Headache and Pain Syndromes

Headache and Pain Syndromes
Lauren Doyle Strauss
Anna Minster
OVERVIEW OF HEADACHES
Introduction
Headache is the most common manifestation of pain in childhood and is the most frequent reason for referral to a child neurologist. Although migraine tends to be most studied and better recognized by parents, tension-type headache is the most common. It is important to recognize, diagnose, and treat the pain of headaches which can often first present in childhood, as recurrent or unremitting headaches can lead to significant disability that can persist into adulthood.
EPIDEMIOLOGY: (1) The prevalence of all types of headache increases with age. According to Bille’s landmark epidemiologic survey conducted in the 1950s in Sweden of 6,000 school children, the prevalence of “headache” ranged from 37% to 51% in 7 yo, rising to 57% to 82% by age 15. Frequent headaches occurred in 2.5% of 7 yo, which increased to 15% with 15 yo.1 (2) With migraines, prevalence increased with age; however, gender predilection changes. In children (3-7 yo), migraine prevalence is 1.2% to 3.2% and is more common in boys. By 7 to 11 y of age, 4% to 11% have migraines, but is now seen equally in boys and girls. Around the age of puberty (studied at 15 y of age), the prevalence increases to 8% to 23%, with girls more frequently affected than boys.2,3 (3) Tension-type headache has the highest lifetime prevalence. Prevalence ranges in different studies from 30% to 78%, which would make it the most common headache type. (4) Chronic daily headache accounts for significant proportion of referrals to headache clinics. Chronic daily headaches of various types have a prevalence of about 1% in adolescents, which increases in adulthood.4 It accounts for 15% to 20% of referrals to headache clinics. (5) Headache is a leading cause of disability. According to the World Health Organization (WHO), migraine alone is 19th among all causes of years lived with disability (YLDs).5 Headache disorders can cause personal suffering, which can lead to impairment of family and social life, and performance in school.
PATHOPHYSIOLOGY: (1) Vascular Theory: First theory proposed by Harold Wolff in 1940. Intracranial vasoconstriction is responsible for the aura of migraine and the rebound vasodilation activates perivascular nociceptive nerves, resulting in headache. (2) Neurovascular Theory: More widely accepted. Cortical spreading depression explains the aura; consists of rapid and nearly complete depolarization of neurons, with massive efflux of K+ ions extracellularly. It originates in the visual cortex and propagates like a wave throughout brain tissue at a rate 2 to 6 mm/min. It can also be a/w relative decrease in blood flow, leading to plasma protein extravasation through the dural vessels, causing activation of the meningeal afferents. Thought to centrally activate the trigeminal nucleus within the brainstem, leading to sensitization of the trigeminal afferents.
Clinical Approach to Headache Patients
HISTORY: Involve both the children and their parents in history taking as often parents are unaware of associated symptoms. Most headaches in childhood are benign, but be cognizant of parents’ anxiety as there may be concerns about malignant causes. A detail-oriented history is crucial for an appropriate diagnosis and to eliminate unnecessary tests (Table 14.1).
TABLE 14.1 Important Details in History Taking

Headache patterns

Acute vs. chronic vs. recurrent, progressive vs. non-progressive, or mixed pattern

Headache description

Onset, location, severity of pain on pain scale, quality (dull, pressure, twisting, sharp, pounding, throbbing, stabbing), frequency, duration, temporal association (time of day or menses cycle)

Aura

Timing, length, type (visual, sensory, language, or motor disturbances)

Associated symptoms

Tinnitus, numbness, paresthesias, weakness, dysarthria, nausea, vomiting, dizziness (light-headedness or vertigo), blurred vision, diplopia, photophobia, phonophobia, fatigue, scotoma, scintillations, transient visual obscurations, eye pain, neck pain, osmophobia

