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|Author: Klaus Podoll, Markus Dahlem and David C. Haas||12. March 2005|
|Edited by: Klaus Podoll, Markus Dahlem and David C. Haas|
Aura symptoms persisting for more than 1 week without radiographic evidence of infarction.
A. The present attack in a patient with 1.2 Migraine with aura is typical of previous attacks except that one or more aura symptoms persists for > 1 week
B. Not attributed to another disorder
Persisting aura symptoms are rare but well documented. They are often bilateral and may last for months or years. Reliably effective treatment is not known though acetazolamide and valproic acid have helped in a few cases. Exclude posterior leukoencephalopathy by diffusion MRI among other things. Exclude 1.5.4 Migrainous infarction by MRI.
© 2003 International Headache Society (quoted from International Headache Society, 2004)
By Klaus Podoll, Markus Dahlem and David C. Haas (retired)
Migraine aura status was not included among the sub-types of migraine listed in the 1988 classification of migraine by the International Headache Society (IHS), probably because the committee members were unaware of this condition. They did, however, list a class called migraine with prolonged aura. To be placed into this class, at least one of the patient's aura symptoms must last more than 60 minutes and less than 7 days.
In his 1982 report of two patients, David C. Haas used the term prolonged migraine aura status. Subsequent articles have used the following terms: 1. Sustained visual aura, 2. Persistent positive visual phenomena, 3. Persistent migraine aura, 4. Migraine aura status, 5. Persistent migrainous visual phenomena.
Finally, the Second Edition of the International Headache Classification by the IHS released in 2003 introduced the term 6. Persistent migraine aura without infarction for the aura status (for a recent review see Agostini and Aliprandi, 2006).
The symptoms are those of a migrainous visual aura that either recurs repetitively hour-after-hour, day-after-day for weeks, months, or years, or does not abate for weeks, months, or years. Aura symptoms other than visual may be a part of the picture, but so far no one has reported a migraine aura status composed soley of non-visual aura symptoms.
Patient 1 of Haas (1982): This 70-year-old man had stereotyped repetitive attacks several times per hour for about 5 weeks. His description of them is enhanced by his painted rendition of their appearance.
Illustration of visual patterns seen in prolonged migraine aura status by patient 1 of Haas (1982).
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.
The 7 patients with "aura status" reported by Haan et al.. (2000) experienced several mostly visual auras daily for weeks, very often without headache. They were asymptomatic between the auras.
Patient of Corral-Caramés et al. (2009): A 8 years-old girl who experiences during a month one to three brief episodes a day during which she relates: "I saw things as little and remote, sometimes they moved; one day I saw my sister’s books turning bigger, and another day my father getting little as a doll; sometimes my doll’s leg swinged, or the blind in the window got up and down". Later these attacks spaced out to one each to days for another two weeks. With no previous episodes of headache, these start two days after the visual distortions disappeared, with clinical features of migraine without aura. There were antecedents of migraine in maternal line, and no previous trauma, epilepsy, drug ingestion or psychiatric disorders. Clinical examination, cranial RMI, and EEG were normal.
Case of Blythe et al. (1986): This 24-year-old woman suffered from a relapsing and remitting neurological disorder for 3 years, her neurological symptoms including a variety of persisting visual disturbances with visual hallucinations of random form dimension ("white spots 'like fireflies'"), increased visual discomfort, increased afterimages and trails ("a streak along the path described by the moving object"). After 6 weeks of treatment with carbamazepine, the duration of the increased afterimages fell by about 30%, but no further reduction occurred over the following 3 months.
Case of Luda et al. (1991): This 65-year-old woman developed "scintillating scotomas" in her right visual field without headache on May 3, 1990 and they were still present and unremitting when the authors reported her problem over 12 months later. The hallucination was described as "scintillating geometrical figures (in the shape of either rings or chains)..."
Patient 3 of Liu et al. (1995): On November 10, 1992, this 29-year-old woman "experienced sudden disorientation followed by stars filling the visual field of both eyes, followed by a diffuse, nonpulsating headache. When the headache ceased, she was left with "constant flashing lights and circles which were worse at night..." These visual symptoms resolved spontaneously in April 1993. Months later, she experienced "zig-zag" lines for 10 minutes followed by a pulsating headache. She also had brief attacks of unilateral paresthesias sometimes followed by headaches both before and after the persistent aura.
Patient 1 of Chen et al. (2001): This 45-year-old woman reported seeing an occasionally flickering coin-sized white spot in her left field of view for 3 months. It began "after" a migraine headache without aura. It prevented reading.
