Pediatric Annals

Complicated Migraine Syndromes and Migraine Variants

Colette Parker, MD

Abstract

Migraine headaches and their variants are the most common episodic disorders of childhood. It is estimated that 5% to 10% of children suffer migraines. Migraine becomes increasingly more common as the child ages, but this increase may be due to underdiagnosis in younger children. There is a female predominance, especially in adolescents, and a strong familial pattern. The typical feature of migraine without aura ("common migraine") is episodic, severe head pain with nausea and vomiting that is relieved by sleep. Over 90% of affected children have a positive family history for similar headaches. Migraine with aura also includes unilateral throbbing pain and an aura consisting of a transient neurologic disturbance, particularly a scintillating scotoma ("classic migraine").

Migraine is a complex event in which there is initially a focal reduction in cerebral blood flow with subsequent spreading oligemia and cortical depression that does not respect vascular territories.1 It has been suggested that migraine is a central dysautonomia with related vascular changes.2 In classic migraine, this causes the aura and head pain. Rarely, the neurologic disturbances can be unusual and mimic stroke, seizures, movement disorders, and other diseases. These form the basis for the syndromes of complicated migraine and migraine variants. This review discusses these rare, sometimes frightening manifestations of migraine in childhood. Table 1 summarizes the classification of migraine.3

Table

The diagnosis of acute confusional migraine is difficult, especially if it is the first migraine event. When there is no history of previous migraine attacks, a family history for migraine may be helpful. One must exclude toxic ingestion, encephalitis, and metabolic encephalopathies such as Reye's syndrome to make this diagnosis.

MIGRAINE VARIANTS

The term migraine variant describes the following paroxysmal events. When these occur in infants and young children, the child may be unable to describe symptoms, but they usually go on to develop more typical migraine attacks or have a strong family history of migraine. Headache may be present.

Benign Paroxysmal Vertigo

A disorder of preschool children and infants, benign paroxysmal vertigo causes sudden and severe vertigo with nystagmus.11 Unlike other migraine syndromes, nausea and vomiting are usually absent. The child will lay motionless during the episode or wedge him or herself between objects to maintain balance. The child is often quite fretful during the event and appears pale. The episode only lasts for a few minutes, and these patient recovers completely. There is no change in consciousness and no complaints of head pain. These stereotypic events are recurrent at irregular intervals. As the child ages, more typical migraine attacks emerge.

Vertigo and dizziness are common complaints in older children and adults during a migraine attack. Vertigo can also be caused by middle and inner ear disease, drugs, trauma, epilepsy, tumor, and Meniere's disease. Benign paroxysmal vertigo can usually be distinguished on clinical grounds because of rhe recurrent, brief attacks in an otherwise healthy child. As with other migraine syndromes, a family history can almost always be obtained.

Cyclic Vomiting

Also called periodic syndrome or abdominal migraine, cyclic vomiting is characterized by recurrent episodes of severe emesis and pallor lasting up to several hours.12 If the episodes are prolonged, patients may suffer from dehydration. If the child is verbal, concurrent complaints of abdominal pain are common. After the episode, these patients often go to sleep and awaken symptom-free. As these children grow older, they usually develop more typical features of migraine headache. The family history is positive for migraine in most cases. As expected in all migraine syndromes, the physical and neurologic examination results are normal between attacks.

Cyclic vomiting from migraine is especially challenging to recognize in the preverbal…

Migraine headaches and their variants are the most common episodic disorders of childhood. It is estimated that 5% to 10% of children suffer migraines. Migraine becomes increasingly more common as the child ages, but this increase may be due to underdiagnosis in younger children. There is a female predominance, especially in adolescents, and a strong familial pattern. The typical feature of migraine without aura ("common migraine") is episodic, severe head pain with nausea and vomiting that is relieved by sleep. Over 90% of affected children have a positive family history for similar headaches. Migraine with aura also includes unilateral throbbing pain and an aura consisting of a transient neurologic disturbance, particularly a scintillating scotoma ("classic migraine").

Migraine is a complex event in which there is initially a focal reduction in cerebral blood flow with subsequent spreading oligemia and cortical depression that does not respect vascular territories.1 It has been suggested that migraine is a central dysautonomia with related vascular changes.2 In classic migraine, this causes the aura and head pain. Rarely, the neurologic disturbances can be unusual and mimic stroke, seizures, movement disorders, and other diseases. These form the basis for the syndromes of complicated migraine and migraine variants. This review discusses these rare, sometimes frightening manifestations of migraine in childhood. Table 1 summarizes the classification of migraine.3

