Basics
Description
- Primary headache: headache without an identifiable underlying etiology; includes but not limited to migraine, tension headache, cluster headache, and other trigeminal autonomic cephalgias; may be episodic or chronic in nature
- Secondary headache: headache attributed to an identifiable underlying etiology
- Other: unusual headaches, especially in children, that include various cranial neuralgias and central and primary facial pain
Epidemiology
- Headache prevalence can approach 30-50% by school age, 60-80% by early adolescence, and as high as 85% by adulthood.
- Tension-type headache is most common type of headache, with migraine second.
- Mean age of onset for migraine is 7 years for boys and 11 years for girls. Migraine precursors such as abdominal migraine, cyclic vomiting syndrome, and benign paroxysmal vertigo of childhood can be seen even younger. These may transform into migraines by puberty.
- Overall migraine reported prevalence ranges between 8 and 23%. Suspicion is that migraine prevalence may be as high as 30-35% due to underreporting. Prevalence of chronic daily headache in younger children is 2-4% and 4-5% in adolescence/adulthood.
- Until puberty, migraines more common in males (55-60%), post puberty in females (75%)
- Genetics plays a role with some reports of 90% family history noted. Genes have been identified for some migraine subtypes including familial hemiplegic migraine.
- Ethnicity plays a role, with majority of reported chronic migraine sufferers being Caucasian.
Diagnosis
Migraine in children is classified into the following groups: �
- Migraine without aura: "Common"� migraine represents most cases
- Migraine with aura: "Classic"� migraine must include aura, a reversible focal neurologic symptom that gradually develop over 5-20 minutes and typically resolve within 1 hour.
- Basilar-type migraine (up to 20% of childhood migraines): often seen migraine with aura with occipital pain often and must include two of the following: dysarthria, vertigo, tinnitus, hyperacusis, diplopia, ataxia, visual symptoms, bilateral paresthesias, and decreased consciousness
- Childhood periodic syndromes that are precursors to migraine: benign paroxysmal vertigo of childhood, cyclic vomiting, abdominal migraine, benign paroxysmal torticollis
- Hemiplegic migraine: type of migraine with aura, with symptoms that must include motor weakness along with one of the following: sensory symptoms, visual symptoms, dysphasic speech
- Others: "Alice in Wonderland syndrome"� has distortions of vision, space, and/or time; confusional migraine has impaired sensorium, agitation, and lethargy.
General migraine diagnostic criteria set by the International Classification of Headache Disorders 3rd edition, beta (ICHD III, beta) in children/adolescents �
- 5 attacks not attributed to another condition
- Lasting 2-72 hours
- 2 of the following:
- unilateral or bilateral pain
- moderate to severe pain
- pulsating quality
- aggravated by routine activity
- 1 of the following:
- photophobia and phonophobia (may be inferred)
- nausea or vomiting
- Aura has separate criteria.
Tension-type headache differs from migraine as follows: more episodes typically seen, lasts 30 minutes to 7 days, typically describes as bilateral pain, pressing/tightening quality, mild to moderate intensity, not worse with activity, not associated with sensitivity to light or sound, nausea, or vomiting �
History
The history should help clarify the diagnosis of which type of headache is present and help guide workup and treatment. �
- The following questions should be asked:
- Is there more than 1 type of headache?
- Have headaches gotten worse?
- How often do they occur?
- Where is the pain located?
- What is the quality of the pain?
- Do the headaches occur at any special time of day?
- What associated symptoms are present?
- Is there a warning sign or aura noted?
- What triggers a headache?
- What helps the headache feel better?
- Any symptoms present between episodes?
- What treatments have been tried? (include dose, duration, and outcome)
- Any migraine precursors noted or other common comorbidities such as motion sickness or psychiatric concerns?
- "Red flags"�: age of onset younger than 3 years of age; first or worst headache; occipital location; recent headache onset; increasing severity or frequency; headache in the morning associated with vomiting; headache causing awakening from sleep; worse with straining; change in mood, mental status, or school performance; or underlying neurocutaneous syndrome or other neurologic concern
- Lifestyle considerations that affect headaches: amount and quality of sleep, hydration status, consistent diet/meals, caffeine consumption, level of fitness and amount of cardiovascular exercise, stress, and anxiety
Physical Exam
- Vital signs: blood pressure, heart rate, and weight. Consider orthostatic blood pressure and pulse for dizziness or syncope; if obese, consider pseudotumor or sleep apnea syndrome.
