Uncommon Migraine

Complicated, variant or less common forms of migraine

A number of complicated and variant forms of migraine are known. While there is some debate over whether certain entities are actually “migraine” – they are generally classified as such because they often have the same triggers as migraine and, in addition to their unusual features, share many features in common with more typical migraine. Unusual forms of migraine may be especially frightening because they mimic life-threatening emergency situations. While generally ‘benign’ (self-limiting and not caused by another disease or condition,) each requires a thorough medical examination. Emergency medical care should be immediately obtained if there is any doubt concerning the origin of symptoms.

Basilar migraine – also called basilar artery or Bickerstaff migraine

Basilar migraine, also known as basilar artery migraine, is rare but potentially dangerous. Basilar migraine always includes aura and is a subtype of migraine with aura. 86% of those with basilar migraine have a family history of basilar migraine. It was previously thought to occur exclusively in teen-aged girls and young women. It has since been recognized in both sexes and all ages but is most common in adolescent girls. Basilar migraine attacks can alternate with typical migraine attacks.

Basilar artery migraine is believed to be associated with, or increase the risk of, strokes or transient ischemic attack (TIA.) Symptoms frequently observed include partial vision loss, double vision, hearing changes, dizziness, vertigo, severe vomiting, slurred speech, loss of coordination, numbness (on one or both sides of the body), weakness, trouble walking (ataxia) and general confusion. These symptoms are usually brief and generally subside with the onset of the actual headache, but may remain for up to several days after headache pain resolves.

Headache pain is located in the back of the head (the occipital area) and at least 2 of the following symptoms must be observed during the aura stage:

  • Ataxia (a severe loss of coordination – especially trouble walking)
  • Bilateral paresthesias (strange feelings or sensations on both sides of the body)
  • Deafness
  • Decreased consciousness
  • Double vision
  • Dizziness
  • Vertigo (room is ‘spinning’)
  • Sudden falling with complete recovery in seconds to minutes (“drop attacks”)
  • Difficulty speaking
  • Ringing in the ears (tinnitus)
  • Vision loss in one or both eyes
  • Fluctuating low-tone hearing loss
  • General weakness

Ocular migraine – also called ophthalmic or retinal migraine

Ophthalmic (retinal) migraine involves repeated episodes of a blind spot or blindness in one eye, usually lasting less than one hour and usually followed by headache. The headache may or may not have a ‘migraine’ quality. It may be simply a dull ache behind the eye that lost vision or it may cause pain in the entire head. A diagnosis of ocular migraine requires a normal eye exam between attacks. In other words, the problem cannot be (is not) in the eye itself.

Hemiplegic migraine – also called familial hemiplegic migraine

Familial hemiplegic migraine or hemiplegic migraine is a subtype of migraine with aura. Hemiplegic migraine is without a family history of same is rare, and probably results from a spontaneous genetic defect. Generally it is an autosomal dominant form of migraine that begins during childhood and which is characterized by extended paralysis of all or part of one side of the body (hemiplegia) that is accompanied by numbness, language difficulty (aphasia: difficulty producing or comprehending written or spoken words) and confusion.

The hemiplegia may start before, during or after the headache phase, and generally lasts from 24 hours to as long as a week. When originating before the headache, the hemiplegia is followed by headache within an hour, usually on the side opposite to the hemiplegia. Hemiplegic migraine is an example of “complicated migraine.” (The migraine is associated with neurologic signs or symptoms that persist beyond the head pain.) Like all complicated migraine, it has dramatic focal features and a persistent neurologic deficit that remains for at least 24 hours after the headache.

Ophthalmoplegic migraine

Ophthalmoplegic (OP) migraine is another rare form of “complicated migraine”. As suggested by the name, OP migraine includes features of both ocular and hemiplegic migraine. OP migraine is characterized by a severe unilateral headache that is associated with pain around the eye and paralysis in the muscles that surround the eye. Recurrent attacks may cause permanent damage. Other symptoms may include droopy eyelid, double vision and other visual problems.

Ophthalmoplegic migraine is believed to result from inflammation of the oculomotor nerve, and might therefore be categorized as a type of neuritis rather than as migraine. The course of individual attacks also suggests neuritis as opposed to migraine – symptoms can last for months.

