Pediatric Annals

CME Article 

The Clinical Spectrum of Migraine in Children

Joseph M. Dooley, MB, BCh, FRCPC; Eric M. Pearlman, MD, PhD

Abstract

Most physicians recognize the characteristic nature of migraine in adults and have acquired a general comfort level with the idea of migraine with or without aura in that population. Although pediatricians are comfortable with the diagnosis of migraine, many find that their comfort is limited to children with the more common varieties, especially migraine without aura. Pediatricians often feel less certain in their ability to diagnose and manage other types of migraine, in part due to the variability of clinical presentations of migraine in children and also due to parental/physician anxiety over the presence of some underlying cause. This is confounded further by the presence of the migraine variants of childhood that are even less well known by most pediatricians. The diagnosis of some these variants is addressed in the most recent consensus statement from the International Headache Society, published in 2003, the International Classification of Headache Disorders-II (ICHD-II).1 The conditions recognized as childhood periodic syndromes that are commonly precursors of migraine include cyclical vomiting syndrome, abdominal migraine, and benign paroxysmal vertigo of childhood. There are, however, other conditions occurring in childhood, which have been associated with migraine by pediatricians and pediatric neurologists on the basis of epidemiological, genetic, or familial links.

Abstract

Most physicians recognize the characteristic nature of migraine in adults and have acquired a general comfort level with the idea of migraine with or without aura in that population. Although pediatricians are comfortable with the diagnosis of migraine, many find that their comfort is limited to children with the more common varieties, especially migraine without aura. Pediatricians often feel less certain in their ability to diagnose and manage other types of migraine, in part due to the variability of clinical presentations of migraine in children and also due to parental/physician anxiety over the presence of some underlying cause. This is confounded further by the presence of the migraine variants of childhood that are even less well known by most pediatricians. The diagnosis of some these variants is addressed in the most recent consensus statement from the International Headache Society, published in 2003, the International Classification of Headache Disorders-II (ICHD-II).1 The conditions recognized as childhood periodic syndromes that are commonly precursors of migraine include cyclical vomiting syndrome, abdominal migraine, and benign paroxysmal vertigo of childhood. There are, however, other conditions occurring in childhood, which have been associated with migraine by pediatricians and pediatric neurologists on the basis of epidemiological, genetic, or familial links.

Eric M. Pearlman, MD, PhD, is Medical Director and Physician-In-Chief, The Children’s Hospital Memorial University Medical Center; Chair, Department of Pediatrics, and Associate Professor of Pediatrics, Mercer University School of Medicine-Savannah Campus. Joseph M. Dooley, MB, BCh, FRCPC, is Professor of Pediatrics, and Head, Pediatric Neurology Division, Dalhousie University and IWK Health Centre, 5850/5980 University Avenue, Halifax, Nova Scotia B3K 6R8.

Address correspondence to: Eric M. Pearlman, MD, PhD, 4700 Waters Avenue, Savannah, GA 31404; e-mail: Pearler1@memorialhealth.com.

Most physicians recognize the characteristic nature of migraine in adults and have acquired a general comfort level with the idea of migraine with or without aura in that population. Although pediatricians are comfortable with the diagnosis of migraine, many find that their comfort is limited to children with the more common varieties, especially migraine without aura. Pediatricians often feel less certain in their ability to diagnose and manage other types of migraine, in part due to the variability of clinical presentations of migraine in children and also due to parental/physician anxiety over the presence of some underlying cause. This is confounded further by the presence of the migraine variants of childhood that are even less well known by most pediatricians. The diagnosis of some these variants is addressed in the most recent consensus statement from the International Headache Society, published in 2003, the International Classification of Headache Disorders-II (ICHD-II).1 The conditions recognized as childhood periodic syndromes that are commonly precursors of migraine include cyclical vomiting syndrome, abdominal migraine, and benign paroxysmal vertigo of childhood. There are, however, other conditions occurring in childhood, which have been associated with migraine by pediatricians and pediatric neurologists on the basis of epidemiological, genetic, or familial links.

The following review includes not include only the more common migraine varieties but also a series of clinical entities that the authors believe fall within the clinical spectrum of migraine or migraine-associated phenomena. It is important for the practicing pediatrician to recognize these entities. Making an accurate diagnosis will lead to reduced physician and parental anxiety about secondary headaches, less unnecessary diagnostic testing and better treatment.

