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SYMPTOMS | PATHOMECHANISMS | HISTORY

Symptoms

Transitory aura symptoms

Persistent aura symptoms

Internet survey

German version

Worsening factor...

Improving factor...

VS and spiritual...

Persistent aura and spirituality

VS and arts

Case vignettes

FAQs

Author: Klaus Podoll, Markus Dahlem, Sofia Greene	28. August 2007
Edited by: Klaus Podoll, Markus Dahlem, Sofia Greene

Migraine Aura Status score

As has previously been mentioned, in the 2nd edition of the International Classification of Headache Disorders the migraine complication persistent aura without infarction is classified with a categorical diagnostic system. The two categorical diagnostic criteria: "(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", and "(B.) Not attributed to another disorder", have to be met with for the diagnosis to be made. All other patients with prolonged aura status symptoms (and it may well be expected that these will represent the majority of cases to be encountered in the spectrum of variant phenotypes of persistent aura without infarction) don't get this diagnosis, meaning that most often they don't get a diagnosis at all; no label, no name, which may have serious consequences in psychological (anxiety resulting from diagnostic uncertaincy), therapeutical (wrong treatments), socioeconomic (unnecessary investigations) and scientific terms (lack of research because of rarity of diagnosed patients).

As a way out, we suggest to classify the spectrum of persistent migraine aura with a diagnostic system that includes both categorical and dimensional diagnostic criteria, based on: The patient's prolonged Migraine Aura Status (MAS) symptom(s), and his history concerning headaches and/or transitory aura symptoms; the presence of these symptoms at the onset of the MAS symptoms; the existence of a prior history of MAS symptom(s); and worsening of MAS symptom(s) with acute attacks of headaches and/or transitory aura symptoms. The Migraine Aura Status score (MAS score), which ranges from 0-13 (see box below), operationalises the weight of clinical arguments to support the notion of a migrainous origin of the patient's prolonged aura, with higher values of the MAS score representing a greater amount of clinical evidence suggesting a migrainous origin of the persistent aura. Thus, a MAS of zero corresponds to the the diagnosis of primary persistent visual disturbance (visual snow phenomenon), which may nevertheless possibly represent a variant phenotype of long-lasting migraine aura (Jäger et al., 2005), whereas a maximum MAS score of 13 corresponds to a patient's history providing a huge amount of clinical evidence to support the diagnosis in question.

On the basis of the MAS score, the presence of persistent aura without infarction can be deemed to be either definite, probable, or possible, according to the diagnostic criteria proposed below.

Diagnostic criteria of definite, probable and possible persistent aura without infarction

A. History of one or more focal neurological symptoms persisting for > 1 week, i.e. migraine aura status (MAS) symptom(s), e.g. visual disturbances, tinnitus, aphasic disturbances, etc.

B. A MAS score ≥ 0 (range 0-10) according to the following criteria from the history:

1. Pre-MAS history of recurring headaches (1 point)
2. Post-MAS history of recurring headaches (1 point)
3. Pre-MAS history of transitory aura symptoms (1 point)
4. Post-MAS history of transitory aura symptoms (1 point)
5. Pre-MAS history of one or more MAS symptom(s) lasting > 1 week that were (a) fully reversible or (b) persistent up to present. (1 point)
6. Onset of MAS with (a) headache attack and (b) no other aura symptoms except MAS (1 point)
7. Onset of MAS with (a) attack of migraine with aura or migraine aura without headache and (b) MAS phenomena not having occurred previously as migraine aura symptom (2 points)
8. Onset of MAS with (a) attack of migraine with aura or migraine aura without headache and (b) MAS phenomena having occurred previously as migraine aura symptom (3 points)
9. Worsening of MAS intensity with attacks of migraine without aura (1 point)
10. Worsening of MAS symptom's intensity with (a) attack of migraine with aura (featuring transitory aura symptoms different than those experienced in MAS) or (b) attack of migraine aura without headache (featuring transitory aura symptoms different to those experienced in MAS ) (1 point)

C. Not attributed to another disorder.

Specify the clinical diagnostic certaincy: The presence of persistent aura without infarction is either definite (MAS score ≥ 4 and criterion B.8), probable (MAS score ≥ 4 without criterion B.8) or possible (MAS score < 4).

Relationship of classification according to MAS score to the classification from Liu et al. (1995)

Liu et al. (1995) classified patients into 3 groups according to the relationship between their persistent positive visual phenomena and their migraine.

Group 1: Persistent positive visual phenomena definitely secondary to migraine. These patients had visual aura with headache, followed by persistent visual phenomena. This criterion corresponds to MAS score ≥ 2 including item B.7(b) or MAS score ≥ 3 including item B.8(b).

Group 2: Persistent positive visual phenomena probably secondary to migraine. These patients had a history of migraine with aura. The temporal relationship between the onset of persistent positive visual phenomena and migraine events is not as clear as that in group 1. However, headaches occurred during the persistent visual phenomena, suggesting some association. This criterion corresponds to MAS score ≥ 3 including item B.1, B.2 and B.3 but not B.7 or B.8.

Group 3: Persistent positive visual phenomena possibly a migraine equivalent. These patients had suggestive migraine histories, but the persistent positive visual phenomena developed without any associated migraine events. This criterion corresponds to MAS score ≥ 1 including item B.1 but not B.2, B7. or B.8.

The application of the suggested diagnostic criteria is demonstrated by the case histories from participants in Sofia Greene's internet survey (pending) and by the collection of case vignettes based on data from various internet resouces (see here).

References

International Headache Society. The International Classification of Headache Disorders, 2nd edition. Cephalalgia 2004; 24 (suppl. 1): 1-160.
Jäger HR, Giffin NJ, Goadsby PJ. Diffusion- and perfusion-weighted MR imaging in persistent migrainous visual disturbances. Cephalalgia 2005; 25: 323-332.
Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology 1995; 45: 664-668.

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Pre-publication research on migraine with aura