case study
CHAPTER
19 Headache H eadach e is a subjective fee ling of pain cau sed by a variety of intracranial and extracrania l factors. It is one of the most com mon compla ints in adults and children, with most h eadaches being self- treated u s ing an over-the-counter (OTC) analgesic. Most head aches are acute and self-limited and are not li fe threatening. One in I 0 people will have a migraine, and fewer than I % of headaches are caused by serious intracranial disease. The goals for the practitioner in evaluating a head ach e are to: • Identify life- threatening causes of headache. • Diagnose treatable disease associated with
some headaches. • Provide symptom relief.
To accomplish t h ese goals, the practitioner needs to conduct a careful history and physical examination even t hough physical find ings will be n ormal fo r the majority ofpatients who report a h eadache.
Pain fro m headache arises from s timul a tion of pain-sensitive structures of the head and brain caused by traction, inflammation, vascu lar dilation, muscle contraction, or dys regulation of ascendin g brainstem serotoner gic systems (Fig. 19. 1 ) . Headaches can be categori z ed as p rimary or secondary. Pri mary headaches are characterized by the ab sen ce of structura l pathology or systemic disease ; they account for more than 90% of all headaches. Secondary headaches are at tributed to an underlying disorder. Primary headaches are of four major types: migraine , tension-type headach e (lTH), c luster headache or othe r trigeminal autonom ic cephala lgias, and other primary headaches. Gen erall y, pain aris ing from di sordered function , damage, or infla mmation of s tructures located anterior to, and above, the tentorium (the fold of dura mater separating the cerebellum from the cerebrum) is felt in the front of the h ead,
but pain felt in the back o f the head ari ses from structures located below the tentoriurn Extracranial structures that are sensitiv~ to pain include the skin , s ca lp, blood ves. sels, facia l muscles, eyes, ears, teeth, nasal cavity, mucous membranes of the mouth and pharynx, and the temporomandibular joint (TMJ). Brain tissue itself is not sensitive to pain, but sensitive structures of the brain include the blood vesse ls , sensory nerves
I
and g angli a. Cranial structures project pain to the sur
face clos e to the sou rce of pain. Pain from extracranial structures is u s ually felt in the immediate region affected (Fig . 19.2). The head is innervated extensively from the first branch of the trigeminal nerve (cranial nerve [CN] V). It has significant anatomical con nections to the upper cervical roots C I to C3, which supply the posterior fossa and neck structures.
Headache has been divided by symptom ato logy into types using the diagnostic criteria revis ed in 20 13 by the Headache C lassifica tion Committee of the International Headache Society ( https://www.ichd-3 .org).
Of the types class ified, only a few are com mon (Box 19. l ) . Headaches c an also be cate goriz ed into acute (new onset), s ubacute, or chronic. Acute headaches warrant close atten· tion . The headache a ssociated with subarach n oid hemorrhage (SAH) has an intense sudden onset. Subacute headaches are lo calized head· ach es preceding neurologic findings, often caus ed by a vascu lar di sorder or a space-occu· pying lesion. Headaches of greatest concern to c linicians are those that are persistent, severe, sudden in ons et, and different from the pa· tient ' s usual headache. The major clue to a s sess ment of chronic headache is a change in t h e u s ual pattern of occurrence. Clinicians can ask patients to keep a headache diary that
254
Chapter 19 • Headache
Hysteria Intrinsic pain tract disorder
Mechanical pressure and distortion Traction
Vasodilation (cerebral)
Vasodilation (extracerebral)
Muscle contraction Inflammation
FIGURE 19.1 Mechanisms of crania l pain. (From Noble J: Textbook of primary care medicine, ed. 3 , St. Louis, 2001, Mosby.)
Chapter 19 • Headache
. . .
C1 ... C2
C3 .· ·.. ..·. _.,.. . ..
.... . .. . .. .·
FIGURE 19.2 Sensory pathways for cranial pain.
includes notes on timing, frequency, and as sociation with sleep, diet, emotional episodes, and other potential contributing factors.
DIAGNOSTIC REASONING: FOCUSED HISTORY
What clues indicate this is a potentially serious, Life-threatening headache?
First assess whether the patient is fully ori ented before proceeding with further history. The Mini-Cog is a three-item recall test for memory and a s imple scored clock drawing that can be administered in about I 0 minutes (https://www.alz.org/ documents_ custom/ minicog.pdf). If the patient shows a mental status deficit, immediate evaluation with a head computed tomography (CT) scan and emergency treatment may be indicated.
Key Questions • How did the headache begin? • What is your age? • Have you had this type of headache before? • On a scale from 0 (no pain) to I 0 (worst
pain ever), how severe is the pain? • Do you have a history of recent trauma to
the bead?
Box 19.1 First Level of the International Classificati of Headache Disorders on
PRIMARY HEADACHES 1. Migraine 2. Tension -type headache 3. Trigeminal autonomic encephalalgias 4 . Other pri mary headaches
SECONDARY HEADACHES 1 . Headache attributed to head or neck
trauma 2 . Headache attributed to cranial or cervical
vascular disorder 3. Headache attributed to nonvascular
intracranial disorder 4. Headache attributed to su bst ance or its
withdrawal 5. Headac he attributed to i n fec tion 6 . Headache attributed to disorder of
homeostasis 7. Headache or facial pain attributed to dis
order of cranium, neck, eyes, ears, nose, sinuses, teeth , mouth, or o ther facial or cranial structures
8. Headac he attributed to psych iatric disorder
CRANIAL NEURALGIAS, CENTRAL AND PRIMARY FACIAL PAIN, AND OTHER HEADACHES 1. Painful cranial neuralgias and other facial
pains 2. Other headache disorders
Data from the Headache Classification Committee of the Internationa l Headache Society: The International Clas sification of Headache Disorders, ed. 3 , Cephalalgia 33:629-808, 2 013.
