Migraine may occur in many variations of its clinical appearance. Ophthalmic migraine (classic migraine) is the most common form and typically presents with attacks of bilateral homonymous scintillating scotomas, transient hemianopic loss of vision and half-sided headaches as well as possibly nausea and other intestinal disturbances. Prodromal symptoms such as euphoria, increased irritability, unusual hunger, or high sensitivity to light, sound, and smell often precede an attack of ophthalmic migraine. A flood of urine of low concentration and pale color follows it. The so-called "common migraine" is the second most recognized type. It closely resembles the ophthalmic (classic) migraine, but it has no visual symptoms. The more unusual types of migraine are the ophthalmoplegic migraine, "migraine accompagnée," abdominal migraine, and ocular migraine. Ophthalmoplegic migraine goes along with transient paresis of eye muscles and thus causes temporary paralytic strabismus. "Migraine accompagnée" (Charcot) is a rare kind of migraine that exhibits transient signs of cerebral irritations or defects such as speech difficulties, sensory or motor defects, and epileptiform convulsions. Abdominal migraine mainly exhibits abdominal symptoms and signs such as pain, nausea, vomiting, and diarrhea. Ocular migraine, finally, causes transient vision and visual field defects in one eye only. Clinically confirmed cases of pure ocular migraine are rare while, on the other hand, many patients with different types of migraine also report episodes of transient unilateral scotomas or visual loss. Any combination of all the components of the different types of migraine seems to be possible, and one patient may suffer various combinations at different times.
Transient arterial spasms in parts of the central nervous system are the initial feature of all types of migraine. The spasms mainly occur in intracranial vessels, but extracranial arteries of the head region may also be involved. Typically, the arterial spasm is followed by distention of the involved arteries. Pain of different types is known to occur in this latter phase of vascular dilation.
Fig. 1: The central retina of the right eye of the present patient 25 minutes after onset of an attack of ocular migraine. Retinal edema and a cherry-red spot are seen, but there are no arteriolar spasms. (Fundus photograph)
To present a clinical observation of recurrent transient unilateral retinal edema and visual loss due to typical ocular migraine in a young man is the purpose of this paper.
This robust, healthy 20-year-old Caucasian male states that he has experienced in his right eye approximately monthly attacks of sudden, painless, complete blindness since the age of 12 years. Over the past two years, these have increased in frequency to about every two and one-half weeks. The duration of the blindness has been 20 to 30 minutes with complete recovery. The patient denies any aura, headache, nausea, or neurologic symptoms accompanying the attack. He knows of no precipitating event except that they may possibly occur more frequently when he is stressed or fatigued. The patient is a light cigarette smoker and very occasionally drinks alcoholic beverages. His past medical history and review of systems are completely normal. The family history is negative - especially for migraine, asthma, epilepsy, diabetes, hypertension, or heart disease.
Physical examination reveals normal blood pressure, with no postural hypotension. Peripheral pulses are normal. No bruits are heard over the head or neck. Cardiac status is normal. Neurologic examination shows no focal signs. Findings from laboratory examination including CBC, ESR, FBS, BUN, cholesterol, triglycerides, protein electrophoresis, antinuclear factor, and T3 and T4 are also normal. Chest x-ray, EKG, skull x-rays, and brain scan reveal no abnormalities. An EEG is interpreted as mildly abnormal because of diffuse shifting theta transients, but no focal or paroxysmal features are present. In summary, results of the history, physical examination, laboratory and x-ray studies, and medicine, neurology, and neurosurgery consultations are all normal.
The patient describes a typical episode as follows. The onset is heralded by blurred vision in his right eye followed shortly by a loss of color vision. Then he notes that the central portion of objects is blacked out with only peripheral portions seen. Finally, all vision disappears and no light can be seen. This development takes 5 to 10 minutes with the duration of amaurosis being about five minutes. Then the above sequence of events reverses in 5 to 10 minutes leaving blurred vision for about 60 minutes. The patient has been seen by ophthalmologists at intervals for evaluation, with all examinations reported to him as showing normal results.
On the morning of his appointment at this Eye Clinic for consultation, quite fortuitously, an attack began at 9:00 A.M. The patient was examined 15 minutes after the onset. Visual acuity was blindness with no light perception in the right eye and 20/20 in the left eye. The right pupil had no direct reaction to light, but it responded consensual ly. The left pupil had a direct reaction, but no consensual reaction. Ophthalmoscopic examination of the right eye revealed very definite retinal edema with a cherryred macular spot (Fig. 1). Retinal vessels appeared to be about normal in both eyes and there were at the time of the first examination no arteriolar spasms seen in the right fundus. The photograph taken at 9:25 a.m. still shows the diffuse retinal edema and a cherry-red spot in the macular region. Ophthalmoscopic examination of the left eye revealed normal findings. Several different observers viewed these findings in the right eye. The first fundus photographs (Fig. 1 ) were taken 25 minutes after the onset of this episode when the patient was regaining vision. Three hours after the onset visual acuity in the right eye had improved to 20/30 and JO with difficulty. The retinal edema had disappeared and the right fundus was normal again. Another photograph of the right fundus was taken (Fig. 2). Follow-up examination shows a vision of 20/20 and J O in both eyes. The pupils were equal and had normal reactions to light. Visual fields, applanation tonometry, fundoscopy, and ophthalmodynamometry are all normal in both eyes.
