What is Persistent Aura Without Infarction? 2023
What Is Persistent Aura Without Infarction?
Very rarely a patient with migraine with aura will experience a persistent visual aura. Persistent is defined as more than one week. The aura is usually present in bilateral visual fields, in central vision, and superimposed on normal vision.
This is theoretically a very scary situation which requires neurologic consultation and an MRI brain scan.
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This is an article by Britt Talley Daniel MD, member of the American Academy of Neurology, the American Headache Society, migraine textbook author, and blogger.
Persistent migraine aura without infarction is a rare but well documented condition. There should be persistence of a migraine aura for more than one week without radiographic evidence of infarction.
The patient should have previously had Migraine with aura and to say “persistent aura” the aura symptoms should have lasted one or more weeks.
Neuroimaging shows no evidence of infarction or stroke.
Not better accounted for by another ICHD-3 diagnosis.
Persistent aura symptoms are rare but well documented. They are often bilateral and may last for months or years.
Related questions.
What is the difference between the auras of Migraine without aura and Persistent aura without infarction?
Lashley’s famous migraine aura, getting bigger with time
Symptoms of migraine with aura should last less than 60 minutes.
ICDH-3 says:
Migraine with aura
Previously used terms:
Classic or classical migraine; ophthalmic, hemiparaesthetic, hemiplegic or aphasic migraine; migraine accompagnée; complicated migraine.
Description:
Recurrent attacks, lasting minutes, of unilateral fully reversible visual, sensory or other central nervous system symptoms that usually develop gradually and are usually followed by headache and associated migraine symptoms.
Diagnostic criteria:
A. At least two attacks fulfilling criteria B and C
B. One or more of the following fully reversible aura symptoms:
visual
sensory
speech and/or language
motor
brainstem
retinal
C. At least three of the following six characteristics:
at least one aura symptom spreads gradually over ≥5 minutes
two or more aura symptoms occur in succession
each individual aura symptom lasts 5-60 minutes
at least one aura symptom is unilateral
at least one aura symptom is positive
the aura is accompanied, or followed within 60 minutes, by headache
D. Not better accounted for by another ICHD-3 diagnosis.
The comment regarding spreading gradually is important in that migraine does this. It moves from place to place on the body. If the aura starts as a visual aura, say a zig-zag line. The line may appear far laterally and slowly move centrally in vision.
A TIA or a stroke or infarction involving the central retinal artery and the retina, produces a visual image that doesn’t move. It comes and then goes usually after 5 to 30 minutes, if it is a TIA (transient ischemic attack), or it comes and it stays, if it is a stroke or infarction. This is an important, usually helpful clinical sign, differentiating the two.
The other important bit here is the ICHD-3 limit of 60 minutes of time. Less than 60 minutes is okay and usually the symptom then remits, like a migraine visual attack should do.
Persistent aura attacks, by definition, have to last longer than 7 days, or a week.
If the migraine aura is a sensory symptom, like paresthesiae or numbness, it may start in the fingers of one hand, on one side of the body. The numbness moves from finger to finger, clearing up in the first involved finger as time goes on.
Brain anatomy for visual fields
Then the numbness goes to the arm, upper arm, and then cheek, lips, gum, and tongue on the same side.
This is the migraine movement characteristic feature.
One-sided or both sided (bilateral)?
A migrainous aura symptom of numbness is usually only on one side of the body, like the right hand, fingers, arm, upper arm, face. This is because the event is occurring in the opposite parietal lobe of the brain.
Left parietal brain symptoms cause only cause numbness that the patient perceives in the right hand, or cheek.
Rarely, the patient perceives numbness on both sides of the body for migraine sensory symptoms.
A migrainous visual aura, however, is usually seen in both eyes, because the event is occurring in the occipital part of the brain, in the back of the head, on one side.
The patient usually only perceives the visual event on one side, however. An aura event originating in the left occipital brain might be perceived in the visual field of the left lateral eye, which will be dominant, but there will be a less noted aura occurring in the nasal visual field of the right eye. Patients should be instructed to notice this.
Neuroimaging for the event.
This should usually be an MRI scan with contrast which is a better test than a CAT scan with contrast to view a stroke.
The patient should have a previous history of migraine with aura events.
This doesn’t always happen, but the neurologist should seek from the patient a history of previous events of migraine with aura.
A frequent possible exception here is a female patient who had previously experienced migraine before, but only migraine without aura events. Then her Obstetrician puts her on an estrogen-based birth control pill and during her next menstrual cycle, she has her first, scary, migraine with aura event which brings her in to see the neurologist.