Severity of morbidity

Missed days of school/work, ED visits

Headache triggers

Food triggers (commonly include food dyes, MSG, chocolate), caffeine use, odors, noisy environment, dehydration, catamenial or hormone association, weather change, certain odors, physical activity, sleep (delayed sleep onset, frequent night awakening, snoring, apnea, periodic limb movements in sleep, excessive sleep), anxiety, mood, history of social stressors, illicit drug use, dental concerns (dental infection/abscess, teeth grinding, emerging wisdom teeth, TMJ pain), history of concussion, history of carsickness

Alleviating factors

Sleep, medications, position (standing vs. supine), cold/warm compress/shower, distraction by a different activity

Medication Hx

Current/failed abortive or prophylactic medications, ask if abortive medications taken at the onset of headache, dosing (important to document maximum doses of failed medications), frequency, specifically ask about OCP

Other treatment Hx

Therapy, acupuncture, massage therapy, exercise, music, yoga, meditation

Family history

History of headaches, migraines, anxiety, depression, sleep apnea, seizures

Previous evaluations

Imaging (CT, MRI), formal ophthalmologic exam, EEG, sleep study, dental evaluation, LP with opening pressure

Parental concerns

What are you concerned about? What do you think is causing the headaches?

Patient concerns

Conduct interview separately

CLASSIFICATION OF HEADACHES: (1) International Headache Society (IHS) developed the International Classification of Headache Disorders, 2nd edition (ICHD-II), which can guide approach (Table 14.2).6 (2) First determine whether the headache is primary or secondary (consider when onset or worsening is associated with a disorder known to cause headaches, as in underlying medical or neurologic conditions).
TABLE 14.2 ICHD-II: Primary and Secondary Headaches

Primary headaches

•

Migraine

–

Migraine without aura

–

Migraine with aura

•

Familial hemiplegic migraine (FHM)

•

Sporadic hemiplegic migraine

•

Basilar-type migraine

–

Periodic syndromes that are commonly precursors of migraine

•

Cyclical vomiting

•

Abdominal migraine

•

Benign paroxysmal vertigo of childhood

•

Status migrainosus

•

Tension-type headache

•

Cluster headache and other trigeminal autonomic cephalalgias

•

Other primary headaches

Secondary headaches

Headache attributed to

•

Head or neck trauma

–

Acute posttraumatic headache

–

Chronic posttraumatic headache

•

Cranial or cervical vascular disorder

•

Nonvascular intracranial disorder

•

Headache attributed to idiopathic intracranial hypertension

–

Headache attributed to low cerebrospinal fluid pressure

–

Headache attributed to intracranial neoplasm

•

Substance or withdrawal from substances

•

Alcohol-induced headache

•

Headache induced by food components and additives

•

Ergotamine-overuse headache

•

Triptan-overuse headache

•

Opioid-overuse headache

•

Caffeine withdrawal headache

•

Infection

–

Headache attributed to intracranial infection

–

Headache attributed to systemic infection

•

Disorders of homeostasis

•

Sleep apnea headache

–

Headache attributed to arterial hypertension

–

Headache attributed to hypothyroidism

•

Disorders of cranium, neck, eyes, ears, nose, sinuses, teeth, or other facial or cranial structures

•

Psychiatric disorders

Cranial neuralgias

•

Cranial neuralgia and central causes of facial pain

Other headaches

•

Other headache, cranial neuralgia, central or primary facial pain

Note: Full listing available on International Headache Society’s Web site www.ihs.org.

The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;24

(suppl 1):9-160. New version of classification (ICHD-III) is expected in late 2013.