Walsh and Hoyt state that "the eye itself can be involved in the "angiospastic" circulatory disturbance of a migraine attack."' These authors also explain that this type of loss of vision in one eye is the result of retinal hypoxia. In the present case of ocular migraine there is the question of at which level the arterial blood supply of the retina was closed by the spastic episode. Was it the central retinal artery or were all four main arterioles involved? The patient has high central branching of the central retinal artery in both eyes. Our clinical examination 15 minutes after the onset of the unilateral blindness revealed no arteriolar spasms in the edematous retina. This could mean that the spastic phase of the attack was over - or it could indicate that the central retinal artery was the site of the spasms in this case. It is of some interest that the patient always has total loss of vision - always in the same eye - and that he has not observed quadrantal or altitudinal field loss. In all other reported cases of ocular migraine in which a physician has been able to observe the ocular fundus during an attack, the retinal arterioles have always been constricted.1"5 The photographs of Heyck taken during a monocular attack seem to document arteriolar spasms.* In cases of partial visual loss in one eye, corresponding arteriolar spasm has been observed by Siegrist and also by Parisotti (Wilbrand-Saenger *).
Many episodes of transient blindness have caused no detectable functional or organic damage in the involved right eye of the present patient. In the literature cases with evidence of permanent damage resulting from ocular migraine have been reported.1'7 Ischemic papillitis, retinal hemorrhages, vitreous hemorrhages, central serous retinopathy, pigmentary changes of the retina, optic nerve atrophy, and permanent pupillary dilation as well as loss of accommodation are included in the list of defects recorded following ocular migraine.1 In the differential diagnosis of transient episodes of monocular blindness associated with retinal edema, carotid artery insufficiency, arterial embolism and high intraocular pressures have to be considered. Clinical evaluation eliminates these causes in the present patient and leaves ocular migraine as the only acceptable diagnosis.
Treatment is indicated, in the present patient in view of the fact that attacks of ocular migraine have been reported to cause permanent ocular damage. This consists, in the first place, in an attempt to stabilize the emotional status of the patient and, in the second place, in medications. Allergies - especially food allergies - have to be studied and treated.' First, rapid acting oral vasodilators were tried, but these failed to influence the duration and severity of the attacks. Currently, amylnitrite inhalation at the beginning of an attack along with tranquilization is being used. Newer and more unusual types of treatment will be tried if the time-honored methods do not help. The fact that there are many new therapeutic approaches to cure migraine every year is, of course, due to the common experience that most of them do not help.
Fig. 2: The normal central retina of the right eye of the present patient 3 hours after an attack of ocular migraine. (Fundus photograph)
Ocular migraine in a 20-year-old male causes total unilateral transient blindness and occurs about once a month. Retinal edema and a cherry-red spot as well as absence of direct light reaction of the pupil were seen during an attack. The special features of ocular migraine in relation to the other more common types of migraine are discussed.
Department of Ophthalmology
University of Michigan
Ann Arbor, Michigan
1. Walsh FB, Hoyt AB: Clinical Neurophthalmology. Baltimore, Williams & Wilkins, 1969, p 1671.
2. Rosenstein AM: Beitrag zu den beiderseitigen Verdunklungen des Sehvermögens mit vorübergehendem ophthalmoskopischem Befund bei Herzklappenfehler. Klin Mbl f Augenheilk 75:357, 1925.
3. Gronvall A: On changes in the fundus oculi and persisting Injuries to the eye in migraine. Acta Ophth 16:602, 1938.
4. Wagener cited by Weber LW, Runge W: Störungen und Veränderungen des Sehapparates by Psychosen and Neurosen. Kurzes Handbuch der Ophthalmologie, Berlin, Julius Springer, 1931, p 800.
5. Heyck H: Kopfschmerz und vegetatives Nervensystem (Migräne und verwandte Kopfschmerzformen). Akt Fragen Psychiat Neurol 4:167, 1966.
6. Wilbrand H, Saenger A: Neurologie des Auges. München, J.F. Bergmann, vol 3, part 2, 1906, ? 968.
7. Conner RCR: Complicated migraine. Lancet, :1072, 1962.
8. Speer F: Allergic factors in migraine. Mod Med 8:100, 1971.