This type patient is a Serious, Immediate, Fragile patient who is now, because of the migraine with aura event and on the pill at great risk for an imminent stroke.
She should be advised to get off the pill that day, and to never take it again.
Literature Review of Persistent Visual Aura Without Infarction
This rare migraine syndrome was first described by Sacks[ii] in 1970. He wrote that “recurrent cycles of paresthesiae may follow one another for hours on end, or alternate with cycles of scotomata, in a migraine “status.’”
Then Haas[iii] in 1982 writing in the Annals of Neurology in an article entitled “Prolonged migraine aura status” described 2 patients with migraine who “experienced a prolonged migraine aura status consisting of frequent recurrence of auras, hour after hour, day after day, for successive weeks.”
One of Haas’ patients was an 18-year-old boy who had experienced typical migraine with aura attacks for a few years. He usually saw a “bright zigzag arc” that moved and twice a “stationary bright arrowhead figure in the left field.” The aura was usually followed by right or left supraorbital headache, photophobia, nausea, and sometimes vomiting. Hass describes what happened next:
In October 1978, he began to experience a new visual phenomenon unassociated with headache. It burst into his vision in the form of concentric gray circles like ripples in a pond. Each attack lasted several seconds. The circles were faint and, though annoying, did not obstruct vision. Originally, he saw a set of concentric circles in front of him, but later saw clustered sets of concentric circles in the right visual field.
For about two months these clusters appeared frequently but irregularly and sometimes followed one another closely for hours. During one two-week period, attacks were unremitting, and he saw about 100 clusters each day. In February 1979, attacks became infrequent, and by April they had disappeared completely.
Thirteen years later Liu, et al,[iv] writing in Neurology in 1995 reported the first large series with 10 patients in a paper entitled “Persistent positive visual phenomenon in migraine.” Since then there have been many articles and comments on treatment.
An article by San-Juan and Zermeno[v] in 2007 in Cephalalgia describes persistent aura in a 28-year-old Mexican patient. This man had a 24-year history of migraine and presented with persistent aura symptoms described as “scintillating scotomas bilaterally associated with photopsias and amaurosis followed by migraine headache.” Neurologic imaging studies were negative, the episode lasted 35 days, and “probably resolved with nimodipine therapy.”
Case report
Wei-Ta Chen, Yung-Yang Lin, Jong-Ling Fuh, Matti S. Hämäläinen, Yu-Chieh Ko, Shuu-Jiun Wang Author Notes
Brain, Volume 134, Issue 8, August 2011, Pages 2387–2395.
Abstract
Persistent aura without infarction, a rare migraine disorder, is defined by aura symptoms that persist for >1 week without radiological evidence of cerebral infarction. To unveil its pathophysiological mechanisms, this study used magnetoencephalography to characterize the visual cortex excitability in persistent aura by comparison with episodic and chronic migraine.
We recruited six patients with persistent visual aura, 39 patients with episodic migraine [12 in ictal phase; 27 in interictal phase (with aura, n = 9; without aura, n = 18)], 18 patients with chronic migraine and 24 healthy controls. Five sequential blocks of 50 neuromagnetic prominent 100 ms responses were obtained, and the dynamic change in visual cortex excitability was evaluated by the percentage changes of individual mean prominent 100 ms amplitudes at blocks 2–5 compared with block 1, with a significant increase indicating potentiation.
We found that in patients with persistent aura, there was significant potentiation during ictal periods (P = 0.009 and 0.006 at blocks 2 and 5, respectively), and the excitability change was inversely correlated with the duration of aura persistence (correlation coefficient −0.812, P = 0.050, block 2). The interictal recordings (n = 3) also showed potentiation. In terms of the other migraine spectrum disorders, persistent aura differed from episodic migraine in the presence of ictal potentiation.
Persistent aura further differed from chronic migraine in the absence of interictal potentiation in chronic migraine. There was a higher percentage change of response amplitude at the end of stimulation (block 5) in persistent aura (43.3 ± 11.7) than in chronic migraine (−7.6 ± 5.5, P = 0.006) and ictal recordings of episodic migraine (−4.9 ± 9.6, P = 0.020).
Normal control subjects had no significant response changes. This magnetoencephalographic study showed that the visual cortex in patients with persistent visual aura maintains a steady state hyperexcitability without significant dynamic modulation. The excitability characteristic supports persistent visual aura as a nosological entity in migraine spectrum disorders and suggests a pathophysiological link to sustained excitatory effects possibly related to reverberating cortical spreading depression.
