Diagnostic criteria of persistent aura

NN [subject #466], Sequence of symptoms at onset of persistent aura without infarction, 2007. © 2007 NN

A 21-year-old female sufferer from migraine with aura wrote: "The current migraine with aura is still present. It started whilst I was driving, I noticed a small patch of shimmering starting in my right eye, in the top right hand corner of my vision. At the time of onset I did not feel well, I had a fever, a headache, was nauseous, dizzy and felt generally unwell. My vision was affected, the aura seemed to cover the top half of my vision, more so towards the right hand side. When I awoke the next morning I had a splitting headache. My aura had changed in the sense that my vision had improved, yet the initial patch of shimmering/twinkling was still there. It was bold and very noticeable."

(NN [subject #466], Email to Klaus Podoll, November 8, 2007)

David C. Haas, MD. © 2006 David C. Haas

Persisting migraine aura symptoms are rare but well documented. They may last for weeks, months or years. These symptoms are characteristic of a type of migraine with aura that was first described in 2 cases by the American neurologist, David C. Haas (1982), under the designation "prolonged migraine aura status".

Grant T. Liu, MD © 2006 Grant T. Liu

It was not until 13 years later that Grant T. Liu and associates (1995) reported the first large series of 10 cases with the given condition in a paper entitled "Persistent positive visual phenomena in migraine".

Illustration of persistent positive visual phenomena seen by patient 4 of Liu et al. (1995). "Here's a drawing from the child in the article. You can use it in your website." (Grant T. Liu, Email to Klaus Podoll, August 14, 2007) © 1995 Grant T. Liu (for larger image see here)

Their patients' "complaints were similar in their simplicity and involvement of the entire visual field and usually consisted of diffuse small particles such as TV static, snow, lines of ants, dots, and rain" (Liu et al., 1995, p. 664).

Logo of the International Headache Society. © 2006 International Headache Society

According to the 2nd edition of the International Classification of Headache Disorders from 2004, persistent aura without infarction is defined by aura symptoms persisting for more than 1 week without radiographic evidence of infarction (for a recent review see Boudreau and Leroux, 2006 [PDF]).

Illustration of visual patterns seen in prolonged migraine aura status by patient 1 of Haas (1982).

Kiki O [subject #264], Transitory visuaI snow occurring as recurring aura symptom during attacks of migraine with aura, 2007. © 2007 Kiki O [more]

Kiki O [subject #264], Persistent visual snow brought on by persistent aura without infarction, 2007. "Whether you open your eyes or keep them shut. You haven't experienced darkness for a long time. You always see more than there really is." © 2007 Kiki O [more]

However, as regards the phenomenology of the persistent aura symptoms, the said ICHD-II criteria require that the present attack in a patient with migraine with aura is typical of previous attacks, except that one or more aura symptoms persist for over 1 week (i.e., the aura symptom that now persists for > 1 week must have occurred earlier in the patient's history as a transient migraine aura symptom). As can be seen from analysing the case histories from migraineurs reporting persisting visual disturbances, or other persisting aura symptoms, this additional criterion is not always met with, and the currently established diagnostic criteria may be too narrow to account for all types of clinical course of the persisting aura symptoms as reported by migraine sufferers, as there is infinite variability in patients.

Metro Dynamics, Window, 2006. "In addition to the persistent 'buzz' or static-like disturbance and floaters, I've depicted the dim dark pulsations that sometimes occur at the lower periphery of my vision. The scene is a view out an airplane window." © 2006 Metro Dynamics [more]

Consider, for example, the following case report: "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've been to a series of doctors over the last six months (allergist, neurologist, neurosurgeon, ophthalmologist). My labs, MRI, and visual field test show nothing and the ophthalmologist found that I have 20/15 vision in both eyes. I am scheduled to fly to Oklahoma to see an neuro ophthalmologist in the next few weeks. I guess I'm posting here to see if anyone has the same symptoms and has gotten a diagnosis and or treatment since this has baffled every doctor I've seen so far. I haven't been able to drive for a month and a half and my life is basically on hold, school, business, everything. I can't focus well enough to read a book. Any leads would be helpful. Thanks, grace" - "During the last ... months I have struggled with blotchy vision and ocassional dizzy spells. My dizziness is not to the extreme of falling, but more as though the room were rocking back and forth like a boat... I am scheduled to see a neuro-ophthalmologist in a few weeks who has suggested a variation of migraine without headache could be responsible for my problems."

(gracefell, Brain Talk Communities – Specific Neurological Conditions (M-Z) – Visual Impairments – tunnel vision, migraine aura?, March 29, 2004; gracefell, Ezboard forum Visual snow or static – Archive – fuzzy, and sometimes very blurry eyesight, April 18, 2004)

As a consequence of the current formulation of the diagnostic criteria of persistent aura without infarction, even a doctor considering this diagnosis in assessing and counselling a migraineur may be left in the position of diagnosing, at best, an "atypical variant" of the said clinical type of migraine with aura; leaving open diagnostic doubts in both doctor and patient, with concern, anxiety and depression being possible consequences of the sufferer's insecure diagnostic status.

HenrikKJ [subject#074], Visual snow, 2006. © 2006 HenrikKJ

HenrikKJ [subject#074], Visual snow, 2006. © 2006 HenrikKJ

"I also suffer from constant strange visuals that have gotten progressively worse over the last four years. Some doctors said migraine, others said no way. None have any real answers for me."

(PatioGazer, Brain Talk Communities – Specific Neurological Conditions (M-Z) – Visual Impairments – tunnel vision, migraine aura?, April 6, 2004; see also here)

References

Boudreau G, Leroux E. The complications of migraine classified under the International Classification of Headache Disorders: a review. Headache Care 2006; 3: 85-90. [PDF]
Haas DC. Prolonged migraine aura status. Ann Neurol 1982; 11: 197-199.
International Headache Society. The International Classification of Headache Disorders, 2nd edition. Cephalalgia 2004; 24 (suppl. 1): 1-160.
Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology 1995; 45: 664-668.

Acknowledgment: We wish to thank David C. Haas for his support and advice whilst pursueing the current project.

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