Migraine Without Aura

Migraine without aura (previously common migraine) accounts for more than 60% of children with migraine. Although headache is the primary, and most recognized, feature of migraine, there are many other features of a migraine attack. Attacks of migraine without aura can be divided into three phases: prodrome, headache, and post-drome. The prodrome often precedes the headache by as much as 24 hours. During this time, the child may appear pale and listless and may experience thirst, food cravings, or anorexia. Mood changes, such as elation, irritability or social withdrawal may also occur. These prodromal symptoms are more common in migraine without aura than in migraine with aura. A prodrome may be subtle and hard to recognize, unless parents are educated about its presence. However, educating parents may allow them to recognize that an attack is imminent within the subsequent 24 hours.

The headache phase is characterized by head pain. Although the presence of unilateral pain is part of the diagnostic criteria for migraine, bifrontal or bitemporal pain is described more commonly than unilateral pain in children. Some children also describe holocranial or occipital pain or have a difficult time localizing the pain. There is no location of pain that precludes a diagnosis of migraine. The pain may have a gradual onset, but in children, this pain often builds more rapidly than in adults. Most episodes of pediatric migraine are brief and may last as short as an hour, although more commonly they last 2 to 3 hours, and often terminate when the child falls asleep. Some children report headaches that can be as brief as 20 minutes, but if untreated, the headaches can last as long as 48 to 72 hours. The headaches force the child to seek the comfort of a quiet, darkened room because of associated photophobia and phonophobia. Headaches are accompanied by anorexia, nausea, and vomiting, although the presence of vomiting without nausea should suggest another cause for the headache, such as increased intracranial pressure. Many children describe “dizziness,” which is more lightheadedness than vertigo, blurred vision, abdominal pain, cold extremities, chills, sweating, or even fever, during the headache phase. During an attack, the child appears ill and pale, often with apparent dark circles around the eyes. Some children continue to have this dark discoloration around the eyes between attacks. This has been called the “migraine facies” and seems similar to the “allergic facies” described in children with allergies.

Migraine headaches are typically aggravated by physical activity, such as going up or down stairs, carrying backpacks, coughing, sneezing, or bending over. Although childhood migraine is often described as less severe than adult migraine, the experience is severe enough to interfere with the child’s activities.2

Headaches can occur at any time, including overnight and early morning hours. About 25% of headaches awaken the child, which can raise concern for the presence of underlying pathology. Attacks are often precipitated by hunger, tiredness, and stress. Stress may be either positive (excitement) or negative. Foods are a precipitant for only 20% of patients. Most find that headaches are more likely to occur when there is a combination of these precipitants.

After the headache, patients are usually exhausted, although some experience elation and enhanced energy.

The diagnostic criteria from the ICHD-II are detailed in Sidebar 1. The management of migraine is discussed by Hershey et al in this issue (see page 416).

Sidebar 1.

  1. At least five attacks fulfilling criteria B-D

  2. Headache attacks lasting 1–72 hours (untreated or unsuccessfully treated)

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

    • Unilateral location
    • Pulsating quality
    • Moderate or severe pain intensity
    • Aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
  4. During headache at least one of the following:

    • Nausea and/or vomiting
    • Photophobia and phonophobia (may be inferred by behavior)
  5. Not attributed to another disorder

Diagnostic Criteria for Childhood Migraine Without Aura

Migraine with Aura

Among children with migraine, about 18% have migraine exclusively with aura, 13% have migraine with and without aura, and an additional 5% only experience the aura, without headache. Children younger than 10 years have difficulty describing their aura and may find it easier to draw their symptoms.3 Should a child have difficulty either describing or drawing the aura, it may be helpful to show them pictures of typical auras. The headaches associated with migraine with aura are the same as those described above for migraine without aura.

The aura represents focal cerebral dysfunction and either immediately precedes or occurs at the same time as the onset of the headache. It usually comes on less than 30 minutes before the headache and is brief, lasting only 5 to 20 minutes. The type of aura depends on the cortical area that is affected.

Visual auras are the most common type described in children. The visual disturbance may consist of blurring of the vision, fortification spectra (zigzag lines), scotomata (field defects, most commonly holes in central vision), scintillations (sparkles or stars), or kaleidoscopic patterns with multiple colors. More complicated visual distortions, such as those described below as the Alice in Wonderland syndrome, also represent migraine auras. If other cortical areas are affected, the aura may manifest as numbness/parasthesia, weakness, confusion, amnesia, or aphasia. The type of aura can vary from one episode to the next. Motor auras tend to last longer than the other aura phenomena. Complicated migraine is associated with neurologic signs or symptoms that persist for at least 24 hours after the headache. Examples include ophthalmoplegic migraine and hemiplegic migraine, as described below.

The major concern for many clinicians is distinguishing a migraine aura from either a seizure or a vascular event. In most situations, the speed of evolution of the symptoms allows easy distinction. In migraine, the symptoms develop over at least 5 minutes in comparison with the much more rapid spread of seizures and vascular accidents. In addition, a migraine aura often has a mix of positive and negative features, such as scotoma and scintillations or numbness and tingling. Aura is also completely reversible. The absence of headache, presence of altered consciousness, or prolonged duration usually necessitate an evaluation to rule out other conditions.

