Retinal Migraine - The Basics

Diagnosing Migraine

One of the difficulties encountered at times when discussing Migraines occurs when a Migraineur is given a diagnosis that isn't actually accurate in diagnostic terms, but is really a descriptive term. Such terms may be used fairly frequently, but they fall short of a diagnosis and may also be used differently from one doctor to another. That's one reason why most doctors diagnose based in the International Headache Society's International Classification of Headache Disorders, 2nd Edition (ICHD-II). A "standard" diagnosis also makes communications and transitions easier when patients need to consult other doctors or change doctors.

There are several terms that are sometimes used, supposedly as Migraine diagnoses, that involve visual symptoms. Most of them aren't actually standard Migraine diagnoses. Retinal Migraine, however, is an actual Migraine diagnosis. What becomes confusing about it is that it's sometimes misused, resulting in a misdiagnosis. The term "retinal Migraine" is often misused to mean any Migraine that involves any visual symptoms or a Migraine with visual symptoms but without the headache phase of the attack. 

Retinal Migraine Symptoms:

Retinal Migraine is Migraine where there are repeated attacks of visual disturbances preceding the headache phase of the Migraine attacks.

A retinal Migraine attack begins with monocular (in one eye) visual symptoms that can include:

1. scintillations (seeing twinkling lights)
2. scotoma (areas of decreased or lost vision)
3. temporary blindness.

The headache phase of a retinal Migraine begins during or within 60 minutes of the visual symptoms. The headache phase presents symptoms consistent with Migraine without aura:

·         Headache duration of 4-72 hours

·         At least two of these characteristics:

1.      unilateral (on one side) location

2.      pulsatile quality (pulsing or throbbing)

3.      moderate or severe pain intensity

4.      aggravation by or causing avoidance of routine physical activity such as walking or climbing stairs

At least one of these characteristics:

1.      nausea and/or vomiting

2.      photophobia (increased sensitivity to light) and phonophobia (increased sensitivity to sound) 

The primary differentiating factors between retinal Migraine and Migraine with aura are:

1. The visual symptoms of retinal Migraine are monocular.
2. Total, but temporary, monocular blindness may occur in retinal Migraine.
    

Diagnosing Retinal Migraine:

There are no diagnostic tests to confirm retinal Migraine. Diagnosis is accomplished by reviewing the patient's personal and family medical history, studying their symptoms, and conducting an examination. Retinal Migraine is then diagnosed by ruling out other causes for the symptoms. With retinal Migraine, it is essential that other causes of transient blindness be fully investigated and ruled out. 

Retinal Migraine Treatment:

For infrequent attacks, medications used for other forms of Migraine are often employed to relieve the other symptoms. These medications can include NSAIDs, antinausea medications, Midrin, ergotamines the triptans. The choice of medications is somewhat affected by the age of the patient. When Migraines are frequent, the same preventive therapies used for other Migraines can be explored.

The more technical explanation:

In the ICHD-II, retinal Migraine is described as, Repeated attacks of monocular visual disturbance, including scintillations, scotomata or blindness, associated with Migraine headache.
The diagnostic criteria for retinal Migraine under ICHD-II are:

A.     At least 2 attacks fulfilling criteria B and C

B.      Fully reversible monocular positive and/or negative visual phenomena (e.g., scintillations, scotoma or blindness) confirmed by examination during an attack or (after proper instruction) by the patient’s drawing of a monocular field defect during an attack

B.

C.     Headache fulfilling criteria B–D for Migraine without aura begins during the visual symptoms or follows them within 60 minutes

D.     Normal ophthalmological examination between attacks 

The relevant diagnostic criteria for Migraine without aura are:

B.      Headache attacks lasting 4–72 hours (untreated or unsuccessfully treated)

C.     Headache has at least two of the following characteristics:

1.      unilateral location

2.      pulsating quality

3.      moderate or severe pain intensity

4.      aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs

D.     During headache at least one of the following:

1.      nausea and/or vomiting

2.      photophobia and phonophobia

 

---

Resources:
"The International Classification of Headache Disorders, 2nd Edition." Cephalalgia 24 (s1). doi: 10.1111/j. 1468-2982.2003.00824.x
Randolph W. Evans, Nina T. Mathew. "Handbook of Headache, Second Edition." Philadelphia: Lipincott Williams & Wilkins. 2005.

 

Ocular, Optical, and Ophthalmic Migraines

Ocular, Optical, and Ophthalmic Migraines
Migraine disease is not only painful and potentially debilitating, it can be confusing. There are different types of Migraine, and some should be approached and treated differently than others. That makes it important that Migraine be properly diagnosed.
In any health field, there needs to be standardization in diagnosing. If every doctor used different diagnostic criteria and classifications, there would be total chaos. It would be impossible to communicate with patients, other doctors, researchers, etc. In the field of Migraine disease and headaches, the gold standard for diagnosis and classification is the International Headache Society's International Classification of Headache Disorders, 2nd Edition (ICHD-II).