• Did you lose con scious ness? • Do you notice other symptoms associated
with the hea dache pain? • Do you have any chronic health problems?
Onset and Severity Sudde n onset of a severe headache, without a history of chronic headache a nd with al· tere d mental status, suggests an intracerebral hemorrhage (ICH) secondary to a ruptured ane urysm or vascular anomaly. Severity of headache is a very subjective m eas ure and can sometimes be difficult to interpret. Headache of an I C H witho ut a history of trauma is rare in chi ldren and adolescents,
0
t the prevalence increases with age, espe b~ lly in people older than 50 years with ciahistory of uncontrolled hypertension or :hose being treated with anticoagulation
therapy. onset of sudden severe headache with
neurologic si~s is an emergency; the patient eeds immediate emergency treatment.
Subarachnoid hemorrhage is often precipi tated by physical activity and is described as the " worst headache ever. " Patients also report a stiff neck and may have a transient toss of consciousness, nausea and vomiting, pbotophobia, pupillary dilation, and seizure. Some patients, who have a leaking aneurysm, may report a headache for several days which will subsequently worsen with neurologic findings.
If SAH is suspected, the patient needs transport to an emergency center for a CT scan and possible surgical intervention be cause early diagnosis and treatment improve prognosis.
History of Trauma Trauma to the head may cause subdural or epidural bleeding. Falls are a common cause of subdural bleeding in older adults. The pa tient may have a brief loss of consciousness followed by a period of lucidity that can last for minutes to days, with subsequent relapse and appearance of neurologic signs. In the older adults, especially after a fall , altered mental status may begin to manifest after I month. This delayed presentation results from the low-pressure leakage of blood from subdural veins. Epidural hematomas are more common in young adults and are often associ ated with skull fracture. The blood vessels that are ruptured in epidural bleeding are the middle meningeal artery or its dural branches. Because of the high arterial pressure, the mental status changes associated with epidu ral bleeds can be dramatic. A patient can go from a period of lucidity to a comatose state over a few hours.
Minor h e ad trauma may result in headache because of s oft tissue or extracranial injury. Pain is often localized to the site of injury and is self-limiting. Anyone who has experienced head trauma should be carefully observed for
Chapter 19 • Headache
at least 24 hours for changing neurologic signs.
Associated Symptoms The entry of infectious organisms, chemjcal agents, and drugs into the subarachnoid space causes inflammation of meningeal structures and associated blood vessels, resulting in a headache. Headache associated with infec tion presents with fever and possibly menin gismus (stiff neck), which can indicate men ingitis or encephalitis. Unilateral upper or lower extremity weakness with loss of man ual dexterity is seen in children with hemi plegic migraine.
An ICH often presents as a sudden and severe " thunderclap" headache associated with confusion, vomiting, lethargy, and focal neu rologic signs; it is caused by a ruptured vascu lar anomaly or an aneurysm. Drowsiness or confusion can be produced by increased intra crarual pressure (ICP) secondary to meningi tis or metabolic disorders.
Brain tumors in children, especially young children, are difficult to diagnose because of the child' s inability to describe headache or diplopia until about 4 years of age. Signs are vague, and the developing skull in an infant may accommodate a pathological condition for some time. However, head ache can be the initial manifestation of brain tumor if followed quickly by neuro logic signs such as vomiting, recurrent morning headaches, reflex asymmetry, and papilledema.
Presence of Chronic Disease Individuals with AIDS are at increa sed risk for cryptococcal meningitis, encephalitis, in trace rebral abscess, and generalized sepsis. Patients being treated with anticoagulation therapy or older adults are at increased risk for headache from a serious cause such as an ICH or acute glaucoma.
Acute glaucoma caused by anticoagula tion, a rare condition, may also occur with retinal detachment.
Headaches secondary to metabolic dis orders can be th e result of hyponatre mia, hypercalcemia, uremia, hypoglycemia , and hypercapnia.
I
Chapter 19 • Headache..
After determining that a headache is not serious, how can I narrow down the causes?
Key Questions • What does it feel like? • Where does it hurt? • What makes it worse? • How long h ave you had this headache? • Can you tell when a headache is developing?
Characteristics of the Pain A moderately intense, constant throbbing headache is associated with dilation of the cervical arteries. Severe pain associated with nausea, vomiting, and altered mental status indicates an expanding lesion such as a tumor, hematoma, edema, or enlargement of the ven tricles secondary to hydrocephalus.
Migraine headache pain is caused by the production of various substances on dilated arteries that sensitize those arteries to pain. The patient usually has a high serum sero tonin level early in the migraine headache. The pain is steady or throbbing and is usually limited to the same side. Migraine headaches are thought to result from an initial phase of intracranial or extracranial vasoconstriction followed by a longer interval of vasodilation. Frequently, the headache takes 3 to 4 hours to reach peak pain levels.
C luster headaches (uncommon in chil dren) are the result of an unknown vascular change that occurs within a period of 5 minutes. The serotonin level is unchanged in cluster headaches. The pain of cluster headaches is described as exp losive and severe.
Tension (muscle contraction) headaches usually occur at school or work, generally when under a significant amount of pressure or stress, and often disappear on weekends and vacations and during periods of relaxation.
Location Pain secondary to trauma or inflammation is perceived as near the site of insult such as the occipital area, nape of neck, bifronta l area, or generalized to the head. Noxious stimulation from any type of disease of the eye, ear, nose, or paranasal sinuses may spread to cau se pain in the head.
