully recovered, he realized that the hallucination was transient and his vision remained unaffected. He was taken to see his pediatrician who made a diagnosis migraine. He cannot recall if he received any medication to stop the attacks. After he left school, the visual aura stopped and he was free of visual aura at college and in the Service. In 1988 episodic visual aura reoccurred with increased frequency. In one month he has had as many as 10 attacks. He attributed the re-emergence of his migraine and the frequency of the aura to "a perpetual stressful situation" following his wife being declared legally blind due to macular degeneration and no longer able to drive. The patient was also growing concerned about his own health. At this time, he started to keep a meticulous diary documenting the features of the visual aura. They appeared to be identical, stereotypic, scintillating scotoma almost always starting in the lower right homonymous quadrant of his visual field. On rare occasions it developed in the left half of his vision. At no time was it followed by headache. His past history was negative for head trauma or neurological symptoms. Family history was negative for migraine. In 1988 he developed: 1. Glaucoma 2. Mild hypertension, treated with a diuretic 3. An essential and action tremor of the upper extremities. In 1993 his medications were: Amitriptyline 10 mg. 1 tablet p.o.q.h.s. Inderal LA 120 mg. 1 tablet p.o.q.d. Hydrochlorothiazide On examination: Visual acuity 20/20 OU, reads J1 corrected Visual fields full OU Pupils normal Eye movements full with no nystagmus Fundus examination showed normal optic discs and fundi OU He was seen in the Movement Disorder Clinic for evaluation of essential tremor. The neurological examination revealed no other abnormality. Electroencephalogram-1993 The EEG at rest consisted of moderate voltage 11 Hertz symmetrical alpha activity and low voltage symmetrical fast activity. Hyperventilation produced essentially no change. Photic strobe stimulation produced no driving response. No epileptiform activity noted. Normal study. Brain MRI with Gadoilium showed bilateral periventricular T2 hyperintensities consistent with a moderate degree of small vessel ischemic disease. RE: Stress: The major stress at home was his wife's health and over the next few years her health deteriorated due to severe arthritis of the hips and the onset of angina. She became house bound. The patient became severely depressed. His primary care physician prescribed antidepression medication from 1993 to 2003. During this time the patient continued to have episodic visual aura without headache and he continued to come to the Neurovisual Clinic as he enjoyed discussing his symptom and getting regular reassurance that the aura was characteristic of migraine and not heralding blindness. |
| Pathology |
Cortical spreading depression (CSD) has been suggested to underlie migraine visual aura. However, it has been challenging to test this hypothesis in human cerebral cortex. Data from functional MRI studies in human visual cortex strongly suggest that an electrophysiological event such as CSD does in fact generate the aura in human visual cortex. (9) Visual aura are characterized by a wave of cerebral hypoperfusion-oligemia that passes across the cortex at the characteristically slow rate of 2 to 6 mm per minute (9-11). Richards suggested that in migraineurs the cortical neurons activated by the leading edge of cortical spreading depression are sensitive ‘line detectors'. His reasoning was based on Hubel and Wiesel's concept of neurons as ‘feature detectors', which in turn was suggested by the results of recording the intracellular activity of single neurons in the primary visual cortex of the cat and monkey brain in response to specific visual stimuli (4-7). (See also ID 947-1 Hubel D Migraine Visual Aura and ID 948-1 Recordings of visual cortical cells to visual stimuli). Richards calculated that the cortical distance for each teichoptic line was 1.2 mm, five times larger than the known diameter of individual orientation columns in the monkey and so he argued that the scale discrepancy suggested that the orientation columns alone were not responsible for the phenomena. Increased activity within lattice-like, long range excitatory connections in different layers of V1, V2 and V4 are considered better anatomical candidates for the generation of visual aura (12). The trigger to provoke visual aura is unknown. In a study of visual processing in migraine subjects with aura, Wray et al found that the migraineurs response time in the low-level tasks was better than normal controls. The data suggest that migraineurs process visual signals to the primary visual cortex [Area V1] more rapidly than non-migraineurs and that Area V1, between attacks of visual aura, may be hypersensitive to visual stimuli. (14) |