Table

TABLE 1Classification of Migraine

TABLE 1

Classification of Migraine

COMPLICATED MIGRAINE

For discussion purposes, complicated migraine headaches are unusual neurologic symptoms or signs during the course of a migraine headache. The signs and symptoms often mimic the transient ischemic attacks of persons with cerebrovascular disease, and indeed patients with complicated migraine are at higher risk for having strokes. Headache almost always accompanies the event but the patient may be aphasie or disoriented and unable to relate head pain. The aura of migraine and the neurologic signs or symptoms can rarely be the only manifestation of the migraine event (migraine sine hemicrania). There may be further diagnostic confusion because seizures also cause transient neurologic disturbances. The difference between a seizure and a migraine event is usually self-evident, but the distinction can be blurred if the child with seizures is found in the post-ictal phase without the ictal event being witnessed. This patient's confusion, aphasia, and hemiparesis (Todd's paralysis) can resemble complicated migraine. However, several features distinguish seizures and complicated migraine. Most often, complicated migraine occurs in a patient who has a history of typical migraine attacks. Also, the evolution of symptoms and signs is slower in migraine, and the patient more often has memory of the event. Unlike with migraine, nausea and vomiting are uncommon with seizures, and the vomiting is more likely to occur during the post-ictus.

Ophthalmoplegic Migraine

Transient paresis of eye movement characterizes ophthalmoplegic migraine.4 This feature is rare at any age, but the onset is usually between 5 and 1 2 years of age. Ophthalmoplegic migraine occurs during an otherwise typical migraine attack. The pain is always ipsilateral to the eye involved. An aura may precede the attack, and nausea and vomiting are common. The child presents with head or unilateral orbital pain associated with ptosis, strabismus, diplopia, and pupillary dilatation. The neurologic symptoms most often occur during the actual time of head pain. The findings are usually that of a third nerve palsy, but all three cranial nerves of extraocular movement can be involved.

The differential diagnosis is quite large, especially in adults. A congenital berry aneurysm must be excluded in patients older than 20 years of age. A number of inflammatory diseases of the orbit can cause extraocular movement disorders as can meningitis (as from a cryptococcal infection, for example). Diabetes and other causes for vasculitis are common ideologies of third nerve palsies as well. Rarely, isolated III, IV, or VI cranial nerve palsies will follow a course similar to Bell's palsy. Intracranial tumors must also be excluded. Also, myasthenia can usually be eliminated on clinical grounds because of the absence of headache.

The diagnosis of ophthalmoplegic migraine is supported by a clinical course of transient headache with nausea and vomiting, resolution of the eye signs, a past history of typical migraine attacks, and a family history of migraine. Every child with a first attack should have an evaluation to eliminate the other considerations listed previously.

Retinal Migraine

The most common visual disturbance in migraine is the aura. This consists of scotomas defined as the sensation of seeing scintillating lights. This phenomenon is caused by ischemia of the contralateral occipital cortex. In contrast, retinal migraine is caused by ischemia of the retinal artery and causes ipsilateral monocular blindness and headache. It occurs in children with a preexisting migraine history and, rarely, can be the only manifestation of a migraine attack. The visual disturbance usually precedes the headache phase and is transient.

The differential diagnosis for retinal migraine includes transient monocular blindness, a common manifestation of cerebrovascular and embolic heart disease. Transient, bilateral visual loss also occurs in patients with severe increased intracranial pressure and is an ominous sign in patients with pseudotumor cerebri. Patients who do not have a typical history of migraine must be evaluated for these disorders.

Hemiplegie Migraine

Hemiplegie migraine causes unilateral weakness of the face and arm with variable involvement of the lower extremity.5 It is one of the more common complicated migraine syndromes. The motor deficit may be preceded by numbness or tingling of the affected limb. The weakness may precede, accompany, or follow head pain. Weakness on the right side can be accompanied by aphasia. If recurrent, alternate sides may be affected. These attacks are usually not associated with precipitating events, but may be seen following otherwise minor head trauma. As in other complicated migraine syndromes, hemiplegie migraine is a rare event in children. Familial hemiplegie migraine is this disorder with a family history of similar attacks. The gene for this disorder has been located on chromosome 19.6 Unlike other focal neurologic signs of migraine, hemiplegia may not be transient but last for several days. Acute stroke must be considered in any patient with the first attack of hemiplegie migraine. A history of migraine and the family history of similar attacks support the diagnosis.

Alternating hemiplegia syndrome1 is a severe disorder beginning in children under 3 years of age. It is unclear whether this syndrome represents a malignant form of migraine or a separate disorder. Children with this disorder have frequent, recurrent, and alternating episodes of hemiplegia, frequently associated with progressive intellectual and motor impairment. Seizures occur in some patients, but the hemiparetic events are random and do not necessarily follow a seizure. Electroencephalography (EEG) often shows slowing, but the imaging study and angiography results are normal.