The diagnostic criteria for migraine with aura are provided in Sidebar 2 (see page 410).

Sidebar 2.

  1. At least two attacks fulfilling criteria B-D

  2. Aura consisting of at least one of the following, but no motor weakness:

    • Fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision)
    • Fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness)
    • Fully reversible dysphasic speech disturbance
  3. At least two of the following:

    • Homonymous visual symptoms1 and/or unilateral sensory symptoms
    • At least one aura symptom develops gradually over ≥5 minutes and/or different aura symptoms occur in succession over ≥5 minutes
    • Each symptom lasts ≥5 and ≤60 minutes
  4. Headache fulfilling criteria B-D for migraine without aura begins during the aura or follows aura within 60 minutes

  5. Not attributed to another disorder

Diagnostic Criteria for Migraine with Aura

Typical Aura with Nonmigraine Headache or Typical Aura Without Headache

The ICHD-II also includes categories for patients who have a typical aura, as described above, but whose headaches do not meet the criteria for migraine without aura or who do not develop headache after a typical aura. Without the presence of headache meeting criteria for migraine, there must be a heightened awareness for other causes of transient neurologic dysfunction. In children, this will often necessitate additional investigation.

Basilar-Type Migraine

Basilar-type migraine is a form of migraine with aura in which the aura reflects brainstem, occipital, or cerebellar dysfunction. The aura is followed by moderate to severe headache, which may be occipital. In most patients, the headache is associated with typical migraine features, such as nausea, vomiting, photophobia, and phonophobia. The age of onset may be as early as 7 years but is more common in adolescence and young adulthood.

The aura in basilar migraine is usually brief, lasting from 5 to 60 minutes, and may consist of vertigo, ataxia, nystagmus, dysarthria, tinnitus/hyperacusia, alterations in consciousness, bilateral parasthesias, diplopia, or visual disturbances (see Sidebar 3). Typically, the visual symptoms start first and may begin in one temporal or nasal field, with subsequent spread to involve both visual fields, reflecting bilateral hemispheric dysfunction. Patients may report hallucinations, with enlargement of body parts, especially the hands and head. Basilar-type migraine is only diagnosed in the absence of motor symptoms. The presence of motor symptoms changes the diagnosis to hemiplegic migraine (see below), although this distinction may be arbitrary.

Sidebar 3.

  1. At least 2 attacks fulfilling criteria B-D

  2. Aura consisting of at least two of the following fully reversible symptoms, but no motor weakness:

    • Visual symptoms simultaneously in both temporal and nasal fields of both eyes
    • Dysarthria →Vertigo →Ataxia →Tinnitus →Decreased level of consciousness
    • Hypacusis →Diplopia →Simultaneously bilateral paraesthesias
  3. At least one of the following:

    • At least one aura symptom develops gradually over 5 minutes and/or different aura symptoms occur in succession over 5 minutes
    • Each aura symptom lasts between 5 and 60 minutes
  4. Headache fulfilling criteria B-D for migraine without aura begins during the aura or follows aura within 60 minutes

  5. Not attributed to another disorder

Diagnostic Criteria for Basilar Migraine

A family history of typical migraine is reported by 86%, and many patients who have basilar-type migraine attacks also have attacks of typical migraine with aura.

The pathogenesis of basilar-type migraine remains unknown. The unusual aura symptoms and the presence of occipital headache often create anxiety. However, the characteristic nature of the attacks, resolution of symptoms, and normal examination between attacks should be reassuring that there is no ominous pathology responsible for the symptoms.

Confusional Migraine

Confusional migraine is an uncommon disorder, and little is known about the pathophysiology. It occurs more commonly in boys with a mean age of onset of 12 years.4 The most common presentation is an adolescent who presents to the emergency room with amnesia and confusion. Impaired speech is common, with the substitution of inappropriate words or phrases. These agitated, combative, and delirious patients are usually suspected of either being under the influence of illicit drugs or of having an encephalopathy. As they become more lucid, they will complain of headache, which may be typical of migraine or be relatively mild.

The events may be precipitated by minor head trauma. Confusional migraine was initially known as “footballer’s migraine,” as the episodes often followed soccer games in which the players repeatedly headed the old, wet, and heavy leather soccer ball. Episodes may last up to 1 to 2 days, with an average duration of 5 hours, and often end with the patient falling asleep. Upon awakening, the patient is normal and is usually amnesic for the event.