Questions often arise about ocular, optical, and ophthalmic Migraines. These questions, however, are difficult if not impossible to answer because there are no such Migraine classifications in the ICHD-II, no such diagnosis listed there. Although there are doctors who use these diagnoses, they use them differently, making it difficult for anyone else to enter a discussion or answer questions.
 
Examples:
Mary Jane reports having been diagnosed with ocular Migraines. Her Migraines typically beginning with six to 18 hours of mood swings, excessive yawning, food cravings, and unusually frequent urination followed by tiny blind spots in her vision (scotoma) and  extreme sensitivity to light (photophobia) and sound (phonophobia). These symptoms are followed by a headache that is on one side (unilateral), throbbing with her pulse (pulsatile), and moderate to severe in intensity. Her ICHD-II diagnosis? Migraine with aura. She sometimes has the same symptoms, but without the headache. The ICHD-II diagnosis for those Migraine attacks is still Migraine with aura, but the descriptive term acephalgic (meaning without head pain) is added, acephalgic Migraine with aura.
Lou has been diagnosed as having optical Migraines. She reports having quickly developing intense headaches on the right side of her head, focused around her eye. She also reports extreme nausea and vomiting. Her optometrist diagnosed her with optical Migraines. Her ICHD-II diagnosis? Migraine without aura.

Dianna was diagnosed with ophthalmic Migraines. Her first symptom was complete blindness in one eye (monocular). This was followed by phonophobia, nausea, and a mild headache. The blindness resolved by the time the headache was over. Her ICHD-II diagnosis? Retinal Migraine.

 
If you've been diagnosed with ocular, optical, or ophthalmic Migraines, you may encounter some confusion when talking with other Migraineurs or seeing doctors other than the doctor who diagnosed your Migraines. The examples above are not meant to be applied to anyone else, but to show how differently terms are used when they're not used with any established criteria. To better educate yourself about Migraine disease, particularly how it affects you, ask your doctor if he's familiar with the International Headache Society's International Classification of Headache Disorders. If he is, he should be able to give you an ICHD-II diagnosis. If not, you may want to seek a second opinion from a doctor who is familiar with the ICHD-II.

Migraine without Aura - The Basics

Migraine is a common disabling primary headache disorder. Epidemiological studies have documented its high prevalence and high socioeconomic and personal impacts. It is now ranked by the World Health Organization as number 19 among all diseases world-wide causing disability.

Migraine is a genetic neurological disease. Because there are several different types of Migraine, and some forms involve different genetic markers, some researchers theorize that it may actually be more than one disease. For now, however, Migraine is divided into two major subtypes, Migraine without aura (MWOA) and Migraine with aura (MWA). There is a single classification under Migraine without aura. MWOA is the most common form of Migraine.

For consistency in diagnosing and classifying head pain disorders, the International Headache Society’s International Classification of Headache Disorders, Second Edition (ICHD-II), is generally accepted as the “gold standard.” The ICHD-II classification criteria for Migraine without aura is:

1.1 Migraine without aura
Previously used terms: Common migraine, hemicrania simplex
Description:
Recurrent headache disorder manifesting in attacks lasting 4–72 hours. Typical characteristics of the headache are unilateral (one-sided) location, pulsating quality (throbbing or varying with the heartbeat), moderate or severe intensity, aggravation by routine physical activity and association with nausea and/or photophobia (increased sensitivity to light) and phonophobia (increased sensitivity to sound).
Diagnostic criteria:

1. At least 5 attacks fulfilling criteria B–D
2. Headache attacks lasting 4–72 hours (untreated or unsuccessfully treated)
3. Headache has at least two of the following characteristics:
   1. unilateral location
   2. pulsating quality
   3. moderate or severe pain intensity
   4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs)
4. During headache at least one of the following:
   1. nausea and/or vomiting
   2. photophobia and phonophobia
   3. Not attributed to another disorder

The Postdrome
Once the headache is over, the Migraine attack may or may not be over. The postdrome (sometimes called postheadache) follows immediately afterward. The majority of Migraineurs take hours to fully recover; some take days. Many people describe postdrome as feeling “like a zombie” or “hung-over.” These feelings are often attributed to medications taken to treat the Migraine, but may well be caused by the Migraine itself. Postdromal symptoms have been shown to be accompanied and possibly caused by abnormal cerebral blood flow for up to 24 hours after the end of the headache stage. In cases where prodrome and/or aura are experienced without the headache phase, the postdrome may still occur. The symptoms of prodrome may include:

• lowered mood levels, especially depression
• or feelings of well-being and euphoria
• fatigue
• poor concentration and comprehension
• lowered intellect levels

A MWOA attack can skip the headache phase. In that case, it’s described as “acephalgic” or “silent” Migraine without aura; the diagnosis is still Migraine without aura.
It’s important to note that you can have more than one type of Migraine. It’s also not unusual to experience both headaches and Migraines. In fact, tension-type headaches can be a Migraine trigger.
If your doctor has diagnosed you with “Migraines,” ask for a more definitive diagnosis. That will make it easier for you to find information and learn about Migraine disease as it applies to you.

