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The Migraine Syndrome in Children 2022 Paediatrics and Child Health
The Migraine Syndrome in Children 2022 Paediatrics and Child Health
The migraine syndrome in umbrella of migraine has become more compelling over the past
decade.3 Their recognition as migraine disorders not only brings
PAEDIATRICS AND CHILD HEALTH 32:10 388 Ó 2022 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
Investigations are only necessary if the clinical presentation is unlike other types of pain, are not comforted by being cuddled.
either atypical, accompanied by headache ‘red flags’ indicating a These features resemble photophobia, vasomotor changes and
secondary cause is possible, or if thorough clinical assessment allodynia seen in migraine.
cannot exclude underlying disorders. Criteria for diagnosis of IC are listed in the ICHD 3 in the ‘ap-
pendix’ section, denoting that sufficient evidence for its inclusion
Migraine with aura is less common than migraine without aura, in the main body has not been confirmed. Criteria for diagnosis of
affecting only 1 in 4 children and young people with migraine. It IC outlined within the ICHD 3 and Rome IV consensus are largely
has similar characteristics to migraine without aura but with similar. Rome IV has excluded the rule of 3 hours, days and weeks
added aura symptoms before or with the headache. Visual dis- for use in clinical practice. These have been retained in ICHD for
turbances are the most common aura symptoms, followed by the purpose of clinical research (Table 1).
sensory disturbances, speech disorder and motor weakness. Evidence to support the close relationship between IC and
Typical aura symptoms usually evolve over at least 5 minutes migraine is demonstrated from epidemiological and follow up
and are followed within 60 minutes by the headache phase. studies of babies with IC. Prospective studies on infants with
Migraine is described as chronic migraine when headaches colic show that they are twice as likely to develop migraine in
occur on at least 15 days per month for at least three consecutive later childhood. Children with migraine are more likely than not
months, with headaches consistent with migraine on at least to have had infantile colic symptoms e one study suggested 79%
eight days per month. Chronic migraine affects 1e2% of ado- compared with a population incidence of 17%. Mothers with
lescents. Girls are slightly more likely to be affected than boys. migraine are more than twice as likely to have offspring with
Chronic migraine is associated with poor response to treatment, infantile colic compared with mothers without migraine.
high impact on quality of life, school attendance, academic The UK National Institute of Health and Clinical Excellence
achievement and participation in sports and leisure activities. recommends soothing strategies such as gentle motion, holding/
Chronic migraine may be compounded by medication overuse. swaddling and white noise. NICE also encourages parents/carers
to look after their own well-being. Neither NICE nor other sys-
Infantile colic (IC) tematic reviews support hydrolysed formulas, probiotic use,
simethicone, lactase drops or herbal remedies since the studies
Most paediatricians are familiar with this common disorder,
investigating these are small and/or prone to bias. Treatment
distressing infants and their carers alike. It is characterized by
with simple analgesics such as paracetamol can relieve pain, but
episodes of relentless crying in infants less than three months of
acute migraine treatments such as sumatriptan have not been
age, lasting for several hours per day (often late in the day or
trialled, and their pharmacological properties in infants have not
evening) on at least three days a week for longer than three
been studied. As such, these cannot be recommended.
weeks. It affects 10e15% of infants, boys and girls equally. The
condition usually peaks at six weeks and decreases by 4e5 mon-
Benign paroxysmal torticollis (BPT)
ths of age.
The aetiology and pathogenesis of infant colic are not known. BPT is a disorder of the childhood migraine syndrome with a
Several factors and mechanisms have been proposed, including median age of onset of 2e3 years. BPT is considered rare but its
infant factors such as sleep disruption, visceral hypersensitivity, true prevalence may be higher due to underdiagnosis and lack of
allergy, gut microflora and dysmotility. Breast-fed infants are less awareness of the condition. In some studies 1% of children with
likely to suffer from colic. Maternal factors implicated in infantile migraine have a history of BPT. BPT causes anxiety due to the
colic include psychological stress, poor physical health and nature of the cardinal symptom of unexplained head tilt. Epi-
smoking during pregnancy, and negative childbirth experiences. sodes of torticollis last for minutes to days and are often asso-
The characteristic clinical features of IC are the episodic nature ciated with distress, pallor, refusal to eat, vomiting, irritability or
of inconsolable crying and distress with complete resolution be- lethargy, ataxia and occasionally, hypotonia. Older children often
tween episodes. Infants with colic enjoy normal growth, weight report headaches during episodes.