Basilar Artery Migraine

Basilar artery migraine syndrome occurs primarily in adolescent girls but may begin much earlier in childhood. Affected children present with recurrent stereotypic attacks caused by temporary dysfunction of the brain stem and cerebellum. Clinical features are ataxia of gait, dysarthric speech, vertigo, and tinnitus.8 Variable bilateral visual complaints range from cortical blindness to blurring of vision or, at times, ophthalmoplegia. Alternating hemiplegia, impairment of consciousness, paresthesias of the face, arms, or legs, sudden loss of postural tone, loss of consciousness, and cardiac arrhythmias may be associated. The complex event is then followed by a headache, which is often severe and localized to the occipital region. The symptoms and signs present suddenly and clear completely. Patients usually have a strong family history of migraine and may later develop a more typical migraine syndrome.

The pathophysiology of basilar migraine is related to reduced blood flow to the brain stem, which accounts for the myriad of symptoms and signs associated with the attacks. There is also an association between basilar migraine and benign occipital epilepsy2 and most patients have occipital slowing on EEG during and immediately after a basilar migraine attack. Others have occipital spikes and coexisting seizures. The basilar migraine attack is perhaps triggered by the seizure. It is not uncommon for patients with epilepsy to have a severe headache and vomiting after a convulsion, further confusing the distinction between basilar migraine and seizures.

This diagnosis is difficult with the first attack. Trauma, vascular disease causing a brain stem infarct, and neoplasm must be excluded. Multiple sclerosis can cause similar signs. However, in this case, they will persist. A history of recurrent events and a family history of migraine are supportive evidence for basilar migraine.

Acute Confusional Migraine

Agitated confusion, disorientation, and combativeness characterize acute confusional migraine.9 The attacks may last from several minutes to several hours, and there is amnesia of the event. Vomiting can occur with an attack, but the child is usually too confused to complain of headache. The patients often enter a deep sleep afterwards. Acute confusional migraine may be the initial manifestation of a migrainous disorder but more often occurs in patients with previous migraine headaches. The disorder is caused by unilateral or bilateral cerebral hemispheric dysfunction. If an EEG is obtained during the event or shortly after, a generally disorganized background is seen often with unilateral temporal and occipital slowing.

Transient global amnesia is a temporary and total loss of memory. It has been described in migraine patients and may be a variant of acute confusional migraine. The Aiice*in-Wonderland syndrome is related to confusional migraine and basilar artery migraine. The patient senses a distortion in body image and time, and can experience visual hallucinations.10

Table

TABLE 2Migraine Prophylaxis

TABLE 2

Migraine Prophylaxis

The diagnosis of acute confusional migraine is difficult, especially if it is the first migraine event. When there is no history of previous migraine attacks, a family history for migraine may be helpful. One must exclude toxic ingestion, encephalitis, and metabolic encephalopathies such as Reye's syndrome to make this diagnosis.

MIGRAINE VARIANTS

The term migraine variant describes the following paroxysmal events. When these occur in infants and young children, the child may be unable to describe symptoms, but they usually go on to develop more typical migraine attacks or have a strong family history of migraine. Headache may be present.

Benign Paroxysmal Vertigo

A disorder of preschool children and infants, benign paroxysmal vertigo causes sudden and severe vertigo with nystagmus.11 Unlike other migraine syndromes, nausea and vomiting are usually absent. The child will lay motionless during the episode or wedge him or herself between objects to maintain balance. The child is often quite fretful during the event and appears pale. The episode only lasts for a few minutes, and these patient recovers completely. There is no change in consciousness and no complaints of head pain. These stereotypic events are recurrent at irregular intervals. As the child ages, more typical migraine attacks emerge.

Vertigo and dizziness are common complaints in older children and adults during a migraine attack. Vertigo can also be caused by middle and inner ear disease, drugs, trauma, epilepsy, tumor, and Meniere's disease. Benign paroxysmal vertigo can usually be distinguished on clinical grounds because of rhe recurrent, brief attacks in an otherwise healthy child. As with other migraine syndromes, a family history can almost always be obtained.

Cyclic Vomiting

Also called periodic syndrome or abdominal migraine, cyclic vomiting is characterized by recurrent episodes of severe emesis and pallor lasting up to several hours.12 If the episodes are prolonged, patients may suffer from dehydration. If the child is verbal, concurrent complaints of abdominal pain are common. After the episode, these patients often go to sleep and awaken symptom-free. As these children grow older, they usually develop more typical features of migraine headache. The family history is positive for migraine in most cases. As expected in all migraine syndromes, the physical and neurologic examination results are normal between attacks.

Cyclic vomiting from migraine is especially challenging to recognize in the preverbal child. Often the gastrointestinal symptoms are so dramatic that no one asks or pays attention to complaints of head pain. This is a diagnosis of exclusion, and other, more common, causes for vomiting must be considered. Because vomiting so often accompanies increased intracranial pressure, all infants need a neuroimaging study to exclude an intracranial mass.