Many patients do not have a history of previous headaches, although typically, even those patients without a history of headaches will subsequently experience migraine in the future. Initial attacks usually prompt an emergent and thorough evaluation for significant pathology, including meningitis/encephalitis, hematoma/contusion, and seizure. However, EEG should be interpreted cautiously, as generalized or focal slowing occurs in confusional migraine. Confusional migraine may represent an overlap between basilar migraine, hemiplegic migraine, and episodic ataxia type 2, as demonstrated by complicated aura, overlap of symptoms and the presence of the CACNA1A gene mutation in patients with confusional migraine.5,6

Hemiplegic Migraine

Hemiplegic migraine can be either autosomal dominant (familial hemiplegic migraine, FHM) or sporadic hemiplegic migraine (SHM). Hemiplegic migraine is characterized by prolonged hemiplegia accompanied by numbness, aphasia, and confusion. The hemiplegia may precede, accompany, or follow the headache, and is typically contralateral to the headache. The symptoms usually last more than 1 hour and rarely can last for more than a day and sometimes up to a week.

The range of clinical features can vary widely, even in patients within the same family. Attacks can include impairment of consciousness, which can range from somnolence or confusion to coma (Sidebar 4, see page 411). Some FHM attacks are associated with cerebellar ataxia, whereas other types may manifest with coma, fever, and meningismus. A further type of FHM involves progressive ataxia, nystagmus, gait unsteadiness, limb incoordination, and dysarthria.

Sidebar 4.

  1. At least two attacks fulfilling criteria B and C

  2. Aura consisting of fully reversible motor weakness and at least 1 of the following:

    • Fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) or negative features (eg, loss of vision)
    • Fully reversible sensory symptoms including positive features (eg, pins and needles) or negative features (eg, numbness)
    • Fully reversible dysphasic speech disturbance
  3. At least two of the following:

    • At least one aura symptom that develops gradually over at least 5 minutes or different aura symptoms that occur in succession over more than 5 minutes
    • Each aura symptom lasts more than 5 minutes and less than 24 hours
    • Headache fulfilling criteria 2 through 4; migraine without aura begins during the aura or follows onset of aura
  4. At least one first- or second-degree relative has had attacks fulfilling these criteria A through E

  5. Not attributed to another disorder

Diagnostic Criteria for Familial Hemiplegic Migraine

Attacks can be provoked by emotional stress, mild head trauma, or exertion. Most patients develop symptoms gradually and recover between attacks, but permanent sequelae, such as dementia, hemiplegia, infarction, and even death can occur. Basilar migraine symptoms (as described above) occur in about two-thirds of patients, and most patients also have attacks of more typical migraine with or without aura.

About half of families with FHM have a missense mutation in a P/Q-type calcium channel gene (CACNA1A) linked to chromosome 19p13.7 FHM2 is due to an abnormality in Na/K pump associated gene in the ATP1A2 gene on chromosome 1q21–23.

Other possible etiologies must be considered, including structural lesions, vasculitis, cerebral hemorrhage, brain tumor, mitochondrial myopathy, and encephalopathy. If hemiparesis is always on the same side, consider a vascular abnormality.

The diagnostic criteria for FHM and sporadic hemiplegic migraine are detailed in Sidebar 3 (see page 411), with the presence or absence of a family history distinguishing the two conditions. Some forms of FHM are responsive to therapy with acetazolamide.

Ophthalmoplegic Migraine

Ophthalmoplegic migraine is better considered as a cranial neuralgia, as there is evidence that it is a demyelinating-remyelinating process. It is characterized by periorbital or retro-orbital pain associated with prolonged oculomotor nerve palsies involving the III, and less commonly, the IV or VI cranial nerves. The ophthalmoplegia may precede, accompany, or follow the headache, which can be mild and non-specific or may be more migraine-like. During an episode, patients may have ptosis and limited ocular adduction. These signs may persist beyond the headache, and with recurrent attacks, patients may be left with a permanent oculomotor deficit. Ophthalmoplegic migraine is no longer included in the ICHD-II classification.

Ophthalmic (Retinal) Migraine

Ophthalmic or retinal migraine is characterized by repeated attacks of monocular scotoma or blindness usually followed by headache. Patients have normal ophthalmologic examination findings between attacks. Attacks, which occur without headache, are representative of acephalic migraine (migraine sine hemicrania).

A positive family history is essential in making the diagnosis, but one should exclude retinal embolism or other ocular abnormalities. The ICHD-II criteria are listed in Sidebar 5 (see page 412).

Sidebar 5.