gain and development. Investigations reveal no other cause for the Episodes of torticollis resolve spontaneously with complete re-
distress. The source of distress is not clear but is assumed to be covery between episodes. Episodes recur every few weeks and
abdominal pain. Babies often have their eyes closed, look pale and, reduce in frequency over 2e3 years until they stop by the age of 5
1. An infant who is less than five months of age when the symptoms A. Recurrent episodes of irritability, fussing or crying from birth to four
start and stop months of age, fulfilling criterion B
2. Recurrent and prolonged periods of infant crying, fussing, or irri- B. Both of the following:
tability reported by caregivers that occur without obvious cause 1. episodes last for 3 hours/day
and cannot be prevented or resolved by caregivers 2. episodes occur on 3 days/week for 3 weeks
3. No evidence of infant failure to thrive, fever, or illness C. Not attributed to another disorder.
Table 1
PAEDIATRICS AND CHILD HEALTH 32:10 389 Ó 2022 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
years in most patients. Early motor developmental delay, if present, consciousness, and there are no abnormal movements or
seems to be transient, and the prognosis for neurodevelopment is posturing to suggest an epileptic event. Between episodes, the
generally favourable. child returns to their normal self, and the neurological examination
The aetiology and pathogenesis of BPT are not well under- is normal.
stood. Around half of children with BPT have a family history of BPV is the most common cause of episodic dizziness in chil-
migraine and/or IC. There are case reports of families with BPT, dren presenting to paediatricians, paediatric neurologists and
migraine and hemiplegic migraine associated with CACN1A and ear, nose and throat specialists. The prevalence of BPV is esti-
PRRT2 channelopathies. Follow up studies showed children with mated at 2e3% of the childhood population and is slightly more
BPT are more likely than unaffected children to develop benign common in girls. The typical age of onset is 3e4 years but may
paroxysmal vertigo of childhood (45e51%), cyclical vomiting range from 18 months to 12 years. Between 21 and 33% of
syndrome (9%) or migraine (as high as 63%) later in childhood. children with BPV are reported to have migraine headaches or a
Torticollis can also occur as a feature of IVth cranial nerve palsy combination of migraine and cyclical vomiting in later childhood
(head tilted to the side of the lesion), posterior fossa (cer- and adolescence. Some will develop ‘vestibular migraine’: at-
ebellopontine) mass lesion, cervical cord lesion (a tumour or tacks of disabling vertigo lasting between 5 minutes and 72h
Chiari malformation) or dystonia. Sternocleidomastoid shortening associated with throbbing headache and its typical associated
as a result of birth injury is a common cause of fixed torticollis. features. Others will have additional symptoms supporting a
The diagnosis of BPT is made on the application of the clinical diagnosis of migraine with brainstem aura. Migraineurs in later
criteria (Box 2) and exclusion of these other possible causes. The childhood are more likely (9%) than would be expected by
episodic nature of torticollis in BPT, alternation of the affected side, chance to have had BPV in earlier life.
association with complete recovery and normal neurological ex- The diagnosis of BPV can be made clinically by applying the
amination in-between episodes help to exclude other causes. ICHD 3 criteria for diagnosis (Box 3). A video recording of an
Videos of the attacks can help to secure the diagnosis. MRI of the episode can help, where the diagnosis is uncertain from the
brain and cervical spine, ictal EEG and ophthalmological assess- history. Other causes of dizziness should be considered,
ment may be required where the diagnosis is uncertain. including central (traumatic brain injury or structural brain ab-
The management of children with BPT is generally symp- normalities) and peripheral (recurrent otitis media, Meniere’s
tomatic and supportive. Vomiting, ataxia and irritability appear disease) causes, as well as emotional and mental health diffi-
to respond to simple analgesia and antiemetics such as culties. Investigations are only required if other causes are sus-
ondansetron and prochlorperazine. There are case reports of pected. Brain imaging will be needed if the child has persistent
response to migraine prophylactic agents such as cyprohepta- non-resolving symptoms, new neurological deficit, squint or
dine, topiramate and riboflavin, but no high-quality drug trials to seizures. Standard and ambulatory electroencephalography
determine efficacy have been conducted. Multi-centre clinical (EEG) is needed if the child’s episodes have features of epileptic
trials are needed if adequate treatment is to be proven. seizures. Inner ear disease should be suspected if symptoms are
associated with hearing loss and/or tinnitus. Routine vestibular
Benign paroxysmal vertigo (BPV) function tests can be practically challenging in young children,
are not usually required to confirm the diagnosis.