- Skin changes consistent with neurocutaneous syndrome
- Sinus tenderness, limitation of jaw excursion, or occipital trigger points
- Complete neurologic exam including vision screen and funduscopic exam: should be normal in primary headaches. Exception is during aura: Exam may show temporary deficit.
- Basic depression and psychiatric screen
- Any abnormality on exam warrants further investigation, as it may suggest secondary headache with possible underlying etiology.
Diagnostic Tests & Interpretation
Practice parameters are established in 2002 for the evaluation of children and adolescents with recurrent headaches. The guidelines address laboratory evaluation, ancillary testing, and imaging. �
Imaging
- Neuroimaging studies (CT or MRI): generally not recommended if neurologic exam is normal and no red flags. If exam is abnormal or red flags are present, imaging should be obtained with determining which study to obtain based on clinical concern and situation.
- CT if there is any suspicion for any acute process such as hemorrhage, but otherwise MRI is preferred.
- Consider risk of imaging (e.g., younger children require anesthesia for MRI; radiation exposure with CT).
- Consider MRA/MRV if vascular cause/etiology in differential diagnosis.
- Neuroimaging guidelines are based on multiple studies with more than 600 images reviewed. Only 3% of those images obtained led to specific treatments geared toward imaging findings, and in all those who had abnormal imaging that required treatment, their neurologic exams were found to be abnormal.
Diagnostic Procedures/Other
- EEG: no role for EEG in routine testing of patients with headache
- Laboratory investigation: generally not recommended in primary headaches
- Lumbar puncture (LP): not recommended unless concern for secondary headache or underlying etiology such as infection, hemorrhage, sinus thrombosis, pseudotumor cerebri, and low-pressure headache. Imaging should be done first to ensure it is safe to perform LP.
Differential Diagnosis
The pattern of headache can help clarify the differential. There are 5 patterns. �
- Acute, 1st severe headache
- CNS infection, cocaine or other substance abuse, medication (methylphenidate, steroids, psychotropic drugs, analgesics, cardiovascular agents), hypertension (usually secondary), hydrocephalus, pseudotumor cerebri (idiopathic intracranial hypertension), post-LP, CNS hemorrhage, ventriculoperitoneal shunt malfunction, sinus thrombosis, migraine, other infection including upper respiratory, somatization
- Acute recurrent headache
- Much of what is listed above in acute, 1st severe headache can be recurrent as well. Additional consideration would include migraine and variants, cluster, and tension.
- Chronic progressive headache
- Brain tumor, chronic CNS infection including abscess, hydrocephalus, vascular malformation, hematoma, sinus thrombosis, idiopathic intracranial hypertension, depression or other psychiatric condition, anemia, rheumatologic diseases
- Chronic nonprogressive or daily headache
- Much of what is listed above in chronic progressive headache can be nonprogressive as well. Additional consideration would include medication overuse, substance abuse including caffeine, chronic infection such as sinusitis, occipital neuralgia, temporomandibular joint syndrome, orthostatic headache, post-LP, other systemic disease, posttraumatic, sleep disorder, tension headache, fibromyalgia.
- Mixed headache: migraine-superimposed tension headache with broad differential including much of what is listed above
Treatment
Medication
Practice parameters are established in 2004 for the pharmacologic treatment of migraine in children and adolescents. The guidelines addressed acute and prophylactic medical management. �
- Acute treatment: generally most effective if given early in the acute headache/migraine
- 1st line is ibuprofen (10 mg/kg PO) - level A.
- Acetaminophen (10-15 mg/kg PO) - level B
- Naproxen sodium 5.5-7.7 mg/kg PO per dose: longer acting NSAID than ibuprofen
- Sumatriptan nasal spray can be 1st line in adolescent migraine - level A.
- Many options noted to be level U (not enough data).
- Additional acute treatments often used especially in refractory patients
- Antiemetics (prochlorperazine, metoclopramide, ondansetron) also enhance effectiveness of analgesics and may abort migraines. Prochlorperazine has best evidence of this class, with up to 95% improvement noted in some studies.
- Triptans: generally safe, but only almotriptan is currently FDA approved for use in children or adolescents. Rizatriptan and zolmitriptan have been shown effective in children and adolescents. Seven triptans are available in various forms, including tablet, nasal spray, dissolvable, and injection.
- Any agent that may lead to vasoconstriction such as triptans and ergotamines are not recommended in basilar, hemiplegic, or any migraine with vascular risk factors.
- Most over-the-counter or prescription pain/headache/migraine medications may cause rebound headaches.
- Status migrainosus
- Migraine lasting >72 hours
- General treatment involved a combination of medications/treatments.