Adults des~ribe ~Hs as a "hatband" . tribution of pam; children describe a g dii. ized headache or discomfort. Most p:~I. describe a cluster headache as sudden
1~ pain beginning over one eye and sprsev~e rapidly to the same side of the face. eadll)g
Orbital pain is seen with increased intra l~ pr~ss~e: Pe~orb~tal pain .may be pr Ocu. with s1nus1t1s, m1grame, or tngerninaJ n eseni gia, or it may be a sign of ocular diseaseeura~ pain is located in the frontotemporal or ~IMJ ral regions and may be unilateral or bilat Jl<). Nonpulsatile headache in the occipital eraJ. paracervical regions is often caused by 3lld traction of muscles of the head and neck. eon.
Aggravating Factors Triggers are present in many patients with mi. graine and include sound, odor, and estrogen fluctuations associated with the menstrual cy. cle. The most common food triggers are red wine, chocolate, and ripe cheese, which are foods high in tyramine or tryptophan. Migraine is usually worse with activity, but patients with TTHs are able to continue their usual routines. Stress can trigger any type ofprimary headache and must be considered a comorbid condition.
Duration Most TTHs last less than 24 hours, a similar duration as migraine headaches. Cluster head aches usually last less than 3 hours and tend to occur in cycles. A s ubacute headache per sists for days and weeks.
Aura and Prodrome In migraine with aura, visual symptoms pre dominate and range from nonspecific blurring to scintillating scotomata with zigzag patterns, diir lopia, and stars or flashes. Aura often precedes the onset or is simultaneous with headache. Prodromal symptoms include fatigue, depressed or euphoric mood, increased or decreased air p e tite, constipation or diarrhea, and yawning.
What does the chronicity ofpain s uggest?
Key Questions • How often do you get a headache? • Can you descr ibe any p attern to the
headache?
dong does the headache last? J-{O\\ .
• }-lave you bad this kind of headache before? • you drink alcohol? Do you take any
0• v . ? rnedications.
frequency . rient with a persistent headache for more
Apa 3 months may demonstrate physical ~~·nos such as papilledema, bilateral or uni flO ~{'CN VI (abducens) palsies, gait or bal tate . . f h 1e disturbances, or spast1c1ty o t e ower ::~reinities. As a rule, in the absence of such yrnptoms, a recurrent headache of more than
; months' duration is rarely related to struc tural or systemic findings. If a headache has bee" present continuously for more than
4 weeks, without accompanying neurologic signs or symptoms, it is most likely psycho !!enic in origin, especially if coupled with ~rolonged school or work absences, increased stress, and depression. This may be the case in a very small number of patients who present with complaints of headache.
Pattern and Duration of Headache Headaches that occur throughout the day sug gest a tension type. Sinus headaches occur after arising and worsen as the day pro gresses, especially when bending forward, and are Jess painful in the evening. Headaches associated with severe hypertension are not common and occur only with a diastolic blood pressure reading of 130 mm Hg or greater. These headaches are occipital, worse on arising, and lessen as the day progresses. Meningeal inflammation produces a pain that
Chapter 19 • Headache
fluctuates throughout the day and night with no clear pattern. Migraine pain is episodic, occurring from several times a week to once a year. Cluster headache pain demonstrates a pattern of attacks that occur daily for several weeks with long periods of remission. The pain is short, often lasting less than 1 hour, but is intense.
Prior History of Headache Headaches can be described as acute (new onset), subacute, or chronic. Acute-onset headaches must be evaluated for organic causes. Subacute and chronic headaches are usually caused by vascular inflammation or muscle tension. Chronic headaches are usu ally described as dull, bilateral, or bandlike. Chronic daily headaches may be mixed, pro duced by a combination of vascular and muscular causes.
Organic lesions may initially produce pain that is intermitte nt, but as the lesion pro gresses, the duration and frequency of the attack increase.
Psychogenic headache pain is daily, con stant, diffuse, and difficult to describe.
Age of Patient at First Onset The age of onset of migraines can be as early as 5 years old. Usually migraine headaches begin at ages 10 to 30 years. New onset of migraine headaches in adults older than age 50 years is unusual. Tension headaches have a usual first-onset age of 8 to 12 years. Cluster headaches have a usual first-onset age of 20 to 40 years.
2EVIDENCE-BASED PRACTICE Association o.f· Migraine He<1d<1che with Cardiovascular Disease
A meta-ana lysis of studies and reviews pub lished si nce 2009 was conducted to evaluate the assoc iation between migraine and cardio vascular disease, including stroke, myocardial infarction, and death caused by cardiovascular disease.
Migrai ne is associated with a twofold increase in risk of ischemic stroke only among people who have migraine with aura. There is a higher
risk among women compared with men, and risk was further magnified for people with migraine who were younger than 45 years o ld, smokers, and women who u sed oral contraceptives. There was no association between a migraine and myo ca rdial infarction o r death ca used by cardiovas cu l ar disease. Too few studies are available to reliably evaluate the impact of modifying factors such as migraine aura on these associations.
Reference: Schurks e t al, 2009.
Chapter 19 • Headache
2 EVIDENCE- BASED PRACTICE Pret•t1lt.!ll<..'t.~ t~f" //et1tlt1clle.\· t1111/ 1ll~~rt1i11e.\ in ( ,lliltlre11 11111/ A. tlo/e.,·<..·e111.,·
Migraine and headache are global disabling conditions causing impaired quality of life not only in adults but also in children and adoles cents. This review covers epidemiological stud ies on migraine and headache in c hildren and adolescents published in the past 25 years. A
Reference: Wober-Bingol , 20 13.
Lifestyle Habits and Medications Alcohol is an important trigger for migraine and cluster h eadaches, althou gh it may relieve a ITH. Smoking and secondhand smoke ex posure can trigger headaches. Headaches may be a side effect of medications the patient 1s taking.
What other symptoms d oes the patient have?
Key Questions • Do you have any nausea or vomiting? • Do you notice any vision changes? • Does light bother you? • Are you dizzy?