| Disease/Diagnosis |
Migraine. |
| Clinical |
This 73 year old man describes his migraine visual aura with difficulty. The features that he emphasizes are: • The visual disturbance is way on the way before I notice it. • The clarity of the hallucination depends on the background. • It begins with a circular area of activity and motion and around the rim of the circle are a series of points. • Occasionally the central circle blots out words (scotoma) if I am reading. • The scotoma gradually enlarges, changes shape and ceases to be a complete figure. • The scotoma moves out to the periphery and eventually out of my field of vision. • The points around the scotoma are bright like an arc of light bulbs. • The aura lasts 20 to 25 minutes. • I usually see one arc at a time that simply moves Serial drawings of his visual aura illustrated a stereotypic pattern of a circle edged with sharp short lines - ‘points'. Comment: Migraine affects some 18% of women, 6% of men and 4% of children. Visual disturbances are the most common aura in migraine occurring in 82% to 90% of subject who experience aura. About 20% of the time visual aura can occur alone without any ensuing headache, as in this patient (8). Typically migraine visual aura consist of poorly localized luminous hallucinations of formless flashes of white or multicolored lights (photopsias), or zig-zag patterns, wavy lines, heat flashes, or spots or stars seen in the central portion of the visual fields or dispersed throughout the entire visual field. The aura may also be a distinct perception of a central or paracentral blind spot (scotoma) edged by an arc of shiny, shimmering, crennelated shapes which taken together constitute a scintillating scotoma. To Dr. Hubert Airy, migraine sufferer and researcher, in 1870, these serrated figures seemed ‘a fortified town with bastions all around it, these bastions being colored most gorgeously' (1). Like the headache associated with it, the scintillating scotoma is usually unilateral and is seen in the homonymous hemianopic fields (2). It is so highly characteristic of migraine that, it is probably pathognomonic. Richards, in 1971, described the scintillating scotoma in detail, basing his quantitative measurements largely on observations provided by his wife. He also combined several sketches of serrated figures to record the general form the arcs take as they expand across the visual field. He not only found that the figures, seen and drawn by Dr. Airy, 100 years earlier, almost exactly matched the figures drawn by his wife, but that the size of the line lengths of the serrated lines was constant (i.e. the distance between the lines was estimated to be 1.2 mm of cortical distance for each line, or about one-quarter of the line length) and that the inner angle between two lines forming a serration averaged 60 degrees over the entire field (Figure 2 (11)). Normally, only one set of discreet lines in visual space is seen at a time moving peripherally at a constant propagation rate of 3 mm/min across the visual cortex (9-11). The characteristic form of the arc, and the development of a scotoma, indicate a wave of intense excitation in the primary visual cortex followed by a period of disinhibition. |
| Presenting Symptom |
Visual Hallucinations |
| Ocular Movements |
Normal |
| Neuroimaging |
Functional MRI (fMRI) performed during spontaneous visual aura in migraineurs has shown moderate focal reductions in cerebral blood flow and volume in the occipital lobe during the aura. The occipital lobe perfusion deficit corresponded anatomically with the reported visual field disturbance and with the side of the subsequent headache. (3) The findings were consistent with blood flow data obtained during a spontaneous migraine headache with an atypical visual disturbance, using PET and Oxygen 15-labeled water. (13) Woods and colleagues described a patient who, during positron emission tomography (PET), fortuitously had an attack of common migraine. Highly sophisticated measurements showed a reduction in blood flow that started in the occipital cortex and spread slowly forward into the temporal and parietal lobes bilaterally. View ID41-1 Migraine PET Study to observe pattern of "spreading oligemia". Courtesy JC Mazziotto, M.D., Ph.D. |
| Treatment |