Patient 2 of Chen et al. (2001):This 24-year-old woman complained of seeing numerous stars persistently flickering in her right visual field for 3 years. At times they formed a single light. This phenomenon developed during a migraine attack. She had suffered migraines from childhood. A visual aura of of bright yellow flickering stars lasting 30-60 seconds had occurred during most of her migraine headaches.
Patient of Spierings (2002): This 41-year-old man with migraine with typical visual aura from childhood developed his typical aura while upset in October 1996. It was unusually vivid and was accompanied this time by tingling in his left upper limb for 30 minutes. This was followed by severe headache with photophobia, generalized weakness, and confusion. The visual disturbance never disappeared, but has persisted to the time of Spiering's report. The patient sees things as though looking through a veil, and "bright-white, flickering, zigzag lines in the periphery of both visual fields" (his typical aura) come and go.
Patient of Oberndorfer et al. (2004): A 21-year-old male with familial hemiplegic migraine since the age of 3 years got a persistent aura without infarction with somnolence and right-sided hemiplegia with full remission within 24 day. Diffusion-weighted magnetic resonance imaging (DWI), perfusion-MR imaging (P-MRI) and [99mTc] hexamethyl-propyleneamine-oxime-single photon emission tomography (HMPAO-SPECT) were performed on day 2, when he was somnolent with right-sided hemiplegia, on day 9 when a mild hemiparesis was still present and on day 24 after recovery. The right central region showed normal findings in DWI, whereas P-MRI and SPECT revealed hyperperfusion on day 2, less marked on day 9, and normal findings on day 24.
Patient of Iizuka et al. (2005): A 35-year-old woman with sporadic hemiplegic migraine presented with headache followed by right arm weakness, right visual field defect, aphasia and confusion that persisted for 1 week.
Patient of San-Juan and Zermeño (2007): A 28-year-old Mexican woman had a 24-year history of migraine headache. In November 2005 she had an attack which started with scintillating scotomas bilaterally associated with photopsias and amaurosis followed by migraine headache. All imaging studies were negative. The episode of persistent aura lasted 35 days.
Patient of Koyama and Kawamura (2007): A 46-year-old Japanese female with a history of migraine with aura since the age of 15 underwent catheter ablation to treat her Wolff-Parkinson-White syndrome. In the middle of the procedure, triggered by the physical stress from the intervention, she developed a persistent aura without infarction with initial strong headache and subsequent persistent visual disturbances including "dazzling spots in the air, similar to the reflection of the water", increased negative afterimages and corona phenomenon. All imaging studies (MRI, SPECT), EEG and VEP were negative. The persistent aura symptoms remained stable over a follow-up period of 4 years.
Patient of Dodick and Roarke (2007): A 43-year-old male had a 16-year history of recurrent but infrequent attacks of hemiplegic migraine and a history of ca. 5 episodes of right hemiparesis, visual aura, severe headache, nausea, vomiting, and diminished level of consciousness lasting up to 3 months. In July 2003, he sustained another episode of persistent aura with right hemiparesis, right hemiapraxis, right hemianopsia and global aphasia with partial remission over the next 2 years. On follow-up, he still had a severe fluent aphasia and right-sided ideomotor apraxis. Despite the absence of cerebral infarctions in subsequent CT examinations, he had persistent evidence of left hemisphere glucose hypometabolism on PET and reduced cerebral blood flow on SPECT imaging.
Continuous, strongly fluctuating variety
Patient 1 of Rothrock (1997): Two months before she was seen by the author, this 61-year-old woman experienced a particularly severe prolonged migraine with "jagged zigzags like crushed broken glass" to the left of a scotoma in her left visual field. This hallucination persisted after the headache ceased. It had been fluctuating in size, without disappearing entirely. In addition, she had developed similarly fluctuating but persistent numbness and tingling in the left face and lips.
They can occur from childhood to old age. All reported patients had previously experienced either migraine headaches with visual auras, migraine visual auras without headache, or migraine headaches without auras, or various combinations of these migraine varieties.
EEGs on 21 patients showed abnormalities in only two, a father and daughter with occipital slow waves. So, the EEG evidence excludes an epileptic cause.
CT and MRI have not shown notable abnormalities. Thus, cerebral infarctions and tumors are well-excluded causes. Likewise, a recent investigation with advanced MR techniques (diffusion- and perfusion-weighted MRI) failed to detect significant changes of water diffusion or cerebral perfusion in the occipital regions, or elsewhere, between two migraine sufferers with a primary persistent visual disturbance (visual snow phenomenon) and two migraineurs with typical visual aura, suggesting that regional changes in cerebral water diffusion and perfusion do not play an important part in the pathophysiology of persistent migraine aura (Jäger et al., 2005).