- Not well studied, but some evidence that the following combinations are successful:
- Fluids, triptan if used successfully prior, NSAID (ibuprofen/naproxen/ketorolac), antidopaminergic (prochlorperazine [Compazine] with best evidence), and Benadryl
- Valproic acid can be used as second tier.
- No definitive evidence for steroids or magnesium despite often being used
- Dihydroergotamine (DHE-45) noted to be highly effective in small studies; can be IV, IM, or intranasal; given with combination of treatments including antiemetic
- Narcotics are not recommended.
- Prophylaxis
- When to start is controversial, but generally if there is more than 1 day per week with disability or dysfunction, daily medication is recommended.
- Goal is reduction in headache frequency or severity by ≥50%. There is no cure for headaches/migraines. Keeping realistic expectations is key to treatment.
- Start at a low dose and titrate as needed while being mindful of adverse effects.
- Can take 4-6 months for effects to be seen
- Choose a drug that may address comorbidities as well. Be careful to avoid drug classes that may exacerbate other medical conditions (e.g., beta-blockers in asthma patients).
- Practice parameters from 2004 very limited: Flunarizine (not available in United States) only prophylactic medication with any recommendation other than level U
- Amitriptyline (TCA) and topiramate (anticonvulsant) most commonly used and studied options in children
- Cyproheptadine (antihistamine) frequently used 1st line in younger children as well as for migraine precursors and GI symptoms
- Other options would include the following:
- Calcium channel blockers: verapamil
- Beta-blockers: propranolol, nadolol
- Tricyclic agents: nortriptyline, imipramine
- SNRI: venlafaxine, duloxetine
- Anticonvulsants: valproic acid, gabapentin, carbamazepine (neuralgias), and oxcarbazepine (neuralgias)
Complementary & Alternative Therapies
- Physical therapy, exercise (aerobic, yoga, Pilates), massage therapy, relaxation techniques (meditation, progressive muscle relaxation, self-hypnosis), stress management, cognitive behavioral therapy, biofeedback
- Some evidence for vitamins, supplements, and herbs such as butterbur (50-75 mg b.i.d.), riboflavin (50-400 mg daily), magnesium oxide (50-200 mg b.i.d.). Of note, supplements and vitamins are not regulated by the FDA. Melatonin as low as 3 mg nightly is reported to be as effective as low-dose amitriptyline.
Additional Reading
- Abu-Arafeh �I, Razak �S, Silvaraman �B, et al. Prevalence of headache and migraine in children and adolescents: a systematic review of population-based studies. Dev Med Child Neurol. 2010;52(12):1088-1097. �[View Abstract]
- Lewis �D. Pediatric migraine. Pediatr Rev. 2007;28(2):43-53. �[View Abstract]
- Lewis �DW, Ashwal �S, Dahl �G, et al. Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2002;59(4):490-498. �[View Abstract]
- Lewis �D, Ashwal �S, Hershey �A, et al. Practice parameter: pharmacological treatment of migraine headache in children and adolescents: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society. Neurology. 2004;63(12):2215-2224. �[View Abstract]
- Papetti �L, Spalice �A, Nicita �F, et al. Migraine treatment in developmental age: guidelines update. J Headache Pain. 2010;11(3):267-276. �[View Abstract]
- Termine �C, Ozge �A, Antonaci �F, et al. Overview of diagnosis and management of paediatric headache. Part II: therapeutic management. J Headache Pain. 2011;12(1):25-34. �[View Abstract]
Codes
ICD09
- 784.0 Headache
- 346.90 Migraine, unspecified, without mention of intractable migraine without mention of status migrainosus
- 307.81 Tension headache
- 339.00 Cluster headache syndrome, unspecified
- 339.02 Chronic cluster headache
- 346.9 Migraine, unspecified
- 339.01 Episodic cluster headache
- 346.00 Migraine with aura, without mention of intractable migraine without mention of status migrainosus
ICD10
- R51 Headache
- G43.909 Migraine, unsp, not intractable, without status migrainosus
- G44.209 Tension-type headache, unspecified, not intractable
- G44.009 Cluster headache syndrome, unspecified, not intractable
- G44.019 Episodic cluster headache, not intractable
- G44.1 Vascular headache, not elsewhere classified
- G44.029 Chronic cluster headache, not intractable
SNOMED
- 25064002 Headache (finding)
- 37796009 Migraine (disorder)
- 398057008 Tension-type headache (disorder)
- 193031009 Cluster headache syndrome (disorder)
- 4473006 Migraine with aura (disorder)
- 128187005 Vascular headache (disorder)