EVALUATION OF HEADACHE: (1) Focused Exam: Vital signs can reveal hypotension, hypertension, or fever. General exam can reveal infectious signs such as sinus tenderness, erythematous tympanic membranes or throat, lymphadenopathy, and nuchal rigidity. Neurologic exam should focus on papilledema, visual acuity, eye movements, visual field testing, and weakness. (2) Need for Imaging: Consider for patients with red flags: waking up from sleep, change in headache quality or intensity, or associated neurologic symptoms or exam findings. (3) Appropriate Brain Imaging: MRI: preferred due to absence of radiation, level of detail, and evaluation of posterior fossa. CT: consider for acute increase of ICP (e.g., hemorrhage, mass lesion, or hydrocephalus), focal neurologic exam, unstable patient, or lack of MRI availability. (4) Vessel Imaging: CTA or MRA should be obtained for the evaluation of arterial dissection, cerebral hemorrhage, or cerebral infarction. If there is concern about a sinus venous, then CTV or MRV should be included. See Neuroimaging chapter for details. (5) Laboratory: CBC, ESR, serum or urine toxicology screens, thyroid function tests, and pregnancy screen. (6) Headache Diary: Extremely helpful in establishing a diagnosis and monitoring improvement. Advise patient to mark intensity on a pain scale, quality, location, timing of menses, triggers, and associated symptoms such as an aura, neurologic symptoms, photophobia, phonophobia, nausea, or vomiting. Teenagers should be encouraged to keep their own diaries. Many sample headache calendars are available on the Internet. Examples include www.childrenshospital .org/az/Site986/Documents/CHBMy_Headache_Diary.pdf (quite detailed), and the American Headache Society’s Committee for Headache Education has daily, weekly, and monthly versions of diaries on their website www.achenet .org/resources/headache_diaries. Many smart phone applications are available to create digital calendars, tables, and graphs, all of which can be electronically included in the medical record. (7) Other Evaluations: If there are signs of meningitis with acute presentation of headache with fever, neck pain, or nuchal rigidity; emergent LP should be done. In idiopathic intracranial hypertension (IIH), therapeutic LP may also need to be done urgently if there are concerns about vision loss; otherwise, it can be done as an outpatient. EEG is not usually recommended unless symptoms overlap with possibly ictal phenomenon such as paresthesias or weakness in one limb, stereotyped simple visual phenomenon, dysarthria, confusion, or decreased responsiveness. Headache can be an aura, ictal or postictal symptom.
PRIMARY HEADACHES
Migraine without Aura
Migraine without aura is the most frequently seen type of migraine.
SYMPTOMS: Often children notice a prodrome of symptoms different from what is considered an aura and can include mood changes, lethargy, yawning, cravings (often chocolate), increased thirst, diarrhea, and constipation prior to the onset of headache.7
DEFINITION: Table 14.3 includes the ICHD-II for children with migraine without aura.6 Migraines can occur frequently, but if >2×/wk, reconsider secondary headache.
Migraine with Aura
DEFINITION: Similar to migraine without aura; however, has preceding “typical aura”: an attack of a reversible focal neurologic symptom that usually develops gradually over 5 to 20 min and lasts <1 h.6 It is possible to have auras without headache.
TABLE 14.3 ICHD-II Criteria for Migraine without Aura

A.

At least five attacks fulfilling criteria B through D

B.

Headache attacks lasting 1-72 h

C.

Headache has at least two of the following characteristics:

1.

Unilateral location, which may be bilateral or frontotemporal (not occipital) in young children

2.

Pulsating or throbbing quality or varying with heartbeat

3.

Moderate or severe pain intensity

4.

Aggravation with, or needing to avoid routine physical activity (e.g., walking, climbing stairs)

D.

During the headache, at least one of the following:

1.

Nausea or vomiting

2.

Photophobia and phonophobia, which may be inferred from behavior in young children

E.