Some differences in children:

1. In children, attacks may last 1–72 hours.
2. The headache of a Migraine attack is commonly bilateral (on both sides) in young children; an adult pattern of unilateral pain usually emerges in late adolescence or early adulthood.
3. In young children, photophobia and phonophobia may be inferred from observing their behavior.
4. The headache of a Migraine attack is usually frontotemporal (front and sides, toward the front, of head). Occipital (lower back of the head) headache in children, whether unilateral or bilateral, is rare and calls for caution in diagnosing as many cases are attributable to structural lesions. (See diagram.)

In MWOA, a Migraine attack can consist of up to three phases:

1. Prodrome
2. Headache Phase
3. Postdrome

The Prodrome
The prodrome (sometimes called preheadache or premonitory phase) may be experienced hours or even days before a Migraine attack. The prodrome may be considered to be the Migraineur's “yellow light,” a warning that a Migraine is imminent. For the 30 to 40% of Migraineurs who experience prodrome, it can actually be very helpful because, in some cases, it gives opportunity to abort the attack. For Migraineurs who experience prodrome, it makes a solid case for keeping a Migraine diary and being aware of one's body.
Potential symptoms of the prodrome are:

• food cravings
• constipation or diarrhea
• mood changes — depression, irritability, etc.
• muscle stiffness, especially in the neck
• fatigue
• increased frequency of urination
• yawning
• neck pain

The Headache
The headache phase is generally the most debilitating part of a Migraine attack. It's effects are not limited to the head only, but affect the entire body. The pain of the headache can range from mild to severe. It can be so intense that it is difficult to comprehend by those who have not experienced it. Characteristics of the headache phase may include:

• headache pain that is often unilateral — on one side. This pain can shift to the other side or become bilateral.
• Although Migraine pain can occur at any time of day, statistics have shown the most common time to be 6 a.m. It is not uncommon for Migraineurs to be awakened by the pain.
• Because trigeminal nerve becomes inflamed during a Migraine, Migraine pain can also occur in the areas of the eyes, sinuses, and jaw.
• This phase usually lasts from one to 72 hours. In less common cases where it lasts longer than 72 hours, it is termed status Migrainous, and medical attention should be sought.
• The pain is worsened by any physical activity.
• phonophobia — increased sensitivity to sound
• photophobia — increased sensitivity to light
• osmophobia — increased sensitivity to odors
• neck pain
• nausea and vomiting
• diarrhea or constipation
• nasal congestion and/or runny nose
• depression, severe anxiety
• hot flashes and chills
• dizziness
• vertigo - sensation of spinning or whirling (not to be confused with dizziness or light-headedness)
• confusion
• dehydration or fluid retention, depending on the individual body's reactions

 

Migraine With Aura – The Basics

Migraine is a common disabling primary headache disorder. Epidemiological studies have documented its high prevalence and high socioeconomic and personal impacts. It is now ranked by the World Health Organization as number 19 among all diseases world-wide causing disability.

Migraine is a genetic neurological disease. Because there are several different types of Migraine, and some forms involve different genetic markers, some researchers theorize that it may actually be more than one disease. For now, however, Migraine is divided into two major subtypes, Migraine without aura (MWOA) and Migraine with aura (MWA). There is a single classification under Migraine without aura. MWOA is the most common form of Migraine. MWA is the second most common, occurring in 25-30% of Migraineurs. Few people have the aura phase with every Migraine attack. Thus, it’s quite common to be diagnosed with both MWA and MWOA.

For consistency in diagnosing and classifying head pain disorders, the International Headache Society’s International Classification of Headache Disorders, Second Edition (ICHD-II), is generally accepted as the “gold standard.” Hemiplegic and basilar-type Migraine are subtypes of Migraine with aura. For the purposes of this article, we’ll be discussing 1.2.1, “typical aura with migraine headache.” The ICHD-II classification criteria:

1.2 Migraine with aura
Previously used terms:
Classic or classical migraine, ophthalmic, hemiparaesthetic, hemiplegic or aphasic migraine, migraine accompagnée, complicated migraine
1.2.1 Typical aura with migraine headache
Description:
Typical aura consisting of visual and/or sensory and/or speech symptoms. Gradual development, duration no longer than one hour, a mix of positive and negative features and complete reversibility characterise the aura which is associated with a headache fulfilling criteria for 1.1 Migraine without aura.
Diagnostic criteria:

1. At least 2 attacks fulfilling criteria B–D
   
2. Aura consisting of at least one of the following, but no motor weakness*:
   1. fully reversible visual symptoms including positive features (e.g., flickering lights, spots or lines) and/or negative features (i.e., loss of vision)
   2. fully reversible sensory symptoms including positive features (i.e., pins and needles) and/or negative features (i.e., numbness)
   3. fully reversible dysphasic speech disturbance
3. At least two of the following:
   1. homonymous visual symptoms1 and/or unilateral sensory symptoms
   2. at least one aura symptom develops gradually over ≥5 minutes and/or different aura symptoms occur in succession over ≥5 minutes
   3. each symptom lasts ≥5 and <60 minutes
4. Headache fulfilling criteria B–D for 1.1 Migraine without aura begins during the aura or follows aura within 60 minutes
5. Not attributed to another disorder

* If the aura includes motor weakness, code as 1.2.4 Familial hemiplegic migraine or 1.2.5 Sporadic hemiplegic migraine. (See Hemiplegic Migraine - The Basics.)

For more detailed information about aura symptoms, please see Anatomy of a Migraine.
Some differences in children:

1. In children, attacks may last 1–72 hours.
2. The headache of a Migraine attack is commonly bilateral (on both sides) in young children; an adult pattern of unilateral pain usually emerges in late adolescence or early adulthood.
3. In young children, photophobia and phonophobia may be inferred from observing their behavior.
4. The headache of a Migraine attack is usually frontotemporal (front and sides, toward the front, of head). Occipital (lower back of the head) headache in children, whether unilateral or bilateral, is rare and calls for caution in diagnosing as many cases are attributable to structural lesions. (See diagram.)

In MWA, a Migraine attack can consist of up to three phases:

1. Prodrome
2. Aura
3. Headache Phase
4. Postdrome

The Prodrome
The prodrome (sometimes called preheadache or premonitory phase) may be experienced hours or even days before a Migraine attack. The prodrome may be considered to be the Migraineur's “yellow light,” a warning that a Migraine is imminent. For the 30 to 40% of Migraineurs who experience prodrome, it can actually be very helpful because, in some cases, it gives opportunity to abort the attack. For Migraineurs who experience prodrome, it makes a solid case for keeping a Migraine diary and being aware of one's body.
Potential symptoms of the prodrome are:

• food cravings
• constipation or diarrhea
• mood changes — depression, irritability, etc.
• muscle stiffness, especially in the neck
• fatigue
• increased frequency of urination
• yawning

The Aura
The aura is perhaps the most talked about of the possible phases. The symptoms and effects of the aura vary widely. Some can be quite terrifying, especially when experienced for the first time. Some of the visual distortions can be exotic and bizarre. It's interesting to note that Migraine aura symptoms are thought to have influenced some famous pieces of art and literary works. One of the better know is Lewis Carroll's "Alice in Wonderland."

While most people probably think of aura as being strictly visual, auras can have a wide range of symptoms, including:

• visual: flashing lights, wavy lines, spots, partial loss of sight, blurry vision
• olfactory hallucinations — smelling odors that aren't there
• paresthesia - tingling or numbness of the face or extremities on the side where the headache develops.
• aphasia - difficult finding words and/or speaking
• confusion
• dizziness
• neck pain
• partial paralysis (only in hemiplegic Migraine)
• auditory hallucinations — hearing things that aren't really there
• decrease in or loss of hearing
• reduced sensation
• allodynia - hypersensitivity to feel and touch
• brief flashes of light that streak across the visual field (phosphenes)

Approximately 25% of Migraineurs experience aura. As with the prodrome, Migraine aura, when the Migraineur is aware of it, can serve as a warning, and sometimes allows the use of medications to abort the attack before the headache phase begins. As noted earlier, not all Migraine attacks include all phases. Although not the majority of attacks, there are some Migraine attacks in which Migraineurs experience aura but no headache. There are several terms used for this experience, including "silent Migraine," "acephalgic Migraine."
The Headache
The headache phase is generally the most debilitating part of a Migraine attack. It's effects are not limited to the head only, but affect the entire body. The pain of the headache can range from mild to severe. It can be so intense that it is difficult to comprehend by those who have not experienced it. Characteristics of the headache phase may include:

• headache pain that is often unilateral — on one side. This pain can shift to the other side or become bilateral.
• Although Migraine pain can occur at any time of day, statistics have shown the most common time to be 6 a.m. It is not uncommon for Migraineurs to be awakened by the pain.
• Because trigeminal nerve becomes inflamed during a Migraine, Migraine pain can also occur in the areas of the eyes, sinuses, and jaw.
• This phase usually lasts from one to 72 hours. In less common cases where it lasts longer than 72 hours, it is termed status Migrainous, and medical attention should be sought.
• The pain is worsened by any physical activity.
• phonophobia — increased sensitivity to sound
• photophobia — increased sensitivity to light
• osmophobia — increased sensitivity to odors
• neck pain
• nausea and vomiting
• diarrhea or constipation
• nasal congestion and/or runny nose
• depression, severe anxiety
• hot flashes and chills
• dizziness
• vertigo - sensation of spinning or whirling (not to be confused with dizziness or light-headedness)
• confusion
• dehydration or fluid retention, depending on the individual body's reactions