Nausea and Vomiting Whereas nausea and abdominal pain are more common in children with migraines, nausea and vomiting are more common in adults. Vomiting can be a sign of increased I C P. Headaches from tumors in the midline, cere bellar, and ventricular areas of the cranium obstruct the normal flow of the cerebrospinal fluid (CSF), producing hydroce phalus, head ache, and early morning vomiting that usu ally occurs without nausea.
Vision Changes Migraine headaches may have an aura that precedes them. A frequently reported visua l aura is a scintillating scotoma, or twinkli n g spots of brightly colored lights. ln children, visual sci ntillation is the most common aura of migraine and often limited to one eye.
C luster headaches are associated with ipsi lateral conjunctival injection, lacrimation, and edema of the eyelid.
total o f 64 cross- sect io nal studies were Id tied, published in 32 different countries.e;ti. estimated overall mean prevalence of head he I w as 5 4.4% (95% confidence interval ~~e 4 3.1-65.8), and the overall mean prevalenc I, migraine was 9 . 1 % (95% Cl , 7 . 1- 11.1). eOf
Photophobia Photophobia is often present with milh'->·
• C>' qJnc h eadaches but 1s not present w ith tensi headaches. Patients with meningitis often ~ port photophobia.
Dizziness Approximately one-third of patients with migraine headaches experience vertigo. The vertigo may appear as an aura, occur during the headache, or occur separately. A young child with vertigo may appear startled or have sudden ataxia.
What do the alleviating and aggravating factors suggest?
Key Questions • Does anything make the headache better? • Does anything make the headache worse?
Alleviating Factors Patients with meningeal irritation obtain par tial r e lief from being recumbent and lying quie tly. Headaches that respond to mild anal gesics are more like ly to be tension headaches. Children obtain complete reli ef after a brief period of rest with a migraine headache, aJ. thoug h rest does not affect a te ns ion headache in children. Some adults experie nce migraine headache r e lief with s leep or rest, particularly in a dark, quiet environment.
Aggravating Factors Increased headache with s neezing or cough· ing may indicate a benign headac he, or it may b e caused by a lesion at the level of the fora men magnum long before clinicaJ signs are
resenL Migraine.heada~bes are made worse P.th exertion. Patients with cluster headaches Wl e worse pain when lying down. Headaches hav . h 1 .tb l are much worse m t e ear y morning and . aprove on arising may indicate a tumor. Be :gn exertiona! headaches_ can. occur du~g coitus. Trigemmal neuralgia pain can be tng
red by s timulation of tbe affected nerve, geoduced by rubbing the face or chewing.pr
What does family his tory indicate?
KeY Questions • Does anyone e lse in the family have head
aches?
family History Tension-type headaches have no family his tory. Migraines have a pos itive family history.
/s there anything else that would help narrow the cause o r causes?
Key Questions Have you been ill recently? Are you taking any medications or vitamins? Could you have been exposed to carbon monoxide (CO)? Do you have working CO and smoke alarms in your home?
Recent Health History Any substance introduced iatrogenically into the ventricular and lumbar fluid s paces can lead to chemical meningitis . Radiographic contrast media, antibiotics, and steroids can cause headache. Lumbar puncture (LP) can cause a severe headach e in 25% of patients. A recent history of epidural anesthesia during child birth or o ther e pidural injections is a ls o associated with headaches . The headache is eased by ly ing down and aggravated by sit ting or standing. C hronic infection, including otitis media , mastoiditis, s inu sitis, dental or pulmonary infection, c ardiovasc ular les ions with shunting, or endocarditis, pre dis pos e s to developme nt of a brain abscess. Hal f of all brain abscesses occur in children with cya notic congenital h eart disease. Penetrating sku ll fractures can also be a portal of e ntry for bacteria a nd contribute to the occurrence of
Chapter19 • Headache
brain abscess. Melanomas may metastasize years after excision and may first be indicated by neurologic changes.
History of Medications Outdated tetracycline use can cause pseudotu mor cerebri ( increased ICP without an intra.cra nial mass or hydrocephalus), as can an exces s ive intake of vitamin A and substances found in some topical acne preparations. Oral cootra cepti ves and overuse of over-the-counter analgesics can cause headache. N-Metbyl-o aspartic acid or N-methyl-o-aspartate (NMDA) receptor antagonist drugs (i.e., memantine, amantadine, phencyclidine [PCP]), used to treat confusion in Alzheimer disease, as an anesthesia, and for the di ssociative anesthesia or eupboriant properties as a recreational drug, can cause headaches.
Withdrawal from certain substances, such as caffeine or nitrates, can also produce headache.
Exposures C arbon monoxide is a tasteless and odorless gas that contributes to many deaths each year.
Exposure to CO may cause a severe, throb bing, generalized headache. Hemoglobin val ues less than l 0 g/dL may cause headache as a result of hypoxia. Home furnaces, hot water beaters, or gas dryers may cause CO exposure because of the incomplete bre akdown of fossil fuel that does not have enou gh o xygen to produce carbon dioxide. Poor ventilation is u sually the cause.
Assess occupational exposure to other tox ins through an occupational hi story. A faulty kerosen e or gas heater may cause headaches that occur during winter months.
DIAGNOSTIC REASONING: FOCUSED PHYSICAL EXAMINATION
Observe the Patient .Assess leve l of alertness and orientation to p e rs on, place, a nd time. Any patient who reports headache and exhibits an ataxic gait, uncoordinated moveme nts, or reduced men ta l a le rtness s hould be immediate ly trans ported to an emergency center for neurologic evaluation.
Chapter 19 • Headache
A patient w h o appears ill or toxic is a sus pect for meningitis. The patient is usually ly ing down with the li g hts off (photoph obia) and may report c hill s . Most toddlers cannot communicate the c h aracteristics of a head ach e but instead become irritable a nd c ranky and rub their eyes a nd head.
