Outcome of definite persistent aura

The outcome of the episodes of persistent aura without infarction was rated as complete remission (CR), partial remission (PR), stable disease (SD) and progressive disease (PD), respectively.

As a single episode of persistent aura can comprise multiple persisting symptoms that may differ in their time course of improvement, stability or worsening, these four categories of outcome need further definition.

Variations in time course of individual symptoms during a single episode of persistent aura without infarction

"While the VS could recover from the episodes [i.e. migraine attacks] in which my vision got worse, and so has moved in waves of getting better and worse (imagine the stock market), the ghost images have never improved (getting worse step by step)."

(Tom9998 [subject #106], Ezboard forum Visual snow or static- Discussion – update, May 21, 2006; additions in square brackets by Klaus Podoll)

To be coded as an episode with complete remission, all persistent symptoms that had occurred or significantly worsened during this episode must have fully remitted or returned to baseline severity. To be coded as an episode with partial remission, at least one of the symptoms that had occurred or significantly worsened during this episode must have improved and none of the symptoms must have progressively worsened over the entire duration of the episode. To be coded as an episode presenting as stable disease, all symptoms that had occurred or significantly worsened during this episode must retain the maximum intensity achieved during the episode and none of them must show a remission, an improvement, a return to baseline intensity or a worsening. Finally, to be coded as an episode presenting as progressive disease, at least one of the symptoms that had occurred or significantly worsened during this episode must have progressively worsened over the entire duration of the episode.

Only 45,8 % of all 166 episodes, sustained by 60 subjects with a diagnosis of definite persistent aura without infarction, show a complete or partial remission, which was in most cases a large remission, whereas in 51,2 % of the episodes one encounters a stable and in 3,0 % a progressive disease. As these rates include the data of a single subject (#175) with 29 episodes of persistent aura fully remitting each time, separate calculations were also made for the 60 first episodes and the 106 further episodes from the 60 subjects. Only 25,0 % of the 52 subjects' first episodes of persistent aura display a complete or large partial remission, whereas 71,7 % of the first episodes show a stable and 3,3 % a progressive disease.

In none of the 40 episodes with complete remission, but in 3 of the 36 episodes with partial remission, the improvement was attributed to the effect of pharmacotherapy.

In one of these observations, the reported pattern of drug administration mimicked a single-case experimental A-B-A-B design. The A-B-A-B design represents an attempt to measure a baseline (the first A), a treatment measurement (the first B), the withdrawal of treatment (the second A), and the re-introduction of treatment (the second B). Thus, after two years of progressive worsening ("unfortunately, many visuals slowly developed ... The thing is worsening: longer afterimage, and more shimmering, night vision is getting worse"), a treatment with Deanxit (a combination of the antidepressive agent melitracene and the neuroleptic drug flupentixole) brought a partial remission of Tadpolefighter's (subject's #8) persistent symptoms over the next four years. "I am very sure the improvement is directly due to the medication. I had tried a few times to stop Deanxit, but after a few days, I would noticed the after-image become more obvious and last longer. Same to the flickering and vibration. Once I continued the medication again, the visual improved."

(Tadpolefighter [subject #8], Ezboard forum Visual snow or static – Discussion - Visual due to depression?, December 11, 2002)

After three months' standing of the visual disturbances of Sheri's (subject's #86) 1st episode of persistent aura, a treatment with Verapamil "reduced the visuals" within a week "and almost eliminated the headaches".

(Sheri [subject #86], Ezboard Forum Visual snow or static - Discussion - Persistent migraine aura, February 18, 2006)

After having suffered from the repetitive variety of persistent aura without infarction with stereotyped daily attacks of expanding fortification spectra for 10 months, SF (subject #85) convinced his neurologist to prescribe a pharmacotherapy with valproic acid (Rothrock, 1997). On 2 months follow-up, SF reported: "I am very pleased to inform you that since I started taking the Sodium Valproate (Depakine Chrono 300) my aura symptoms have diminished dramatically. They have not gone entirely, but the change has been dramatic. It seems that the symptoms only return when I am very tired or stressed. I have attached my diary which I am still keeping. The diary clearly shows the reduction in symptoms. Thank you very much for your help. I will keep you informed of my progress."