Not attributed to another disorder

AURA: Approximately 14% to 30% of children who have migraine report visual disturbances, distortions, or obscurations before or as the headache begins.8 Visual phenomena are the most common, but there are other types (Table 14.4).
OTHER CONSIDERATIONS: (1) Complicated visual perceptions or spreading paresthesias can also be seen in epilepsy, which should be considered. (2) Alice-in-Wonderland syndrome is rare and usually only seen in children. It includes bizarre visual hallucinations where objects can appear larger (macropsia), smaller (micropsia), distorted (metamorphopsia), or distant (teleopsia). (3) Retinal migraine, also called ocular or ophthalmic migraine, is seen more often in adolescents than in children. Sudden onset of monocular visual disturbances last <1 h and are associated with ipsilateral retro-orbital pain. Fundoscopic exam at the time of the headache can show constriction of retinal veins and arteries and retinal pallor. Evaluation of hypercoaguable states or embolic sources should be considered.
TABLE 14.4 Typical Auras Seen in Migraine with Aura

Visual

Negative scotoma (area of absent vision)

Scintillating scotoma (aka fortification scotoma, teichopsia)

Photopsia (flashing lights)

Visual field deficits—hemi- or quadrantanopsia

Alice-in-Wonderland syndrome: macropsia, micropsia, metamorphopsia, teleopsia

prosopagnosia (inability to recognize faces)

Sensory

Paresthesias

Dysesthesias (unpleasant sensation, including burning, wetness, itching, electric shock)

Perioral ± hand numbness (chiro-oral)

Motor

Hemiparesis, monoparesis

Language

Aphasia, dysphasia

Psychiatric

Confusion, amnesia

Auditory

Tinnitus, hypacusia (impaired hearing)

Basilar-Type Migraine
CLINICAL FEATURES: Complicated migraine with aura where the headache is usually occipital and preceded by brainstem-related symptoms with pallor, visual disturbances, vomiting, ataxia, vertigo, and clumsiness. Mean age of onset is 7 y. Motor weakness is not an associated symptom, and if present, consider hemiplegic migraine.
DIAGNOSIS: See Table 14.5 for ICHD-II diagnostic criteria.
DIFFERENTIAL DIAGNOSIS: Drug ingestion, arteriovenous malformations, congenital brain malformations such as Chiari and Dandy-Walker, or vertebral dissection or thrombosis.
Familial Hemiplegic Migraine (FHM)
DEFINITION: An AD genetic form of migraine with an aura that includes some degree of hemiparesis.
DIAGNOSIS: Hemiplegic migraine is characterized by aura of visual field deficits, hemisensory changes, hemiplegia, and aphasia that precedes the headache by 30 to 60 min, but can persist after headache resolution. Some FHM patients can have atypical severe attacks with signs of diffuse encephalopathy that can lead to coma.
FOUR SUBTYPES: Recent genetic discoveries have subdivided FHM into four subtypes. (1) FHM1 accounts for about 50% of cases and is caused by mutations in a gene coding for the P/Q-type calcium channel α subunit, CACNA1A. (2) FHM2 is the second most common caused by mutations in the Na+/K+-ATPase gene ATP1A2. (3) FHM3 is a rare subtype of FHM caused by mutations in a sodium channel α-subunit coding gene, SCNA1. (4) There are many sporadic and FHMs that do not fit into FHM 1-3 subtypes and are considered to be FHM4. It is suspected that new mutations will be found on chromosome 1q.
DIFFERENTIAL DIAGNOSIS: (1) FHM often first presents in adolescence, and intracranial hemorrhage, stroke, tumor, atriovenous malformation, acute disseminated encephalomyelitis (ADEM), and CNS infection must be excluded with urgent brain imaging. A hypercoagulable workup should be done. (2) Seizures are a possibility, and an EEG should also be considered. (3) Alternating hemiplegia of childhood, which is currently in the appendix of the ICHD-II, has been debated as a variant of hemiplegic migraine, a periodic syndrome in childhood, or a form of epilepsy. Some cases have been associated with an ATP1A2 gene mutation, which is also seen in FHM2. It presents <18 mo of age, with a hemiparesis that alternates with each attack and is a/w nystagmus, tonic spells, dystonic posturing, choreoathetoid movements, and autonomic disturbances.6 Acute attacks are usually aborted with sleep.
TABLE 14.5 ICHD-II Criteria for Basilar-Type Migraine

A.

>2 attacks fulfilling criteria B-D

B.