Muscle spasm m ay cause tilting of the head or lifting of the s h ou lde r when t h ere is a p osterior fossa tumor, cervica l spine disease, or whiplash injury. Ptosis of the eyelid may accompany a c luster h eadac he or brain tumor. Blink ing a nd sq uinting of the eyes indicate photophobia.
Take Vital Signs and Obtain Growth Parameters Take temperature, blood pressure, and pulse m easure m e nts. Fever may be the on ly sign of infection. Bradycardia and narrowing of pu lse pressure are signs of increased I CP. In children, if the plotted height and weight chart is signifi cantly below average, consider a hypotha lam ic neoplasm. Plot h ead ci rcumference to assess for normal s kull growth. Macrocephaly may indicate hydrocephalus or a brain tumo r.
Palpate and Percuss the Skull Pa lpate for symmetry of contour, tenderness, and lesions on the scaJp, face, and neck. Pal pate the temporal arteries for quality of pulse and tenderness. Focal tenderness and in.dura tion are seen in TTHs. Tenderness over nodular temporal arteries is a sign of temporal arteritis .
Brain abscesses cau se pain by localized traction and produce tenderness on skull per c ussion over the area involved.
Auscultate the Cranium Intracranial arteriovenous malformations may mimic migraine. Auscultate the orbit a nd skull to evaluate for cranial bruits.
Inspect the Ears, Eyes, Nose, Mouth, and Temporomandibular Joint A thorough examination of the face, head, and neck structures is n eeded to detect organic disease. Examin e the ears for signs of infection.
Anisocoria is when right a nd left pupils are not eq ual. It can b e benign but is assoc i ated with eye infection or injury or increased
intracraniaJ pres~ure. I psi lateral lacrirna . ptosis, and pup1Jlary co n s triction ar h0ri, with c luster headache. Test extraocula e Seen m e nt ( EOM) in a ll fields of gaz e. If ar~~ve. canno t look compl etely to the right or 1e11t left (lateral gaze), suspect a CN VJ the possib_Jy the resu~t of inc~eased IC P. If l~lsy, a r e painful, consider o pti c neuritis. Ms
Observe nasal mucosa fo r redness swelling. Rhinorrhea a nd congestion are alld with sin u s headaches. Observe teeth and seen mucosa because upper molar disease and .:1
dentiti o n can cause h eadac h e. Tapping on ~ teeth or biting down on a tongue blade c e li c it pain from sinusitis. an
Temporomandibular joint instability can cause headache pain. See Chapter 15 for discussion of examination techniques. a
En larged pupils seen during a headache ind icat e migraine; however, if they outlast a h eadach e , then o rga ni c disease s hould be suspected.
Upper motor neuron facial weakness may be present in hemiplegic migraine.
Perform Ophthalmoscopy On ophthalmoscopic examination, note con tour of the o ptic disc and clarity of margins. Note the disc for papilledema and inspect vascu lature for hemorrhage or exudate, venous pulsations, and arterial spasm.
Papi IIedema is often cau sed by an expand· ing intracranial mass a nd increased ICP. Optic disc atrophy s u ggests a chronically increased ICP or a lesion in the optic chiasm.
M eningitis does not produce fundus changes. Retinal h e morrhage in children may indicate abuse.
Assess Cranial Nerve Function A complete assessment of CN function may provide evid e nce for more seriou s causes of h eadac h es secondary to inflammation, t:rac· tion, or metabo li c imbalance. • CN I : Assess for smeJI. The sense of
sme ll may b e lost when the olfactory nerve i s damaged by head injury or by a tumor in the v ici ni ty of the olfactory groove. Herpes s implex encephalitis can l ead to a destru c tion of the olfactory cortex or olfactory nerve.
....
cN JI: Check visual acuity. Rarely does ' r vision contribute to a headache. Poor ~on may contribute to eye pain, but chil ;10 equate this with b~adache. Double
. · 0 0
may be the presenting ocular symp ~~
of increased ICP caused by a un1- .
01w . ~ lateral CN VI palsy or a postenor .1.ossa
Jesion. cNs JI/, JV, and VI: Check visual fields. Headaches as a result of pituitary tumors are usually associated with defects in the peripheral vision. Unilateral or hom onymous hemianopsia (a loss of the same half of the visual field of both eyes) can occur with migraines or brain rumor headaches when the tumor is in the occipital lobes or adjacent to the visual pathways. A half-field defect is seen with parietal lobe tumor. CN III palsy can cause an enlargement of the pupil from compression of the nerve by an expanding lesion. The dilated pu pil is always on the side of the expand ing les ion. CN VI palsy (inability to move eyes in a lateral direction) may be found with acute hydrocephalus or cerebral edema. Nystagmus suggests a brainstem or cerebellar lesion and is usu ally ipsilateral. Lateral gaze nystagmus is also present with an elevated blood alcohol level. Vertical and rotatory nys tagmus suggests central posterior fossa abnormality.
• CN V: Test jaw strength, pain, and touch sensation to face. Trigeminal neuralgia pain can be triggered by stimulation of the affected nerve.
• CN VII: A s k the patient to frown , raise eyebrows, show teeth, close eyes against res istance, and puff out cheeks. Test taste on the anteritor two thirds of the tongue for sweet and salt discrimination . Sali vary and lacrimal glands are innervated by CN VII.
• CN VJ/I : Test hearing acuity. Unilateral deafness should be investigated to rule out acoustic neurorna.
• CNs IX a nd X: Observe swallowing and uvula rise.
• CN XI: Test trapezius strength and stemo cleidomastoid strength against resistance.
Chapter 19 • Headache
• CN XII: Test tongue strength. An intracra nial vascular event may cause a hemiple gia or hemiparesis that can be assessed by observing the protruded tongue drift later ally or by the inability to hold position against resistance.