Infantile Torticollis

Infantile torticollis may be caused by a variant of migraine when it presents during the first year of life, with recurrent episodes of head tilt that may alternate in direction.13 Attacks may last from hours to days. Often the attacks of torticollis are associated with nausea, vomiting, pallor, and agitation. Older children may have ataxia as well. This association has led some to draw a correlation with benign paroxysmal vertigo. Other paroxysmal movement disorders should be considered. These are discussed elsewhere in the issue of Pediatric Annals. Sandifer syndrome is torticollis associated with severe gastroesophageal reflux, usually in children with cerebral palsy. Posterior fossa masses can also cause unusual posturing of the head.

DIAGNOSIS

Diagnosing the various syndromes discussed previously is problematic in that there is no specific test for migraine. In almost every instance, migraine is diagnosed by excluding the many other causes for the particular symptoms. Routine laboratory and neuroimaging study results are invariably normal, although transient ischemic findings are sometimes seen on magnetic resonance imaging. The EEG finding is more likely to be abnormal in children with migraine than in otherwise healthy children, but the abnormalities are not specific. During a migraine attack, the EEG often shows nonspecific slowing.

Key to the diagnosis is a history of migraine headaches in the family. Often parents are unaware of their own diagnoses and attributes their headaches to sinus disease or allergies. A history of "sick headaches" should always be sought in the parents, siblings, and grandparents. Often parents do not remember having headaches or other paroxysmal events in their childhood, while the grandparents will recall these.

Paroxysmal events during infancy, such as cyclic vomiting, can often be a clue to later migraine phenomenon. Somnambulism and motion sickness are much more common in children with migraine than those without. Migraine events normally occur while the child is awake. Unusual episodes occurring during sleep are probably not migraine.

MANAGEMENT

Patients with complicated migraine almost always also have common or classic migraine headaches, and should be treated accordingly. Some neurologists recommend treating patients with complicated migraine more aggressively because of the rare but potentially serious possible complications of stroke, and in the case of basilar migraine, of cardiac arrhythmia. Prophylactic treatment is preferred in children. Abortive therapy is often difficult because children do not always recognize or report early signs of migraine, nor do they commonly take timely action to prevent attacks with abortive drugs such as sumatriptan. Adolescent girls with complicated migraine syndromes should be advised to avoid oral contraceptives because of the increased risk of stroke. Table 2 outlines the commonly used drugs for migraine prophylaxis. If there are known triggers for the event, they should be avoided. The September 1995 issue of Pediatric Armais provides additional information about the management of migraine headaches.

Paroxysmal vertigo and torticollis do not normally require therapy. Cyclic vomiting can be protracted in some infants to the extent of dehydration. For these infants, a trial of cyproheptidine is indicated.

REFERENCES

1. Goadsby PJ. Current concepts of the pathophysiology of migraine. Neuroi Clin. 1997;15:27-42.

2. Gordon N. Migraine, epilepsy, post-traumatic syndromes, and spreading depression. Dev Med ChOd Neuroi. 1989;31:682-689.

3. Olesen J, Headache Classification Committee of the International Headache Society, Classification and diagnostic criteria for headache disorders, cranial neuralgia, and facial pain. Cephalgia. 1938;8(supp| 7):l-96.

4. Bickerstaff ER. Ophthalmoplegic migraine. Ret, Neuroi. 1964;110:582-588.

5. Bradshaw P, Parsons M. Hemiplegie migraine, a clinical study. QfM. 1965;34:65-85.

6. Joutel A, Bousser MG, Biousse V, et al. A gene for familial hémiplégie migraine maps to chromosome 19. Nat Getwt. 1993;5:40-47.

7. Vertet S, Steele JC. Alternating hemiplegia in childhood: a report of eight patients with complicated migraine beginning in infancy. Pediatrics. 1971;47:675-680.

8. Bickerstaff ER. Basilar artery and the migraine-epilepsy syndrome. Proc R Soc Med. 1962;55:167-169.

9. Gascon G, Barlow CF. Juvenile migraine presenting as an acute confusional state. Pediatrics. 1970;45:628-635.

10. Golden GS. The Alice m Wonderland syndrome in juvenile migraine. Pediatrics. 1979;63:517-519.

11. Feniche! GM. Migraine as a cause of benign paroxysmal vertigo of childhood. J Pediatr. 1967;71:114-115.

12. Hammond J . The late sequelae of recurrent vomiting of childhood. Dev Med Child Neurol. 1974;16:15-22.

13. Deonna T, Martin D, Benign paroxysmal torticollis in infancy. Arch Dis Child. 1981;56:956-959.

TABLE 1

Classification of Migraine

TABLE 2

Migraine Prophylaxis

10.3928/0090-4481-19970701-07

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