Persistent Aura without Infarction

Very rarely a patient with migraine with aura will experience a persistent visual aura, which is usually present in bilateral visual fields and in central vision, superimposed on normal vision.  ICHD II[i] notes the typical aura should persist longer than a week and have no radiographic evidence of infarction.  The lead in picture shows an example of "visual snow" which may come with this syndrome.

Literature Review

This rare migraine syndrome was first described by Sacks[ii] in 1970.  He wrote that “recurrent cycles of paraesthesiae 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.”

The duration  of visual aura may be months or years and may be relieved by treatment with acetazolamide (Haan),[vi] lamotrigine (Chen),[vii] furosemide (Rozen),[viii] topiramate (Haas email),[ix] or valproic acid (Rothrock).[x]

The attacks may be continuously repetitive Haas,[xi] continuous-Blythe,[xii] Luda,[xiii] Liu,[xiv] Chen,[xv] Spierings,[xvi] Iizuka,[xvii] San-Juan and Zermeno,[xviii] or continuous with strong fluctuation, Rothrock.[xix]  Haas[xx] has referred to the phenomenon as “migraine aura status.”  Persons affected with these attacks are aged childhood to old age and they have experienced migraine with visual aura, migraine with visual aura without headache, or migraine without aura.

EEGs have been performed on 21 patients and 2 cases showed occipital slow waves but no epileptic activity.  CAT and MRI scans have not show anything wrong and diffusion- and perfusion-weighted MRI[xxi] were also normal.  SPECT scans have revealed a reduction in cerebral perfusion in most patients with the syndrome who have been scanned (Relja).[xxii] 

Patients typically describe what they see as “visual snow.”  Blythe’s[xxiii] patient described "white spots like fireflies.” Liu’s[xxiv] ten patients described what they saw as:

 “a million dots, black cracks and lines, blobs of white and gray, blue squares, bubbles, carpet background, circles, clouds, comets, grainy vision, heat waves, flashing or flickering lights, lines of ants, photopsias, rain-like pattern, snow, or TV static.”

Haas’[xxv] patient drew a picture of what he saw and Haas described the patient’s experience:

“Each repetition began with slowly undulating thick gray lines, which changed in a few minutes into a pinwheel of bright whirling color in his left visual field. Several minutes later this image slowed down and disappeared. After more than a week of suffering these hallucinations, he also developed brief attacks of “electrical” paresthesias in his left hand. These were less frequent than the visual phenomena and alternated with them irregularly. Throughout his ordeal, he had a dull headache over his right eye.”

Drawing by patient 1 of Haas (1982)

 

 

Persistent positive visual phenomena seen by patient 4 of Liu, et al. (1995).

Visual snow-patient of Henrik

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’[xxix] 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 splotchness in my peripheral vision never goes away. It didn't seem to be preceeded by anything.  I can't pinpoint a possible trigger.”

[i] The International Classification of Headache, 2nd Edition.  Persistent aura without infarction. Cephalalgia.  2004. 24;(Supplement 1):32.

[ii] Sacks OW: Migraine. The Evolution of a Common Disorder. Berkley and Los Angeles, University of California Press.  1970.

[iii]  Haas DC.  Prolonged migraine aura status. Ann Neurol.  1982;11:197-199.
[iv] Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS.  Persistent positive visual phenomena in migraine. Neurology.  1995;45:664-668.

[v] 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.
[vi] Haan J, Sluis P, Sluis LH, Ferrari MD.  Acetazolamide treatment for migraine aura status.  Neurology. 2000;55:1588-1589.

[vii] Chen WT, Fuh JL, Lu SR, Wang SJ.  Persistent migrainous visual phenomena might be responsive to lamotrigine.  Headache.  2001;41:823-825.

[viii] Rozen TD.  Treatment of a prolonged migrainous aura with intravenous furosemide.  Neurology. 2000;55:732-733.

[ix] Haas DC, internet site.  Persistent migraine aura without infarction. http://www.migraine-aura.org/content/e25968/e26078/e26305/index_en.html

[x] Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-262.

[xi]  Haas DC. Prolonged migraine aura status. Ann Neurol.  1982;11:197-199.

[xii] Blythe IM, Bromley JM, Ruddock KH, Kennard C, Traub M. A study of systematic visual perseveration involving central mechanisms. Brain. 1986;109:661-675.

[xiii] Luda E, Bo E, Sicuro L, Comitangelo R, Campana M. Sustained visual aura: a totally new variation of migraine. Headache. 1991;31:582-583.

[xiv] Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS.  Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.

[xv] Chen WT, Fuh JL, Lu SR, Wang SJ. Persistent migrainous visual phenomena might be responsive to lamotrigine. Headache. 2001;41:823-825.

[xvi] Spierings EL.  Flurries of migraine (with) aura and migraine aura status. Headache.  2002;42:326-327.

[xvii] 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.

[xviii] 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.
[xix] Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-262.

[xx] 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

[xxi] Jager HR, Giffin NJ, Goadsby PJ. Diffusion- and perfusion-weighted MR imaging in persistent migrainous visual disturbances. Cephalalgia. 2005; 25: 323-332.
[xxii] 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.

[xxiii] Blythe IM, Bromley JM, Ruddock KH, Kennard C, Traub M. A study of systematic visual perseveration involving central mechanisms. Brain. 1986;109:661-675.

[xxiv] Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS.  Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.

[xxv] Haas DC.  Prolonged migraine aura status.  Ann Neurol.  1982;11(2):197-199.

[xxvi] Luda E, Bo E, Sicuro L, Comitangelo R, Campana M. Sustained visual aura: a totally new variation of migraine. Headache. 1991;31:582-583.

[xxvii] Liu GT. Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS.  Persistent positive visual phenomena in migraine. Neurology. 1995;45:664-668.

[xxviii] Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261-262.

[xxix] 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