  1. At least two attacks fulfilling criteria B and C

  2. Fully reversible monocular positive and/or negative visual phenomena (eg, scintillations, scotomata 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

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

  4. Normal ophthalmological examination between attacks

  5. Not attributed to another disorder

Diagnostic Criteria for Ophthalmic (retinal) Migraine

Periodic Syndromes of Childhood that Are Precursors to Migraine

Cyclic Vomiting Syndrome

Children with cyclical vomiting typically present with recurrent periodic attacks of sudden unexplained severe vomiting (see Sidebar 6, page 412). Onset is usually in the preschool child but can occur at any age, including adulthood. Attacks often begin during the night or on awakening, and the child may vomit as frequently as every 10 minutes. During the attack, the child may appear withdrawn or irritable, and there may be associated abdominal pain, diarrhea, fever, and headache.

Sidebar 6.

  1. At least five attacks fulfilling criteria B and C

  2. Episodic attacks, stereotypical in the individual patient, of intense nausea and vomiting lasting from 1 hour to 5 days

  3. Vomiting during attacks occurs at least 4 times/hour for at least 1 hour

  4. Symptom-free between attacks

  5. Not attributed to another disorder

Diagnostic Criteria for Cyclical Vomiting

The episodes of cyclic vomiting usually last a few hours but can last up to several days. Precipitants are similar to those of migraine, with illness, stress, and tiredness being among the most frequently identified precursors.

Between episodes, patients are symptom-free, but episodes usually recur every few months, and severe episodes may lead to dehydration, which often requires intravenous rehydration.

Headache may occur in some children as a symptom of cyclic vomiting syndrome, but as cyclic vomiting resolves in adolescence, it is often replaced by migraine. Cyclic vomiting is more common in girls, and there is often a family history of migraine.

Most pediatricians arrive at a diagnosis of cyclic vomiting syndrome only after other causes of vomiting have been excluded. However, as is the case with migraine, characteristic attacks with normal intervals should not require significant diagnostic evaluations.

Abdominal Migraine

Episodes of abdominal migraine are characterized by central abdominal pain, dull in nature, severe enough to interfere with normal activities, commonly associated with anorexia, nausea, vomiting, and pallor, lasting for at least 1 hour, and with complete resolution between attacks. The child is otherwise well and healthy, with normal growth and development. Diagnosis should be made after exclusion of gastroesophageal reflux (GERD), peptic ulcers, inflammatory bowel disease, constipation, food intolerance, and renal disorders.

Approximately 10% of schoolchildren between 5 and 15 years reported recurrent abdominal pain, and approximately 5% reported episodes fulfill the criteria for the diagnosis of abdominal migraine. Abdominal migraine affects boys and girls equally, with a peak age of onset at 10 years. It is important to recognize that approximately 5% to 10% of children with recurrent abdominal pain have an underlying organic cause for their pain.

Episodes usually last for about 12 hours. Attacks are accompanied by pallor, anorexia, and vomiting. As with migraine, the episode often terminates as the child falls asleep and awakens feeling well. Approximately 70% of patients subsequently develop migraine.8

The ICHD-II diagnostic criteria are listed in Sidebar 7 (see page 412), and the alternate criteria from the Rome III Functional Gastrointestinal Disorder Child/Adolescent Committee are detailed in Sidebar 8.9

Sidebar 7.

  1. At least five attacks fulfilling criteria B to D

  2. Attacks of abdominal pain lasting 1 to 72 hours (untreated or unsuccessfully treated)

  3. Abdominal pain has all of the following characteristics

    • Midline location, periumbilical or poorly localized

    • Dull or “just sore” quality

    • Moderate or severe intensity

  4. During abdominal pain, at least two of the following occur

  5. Not attributed to another disorder

Diagnostic Criteria for Abdominal Migraine

Sidebar 8.

Must include all of the following:

  1. Paroxysmal episode of intense, acute periumbilical pain that lasts 1 hour or more

  2. Intervening periods of usual health lasting weeks to months

  3. The pain interferes with normal activities.

  4. The pain is associated with two or more of the following:

    • Anorexia

    • Nausea

    • Vomiting

    • Headache

    • Photophobia

    • Pallor

  5. No evidence of an inflammatory, anatomic, metabolic, or neoplastic process considered that explains the subject’s symptoms

Note: Criteria fulfilled two or more times in the preceding 12 months13

Benign Paroxysmal Vertigo of Childhood (BPV)

Benign paroxysmal vertigo (BPV) of childhood is characterized by sudden episodes of apparent fear, during which the toddler refuses to walk and usually grabs on to the nearest adult or object (see Sidebar 9, page 413). The toddler may refuse to stand, and verbal children may complain of dizziness and nausea. It occurs primarily in children 2 to 6 years. The child may appear pale and sweaty, and there may be associated photophobia and phonophobia. The attack lasts from seconds up to 5 minutes and rarely can last longer. There may be nystagmus during, but not between, the attacks. There is no alteration of consciousness during the episodes and the child is normal interictally. The frequency varies from daily up to every 2 to 3 months.