Patients with episodic migraine visual aura without headache do not require treatment. |
| Etiology |
Migraine is a complex genetic disorder. |
| Date |
1997 |
| References |
1. Afridi SK, Giffin NJ, Kaube H, Friston KJ, Ward NS, Frackowiak RSJ, Goadsby PJ. A Positron Emission Tomographic Study in Spontaneous Migraine. Arch Neurol 2005;62:1270-1275. http://www.ncbi.nlm.nih.gov/pubmed/16087768 2. Afridi SK, Matharu MS, Lee L, Kaube H, Friston KJ, Frackowiak RSJ, Goadsby PJ. A PET study exploring the laterality of brainstem activation in migraine using glyceryl trinitrate. Brain 2005;128:932-939. http://www.ncbi.nlm.nih.gov/pubmed/15705611 3. Airy H. On a distinct form of transient hemiopsia. Philos Trans R Soc Lond 1870; 160:247-264. 4. Bahra A, Matharu MS, Buchel C, Frackowiak RSJ, Goadsby PJ. Brainstem activation specific to migraine headache. Lancet 2001;357:1016-1017. http://www.ncbi.nlm.nih.gov/pubmed/11293599 5. Baumgartner G. Neuronal mechanisms of the migrainous visual aura. In: Clifford Rose F, editor. Physiological aspects of clinical neurology. Oxford: Blackwell Scientific Publications 1977;111-121. 6. Cutrer FM, Sorensen AG, Weisskoff RM, Ostergaard L, Sanchez del Rio M, Lee EJ, Rosen BR, Moskowitz MA.. Perfusion-weighted imaging defects during spontaneous migraine aura. Ann Neurol 1998, 43:25-31. http://www.ncbi.nlm.nih.gov/pubmed/9450765 7. Hadjikhani, N. Sanchez del Rio M, Wu O, Schwartz D, Bakker D. Fischl B, Kwong KK, Cutrer FM, Rosen BR, Tootell RBH, Sorensen AG, Moskowitz MA. Mechanisms of migraine aura revealed by functional MRI in human visual cortex. Proc Natl Acad Sci USA 2001;98:4687-4692. http://www.ncbi.nlm.nih.gov/pubmed/11287655 8. Hubel DH, Wiesel TN. Receptive fields and functional architecture of monkey striate cortex. J Physiol (Lond) 1968; 195:215-243. http://www.ncbi.nlm.nih.gov/pubmed/4966457 9. Hubel DH, Wiesel TN. Sequence regularity and geometry of orientation columns in the monkey striate cortex. J Comp Neurol 1974a; 158:267-293. http://www.ncbi.nlm.nih.gov/pubmed/4436456 10. Hubel DH, Wiesel TN. Uniformity of monkey striate cortex: a parallel relationship between field size, scatter and magnification factor. J Comp Neurol 1974b; 158:295-305. http://www.ncbi.nlm.nih.gov/pubmed/4436457 11. Jensen K, Tfelt-Hansen P, Lauritzen M, Olesen J. Classic migraine: a prospective recording of symptoms. Act Neurol Scand 1986; 73:359-362. http://www.ncbi.nlm.nih.gov/pubmed/3727912 12. Lashley KS. Patterns of cerebral integration indicated by the scotomas of migraine. Arch Neurol Psychiatry 1941; 46:331-339. 13. Lauritzen M. Pathophysiology of the migraine aura: the spreading depression theory. Brain 1994;117:199-210. http://www.ncbi.nlm.nih.gov/pubmed/7908596 14. Leao AAP. Spreading depression of activity in cerebral cortex. J Neurophysiol 1944, 7:379-390. 15. Olesen J, Larsen B, Lauritzen M. Focal hyperemia followed by spreading oligemia and impaired activation of rCBF in classic migraine. Ann Neurol 1981;9:344-352. http://www.ncbi.nlm.nih.gov/pubmed/6784664 16. Richards W. The fortification illusions of migraines. Sci Am 1971; 224(5): 88-96. http://www.ncbi.nlm.nih.gov/pubmed/5552581 17. Rockland KS, Lund JS. Intrinsic laminar lattice connections in primate visual cortex. J Comp Neurol 1983; 216:303-318. http://www.ncbi.nlm.nih.gov/pubmed/6306066 18. Storer RJ, Akerman S Goadsby PJ. Calcitonin gene-related peptide (CGRP) modulates nociceptive trigeminovascular transmission in the cat. Brit J Pharm 2004;142:1171-1181. http://www.ncbi.nlm.nih.gov/pubmed/15237097 19. Vijayan N, O'Brien MD, Blau JN, Rastegar D, Woods RP, Iacoboni M, Mazziotta JC, Olesen J. Spreading Cerebral Hypoperfusion during Migraine Headache. N Engl J Med 1995;332:1515-1518. http://www.ncbi.nlm.nih.gov/pubmed/7739695 http://www.ncbi.nlm.nih.gov/pubmed/7739696 http://www.ncbi.nlm.nih.gov/pubmed/7739697 http://www.ncbi.nlm.nih.gov/pubmed/7739698 20. Woods RP, Jacoboni M and Mazziotta JC. Bilateral spreading cerebral hypoperfusion during spontaneous migraine headache. New Eng J Med 1994; 331:1689-1692. http://www.ncbi.nlm.nih.gov/pubmed/7969360 21. Wray SH, Mijovic-Prelec D, Kosslyn SM. Visual processing in migraineurs. Brain. 1995 Feb;118 ( Pt 1):25-35. http://www.ncbi.nlm.nih.gov/pubmed/7895008 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
3/4" Umatic master videotape |
| Relation is Part of |
10-1, 926-4, 932-5, 947-1 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Shirley H. Wray Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s62r6p8v |
| Setname |
ehsl_novel_shw |
| ID |
188514 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s62r6p8v |