BPV is characterized by episodes of sudden fearfulness, unstead-
Due to its name and acronym, BPV can be confused with benign
iness and reluctance to stand up or walk. During an episode of BPV,
paroxysmal positional vertigo (BPPV), from which it is aetiologi-
the child holds on to furniture or their carer’s legs, or simply lies
cally and clinically distinct. BPPV is seen almost exclusively in
down on the floor. The child looks pale, feels sick and occasionally
vomits. An observant parent or clinician may note horizontal
nystagmus. In younger children, vertigo is inferred from their
behaviour. Older children may describe unsteadiness or a feeling ICHD-3 (2018) criteria for the diagnosis of BPV1
of a spinning of the room around them. The child does not lose A. At least five attacks fulfilling criteria B and C
B. Vertigo* occurring without warning, maximal at onset and
resolving spontaneously after minutes to hours without loss of
ICHD 3 (2018) criteria for the diagnosis of BPT1
consciousness
A. Recurrent attacks in a young child, fulfilling criteria B and C C. At least one of the following five associated symptoms or signs:
B. Tilt of the head to either side, with or without slight rotation, 1. nystagmus
remitting spontaneously after minutes to days 2. ataxia
C. At least one of the following five associated symptoms or signs: 3. vomiting
1. pallor 4. pallor
2. irritability 5. fearfulness
3. malaise
D. Normal neurological examination and audiometric and vestibular
4. vomiting
functions between attacks
5. ataxia
E. Not attributed to another disorder**.
D. Normal neurological examination between attacks *Infer vertigo from behaviour **Exclude posterior fossa tumours,
E. Not attributed to another disorder. seizures and other ENT disorders.
Box 2 Box 3
PAEDIATRICS AND CHILD HEALTH 32:10 390 Ó 2022 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
A. At least five attacks of abdominal pain, fulfilling criteria BeD At least 2 attacks over 6 months must include all the following:
B. Pain has at least two of the following three characteristics: 1. Paroxysmal episodes of intense acute periumbilical pain, midline or
1. midline location, periumbilical or poorly localized diffuse abdominal pain lasting 1 hour or more
2. dull or ‘just sore’ quality 2. Episodes are separated from weeks or months
3. moderate or severe intensity 3. The pain is incapacitating interferes with normal activities
C. At least two of the following four associated symptoms or signs: 4. Stereotypical pattern and symptoms in individual patient
1. anorexia 5. The pain is associated with 2 or more of the following:
2. nausea a. Anorexia
3. vomiting b. Nausea
4. pallor c. Vomiting
D. Attacks last 2e72 hours when untreated or unsuccessfully treated d. Headache
E. Complete freedom from symptoms between attacks e. Pallor
F. Not attributed to another disorder 6. After appropriate evaluation, the symptoms cannot be fully
explained by another medical condition
Table 2
Invesgaons are not necessary Invesgaons may be necessary according to system involved
PAEDIATRICS AND CHILD HEALTH 32:10 391 Ó 2022 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
‘burning’ abdominal pain, pain lasting less than an hour, the Cyclical vomiting syndrome (CVS)
presence of symptoms in between attacks, pain that does not
CVS is closely related to migraine and has been discussed in a
interfere with daily activities, and symptoms attributed to
previous article published in this journal in 2020. The prevalence
another gastrointestinal disease as constipation, diarrhoea and
of CVS has been estimated from population-based studies to be
weight loss. Criteria for the diagnosis of AM are similar in both
around 2% of school-aged children. The peak age of onset is five
the ICHD-3 and Rome IV for Functional Gastrointestinal disor-
years, but CVS has also been described in people of all ages, from
ders (Table 2).
infancy to old age. CVS is characterized by recurrent episodes of
Investigations are only necessary if the clinical presentation is
intense nausea and vomiting lasting between 2 hours and three
atypical or there are additional symptoms or findings on the
days. Episodes are often unprovoked and occur with stereotypic
clinical examination that may indicate an alternate diagnosis
periodicity and clinical presentation.
(Figure 1).
Akin to migraine headache, episodes display three phases:
Investigations are usually unnecessary with the typical clinical
premonitory symptoms of mood change, yawning, food cravings
presentation, but if other underlying conditions cannot be
and heightened perception, an ictal phase of intense nausea,
excluded safely on clinical grounds, first-line investigations may
vomiting, lethargy, pallor, abdominal pain and/or headache
include full blood count, serum electrolytes, urea, creatinine, liver
followed by a recovery phase. Vomiting can be severe enough to
function tests, C-reactive protein (CRP), urinalysis and culture,
cause dehydration requiring hospital admission for intravenous
and abdominal ultrasound scan.
fluid replacement.