Sidebar 9.

  1. At least two attacks fulfilling criterion B

  2. Multiple episodes of severe vertigo,1 occurring without warning and resolving spontaneously after minutes to hours

  3. Normal neurological examination; audiometric and vestibular functions between attacks

  4. Normal electroencephalogram

Diagnostic Criteria for Benign Paroxysmal Vertigo

Precipitants are sometimes identified and are usually similar to those for a more typical migraine, although labyrinthine stimulation, such as swinging, may also precipitate events.

A family history of migraine is found in less than 40%, but many children will have migraine, especially basilar migraine, as they get older.10,11 BPV resolves before school entry, although it rarely persists into the teenage years. More commonly, the evolution is to more typical migraine.12 Because the attacks are brief, no therapy is usually needed beyond reassurance.

Paroxysmal Torticollis of Infancy

This is probably the most rare of the migraine-associated phenomena. Attacks consist of sudden onset of head tilt, sometimes with accompanying vomiting. Some consider this as a paroxysmal dyskinesia, although others regard it as a precursor to basilar migraine. Some patients have been found to have a mutation in the CAC-NA1A gene, which is also associated with hemiplegic migraine.13 Attacks usually occur in infants younger than 8 months and are more common in girls. Episodes may last from hours to days, although, occasionally, they are brief. Resolution can be expected before school entry. It is important to exclude GERD (Sandifer syndrome) and posterior fossa abnormalities in the differential diagnosis of these children. There are no proven therapies. Although not included in the ICHD-II classification, the criteria for diagnosis are provided in Sidebar 10.

Sidebar 10.

  1. Episodic attacks, in a young child, with all of the following characteristics and fulfilling criterion B:

    • tilt of the head to one side (not always the same side), with or without slight rotation

    • lasting minutes to days

    • remitting spontaneously and tending to recur monthly

  2. During attacks, symptoms and/or signs of one or more of the following:

    • pallor
    • irritability
    • malaise
    • vomiting
    • ataxia
  3. Normal neurological examination between attacks

  4. Not attributed to another disorder

Diagnostic Criteria for Benign Paroxysmal Torticollis

Alice in Wonderland Syndrome

Alice in Wonderland syndrome is an episodic phenomenon during which the patient reports distortions of perception. Typically the episodes do not occur at the time of a headache, although the patients either have a history of migraine or subsequently develop migraine. The classical description is of a perception that either the patient’s own body or objects in the environment appear smaller (micropsia) or larger (macropsia) than reality. The patient is aware that the perception is false, and there is no alteration of consciousness.14 Distortions may also involve time, with a sense of increased or decreased speed involving the patient’s movements or speech or those of others.15 Complex partial seizures are the main differential diagnosis. Therapy, beyond reassurance, is almost never needed.

Other Phenomena

A number of other features are also more common in migraineurs. They are more prone to motion sickness and sleep disturbances, such as sleep-walking. Many patients with migraine (82%) also report an aversion to objects with a “busy” striped-pattern. Ice cream ingestion can lead to headache in more than 90% of those with migraine.16

Summary

Migraine is an intriguing disorder, as much of its story remains untold. In the past decade, our insights have advanced greatly as we gain a better understanding of the pathophysiology of many aspects of migraine. The future will offer further knowledge regarding the etiology of the various components of the migraine subtypes and variants. Some will be reclassified as channelopathies with new and more specific therapies. In the interim, our primary responsibilities are to recognize the treatable and the benign. Awareness of the clinical features allows a more rational approach with the knowledge we currently have. Although evidence-based therapies are few in migraine, the encouragement to identify and subsequently avoid precipitants, in addition to the use of anecdotally supported medications, allows us to help most children and adolescents with migraine.