The Postdrome
Once the headache is over, the Migraine attack may or may not be over. The postdrome (sometimes called postheadache) follows immediately afterward. The majority of Migraineurs take hours to fully recover; some take days. Many people describe postdrome as feeling “like a zombie” or “hung-over.” These feelings are often attributed to medications taken to treat the Migraine, but may well be caused by the Migraine itself. Postdromal symptoms have been shown to be accompanied and possibly caused by abnormal cerebral blood flow for up to 24 hours after the end of the headache stage. In cases where prodrome and/or aura are experienced without the headache phase, the postdrome may still occur. The symptoms of prodrome may include:

• lowered mood levels, especially depression
• or feelings of well-being and euphoria
• fatigue
• poor concentration and comprehension
• lowered intellect levels

See Anatomy of a Migraine for a complete description of these phases and their symptoms.
A MWA attack can skip the headache phase. In that case, it’s described as “acephalgic” or “silent” Migraine with aura; the diagnosis is still Migraine with aura.
It’s important to note that you can have more than one type of Migraine. It’s also not unusual to experience both headaches and Migraines. In fact, tension-type headaches can be a Migraine trigger.
If your doctor has diagnosed you with “Migraines,” ask for a more definitive diagnosis. That will make it easier for you to find information and learn about Migraine disease as it applies to you.

 

Resources:
¹ The International Headache Society. "International Classification of Headache Disorders, 2nd Edition." Cephalalgia, Volume 24 Issue s1. May, 2004. doi:10.1111/j.1468-2982.2003.00823.x.
² Young, William B., MD; Silberstein, Stephen D., MD. "Migraine and Other Headaches." AAN Press. St. Paul. 2004.
³ Calhoun, Anne H., MD; Ford, Sutapa, PhD; Millen, Cori, DO; Finkel, Alan G., MD; Truong, Young, PhD; Nie, Yonghong, MS. "The Prevalence of Neck Pain in Migraine." Headache. Published Online: Jan. 20, 2010.
  

What Is a Complex or Complicated Migraine?

People often ask what "complex Migraines" or "complicated Migraines are." That's a difficult question to answer because it varies depending on who's using those terms.

In most areas of medicine, terminology and diagnostic classifications are standardized so that diagnoses are uniform and when one is mentioned, logical discussion or search for information can follow. That applies in the field of what's generally called "headache medicine," the diagnosis and treatment of Migraine and other headache disorders, as well. The International Headache Society's International Classification of Headache Disorders, 2nd Edition, (ICHD-II) is the gold standard for diagnosing and classifying headache disorders.

Under ICHD-II, there is no classification of "complex" on "complicated" Migraine. Sometimes, "complex" or "complicated" are used as descriptive terms, as opposed to diagnoses, to describe a Migraine attack that is more complex or complicated than they consider to be "normal" or "average." If you hear or see someone using those terms meaning them to be diagnoses of a particular form of Migraine, it's quite difficult to know what they really mean. Since there's no defined criteria or reference for either of them as forms of Migraine, what one person means may be very different from what another person means.
It's helpful to know the types of Migraine that are recognized in the ICHD-II. They are:

1.1 Migraine without aura

1.2 Migraine with aura

1.2.1 Typical aura with migraine headache
1.2.2 Typical aura with non-migraine headache
1.2.3 Typical aura without headache
1.2.4 Familial hemiplegic migraine (FHM)
1.2.5 Sporadic hemiplegic migraine
1.2.6 Basilar-type migraine       

1.3 Childhood periodic syndromes that are commonly precursors of migraine

1.3.1 Cyclical vomiting
1.3.2 Abdominal migraine
1.3.3 Benign paroxysmal vertigo of childhood

1.4 Retinal migraine

1.5 Complications of migraine

1.5.1 Chronic migraine
1.5.2 Status migrainosus
1.5.3 Persistent aura without infarction
1.5.4 Migrainous infarction
1.5.5 Migraine-triggered seizures

1.6 Probable migraine

1.6.1 Probable migraine without aura
1.6.2 Probable migraine with aura
1.6.5 Probable chronic migraine

If you've been diagnosed with "complex Migraine, "complicated Migraine," or any form of Migraine not listed above, please ask your doctor for an accurate and full diagnosis. It may not seem that it should matter, but it does. Here's just one reason why: Triptans (Imitrex, Maxalt, Zomig, and the others) and ergotamines (D.H.E. 45, Cafergot, and Migranal Nasal Spray) are usually not prescribed for people with basilar-type or hemiplegic Migraines because there's concern about their vasoconstrictive (constricting blood vessels) properties.