SPECT scans have shown decreased cerebral perfusion in most of the patients scanned, and in those with phenomena in just one hemifield the decreases have been on the side opposite the perceptions (e.g. left field phenomena, right cerebral perfusion decrease). In some scans the perfusion decreases have been limited to the occipital lobe. These findings suggest that the affected cerebral regions are metabolically depressed, and they are compatible with the data from more quantitative tests in patients with typical migrainous auras. Relja et al. (2005) reported the first case of persistent aura without infarction studied with both brain SPECT and perfusion MRI, documenting decreased left fronto-parieto-occipital and right occipital blood perfusion at the onset of the persistent migraine aura, whereas a control of the perfusion MRI, performed 7 months after symptom onset and almost complete extinction of symptoms, was normal.
Afridi et al. (2005) presented the first magnetic resonance spectroscopy (MRS) study of 2 patients with persistent hemiparetic migraine aura. The results provided evidence for a difference in metabolic ratios between the proposed affected cortical region and the contralateralcortical region. The metabolic implications of this are, as yet, unclear but may involve altered energy metabolism.
Patient number 1 reported by Haas in 1982 (see above) had his frequent repetitive visual attacks fade away quickly 2 days after he began taking cyproheptadine and after he failed to improve on acetylsalicylic acid. Three of the 7 patients of Haan et al. (2000) (see above) with repetitive attacks similar to those of Haas were treated with acetazolamide (250 mg twice or thrice daily) and all 3 experienced cessation of their auras in a matter of days. When the drug was discontinued or its dosage was lowered, the auras returned. None of their 7 patients responded to valproate or propranolol.
Rothrock (1997) reported the disappearance of persistent auras during treatment with divalproex in 2 patients, and Chen et al. (2001) reported the disappearance of persistent auras during treatment with lamotrigine in 2 patients. A host of other drugs have been unsuccessful, including phenytoin, phenobarbital, carbamezepine, acetylsalicylic acid, inbuprofen, verapamil, nifedipine, nimodipine, flunarazine, amitriptyline, clonazepam, fluoxetine, sertraline, baclofen, and buspirone. However, topiramate (Topamax) may be effective for some patients,as indicated in the following e-mail message received by David C. Haas on June 14, 2002:
"I've suffered from Migraine Aura Status (although I didn't know to call it other than just Migraine) for approximately 2 1/2 years. I've been treated quite successfully for this with Topamax. I began taking 50 mg twice a day in January 2001 and within a couple of weeks the visual phenomenon cleared up. This spring we decreased the dosage to 25 mg in the morning kept the night dosage at 50 mg and within a week the visual symptoms began to return. I also experienced a migraine headache during this time. Needless to say, I'm back to the original dose and the symptoms have subsided."
Two continuous, prolonged migrainous visual auras, of 11 and 5 days duration were reported by Rozen (2000) to disappear within a few hours after an intravenous dose of 20 mg of furosemide. This drug was chosen, because it has been shown to inhibit the generation and duration of cortical spreading depression (the putative physiological cause of migrainous auras) induced by potassium in the cat. Although these two patients did not have migraine aura status as discussed above, perhaps they would have if left untreated.
The continuous persistent aura of 35 days duration of the 28-year-old Mexican woman reported by San-Juan and Zermeño (2007) probably resolved with nimodipine therapy.
The evidence so far suggests that acetazolamide may be the premier drug for patients with the repetitive form of aura status, and that divalproex (valproate), lamotrigine, or topiramate should be first choices for patients with the continuous form. When these oral drugs are ineffective, an intravenous injection or injections of furosemide should be tried.
David C. Haas has grouped both the repetitive and continuous forms of the prolonged visual migrainous auras under the rubric of migraine aura status since they both share the characterizing feature of affecting persons for weeks, months, or longer. Since the word "status" as used in "status epilepticus" means a prolonged condition, whether the seizures are repetitive or unremitting, its use for both of the prolonged aura states seems appropriate. Further studies might tell us whether these two aura states are basically the same condition or not.
Most of the materials of this webpage have originally been presented on the Upstate Medical University website Headache Cybertext: for the diagnosis and treatment of headaches and migraines by David C. Haas, removed from the web after his retirement December 31, 2004 (lost webpage, December 31, 2004).
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