Both AM and migraine headache commonly co-exist more
The diagnosis of CVS is clinical, based on fulfilling the criteria
than would be expected by chance. Population-based studies
set out by ICHD-3 and the Rome IV consensus (Table 3). How-
have identified common triggers (tiredness, travel, emotional
ever, as in the case with AM, the diagnosis of CVS requires
stress and missed meals), similar associated symptoms (nausea,
careful clinical assessment to exclude other metabolic, intracra-
vomiting, dizziness, intolerance to light, noise and exercise) and
nial, renal and gastrointestinal causes of vomiting (Figure 1).
common relieving factors (rest, sleep and simple analgesia). One
Because of the wide range of underlying conditions and lack
third to one half of children with AM develop migraine head-
of a specific biomarker of CVS, the choice of investigations is
aches as they reach adolescence. There is a strong family history
often challenging, and tests should be selected based on clinical
of migraine in first degree relatives.
presentation and suspicion.4
On making the positive diagnosis of AM, children and parents
Like other migraine syndrome disorders, the pathogenesis of
should be given an explanation of the disorder and its relation-
CVS is not well understood. Proposed pathophysiological mecha-
ship to migraine. Pharmacological treatment of acute episodes
nisms common to both CVS and abdominal migraine include
consists of simple analgesia and antiemetics if necessary. Chil-
mitochondrial polymorphisms and ion channelopathies such as
dren should be advised to take rest and maintain adequate hy-
that involving ryanodine (RYR2). Triggers to attacks and relieving
dration during attacks. Nasal sumatriptan can be tried in children
factors of CVS resemble AM and migraine headaches. Associated
over 12 years of age. Non-pharmacological prevention may
features such as tiredness, mood changes and sensitivity to light/
include advice on a healthy lifestyle, including regular meals,
noise are common to all these conditions. Long term follow-up of
sleeping routine and exercise. Preventive treatment may be
young children with CVS shows that 79% have migraine head-
needed if the episodes of AM are frequent, prolonged, distressing
aches in adolescence, more than would be expected by chance.
and have a poor response to acute treatment. Unfortunately,
The main aim of treating acute episodes is to stop vomiting
evidence for preventive treatment of AM is derived from open
and prevent dehydration. Both sumatriptan (either by subcu-
studies or small trials. Medications commonly used are pizotifen,
taneous injection or nasal spray) and a reliably absorbed anti-
amitriptyline or flunarizine, which should be tried for at least 6
emetic formulation such as orodispersible ondansetron should be
e8 weeks before its effectiveness can be correctly judged.
given as soon as possible after symptom onset. Co-use of
A. At least 5 attacks of intense nausea and vomiting, fulfilling criteria Must include all of the following:
B and C 1. The occurrence of 2 or more periods of intense, unremitting
B. Stereotypical in an individual patient and recurring with predictable nausea and paroxysmal vomiting, lasting hours to days,
periodicity within a 6-month period.
C. All of the following: 2. Episodes are stereotypical in each patient
1. nausea and vomiting occur at least 4 times per hour 3. Episodes are separated by weeks to months, with return to
1. attacks last 1 hour and up to 10 days baseline health between episodes.
2. attacks occur at least 1 week apart 4. After appropriate medical evaluation, the symptoms cannot
D. Complete freedom from symptoms between attacks. be attributed to another condition.
E. Not attributed to another disorder.
Table 3
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Age of onset 0e3 months 1e2 years 2e5 years 4e7 years 5e10 years >2 years
Duration of 2e4 hours 2e48 hours 2e12 hours 2e72 hours 2e72 hours 2e72 hours
episode
Sensory Allodynia Photophobia Dizziness Photophobia, Phonophobia Photophobia, Photophobia,
impairment Phonophobia Phonophobia,
Allodynia
Vasomotor Pallor Pallor Pallor Pallor Pallor Pallor
GI upset Possible abdominal Nausea, Nausea, Abdominal pain, Nausea, Nausea, Vomiting Nausea, Vomiting
pain Vomiting Vomiting Vomiting
Between Normal Normal Normal Normal Normal Normal
episodes
Table 4
PAEDIATRICS AND CHILD HEALTH 32:10 393 Ó 2022 Elsevier Ltd. All rights reserved.