References

  1. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Headache Classification Committee of the International Headache Society. Cephalalgia. 1988;8Suppl 7:1–96.
  2. Hershey AD. What is the impact, prevalence, disability, and quality of life of pediatric headache?Curr Pain Headache Rep. 2005;9(5):341–344. doi:10.1007/s11916-005-0010-y [CrossRef]
  3. Lewis DW, Middlebrook MT, Mehallick L, Rauch TM, Deline C, Thomas EF. Pediatric headaches: what do the children want?Headache. 1996;36(4):224–230. doi:10.1046/j.1526-4610.1996.3604224.x [CrossRef]
  4. Strain JR, Guo Y, Rothner AD. Acute confusional migraine (ACM): a review of 90 cases seen in children and adolescents. Presented at: 35th Annual Meeting of the Child Neurology Society. , PA, USA. , October 2006. (Abstract No. H-04).
  5. Lewis DW, Pearlman E. The migraine variants. Pediatr Ann. 2005;34(6):486–468, 490–492, 494–497.
  6. Cleves C, Parikh S, Rothner AD, Tepper SJ. Link between confusional migraine, hemiplegic migraine and episodic ataxia type 2: hypothesis, family genealogy, gene typing and classification. Cephalalgia. 2009Jul17. [Epub ahead of print] doi:10.1111/j.1468-2982.2009.01958.x [CrossRef]
  7. Ophoff RA, Terwindt GM, Vergouwe MN, et al. Familial hemiplegic migraine and episodic ataxia type-2 are caused by mutations in the Ca2+ channel gene CACNL1A4. Cell. 1996;87(3):543–552. doi:10.1016/S0092-8674(00)81373-2 [CrossRef]
  8. Dignan F, Abu-Arafeh I, Russell G. The prognosis of childhood abdominal migraine. Arch Dis Child. 2001;84(5):415–418. doi:10.1136/adc.84.5.415 [CrossRef]
  9. Rasquin A, Di Lorenzo C, Forbes D, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology. 2006;130(5):1527–1537. doi:10.1053/j.gastro.2005.08.063 [CrossRef]
  10. Lindskog U, Odkvist L, Noaksson L, Wallquist J. Benign paroxysmal vertigo in childhood: a long-term follow-up. Headache. 1999;39(1):33–37. doi:10.1046/j.1526-4610.1999.3901033.x [CrossRef]
  11. Abu-Arafeh I, Russell G. Paroxysmal vertigo as a migraine equivalent in children: a population-based study. Cephalalgia. 1995;15(1):22–25; discussion 4. doi:10.1046/j.1468-2982.1995.1501022.x [CrossRef]
  12. Cuvellier JC, Lépine A. Childhood periodic syndromes. Pediatr Neurol. 2010;42(1):1–11. doi:10.1016/j.pediatrneurol.2009.07.001 [CrossRef]
  13. Giffin NJ, Benton S, Goadsby PJ. Benign paroxysmal torticollis of infancy: four new cases and linkage to CACNA1A mutation. Dev Med Child Neurol. 2002;44(7):490–493. doi:10.1111/j.1469-8749.2002.tb00311.x [CrossRef]
  14. Evans RW, Rolak LA. The Alice in Wonderland Syndrome. Headache. 2004;44(6):624–625. doi:10.1111/j.1526-4610.2004.446013.x [CrossRef]
  15. Dooley J, Gordon K, Camfield P. “The rushes.” A migraine variant with hallucinations of time. Clin Pediatr (Phila). 1990;29(9):536–538. doi:10.1177/000992289002900912 [CrossRef]
  16. Raskin NH, Knittle SC. Ice cream headache and orthostatic symptoms in patients with migraine. Headache. 1976;16(5):222–225. doi:10.1111/j.1526-4610.1976.hed1605222.x [CrossRef]

CME Educational Objectives

  1. Evaluate the varying and unique clinical presentations of migraine in children.

  2. Explain the diagnostic criteria for migraine in children.

  3. Identify the features of the periodic syndromes that are precursors for migraine.

Sidebar 1.

  1. At least five attacks fulfilling criteria B-D

  2. Headache attacks lasting 1–72 hours (untreated or unsuccessfully treated)

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

    • Unilateral location
    • Pulsating quality
    • Moderate or severe pain intensity
    • Aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
  4. During headache at least one of the following:

    • Nausea and/or vomiting
    • Photophobia and phonophobia (may be inferred by behavior)
  5. Not attributed to another disorder

Diagnostic Criteria for Childhood Migraine Without Aura

Sidebar 2.

  1. At least two attacks fulfilling criteria B-D

  2. Aura consisting of at least one of the following, but no motor weakness:

    • Fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision)
    • Fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness)
    • Fully reversible dysphasic speech disturbance
  3. At least two of the following:

    • Homonymous visual symptoms1 and/or unilateral sensory symptoms
    • At least one aura symptom develops gradually over ≥5 minutes and/or different aura symptoms occur in succession over ≥5 minutes
    • Each symptom lasts ≥5 and ≤60 minutes
  4. Headache fulfilling criteria B-D for migraine without aura begins during the aura or follows aura within 60 minutes

  5. Not attributed to another disorder

Diagnostic Criteria for Migraine with Aura

Sidebar 3.

  1. At least 2 attacks fulfilling criteria B-D

  2. Aura consisting of at least two of the following fully reversible symptoms, but no motor weakness:

    • Visual symptoms simultaneously in both temporal and nasal fields of both eyes
    • Dysarthria →Vertigo →Ataxia →Tinnitus →Decreased level of consciousness
    • Hypacusis →Diplopia →Simultaneously bilateral paraesthesias
  3. At least one of the following:

    • At least one aura symptom develops gradually over 5 minutes and/or different aura symptoms occur in succession over 5 minutes
    • Each aura symptom lasts between 5 and 60 minutes
  4. Headache fulfilling criteria B-D for migraine without aura begins during the aura or follows aura within 60 minutes

  5. Not attributed to another disorder

Diagnostic Criteria for Basilar Migraine

Sidebar 4.