Examine the Neck Ask the patient to perform full range of motion (ROM) of the neck to observe for stiffness or difficulty with movement, which may indicate muscle tension or meningismus. If there is a history of trauma involved, the patient should only perform an active ROM examination. A passive ROM may worsen the acute injury, and the patient's neurologic function in the presence of a neck injury and should be avoided.
Test for Meningismus Normally, the chin can be flexed passively to touch the chest. If neck stiffness (nuchal ri gidity) is present, this maneuver is not possi ble. With the patient supine, attempts to flex the neck cause involuntary hip flexion, and the hips rise (Brudzinski sign). Attempts to extend the knee joint when the hip joint is flexed may cause the other limb to flex at the hip (Kemig sign).
Assess Motor Strength and Coordination of Extremities Asymmetrical increase in muscle tone on the affected side, contralateral to the hemisphere lesion, suggests a cerebral lesion.
Patients who exhibit forearm drift with arms extended and eyes closed may have a motor neuron or cerebellar disturbance with a n expanding intracranial lesion.
Test Balance and Gait Midline cerebellar abnormalities cause marked ataxia. The patient has difficulty standing on the ipsilateral leg and has a tendency to fall or stumble toward the side of the lesion. The gait is also wide-based and halting, and the patient turns with jerky movements. Minimal disturbance is ob served when the patient hops on either foot or stands tandem (one foot behind the other).
Chapter 19 • Headache
Assess Deep Tendon Reflexes Note asymmetry, absence of reflexes, or hy peractive responses. Increase in, or asymme try of, reflexes is seen with cerebral lesions. The plantar or Babinski response is often present with cerebral le s ions.
Have Children Draw Pictures of Their Headaches Having a chi ld draw a headache is an inexpen sive and accurate way to help diagnosis head aches. The child is given a plain piece of paper and asked to draw a picture of how his or her headache felt before any history of the headache is taken. These drawings help to diagnose mi graine headaches. Drawings for migraines in clude visual images such as flashing. Showing the lights being turned off, a dark room, or a blanket over the head depicts photophobia. The need to lie down is also associated with migraine headache. Images for nonmigraine headaches show pictures of pounding or tight headbands. Even very young children (age 4 years) are able to draw stick figures with significant detail.
LABORATORY AND DIAGNOSTIC STUDIES
Complete Blood Count A complete blood count with differential is obtained to detect major blood dyscrasias. Hypoxia secondary to severe anemia can cause headache. In bacterial meningitis the polymorphonuclear leukocytes will be high with a left shift.
Blood Cultures Blood c ultures should be drawn in a patient who has a fever, headache, nuchal rigidity, and altered mental status.
Computed Tomography Scan Computed tomography scanning is a noninva sive diagnostic tool used to detect intracranial disease and s h ou ld be done with sudden-onset severe headaches that occur every day or head ache associated with abnormal neurologic s igns.
Magnetic Resonance Imaging A magnetic resonance image changes over time as red blood cells lyse and hemoglobin
degrades . It is the first imaging choice for a brain abscess.
Lumbar Puncture Lumbar puncture can meas ure CSF pressure directly and can be analyz ed for normal Val. ues of components that are a ltered by disease such as lymphocytes, glucos e , protein, and the presence of bacteria. An LP is performed when a central nervous system (CNS) infec. tion i s suspected but is contraindicated if there is suspic ion of increased ICP.
Erythrocyte Sedimentation Rate E rythrocyte sedimentation rate (ESR) is a nonspecific test that is elevated in the pres ence of inflammation. An ESR should be performed when temporal arteritis is suspected.
Skull Radiograph A radiograph of the skull is u seful in posttrau. matic headache. Specific vie w s must be ob tained to better observe intracranial structures such a s the pituitary gland or paranasal sinuses.
DIFFERENTIAL DIAGNOS I S Primary Headaches
Tension-type h eadache (muscle)
Ten s ion-type headache is the mos t common type of headache in adults and occurs most often in women. The exact mechanism of tension headache i s uncertain but is related to sustained muscle contraction. Tension headache produces a bilateral pain, general or localiz ed, often described a s a frontotem· poral bandlike distribution. The discomfort is described as a m il d to mode rate, non· throbbing pain, tightness, or pressure with a gradua l onset. It may last for hours or days, and recurrences may extend over weeks or months. It is associated w i th hunger, depres· s ion, or stress.
Migraine without aura (common)
About 20% of adults experience migraines, and episodes are not uncommon in children as young as 5 years old. The headache is unilateral
Chapter 19 • Headache
d throbbing and most often accompanied by an ea photophobia, and exacerbation from oaUS ·ca'I activity. The headache is usuaJly fron phys1 . . . .
r periorb1tal. The onset ts rapid, and cre wI o h M. .do is within ours. igrames may recur seen ft M. . h dda
1 ·ly weekly, or less o en. 1grame ea aches
m' ost commonly found in adults 25 to are 34 years of age and are rare during pregnancy. Chronic migraine is present when attacks occur more than 15 days in a month.
Migraine with aura (classic)
Neurologic signs that indicate cortical or brainstem involvement precede classic mi Q11line headaches. Bright lights, noise, or ten ;ion may precipitate headaches. Auras may include v isual disturbances (e.g., scintillating scotoma: a pattern of twinkling colored lights), ascending paresthesias or numbness, weak ness, and aphasia. The pain may be associated with photophobia, phonophobia (noise sensi tivity), nausea, and vomiting. Aura usually precedes but may accompany a headache or occur without headache.
Mixed headache
Mixed headaches are a combination of mus cular contraction and vascular dysfunction. The headache is experienced as a throbbing, constant pain during waking hours with symptoms of tightness, pressure, and muscle contraction. A family history of migraine is common.
