migraine with focal aura

Last edited 11/2020 and last reviewed 03/2023

With classical migraine syndrome (migraine with aura) (1,2):

• the attack may be preceded for several hours by prodromal symptoms
• an attack begins with an aura often consisting of bright specks of light - other visual symptoms such as hemianopia, expanding scotoma, constricted visual fields or blindness may occur
• aura usually precedes the headache, but may accompany it and usually lasts 15-20 min headache may be throbbing and lateralised over one eye - other patterns include occipitally localised or generalised; stooping or straining worsens the headache
• attack may be accompanied by a combination of malaise, nausea, vomiting, photophobia, pallor and later diuresis
• headache may last from several hours to a few days
• diagnosis requires at least two attacks, both fulfilling the following criteria:
  • aura has at least one of the following characteristics (but no motor weakness)
    • fully reversible visual symptoms (e.g. flickering lights, spots or lines; or loss of vision)
    • fully reversible sensory symptoms (e.g. paraesthesiae, numbness)
  • the aura, in addition, has at least two of the following features
    • visual symptoms are homonymous (affect both visual fields on the same side), or the sensory symptoms are unilateral
    • at least one symptom develops gradually over more than 5 minutes, or different symptoms occur in succession over more than 5 minutes
    • each symptom lasts 5-60 minutes
  • the headache begins during the aura, or follows the aura within 60 minutes
  • the attack is not attributable to another disorder

A more comprehensive definition of classical migraine is provided by the International classification of headache disorders (3)

• At least two attacks that meet the following three criteria:
• One or more of the following fully reversible aura symptoms:
• Visual
• Sensory
• Speech, language, or both
• Motor
• Brain stem
• Retinal
• At least three of the following six characteristics:
• At least one aura symptom spreading gradually over a period >=5 minutes
• Two or more aura symptoms occurring in succession
• Each aura symptom lasting 5–60 minutes (*)
• At least one unilateral aura symptom (**)
• At least one positive aura symptom (***)
• Headache accompanying the aura or following the aura within 60 minutes

Not better accounted for by another ICHD-3 diagnosis

• (*) When, for example, three symptoms occur during an aura, the acceptable maximal duration is 3-60 minutes. Motor symptoms may last up to 72 hours.
• (**) Aphasia is always regarded as a unilateral symptom; dysarthria may or may not be.
• (***) Scintillations and pins and needles are positive symptoms of aura
  
  
• aura is the complex of neurological symptoms that occurs usually before the headache of "Migraine with aura"
• may begin after the headache phase has commenced, or continue into the headache phase
• visual aura is the most common type of aura - occurring in over 90% of patients with migraine with aura, at least in some attacks
• visual aura may move across the visual field, and may cross the midline. Examples of visual aura are:
  • fortification spectra, which are often 'C'-shaped
  • a zigzag figure near the point of fixation that may gradually spread right or left and assume a laterally convex shape with an angulated scintillating edge, leaving absolute or variable degrees of relative scotoma in its wake
  • scotoma
• scotoma without positive phenomena may occur; this is often perceived as being of acute onset but, on scrutiny, usually enlarges gradually
• objects may rotate, oscillate, or 'boil'
• simple flashes, specks, and shimmerings
  
  
• paraesthesiae are the second most common type of aura. Numbness usually starts in the hand, migrates up the arm, then involves the face, lips, and tongue. The leg is sometimes involved. Numbness may follow the paraesthesia. Sensory auras rarely occur alone, and usually follow visual auras
  
  
• speech disturbances are the third most common type of aura
• usually aphasic but often hard to categorize - these are less frequent than either visual aura or sensory disturbances
• patients with visual aura occasionally have symptoms in the extremities and/or speech symptoms. Conversely, patients with symptoms in the extremities and/or speech or language symptoms almost always also experience visual aura symptoms at least during some attacks
  
• most people who have migraine with aura also have episodes of migraine without aura



• in children and adolescents, less typical bilateral visual symptoms occur that may represent an aura (3)
  
  
• distinction between migraine with visual aura, migraine with hemiparaesthetic aura and migraine with speech and/or language aura is probably artificial, and therefore not recognized in this classification: they are all coded as 1.2.1 Migraine with typical aura (3)
  
  
• if multiple aura symptoms
• usually follow one another in succession, beginning with visual, then sensory, then aphasic; but the reverse and other orders have been noted


• duration of aura symptoms
• most aura symptoms last up to one hour - however motor symptoms are often longer lasting (3)
• with aura symptoms arising from the brainstem are coded as "Migraine with brainstem aura", but they almost always have additional typical aura symptoms
• when aura includes motor weakness, the disorder should be coded as " Hemiplegic migraine" or one of its subforms
  
  
• prodromal symptoms in migraine with aura
• may begin hours or a day or two before the other symptoms of a migraine attack with aura
• include various combinations of fatigue, difficulty in concentrating, neck stiffness, sensitivity to light and/or sound, nausea, blurred vision, yawning and pallor
• term ‘prodrome’, which has replaced ‘premonitory phase’ or ‘premonitory symptoms’, does not include aura

NICE state with respect to aura and migraine:

Suspect aura in people who present with or without headache and with neurological symptoms that:

• are fully reversible and
• develop gradually, either alone or in succession, over at least 5 minutes
• and last for 5-60 minutes

Diagnose migraine with aura in people who present with or without headache and with one or more of the following typical aura symptoms that meet the criteria above:

• visual symptoms that may be positive (for example, flickering lights, spots or lines) and/or negative (for example, partial loss of vision)
• sensory symptoms that may be positive (for example, pins and needles) and/or negative (for example, numbness)
• speech disturbance

Reference:

1. Patient Information Collection.Migraine Headaches: How to Deal with the Pain.American Family Physician 2005; 72: 9
2. Silberstein SD, Lipton RB and Goadsby PJ (2002). Headache in clinical practice. 2nd edition.London: Martin Dunitz.
3. Headache Classification Committee of the International Headache Society (IHS). The international classification of headache disorders, 3rd edition. Cephalalgia 2018; 38: 1-211
4. NICE (September 2012). Headaches - Diagnosis and management of headaches in young people and adults