OP migraine is usually diagnosed in childhood. In addition to those symptoms related directly to the oculomotor nerve, OP migraine is associated with severe one-sided (unilateral) headache. ves. Ophthalmoplegia may precede, accompany, or follow the headache; recurrent episodes may cause permanent oculomotor deficit.

Acephalic migraine – also called silent migraine or migraine equivalent

Acephalic migraine is aura without headache. The most common form of aura in silent migraine is visual. Silent migraine most often affects women. A positive family history of migraine is essential in confirming the diagnosis. Acephalic migraine may be a variation of ophthalmic migraine.

Status migrainosus

In contrast to migraine without headache, status migrainosus is a migraine headache lasting longer than seventy-two hours. While not really a separate entity, status migrainosis merits special attention. Sufferers may become dehydrated, especially when the headache is accompanied by vomiting. Hospitalization may be required, either for pain control or medical treatment such as IV rehydration.

Forms of migraine seen most often or exclusively in children

Migraine disease in childhood may differ substantially from that seen in adults. The time course is different, often shorter, and unique symptoms may be present. Headache is less often a central feature and, when present, is more often on both sides of the head. There is debate over whether certain conditions represent ‘true’ migraine, and some conditions are referred to as “migraine equivalents”. Migraine equivalents often go unrecognized or are misdiagnosed. They are often forerunners of the typical migraine. Migraine equivalents and complicated migraine in children may alternate with more typical migraine types.

Abdominal migraine

Abdominal migraine is characterized by recurrent bouts of generalized abdominal pain, often associated with general nausea and vomiting, but without headache. Abdominal migraine may alternate with typical migraine. it gradually lessens in most children with advancing age and is most often ‘replaced’ by the occurrence of typical migraine. Abdominal migraine is the most common form of atypical migraine in children.

Confusional migraine

Confusional migraine is uncommon. It most often occurs in the second decade of life. Attacks are sometimes precipitated by minor head trauma and are characterized by the rapid development of confusion and agitation. When affected by confusional migraine, children express a variety of symptoms and may become delirious, restless and/or combative. They may appear to be in pain but do not complain of headache. Attacks generally last less than 6 hours and are followed by deep sleep. Upon awakening, the child is normal and, most remarkably, has no memory of the time during the attack. The confusional migraine attacks tend to recur over months to years, but are eventually replaced by typical migraine in most children. This form of migraine is often very difficult to diagnose and is often misdiagnosed as a psychiatric or behavioral problem.

Migraine-associated cyclic vomiting syndrome – also called periodic syndrome

There is substantial debate over the relation of this condition to migraine. The syndrome is characterized by recurrent periods of intense vomiting separated by symptom-free intervals. Migraine-associated cyclic vomiting most often manifests with a rapid onset, often in early morning hours. Symptoms usually last 6-48 hours. Associated symptoms may include abdominal pain, nausea and lethargy. Symptoms more commonly associated with typical migraine may also occur, such as extreme sensitivity to light (photophobia) and sound (phonophobia.) Headache occurs in roughly 40% of attacks, but is more often associated with bouts of cyclic vomiting as the child becomes older.

This syndrome most often begins when the child is a toddler and continues through adolescence or early adulthood. It may rarely begin during the adult years. Girls are more often affected than boys. There is usually a family history of migraine. Some children with cyclic vomiting syndrome respond to antimigraine drugs.

Benign paroxysmal vertigo of childhood

Paroxysmal means that there is sudden onset of symptoms. Benign means that the condition does not result from and is not caused by some other condition, especially that it is not an indication of a more serious condition or disease.

Benign paroxysmal vertigo (BPV) is characterized by short periods of vertigo (room is ‘spinning’.) Balance is affected and nausea results. BPV is most often diagnosed in children between the ages of 2 and 6. BPV is not associated with hearing loss, tinnitus (ringing in the ears) or loss of consciousness. Symptoms usually last only a few minutes. Children with BPV often develop typical migraine as they grow older.

Paroxysmal torticollis of infancy

Torticollis defines a condition in which the neck is twisted, the head is tipped to one side, while the chin is turned to the other and generally pressed against the shoulder area.

Paroxysmal torticollis of infancy is a rare disorder characterized by repeated episodes of such head tilting that is associated with nausea and vomiting, as well as signs indicating headache. Attacks occur only during infancy and last from hours to days. it is reported that infants with this condition are more likely to develop typical migraine as they grow older.


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