Aura consisting of >2 of the following fully reversible symptoms, but no motor weakness:

Dysarthria, vertigo, tinnitus, hypacusia, diplopia, ataxia, visual symptoms simultaneously in both temporal and nasal fields of both eyes, decreased level of consciousness, simultaneously bilateral paresthesias

C.

At least one of the following:

1.

At least one aura symptom develops gradually over ≥5 min and/or different aura symptoms occur in succession over ≥5 min

2.

Each aura symptom lasts ≥5 and ≥60 min

D.

Headache must fulfill criteria for migraine without aura (see Table 14-3) and begin during the aura or follow aura within 60 min

E.

Not attributed to another disorder

Childhood Periodic Syndromes
There are several syndromes felt to be precursors to migraines which in the past used to be referred to “migraine variants.” Often there will be a strong family history of migraines, motion sickness, or periodic syndromes. The ICHD-II includes three conditions: benign paroxysmal vertigo, cyclic vomiting syndrome (CVS), and abdominal migraine (AM). A fourth syndrome, paroxysmal torticollis, has recently been linked to migraine with molecular genetic testing and is included in the appendix of ICHD-II. With the associated symptoms related to these syndromes, it is important to exclude other pathologic diagnoses (Table 14.6).
Tension-Type Headaches (TTH)
DEFINITION: The most prevalent type of primary headache. Dx can be made after >10 headaches with associated symptoms occurring over at least 3 mo. (1) It is differentiated by the ICHD-II as infrequent (<1 d/mo on average), frequent (1 to 15×/mo), and chronic (≥15×/mo). (2) The headache pain usually has a bilateral location, pressing or tightening quality, and mild to moderate intensity. (3) No associated aura, nausea, or vomiting, but occasionally photophobia or phonophobia. (4) New daily persistent headache is a TTH where patients with no prior headache history often identify exactly when their headache started and headaches continue daily for >3 mo. No underlying secondary cause should be identified.
Chronic Daily Headache
Chronic daily headache is not a diagnosis but rather a collective term and includes chronic TTH, new daily persistent headache, chronic migraine, and hemicrania continua. (1) Chronic migraine must be present for ≥15 d/mo for 3 mo to meet criteria. (2) Hemicrania continua is characterized by daily continuous unilateral pain with conjunctival injection and/or lacrimation, nasal congestion and/or rhinorrhea, or ptosis and/or miosis which persists for >3 mo. Treatment with indomethacin always leads to resolution.
OTHER CONSIDERATIONS: In all types of chronic headaches, it is important to rule out secondary causes and, in particular, medication or analgesic overuse.
SECONDARY HEADACHES
Idiopathic Intracranial Hypertension
Idiopathic intracranial hypertension, formerly known as pseudotumor cerebri, is an important entity to diagnose in the setting of chronic headache. The etiology is not well understood, but is thought to be an impaired reabsorption of CSF.
CLINICAL FEATURES: The headache pain is usually of diffuse and pounding quality that worsens with Valsalva or recumbent position and can be progressive in severity. Symptoms can include diplopia, transient visual obscurations, and pulsatile tinnitus.
TABLE 14.6 Periodic Syndromes in Childhood

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Jun 20, 2016 | Posted by in NEUROLOGY | Comments Off on Headache and Pain Syndromes

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Cyclic Vomiting

Abdominal Migraine

Benign Paroxysmal Vertigo of Childhood

Benign Paroxysmal Torticollis

Description

Episodic attacks of N/V, more common at night or early morning, usually triggered by stress or illness, usually lasting 24-48 h, occurring every 2-4 wk

Episodic attacks of acute midline, moderate to severe intensity abdominal pain assoc. with pallor, flushing, N/V without diarrhea or constipation, lasting 1-72 h

Brief episodes of sudden-onset unsteadiness ± fear, nystagmus, dizziness, clumsiness, nausea, or unilateral throbbing H/A

Episodes of sudden-onset head tilt secondary to cervical dystonia ± vomiting and ataxia that can last hours to days

Age