Cluster headache
Cluster headaches are of vascular origin and are less common than migraines. The onset is abrupt, often during the night, and the se verity inc reases steadily. The pain is unilat eral, ocular, o r periocular, and described as burning, piercing, or neuralgic. C luster head aches occur more often in men and last 15 minutes to 2 hours. The episodic recur rences are " clustered" in cyc les of days or weeks w ith remission lasting months to years. Associated symptoms include ipsilat eral rhi norrhea, conjunctival injections , facial sweating, ptos is, and eyelid edema. Alcohol
ingestion, stress, or vasodilation secondary to wind or heat exposure may precipitate the pain.
B e nign exertional headache
These headaches occur suddenly and are related to coughing, sneezing, straining, running, or orgasm. Headache is the result of stretching the pain-sensitive structures in the posterior fossa . They are more com mon in men. The onset is sudden and "splitting" and pain may last from seconds up to 30 minutes. They should be distin guished from headache of SAH or arterial dissection.
Secondary Headaches
Infectious origin
Sinusitis Sinusitis is frequently associated with a sore throat irritated by postnasal discharge, facial or tooth pain, or a headache over the affected sinus that increases in intensity with cough ing or bending forward. There frequently are a cough that worsens in a lying position, morning periorbital swelling, fever, malaise, and recent upper respiratory tract infection. The maxillary s inuses are the most often af fected. Whereas pain in the temporal and periorbital area suggests frontal sinusitis, maxillary sinusitis produces pain below the eye, in the upper teeth, or both. Ethmoid si nusitis produces medial orbit pain.
Dental Disorders Patients with dental abscess, nerve root dys function, or infection may have headache and facia l pain located near the site of the lesion. Tenderness elicited by tapping on the maxil lary teeth with a tongue blade may indicate dental root infection or max illary sinusitis. Inspection of the mouth may reveal ulceration or infection of pain-sensitive structures in the oral mucosa and gingiva.
Pharyngitis Bacterial infection may irritate pain-sensitive structures in the oropharynx. leading to headache.
i
Chapter 19 • Headache
Otitis Media Recurrent otitis media with sequelae of mas toiditis or chronic infection may result in headache. Signs of otitis will be seen on ex amination of the tympanic membrane.
Meningitis Bacterial meningitis begins as bacteria colo nize in the nasopharynx and enter the CNS through the dural venous sinuses or choroid plexus into the subarachnoid space. Common causal organisms in adults are Staphylococcus pneumoniae and meningitidis. In children, common organisms are S. pneumoniae and Haemophilus injluenzae; in neonates, com mon organisms group B Streptococcus spp. and Escherichia coli. Bacterial meningitis is usually accompanied by severe systemic tox icity and mental status changes (encephalitis). In contrast, aseptic meningitis caused by en teroviruses or mumps virus produces a mild illness sometimes without fever. Photophobia and stiff neck are present in varying degrees. The person usually appears ill with a severe headache, fever, chills, myalgias, photopho bia, and stiff neck. The Brudzinski and Kernig signs may be positive. A petechial skin rash may suggest meningeal disease. Patients may progress to coma and have seizures.
Neurogenic origin
Trigeminal Neuralgia The pain associated with malfunction of the tri geminal nerve (CN V) is characterized by epi sodes of a series of bursts or jabs of sharp elec trical, stabbing pain lasting seconds that occur repeatedly over minutes or hours with a minute or so of relief between episodes. The pain is limited to the distribution of the three branches of CN V. Headaches caused by trigeminal neu ralgia are stimulated by sensory stimuli to the involved nerves, produced by rubbing or touch ing the face or swallowing. Trigeminal neuralgia usually occurs in women and individuals older than age 55 years. In younger patients, episodes may indicate multiple sclerosis.
Optic Neuritis Optic neuritis refers to a variety of conditions that affect the optic nerve and reduce visual
function . Disorders include demyeli . disease (e.g., multiple sclerosis}, intl:3hna tion, viral illness, metabolic disordersnillli. toxin exposure. The patient has an acute' ~ of blurred vision with extraocular motion~ that precedes the visual changes by ~to days. Ophthalmoscopic examination revca~ slightly elevated (hyperemic) disc and a blurred disc margin. Treatment is focUSed the underlying disease. 0q
Cervical Spine Disorders
The three upper cervical nerves are sensory pathways for pain sensation felt in the P<>ste. rior bead and ipsilateral temporal and eye ar. eas (see Fig. 19.2). Disturbances in the neck may cause muscle spasms and pressure 00 other neck structures. Patients with neck. related headache have pain associated with motion of the neck. Downward pressure on the head makes the pain worse and may cause it to travel down the arms.
Temporal Arteritis (Giant Cell Arteritis) Temporal arteritis is a vasculitis of the oph thalmic and posterior ciliary branches of the internal carotid artery. It almost always af fects people older than age 50 years. It pro duces a sharp, localized pain over a tender, nodular temporal artery. Other symptoms include fever, malaise, anorexia, weight loss, or polymyalgia rheumatica. Ischemic jaw pain and face pain are rare but highly sugges· tive. Headaches precede the major danger of temporal arteritis (blindness) by weeks. Unilateral blindness may occur suddenly and is not reversible. Left untreated, blindness may occur in the other eye. An ESR greater than 50 rmn/hr is almost always present. Suspected temporal arteritis is an emergency, and the patient needs referral to an emergency center for immediate evaluation and treannent.
