Review z Paediatric headache Diagnosis and management of headache in children and adolescents Ishaq Abu-Arafeh MBBS, MD, MRCP, FRCPCH As part of our series on managing neurological and psychiatric conditions in children and adolescents, Dr Abu-Arafeh discusses paediatric headaches. eadache is a common complaint in children 1. Migraine without aura Hand adolescents with an overall prevalence of 2. Migraine with aura around 60%. Tension-type headache and migraine • Migraine with typical aura are the most common types of primary headache. • Migraine with brainstem aura The diagnosis of headache disorders is usually made • Hemiplegic migraine on clinical assessment and the application of accept- • Retinal migraine able classification and diagnostic criteria, and inves- 3. Chronic migraine tigations are rarely necessary. 4. Complications of migraine Available and licensed medications for the treat- • Status migrainosus ment of headache in children are limited, but when • Persistent aura without infarction • Migrainous infarction used alongside non-pharmacological measures, chil- • Migraine aura-triggered seizure dren can achieve good control of their headache. 5. Probable migraine • Probable migraine without aura Introduction • Probable migraine with aura Headache affects children of all ethnic and socioeco- 6. Episodic syndromes that may be associated with nomic groups. In a systematic review of population- migraine based studies, around 60% of children and adolescents • Cyclical vomiting syndrome had headache over the past three to 12 months with • Abdominal migraine more girls affected than boys over the age of 12 years.1 • Benign paroxysmal vertigo Episodic tension-type headache (TTH) is the most • Benign paroxysmal torticollis common headache disorder with a prevalence of Table 1. Classification of migraine – ICHD-3 beta.11 12–25% followed by migraine at around 8%.1–4 Chronic TTH affects around 1% of children (mainly Classification of headache disorders adolescents).5 Other primary headaches are rare and The International Classification of Headache reliable data are hard to find. Chronic daily headache, Disorders, now in its third edition (ICHD-3 beta), pro- commonly due to TTH, is a common cause of refer- vides a comprehensive, reliable and most utilised tool ral to specialist clinics and may be disproportionately for the assessment of headache disorders.11 Although represented.6 ICHD-3 is heavily biased towards headache in adults, Primary headaches have a complex genetic basis the system is applicable and reliable in paediatric and follow the biopsychosocial model in clinical pres- practice with minor modifications. entation, trigger factors, comorbidity and in response Headache disorders are divided into primary to treatment.7 The trigeminocervical complex in the and secondary headaches. Primary headaches brainstem, with its extensive network of neuronal con- include: (1) migraine; (2) tension-type headache; (3) nections to the cerebral cortex, the thalamus, the vas- trigeminal autonomic cephalalgias; and (4) other cular system and the dura matter, plays an important rare primary headaches. Secondary headaches in chil- role in the pathogenesis of migraine attacks and pos- dren are uncommon, but it is essential to make sibly other headache disorders.8,9 Physiological, envi- early diagnosis of serious neurological disorders ronmental or emotional factors may trigger a such as brain tumours and idiopathic intracranial micro-neuroinflammation in genetically predisposed hypertension. individuals, causing an increase in vascular perme- The new classification of migraine (Table 1) intro- ability, plasma extravasation and neurotransmitters duces chronic migraine as an important subtype of release leading to the secondary neurological, sen- migraine and expands on the childhood abdominal sory and pain phenomena.10 migraine, cyclical vomiting, paroxysmal torticollis and 16 Progress in Neurology and Psychiatry July/August 2014 www.progressnp.com Paediatric headache z Review paroxysmal vertigo. TTH is classified according to the presentation, by excluding other conditions such as frequency of attacks as infrequent, frequent and occipital epilepsy and vascular malformations.12 chronic TTH. Hemiplegic migraine Causes of headache in children and adolescents Hemiplegic migraine is a type of migraine with motor The diagnosis of headache disorders is based on a clin- aura. During migraine attacks the patients report uni- ical history, clinical examination and prospective lateral weakness with or without slurring of speech. headache diaries. History should include: the duration Familial hemiplegic migraine (FHM) is autosomal of headache disorder; frequency and duration of attacks; dominant, but sporadic cases are also