  1. At least two attacks fulfilling criteria B and C

  2. Aura consisting of fully reversible motor weakness and at least 1 of the following:

    • Fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) or negative features (eg, loss of vision)
    • Fully reversible sensory symptoms including positive features (eg, pins and needles) or negative features (eg, numbness)
    • Fully reversible dysphasic speech disturbance
  3. At least two of the following:

    • At least one aura symptom that develops gradually over at least 5 minutes or different aura symptoms that occur in succession over more than 5 minutes
    • Each aura symptom lasts more than 5 minutes and less than 24 hours
    • Headache fulfilling criteria 2 through 4; migraine without aura begins during the aura or follows onset of aura
  4. At least one first- or second-degree relative has had attacks fulfilling these criteria A through E

  5. Not attributed to another disorder

Diagnostic Criteria for Familial Hemiplegic Migraine

Sidebar 5.

  1. At least two attacks fulfilling criteria B and C

  2. Fully reversible monocular positive and/or negative visual phenomena (eg, scintillations, scotomata 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

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

  4. Normal ophthalmological examination between attacks

  5. Not attributed to another disorder

Diagnostic Criteria for Ophthalmic (retinal) Migraine

Sidebar 6.

  1. At least five attacks fulfilling criteria B and C

  2. Episodic attacks, stereotypical in the individual patient, of intense nausea and vomiting lasting from 1 hour to 5 days

  3. Vomiting during attacks occurs at least 4 times/hour for at least 1 hour

  4. Symptom-free between attacks

  5. Not attributed to another disorder

Diagnostic Criteria for Cyclical Vomiting

Sidebar 7.

  1. At least five attacks fulfilling criteria B to D

  2. Attacks of abdominal pain lasting 1 to 72 hours (untreated or unsuccessfully treated)

  3. Abdominal pain has all of the following characteristics

    • Midline location, periumbilical or poorly localized

    • Dull or “just sore” quality

    • Moderate or severe intensity

  4. During abdominal pain, at least two of the following occur

  5. Not attributed to another disorder

Diagnostic Criteria for Abdominal Migraine

Sidebar 8.

Must include all of the following:

  1. Paroxysmal episode of intense, acute periumbilical pain that lasts 1 hour or more

  2. Intervening periods of usual health lasting weeks to months

  3. The pain interferes with normal activities.

  4. The pain is associated with two or more of the following:

    • Anorexia

    • Nausea

    • Vomiting

    • Headache

    • Photophobia

    • Pallor

  5. No evidence of an inflammatory, anatomic, metabolic, or neoplastic process considered that explains the subject’s symptoms

Note: Criteria fulfilled two or more times in the preceding 12 months13

Sidebar 9.

  1. At least two attacks fulfilling criterion B

  2. Multiple episodes of severe vertigo,1 occurring without warning and resolving spontaneously after minutes to hours

  3. Normal neurological examination; audiometric and vestibular functions between attacks

  4. Normal electroencephalogram

Diagnostic Criteria for Benign Paroxysmal Vertigo

Sidebar 10.

  1. Episodic attacks, in a young child, with all of the following characteristics and fulfilling criterion B:

    • tilt of the head to one side (not always the same side), with or without slight rotation

    • lasting minutes to days

    • remitting spontaneously and tending to recur monthly

  2. During attacks, symptoms and/or signs of one or more of the following:

    • pallor
    • irritability
    • malaise
    • vomiting
    • ataxia
  3. Normal neurological examination between attacks

  4. Not attributed to another disorder

Diagnostic Criteria for Benign Paroxysmal Torticollis

Authors

Eric M. Pearlman, MD, PhD, is Medical Director and Physician-In-Chief, The Children’s Hospital Memorial University Medical Center; Chair, Department of Pediatrics, and Associate Professor of Pediatrics, Mercer University School of Medicine-Savannah Campus. Joseph M. Dooley, MB, BCh, FRCPC, is Professor of Pediatrics, and Head, Pediatric Neurology Division, Dalhousie University and IWK Health Centre, 5850/5980 University Avenue, Halifax, Nova Scotia B3K 6R8.

Address correspondence to: Eric M. Pearlman, MD, PhD, 4700 Waters Avenue, Savannah, GA 31404; e-mail: .Pearler1@memorialhealth.com

10.3928/00904481-20100623-09

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