Retinal Migraine Headache


Article Author:
Yasir Al Khalili
Sameer Jain


Article Editor:
Kevin King


Editors In Chief:
Rodrigo Kuljis
Oleg Chernyshev
Aninda Acharya


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Kyle Blair
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
4/20/2019 9:45:43 AM

Introduction

Migraine is a common and well-documented disorder first described in 1882 by Dr. Galezowski. Ocular or retinal migraines are generally defined as a transient monocular scotoma or loss of vision which is accompanied or followed by a headache within 60 minutes of visual symptoms onset. In some cases persistent monocular visual loss and abnormal ophthalmological findings have been reported. The symptoms are usually transient. The pathophysiology of those persistent deficits is not clear. [1][2] 

Etiology

Based on theories and pathophysiology of a retinal migraine, precipitating factors for a retinal migraine are same for a migraine, with and without aura. Factors include, but are not limited to, emotional stress, high blood pressure, and hormonal contraceptive pills, as well as exercise, being at a higher altitude, dehydration, smoking, low blood sugar, and hyperthermia. Co-morbidity with lupus, atherosclerosis, and sickle cell disease increase the risk of having a retinal migraine [1][2].

Epidemiology

A retinal migraine is a rare disorder, but the prevalence is not known. Data specific to retinal migraines do not exist, but migraines, in general, have a prevalence of 18.2% in females and 6.5% in male patients, with a higher prevalence in whites followed by blacks followed by Asians.  Retinal migraine can start as early as 7 years of age, but most cases start in the second decade and peak in the fourth decade of life. Based on a study by Pradhan et al., it was found that 50% of retinal migraine patients said the vision loss was complete in one eye, up to 20% said it was just blurring, 12% reported an incomplete loss, 7% dimming, and 13% scotoma. More than 75% of patients had a headache on the same side as the vision disturbance within an hour. [3][2]

Twenty-nine percent of retinal migraine patients have a history of migraine headache, and 50% have a family history of migraine headache. [2]

Pathophysiology

The pathophysiology of a migraine remains controversial. One theory of ocular migraine is that it is due to vasospasm within the retinal or ciliary vasculature while others think it is a spreading depression of the neuron in the retina that is similar to the spreading depression of the cerebral cortex. The spreading depression of the cerebral cortex is usually seen in the visual aura of a classic migraine; it has been observed in patients having an episode of a retinal migraine, vasoconstriction of both veins, and arteries that could be diffuse or segmental. It also can be noticed by ocular hypoperfusion on fundoscopy. Fluorescein angiography can confirm the diagnosis. The fluorescein angiogram shows delayed filling or occlusion of the central retinal artery and its branches with either a normal ciliary circulation or patchy choroidal defects and capillary dropout. [4][1][2] 

Retinal migraine attacks are precipitated by similar factors as a migraine with aurae such as stress, smoking, hypertension, hormonal contraceptive pills, exercise, bending over, high altitude, dehydration, hypoglycemia, or excessive heat. Strong family history in these patients suggests that a retinal migraine has a genetic predisposition but no clear pattern of inheritance has been described. The vasospasm theory is controversial due to the complexity of the retinal vascular supply. Retina has dual circulation, central retinal artery supplies, inner retinal layers, lacks adrenergic innervation, maintains sensory nerves, and is auto-regulated. The choroidal circulation supplies the posterior retina including the photoreceptors it carries adrenergic fibers without autoregulation. [1][2] 

History and Physical

The patient will present with a wide range of symptoms. Many patients describe positive symptoms such as flashing lights and scintillating scotomas. The negative symptoms are black, shaded, white areas of different sizes of scotomata. Attacks happen a few times per day and can last from 5 to 20 minutes. A retinal migraine shares many properties with a migraine with an aura and acephalgic migraine. Many times, these are difficult to differentiate. However, it is less common to have monocular vision disturbance in an acephalgic migraine or a migraine with aura classic migraine. The visual symptoms during a retinal migraine usually do not last as long as with a visual aura of migraine. Physical examination will show monocular visual acuity loss and/or loss of the visual field, all other cranial nerves and the rest of the neurological examination will be normal. [5][2] 

Evaluation

The attacks must fulfill criteria for a migraine with aura. The aura must be fully reversible and monocular, that is, positive or negative phenomena confirmed during an attack by visual field examination or patient’s drawing of a field defect and at least 2 of the following [2] 

  • Spreading gradually over 5 minutes or longer
  • Symptoms last 5 to 60 minutes
  • A headache within an hour of visual symptoms

Treatment / Management

If the attacks are infrequent, such as one per month, then treatment is not necessary. When attacks are more frequent, first-line therapy starts with lifestyle changes that include avoiding dietary triggers such as alcohol and caffeine, controlling stressors like high blood pressure, and ceasing to smoke. If that does not help, then the patient must start a diary to help evaluate the success of the therapy and initiate a prophylaxis therapy. It is usually recommended to avoid ergot and beta-blockers in retinal migraine due to the increased incidence of irreversible vision loss. Calcium channel blockers such as nifedipine and verapamil (most effective) are the mainstay of treatment here. Contraindications to calcium blockers include congestive heart failure, hypotension, sick sinus syndrome, cardiac conductive defects, concomitant, and renal or hepatic failure. Other medications such as coumadin and heparin have been used in isolated cases of patients with antiphospholipid antibody syndrome and retinal migraine. Aspirin and antiepileptic drugs have all been shown to reduce the severity of attacks. Abortive therapy is not used in this condition due to the brief duration of episodes; the main focus of treatment would be to reduce the recurrence of attacks. Medications such as Triptans, ergots, and beta blockers should be avoided in migraines with transient vision loss since there is a concern for exacerbation of vasoconstriction and increasing the risk of potential irreversible visual loss. [1][2] 