(SF [subject #85], Email to Klaus Podoll, February 11, 2007)

The episodes of persistent aura without infarction showing a continuous progressive disease course may actually represent a sequence of discrete "micro-episodes" (not fulfilling the defined criteria of a distinct "episode" of persistent aura) brought on by recurrent attacks of migraine with aura, as suggested by the four following posts from email correspondence and internet forums devoted to visual snow.

Outcome of episode of persistent aura: Progressive disease

"Indeed it seems to me that, as time passes (and thus as the number of experienced ocular migraine attacks increases), my eyes do not get better but worse."

(History student, Forum Augen 8453, Subject: Augenmigräne und andere Augenprobleme, Oktober 25, 2003)

"I have suffered from a similar shimmering visual disorder... It has progressively worsened over 15 years with cumulative migraine events ... neurologists and ophthalmologists are befuddled by the symptoms..."

(macdonir, Ezboard forum Visual snow or static – Archive – shimmering vision, January 12, 2004)

"Since then [1999] my vision/balance has never returned to normal and I'm not suggesting that my symptoms are one ongoing migraine aura, but I suspect that my vestibular/visual system (or the part of the brain that's desperately trying to compensate for the damage) keeps getting injured by migraine events which keep recurring or inflamming the problem."

(jimjones235 [subject #172], Ezboard forum Visual snow or static – Discussion – New here, September 4, 2006)

"I have had visual snow for over 30 years – I am an otherwise healthy 44 year old male... I am convinced VS is migraine related since my VS started with the onset of my first migraine when I was a teenager and gets worse after each attack."

(Scott [subject #261], Email to Klaus Podoll, January 2, 2007)

Wang et al. (2008) published a systematic analysis of 29 patients (23 from the literature and 6 own) in terms of demographics, headache and visual symptom profiles, treatment regimens and outcomes. Even though the majority of patients had headache improvement, only 8 had complete resolution of persistent aura symptoms, without definite relevance to any specific agent. Patients with complete resolution of persistent aura symptoms tended to have scotoma, unilateral/homonymous involvement of visual symptoms, a higher number of symptoms more typical of migraine visual aura and a shorter duration of illness compared with those without. In conclusion, the prognosis of persistent aura was poor, and a higher number of symptoms more typical of migraine visual aura predicted a better outcome. For those with a potential for complete resolution, improvement would occur early in the course.

Peatfield et al. (2009) sought to assess the prognosis of persistent aura without infarction on the basis of a retrospective case series of 15 patients with symptoms lasting a minimum of 2 months, without permanent physical signs or CT or MRI scan abnormalities. These were collected from the case records of the Princess Margaret Migraine Clinic, Charing Cross Hospital, from 1980 to 2009. The authors reviewed the records and saw or telephoned the patients when necessary, to obtain an updated diagnosis. The persistent aura symptoms included blurring and shimmering of vision, with wiggly, twig-like or zigzag lines, persistent afterimages, and weakness of one leg with speech arrest. Headache was never a significant problem. The symptoms in 4 of the patients had resolved after a mean of 7 months. Another 4 were lost to follow-up, and the symptoms in the remaining 7 were continuing for between 2 months and 10,5 years. Thus, about a quarter (26,7%) of the patients completely remitted spontaneously within a few months and the remainder seem to continue indefinitely. None of the patients came to any long-term harm.

References

Peatfield R, Weatherall M, Mehta A, Waldman A. The prognosis of persistent migrainous aura. Cephalalgia 2009; 29 (Suppl. 1): 129.
Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology 1997; 48: 261-262.
Wang YF, Fuh JL, Chen WT, Wang SJ. The visual aura rating scale as an outcome predictor for persistent visual aura without infarction. Cephalalgia 2008; 28:1298-1304.