Luda’s[xxvi] patient saw "scintillating geometrical figures in the shape of either rings or chains…” Liu’s[xxvii] patient, a 29-year-old woman “experienced sudden disorientation followed by stars filling the visual field of both eyes, followed by a diffuse, non-pulsating headache.” These stars lasted five months and then disappeared.
Rothrock[xxviii] saw a patient who had a severe prolonged migraine for 2 months associated with “jagged zigzags like crushed broken glass to the left of a scotoma in her left visual field.” Some patients have experienced numbness and tingling on the same side of the body as the visual image.
The following is a detailed description by one of Hass’ affected patients.
“I am a 24-year-old female who started feeling 'out of it' around eight months ago. I've finally identified the out of it feeling as being a change in my vision. It's as though I have tunnel vision. I can make out whatever I am focusing on but everything around it seems splotchy.
It seems to have gotten worse over a period of time and six weeks ago I had an episode of flashing lights, blind spots, and wavy lines in my right hand field of vision (both eyes) for about 30 minutes (sounds like the symptoms of an optic migraine). The splotchiness in my peripheral vision never goes away. It didn't seem to be preceded by anything. I can't pinpoint a possible trigger.”
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References
Sacks OW: Migraine. The Evolution of a Common Disorder. Berkley and Los Angeles, University of California Press. 1970.
Haas DC. Prolonged migraine aura status. Ann Neurol. 1982;11:197-199.
Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.
San-Juan OD, Zermeno PF. Migraine with persistent aura in a Mexican patient: case report and review of the literature. Cephalalgia. 2007;27 (5),456–460.
Haan J, Sluis P, Sluis LH, Ferrari MD. Acetazolamide treatment for migraine aura status. Neurology. 2000;55:1588-1589.
Chen WT, Fuh JL, Lu SR, Wang SJ. Persistent migrainous visual phenomena might be responsive to lamotrigine. Headache. 2001;41:823-825.
Rozen TD. Treatment of a prolonged migrainous aura with intravenous furosemide. Neurology. 2000;55:732-733.
Haas DC, internet site. Persistent migraine aura without infarction. http://www.migraine-aura.org/content/e25968/e26078/e26305/index_en.html
Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-262.
Haas DC. Prolonged migraine aura status. Ann Neurol. 1982;11:197-199.
Blythe IM, Bromley JM, Ruddock KH, Kennard C, Traub M. A study of systematic visual perseveration involving central mechanisms. Brain. 1986;109:661-675.
Luda E, Bo E, Sicuro L, Comitangelo R, Campana M. Sustained visual aura: a totally new variation of migraine. Headache. 1991;31:582-583.
Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.
Chen WT, Fuh JL, Lu SR, Wang SJ. Persistent migrainous visual phenomena might be responsive to lamotrigine. Headache. 2001;41:823-825.
Spierings EL. Flurries of migraine (with) aura and migraine aura status. Headache. 2002;42:326-327.
Iizuka T, Sakai F, Suzuki K, Igarashi H, Suzuki N. Implication of augmented vasogenic leakage in the mechanism of persistent aura in sporadic hemiplegic migraine. Cephalalgia. 2006; 26: 332-335.
San-Juan OD, Zermeno PF. Migraine with persistent aura in a Mexican patient: case report and review of the literature. Cephalalgia. 2007;27 (5),456–460.
Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-262.
24-year-old female- in Haas DW. Persistent migraine aura without infarction. http://www.migraine-aura.org/content/e27891/e27265/e42285/e42286/index_en.html
Jager HR, Giffin NJ, Goadsby PJ. Diffusion- and perfusion-weighted MR imaging in persistent migrainous visual disturbances. Cephalalgia. 2005; 25: 323-332.
Relja G, Granato A, Ukmar M, Ferretti G, Antonello RM, Zorzon M. Persistent aura without infarction: decription of the first case studied with both brain SPECT and perfusion MRI. Cephalalgia. 2005; 25: 56-59.
Blythe IM, Bromley JM, Ruddock KH, Kennard C, Traub M. A study of systematic visual perseveration involving central mechanisms. Brain. 1986;109:661-675.
Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.
Haas DC. Prolonged migraine aura status. Ann Neurol. 1982;11(2):197-199.
Luda E, Bo E, Sicuro L, Comitangelo R, Campana M. Sustained visual aura: a totally new variation of migraine. Headache. 1991;31:582-583.
Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.
Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-262.
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Britt Talley Daniel MD