recognised. severity of pain; site of maximum pain; quality of pain if FHM1 is associated with at least 20 different missense the child is able to describe this; known trigger factors; mutations in the voltage gated calcium channel and warning symptoms. Enquiry is made on the pres- (CACNA1A) on chromosome 19p13. FHM2 is associ- ence or absence of anorexia, nausea, vomiting, light and ated with a mutation in the Na/K-ATPase gene ATPA2 noise intolerance and other sensory, motor or auto- on chromosome 1q21. The FHM3 form is associated nomic symptoms during attacks, and also on relieving with mutations in the sodium channel SCN1A on factors such as rest, sleep and medication. Treatment chromosome 2q24.13–15 The clinical presentation of history should also include dosages, format and route hemiplegic migraine may mimic a stroke. Full inves- of administration of rescue medications, and dosages tigation including magnetic resonance imaging and and length of courses of preventative treatment. magnetic resonance angiogram may be necessary before the diagnosis is made in order to exclude Migraine intracranial lesions and cerebrovascular diseases. Around 80% of children with migraine have migraine without aura.5 There are no clear triggers of migraine Chronic migraine attacks in the majority of children, but around one- Chronic migraine is characterised by headache third of patients may report stress, missing a meal, or attacks occurring on at least 15 days per month, of lack of sleep. Aura symptoms (commonly visual and which at least eight days per month are of typical less often sensory or motor) precede headache in migraine, over at least three months. Chronic 15–20% of patients. migraine often evolves from episodic migraine over The headache often builds up in severity over 30–60 an average period of two years and can be compli- minutes reaching maximum intensity within 1–2 hours. cated by medication overuse headache. Chronic The pain is described as severe (stops all activities) or migraine is associated with significant impact on qual- moderate (stops some but not all activities) and throb- ity of life, school attendance and education.16 bing in quality, but many children cannot describe pain. The site of maximum pain is often on the fore- Tension-type headache head unlike the unilateral headache in adult patients. TTH is characterised by mild (no interference with During attacks, the child is pale, loses appetite, feels normal activities) to moderate (interferes with some nauseated, may vomit, and complains of intolerance to but not all activities) intensity of headache. The pain light, noise and exercise. The child may feel better after A. At least 5 attacks fulfilling criteria B–D rest and sleep, and returns to normal self between B. Headache attacks lasting 2–72 hours (untreated or attacks. Criteria for the diagnosis of migraine without unsuccessfully treated) aura are shown in Table 2. Migraine with typical visual C. Headache has at least 2 of the following 4 charac- aura is easily distinguishable and relatively common, teristics: but in some children atypical aura and specific syn- 1. Unilateral location dromes can pose difficulties in diagnosis and treatment. 2. Pulsating quality 3. Moderate or severe pain intensity Alice in Wonderland syndrome 4. Aggravation by or causing avoidance of routine The Alice in Wonderland syndrome is a type of physical activity migraine with intense visual aura manifested by micro - D. During headache at least 1 of the following: 1. Nausea and/or vomiting psia (images look smaller than their real sizes), macro- 2. Photophobia and phonophobia psia (images look magnified), or a combination of both. E. Not better accounted for by another ICHD-3 The intensity of distorted images can be distressing to the child and may overshadow the pain and other asso- Table 2. Criteria for the diagnosis of migraine without aura – ICHD-3 ciated symptoms. Diagnosis is made, especially at early beta.11 www.progressnp.com Progress in Neurology and Psychiatry July/August 2014 17 Review z Paediatric headache A. At least 10 episodes of headache occurring on Investigations fulfilling criteria B–D Neuroimaging is not necessary in the majority of chil- B. Lasting from 30 minutes to 7 days dren with typical clinical history and in the absence C. At least 2 of the following 4 characteristics: of features of increased intracranial pressure or signs 1. Bilateral location of cerebellar dysfunction. 2. Pressing or tightening (non-pulsating) quality Measurement of cerebrospinal fluid opening pres- 3. Mild or moderate intensity sure is necessary if idiopathic intracranial hyperten- 4. Not aggravated by routine physical activity such as walking or climbing stairs sion is suspected. Electroencephalography is only D. Both of the