If your doctor can't or won't give you an accurate and full diagnosis or give you a good reason why not, you need to find a new doctor.

 

Basilar-Type Migraine - The Basics

Key Points:

1. A Migraine-type defined by the presence of Migraine headache with neurological symptoms which begin either in the base of the brain or from both sides of the brain at the same time; i.e. brainstem or both cerebral hemispheres. 
2. Many compare this Migraine type more to hemiplegic Migraine (Migraine type with weakness) than to Migraine with typical aura (99% visual symptoms). Those with less understanding of this Migraine consider it as “atypical or complicated” aura.
3. All patients describe visual symptoms, nearly 2 of 3 sensory and least often language or aphasic aura. Vertigo is the most frequent symptom type.
4. In actuality, nearly all basilar-type Migraineurs suffer typical Migraine with aura as well; just more frequent sensory and speaking difficulties of longer duration and intensity.
5. Obtain a correct diagnosis, optimal treatment plan, and prevent as many Migraines as possible to reduce disability and improve prognosis.

Introduction
If you've heard of this type of Migraine before, you've probably heard or seen the term basilar artery Migraine (BAM). Under the International Headache Society's International Classification of Headache Disorders-2004, the new designation for this type of Migraine is basilar-type Migraine (BTM). It has also been called Bickerstaff syndrome, brainstem Migraine, and vertebrobasilar Migraine. The term BAM is actually misleading as it implies that the basilar artery is the origin of the attack. It was termed basilar by Bickerstaff in 1961-62. He reported his beliefs that the events of BTM were the result of short term narrowing or spasm of the basilar artery. Reduced blood flow or “ischemia” followed and was believed to increase risk for serious events. This belief remained a concern for over 3 decades. It even resulted in the exclusion of BAM patients from clinical trials of triptans for Migraine. The absence of BAM patients in trials led the Federal Drug Administration (FDA) to contraindicate use of triptans in patients with BAM.

Basilar-Type Migraine - The Basics

(Page 2)
Migraine in now known as a common but complex genetic disorder involving environmental factors. The nerves are the cause in BTM as they are in other Migraine types. As with all Migraine, there is a blood vessel component once Migraine begins, but Migraine begins in nerves. Bickerstaff suggested that BTM was most common in adolescent females. Now BTM is known to affect all age groups. BTM does exhibit the same female predominance seen overall in Migraine; three times as many female sufferers as male.

Symptoms of BTM
Basilar-type Migraine is a Migraine-type that has aura symptoms originating from the base of the brain or both sides of the brain at the same time, but with no motor weakness. These areas are named brainstem and cerebral hemispheres of the brain. In a study of familial Migraine, 95% of those meeting criteria for BTM also met criteria for Migraine with aura. In this BTM group 50% of all auras met BTM criteria. The most frequent symptom in BTM is vertigo. Among all BTM subjects 31% reported two, 45% three, 8% four, 8% five, and 8% six aura symptoms. All patients (100%) described visual aura; 40% with symptoms in both fields of vision and 60% with only one side of vision affected. Temporary blindness can be reported, which is one reason BTM can be quite scary. Symptoms involving sensation or sensory aura occur in 61% with the same involvement on both (40%) or one side (60%) as visual aura. Symptoms involving ability to form words or sentences, called aphasic aura, were present in 40%. Visual aura was the most common initial symptom in 2 of 3. Headache accompanied or followed aura in 98% and met criteria for Migraine or probable Migraine in 98%. The median length of aura of BTM in this study was 60 minutes, but with as short as 2 minutes and as long as 72 hours. The authors of this data concluded that there is insufficient evidence to distinguish basilar-type Migraine as a disease independent of Migraine with typical aura. 

• Aura consisting of at least two of the following fully reversible symptoms, but no motor weakness: 
  • impairments or clumsiness in the speaking of words due to diseases that affect the mouth, tongue, or throat muscles (dysarthria)
  • feeling of spinning (vertigo)
  • noise in the ears (tinnitus)
  • impaired hearing (hypacusia)
  • double vision (diplopia)
  • typically spots or flashes simultaneously in both temporal and nasal fields of both eyes
  • in-coordination of limbs or walking (ataxia)
  • decreased level of consciousness (state of being alert)
  • paresthesias (abnormal or unpleasant sensation often described as numbness or as a prickly, stinging, or burning feeling) at the same time on both sides of the face, arms or legs
• At least one of the following:
  • at least one aura symptom develops gradually over five or more minutes and/or different aura symptoms occur in succession over five or more minutes
  • each aura symptom lasts five or more and 60 minutes or less (note this length does not hold up to patient histories)
• headache meeting criteria of Migraine without aura begins during the aura or follows aura within 60 minutes  