Differential Diagnosis

The most important initial step in evaluating a patient with suspected retinal migraine is to determine if the visual symptoms are monocular or binocular. Once it is confirmed that it is a monocular visual loss, then the healthcare professional has to rule out all other causes of monocular vision loss because a retinal migraine is a diagnosis of exclusion. The other diagnoses that need to be considered are amaurosis fugax as in embolic phenomena of carotid or cardiac source, increase intracranial pressure, orbital apex mass, optic neuritis, giant cell arteritis, migraine with visual aura or anterior ischemic optic neuropathy. [5][2] 

Prognosis

Although a retinal migraine is considered a benign condition, permanent symptoms can persist after the acute attacks. [1][2]

Complications

Complications of a retinal migraine include central retinal artery occlusion (CRAO), retinal infarction, central retinal venous occlusion, branch retinal artery occlusion (BRAO), retinal hemorrhages that can lead to edema of the retina and disc, ischemia of choroid or optic nerve, and vitreous hemorrhage. Many of those could lead to irreversible vision loss in the patient. It is important to avoid the use of Triptans, ergots, and beta blockers in migraines with transient vision loss secondary to risk of potentiating vasoconstriction and increasing the risk of irreversible visual loss. [1][2] 

Deterrence and Patient Education

It is critical to educate the patients about the red flags of vision loss. A visual loss that patients describe as darkness require immediate medical attention and an emergency room visit. Patient must understand that this could be a sign of a stroke or an irreversible eye condition. Visual changes that are more consistent with migraine phenomenon are usually positive such as flashing light. Patient must also be taught that those could come without a headache or any pain. Preventive therapy is important to reduce the frequency of attacks and severity and must be taken on a daily basis. [1][2] 

Pearls and Other Issues

  • A retinal migraine a rare phenomenon that usually affects monocular vision transiently.
  • Duration of symptoms on average is 5 to 20 minutes.
  • The prognosis for an ocular migraine is good.
  • The frequency and intensity of the headache typically decrease.
  • During prolonged periods of retinal, choroidal, or optic nerve hypoxia, permanent visual loss may occur.

Enhancing Healthcare Team Outcomes

The diagnosis of retinal migraine is one of exclusion and all other causes of vision loss should initially be considered. All member of the healthcare team should be vigilant and refer the patient for immediate and emergent assessment for stroke or other thromboembolic cause for their symptoms if the patient presents with visual loss or changes.  [2] 


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Retinal Migraine Headache - Questions

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Retinal migraines are characterized by which of the following?



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Which of the following is a characteristic of retinal migraines?



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Which group of patients most commonly is diagnosed with retinal migraines?



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A 57-year-old female with a left-sided migraine headache diagnosed 2 years ago presents for left-sided vision loss that started abruptly and resolved within 2 to 3 minutes. Vision loss was associated with headache she denies any focal neurological symptoms and no persistence in her vision loss. Based on International Headache Society guidelines, why can't she be diagnosed with a retinal migraine?



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A 59-year-old female with history of congestive heart failure and retinal migraine presents complaining of 2 episode of transient vision loss in her right eye associated with unilateral throbbing headache with nausea and vomiting. These episodes happened 3 times in the past year. What is the best step in management of this patient?



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Retinal migraine is also known as ocular migraine. Which of the following is another name for this condition?



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Although not very likely to happen, what would the fluorescein angiogram show during a retinal migraine attack?



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A 37-year-old female with a family history of migraine presents to the emergency room with a severe throbbing headache on the right and monocular vision loss on the right. What percentage of patients will present with this classic presentation of a headache within an hour of visual symptoms that are same-sided?



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Retinal Migraine Headache - References

References

Pradhan S,Chung SM, Retinal, ophthalmic, or ocular migraine. Current neurology and neuroscience reports. 2004 Sep     [PubMed]
Reggio E,Chisari CG,Ferrigno G,Patti F,Donzuso G,Sciacca G,Avitabile T,Faro S,Zappia M, Migraine causes retinal and choroidal structural changes: evaluation with ocular coherence tomography. Journal of neurology. 2017 Mar     [PubMed]
MacGregor EA, Migraine (Japanese Version). Annals of internal medicine. 2017 Apr 4     [PubMed]
Pula JH,Kwan K,Yuen CA,Kattah JC, Update on the evaluation of transient vision loss. Clinical ophthalmology (Auckland, N.Z.). 2016     [PubMed]
Ahmed M,Boyd C,Vavilikolanu R,Rafique B, Visual symptoms and childhood migraine: Qualitative analysis of duration, location, spread, mobility, colour and pattern. Cephalalgia : an international journal of headache. 2018 Dec     [PubMed]

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