1. Always 2 or more basilar-type aura symptoms

2. Always visual aura characterized by positive or negative features

3. Sometimes with sensory aura characterized by positive or negative features

4. Sometimes with either aphasia or dysarthria

5. Aura symptoms almost always develop gradually, or occur in succession, over > 5 minutes

6. Aura duration 5-120 minutes

7. Headache with Migraine features begins after onset of aura

8. Age of onset < 50 years

9. Almost always co-existing attacks of Migraine with typical aura

  
Correct Diagnosis
Migraine experts caution that when there is motor weakness, great care be taken to arrive at the proper diagnosis. At times it can be difficult to differentiate between Migraine types. Basilar-type Migraine, hemiplegic Migraine (HM) and non-familial Migraine with unilateral (one-sided) motor symptoms (MUMS) with give-way weakness are several examples. The classification committee for the ICHD-2004 comments on similarities of HM and BTM indicating that basilar symptoms are common in HM. If motor weakness is present the current ICHD-2004 criteria require diagnosis as familial hemiplegic or sporadic (non-familial) hemiplegic Migraine. Comments are made that motor weakness can be difficult to tell apart from sensory problems. Another reason an expert Migraine and headache provider is needed in diagnosis is that many of the symptoms of BTM mimic other far more serious medical conditions. It is essential that the diagnosis be definitive and correct. This may require imaging as discussed next. If the provider making the diagnosis is hesitant about it, definitely seek a second opinion from another provider. Since features and associations of BTM are not well known to many providers, seeing a Migraine and headache specialist is advisable when possible. You may achieve best advice if this provider is certified by the United Council of Neurological Subspecialties (UCNS). See achenet.org Find a Healthcare Professional. It is also important to continue medical treatment as advised by your doctor and not skip follow-up appointments. Upon correct diagnosis BTM sufferers should consider having medical identification of some kind on their person as many providers will not recognize BTM. This will alert them to refresh what they know.

Tests
Get a head CT scan if recent bleeding in the brain is a concern. Otherwise evaluation should be a brain MRI with and without intravenous (IV) contrast. Avoid contrast with proven allergy or kidney function less than 30 GFR (unlikely due to age, gender of most patients). In diagnosing BTM imaging helps rule out:

• space-occupying lesions of the brain
• brainstem arteriovenous malformation (AVM): a congenital defect consisting of a tangle of abnormal arteries and veins with no capillaries in between. The blood pressure in the veins is higher than normal and may result in a rupture of the vein and bleeding into the brain.
• vertebrobasilar disease
• stroke

An EEG is often performed to rule out seizure disorders which are especially a consideration with new events, confusion or change in the alert state and younger patients.

Treatment
Migraine-specific medications such as the triptans and ergotamines are contraindicated for BTM. This is because they were not studied in scientific trials of Migraine. This is due to a belief at the time that artery narrowing or spasm was the cause of these symptoms. The triptans and ergotamines are known to constrict blood vessels and were believed to likely cause safety issues if used. Such beliefs however did not lead to the exclusion of Migraine with aura patients. As described above, BTM is essentially a Migraine with aura subtype. Three Migraine and headache specialists in 2001 reported on 13 patients with basilar-type Migraine, familial hemiplegic Migraine, or Migraine with prominent or prolonged aura who had received triptans. No harm was done (no adverse events) with excellent relief of headache and symptoms. They concluded that the contraindication of triptans in basilar-type Migraine should be reconsidered. They also wrote that prominent or prolonged aura may not represent a reasonable contraindication to triptan therapy. In a larger group of patients meeting criteria for BTM, no increased incidence of adverse events was reported following inadvertent or intentional triptan exposure. To avoid the historical restriction on artery narrowing drugs a combination of nonsteroidal anti-inflammatory with and an antiemetic phenothiazine is often used and can be effective. It is also reasonable to discuss your treatment needs with your provider and obtain an expert opinion if offered only a non-specific treatment. An effective treatment plan will require acute use typically early in less than 15-60 minutes and when possible at mild pain.



Of the preventive medications, there is some evidence in children for topiramate success. Many use a calcium channel blocker with benefit although this is based on experience only. Otherwise, BTM is generally managed with traditional preventatives although many recommend that beta blockers be avoided due to rare reports of complicating events.
Disability and Prognosis
As with other forms of Migraine, BTM can be disabling. Because of the neurological symptom types, with vertigo the most frequent, BTM is often more debilitating than Migraine with aura due to aura intensity, number of symptoms and longer length. Perhaps fortunately, the majority of BTM patients are older children, adolescents or young adults. Basilar-type Migraine can mean special problems for people in the traditional work force or trying to care for young children. For many, however, aura in BTM commonly becomes more typical during later mid-life. While disabling, symptoms of BTM are usually more frightening than harmful. A concern or myth about stroke risk has existed for decades. There is no evidence that BTM patients have any greater stroke (cerebrovascular) risk than Migraine with typical aura. Migraine with aura does have a slightly higher stroke risk than Migraine without aura in those younger than 45, so optimal prevention and knowledge of stroke risk factors and their control is important. As a BTM patient, if others are not educated about BTM, it is particularly important that efforts be made to inform them.

Summary
Basilar-type Migraine is one type of Migraine with aura; it is one of the most frightening of head pain disorders. As with other forms of Migraine, it is necessary to have an accurate diagnosis and effective treatment plan. This requires use of that treatment as early as possible when pain is mild without waiting to learn how extreme the pain will become. Severe BTM will often require seeking emergency care. Unfortunately, ED/ERs will often fail to consider BTM as your diagnosis. It may be reasonable that BTM sufferers have medical identification of some kind on their person; consider a Medical ID as important. If providers do consider it, they typically will not know how to treat it. Access to a Migraine and headache specialist is also important. Once diagnosed with BTM, it is important (as with any form of Migraine) to minimize the frequency of attacks through optimal prevention. Also contact your provider if your symptoms or Migraine pattern change. Without consulting a knowledgeable provider, it's impossible to be sure that new symptoms or changes in pattern are attributable to BTM, and that no other condition is present. While BTM isn't cause to panic, be sensible and take good care of yourself.
 
Resources:
The International Headache Society. "International Classification of Headache Disorders, 2nd Edition." Cephalalgia, Volume 24 Issue s1. May, 2004. doi:10.1111/j.1468-2982.2003.00823.x
Kaniecki RG. Basilar-type Migraine. Curr Pain Headache Rep. 2009;13:217-220.
Kirchmann M, Thomsen L, Olesen J. Basilar-type Migraine: Clinical, epidemiologic, and genetic features. Neurology 2006;66: 880-886.
Klapper J, Mathew N, Nett R. Triptans in the treatment of basilar Migraine and Migraine with prolonged aura. Headache. 2001;41:981-984.
Tepper, Stewart J., M.D. Understanding Migraine and Other Headaches. University of Mississippi Press, 2004.
Young, William B. and Silberstein, Stephen D. Migraine and Other Headaches. St. Paul, Minnesota: AAN Press, 2004.
Edited by Frederick R. Taylor, MD August 14, 2010
Medical review by John Claude Krusz, PhD, MD
 

 

Abdominal Migraine

Abdominal Migraine is a form of Migraine seen mainly in children. It's most common in children ages five- to nine-years-old, but can occur in adults as well. Abdominal Migraine consists primarily of abdominal pain, nausea, and vomiting. It was recognized as a form of Migraine disease as links were made to other family members having Migraines and children who had this disorder grew into adults with Migraine with and without aura. Most children who experience abdominal Migraine eventually develop Migraine with aura and/or Migraine without aura. The diagnostic criteria for abdominal Migraine, as established by the International Headache Society, are:

A. At least 5 attacks fulfilling criteria B–D

B. Attacks of abdominal pain lasting 1-72 hours (untreated or unsuccessfully treated

C. Abdominal pain has all of the following characteristics:

• midline location, periumbilical or poorly localised
• dull or ‘just sore’ quality
• moderate or severe intensity

During abdominal pain at least 2 of the following:

• anorexia
• nausea
• vomiting
• pallor

Not attributed to another disorder (1)

Note:
(1) In particular, history and physical examination do not show signs of gastrointestinal or renal disease or such disease has been ruled out by appropriate investigations.
Comments:
Pain is severe enough to interfere with normal daily activities. Children may find it difficult to distinguish anorexia from nausea. The pallor is often accompanied by dark shadows under the eyes. In a few patients flushing is the predominant vasomotor phenomenon. Most children with abdominal migraine will develop migraine headache later in life.

Diagnosis:
As with any form of Migraine, there is no diagnostic test to confirm abdominal Migraine. Diagnosis is achieved by reviewing both family and patient medical history, evaluating the symptoms, and performing an examination to rule out other causes of the symptoms. Other conditions that should be ruled out to arrive at a diagnosis of abdominal Migraine include: urogenital disorders, peptic ulcer, cholecystitis (gall bladder), duodenal obstruction, gastroesophageal reflux, Crohn's disease, and irritable bowel syndrome. If there is any alteration in consciousness, seizure disorders should also be ruled out.

Treatment:
For infrequent abdominal Migraine attacks, medications used for other forms of Migraine are often employed. These medications can include NSAIDs, antinausea medications, Midrin, and the triptans. The choice of medications is somewhat affected by the age of the patient. When abdominal Migraines are frequent, the same preventive therapies used for other Migraines can be explored.