Metabolic origin
Carbon Monoxide Poisoning Carbon monoxide is a colorless, odorless gas with an affinity for binding with hemoglo bin to produce carboxyhemoglobin (COHb), which impairs oxygen transport. Symptoms are nonspecific and are dose related. Low
Chapter 19 • Headache
oHb levels may produce mild dyspnea and ~ ttess across the head; however, as COHb uo 11 I
::: entration increases, the headache be conc d . . d . h es more severe an ts associate wit com &'. • d d . d .d'zziness, nausea, I.atlgue, an 1mme v1 .1 As COHb levels rise, symptoms increase
11SIO · • in severity and lead to loss of consciousness nd seizures. Blood gases and COHb blood ~evels are diagnostic. History may suggest recent smoke inhalation or similar symptoms in multiple family members.
severe Hypoglycemia Hypoglycemia is more likely to occur in indi viduals with type 1 diabetes but can occur in anyone taking oral hypoglycemic agents , in younger people who experience reactive hy poglycemia, or in people who have ingested excessive amounts of alcohol. A dietary and medication history may lead to a specific causative factor. Headache is generaliz ed and bilateral and is associated with dizziness and a sense of not fee ling well. Some people with diabetes may have nocturnal hypoglycemia and report nightmares and vivid dreams, night sweats, and a headache on awakening. Blood glucose levels can confirm the presence of hypoglycemia.
Drug Withdrawal Withdrawal from prolonged use of steroids may cause migraine headaches. Nitrites may precipitate headache. Other drugs causing cranial dilation and an aftereffect of rebound vasoconstriction include hydralazine, alcohol, histamine, nicotinic acid, and caffeine.
Dietary Ingestion A mild to moderately severe generalized headache may occur after ingestion of tyra mines (e.g., aged cheese, red wine), monoso dium glutama te, and nitrites in smoked meats. A headache diary will help identify the pattern of headache related to specific foods.
Cerebrovascular origin
lntracranial Tumor Primary intracranial tumors are more common in children than adults. Brain metas tases from Primary s ites in the lung, breast, or kidney are
more common in adults. Pain is constant and progressive, is felt in a discrete location, changes with head position, and awakens the person from sleep. Objective neurologic signs are present in 98% of all children with brain tumors .
Hydrocephalus Hydrocephalus is an excessive collection of CSF in the ventricles of the brain and can be caus ed by tumors or cysts . If fontanels are still open, hydrocephalus will cause an enlargement of the head on measurement. Headache will be progressive and may be as sociated with neurologic findings and mental status changes similar to those observed with dementia. Radiographic techniques are diag nostic, and LP may detect increased CSF pressure.
Subdural Hematoma Acute subdural hematoma produces a sudden, severe headache and may be a s sociated with a history of head trauma, exertional physical activity, or pharmacologic anticoagulation. There is transient loss of consciousness, stiff neck, nause a , vomiting, photophobia, pupil lary dilation, and pain over the eye. It is es sential to obtain a thorough history of trauma. Posttrauma headache can occur hours or a day after injury.
Pseudotumor Cerebri Teenagers being treated with topical acne preparations, menopausal women, and indi viduals ingesting large amounts of vitamin A are at increased risk for pain from pseudotu mor cerebri. Papilledema will be present in many cases, but without it, the headache may be diagnosed as mixed type. A neurology re ferral is indicated to ensure that no local ob struction is present before an LP is done to assess for increased ICP. An LP sometimes leads to herniation of the brainstem.
Brain Abscess Onset of pain can be gradual or severe, deep, and aching in nature, often worse in morning and a ggravated by coughing or straining. Pain is usually localized to the side of the a b s cess. Other signs of increased ICP may b e present,
Chapt.er 19 • Headache
such as papilledema and widening pulse pres sure. There may be a recent history of head injury, infections (e.g., dental abscess, otitis media, or sinusitis), or assault to the CNS .
lntracerebral Hemorrhage Intracerebral hemorrhage may result in a stroke or sudden coma and is associated with neurologic findings defined by the site of bleeding. A person may present with a
::: DIFFERENTIAL DIAGNOSIS OF Co111111011
CONDITION HISTORY
sudden-onset, severe headache, ~ith or With. out a history of trauma. The seventy ofS)'rnp. toms from bleeding intracranial aneurysms is correlated to the rate of h~morrhage and graded from I (asymptomatic to minimal headache with nuc!1~l . rigidity)_ to_ V (deep coma, decerebrate ngid1ty). Genatnc patients with AIDS and patients prescribed anticoagu. lation therapy are at increased risk for IC}f CT scan is diagnostic. ·
Cau!•ies t~f· Het1tlt1che
PHYSICAL FINDINGS DIAGNOSTIC STUDIES - ~
PRIMARY HEADACHES WITHOUT STRUCTURAL OR SYSTEMIC PATHOLOGY -
Tension-type Common in adults; bilateral headache pain, general or localized (muscle) in bandlike distribution;
history of anxiety, stress, or depression
Migraine More common in children; without aura unilateral, throbbing (common) pain; nausea
Migraine with Pain precipitated by aura (classic) environmental stimuli;
visual disturbances (scintillating scotoma) precede pain
Mixed headache Throbbing, con stant pain during waking hours; muscle tightness; family history of migraine
Cluster Rare in children; abrupt, headache nighttime onset; unilateral
periorbital pain that is severe
Benign Sudden onset related exertional to physical exertion, headache Valsalva maneuver, or
coitus
Normal physical exami- None nation ; neck muscle t ightness or fascicu lations may be palpated
Photophobia and None phonophobia
Nausea and vomiting, None photophobia and phonophobia
Mix of findings related None to tension and migraine headache pain
lpsilateral rhinorrhea, None nasal stuffiness, con junctival injection , sweating, ptosis
Normal physical May need to dis· examination tinguish from
su barachnoid hemorrhage with CT scan
SECONDARY HEADACHES WITH STRUCTURAL OR SYSTEMIC PATHOLOGY
INFECTIOUS ORIGIN Sinusitis Frontal , upper molar, or Low to no fever ; pain on Radiographs
periorbital pain; cough, palpation of frontal , (Waters view) rhinorrhea maxillary sinuses;
purulent nasal or postnasal discharge
Dental disorders Localized pain in jaw and Malocclusion, caries, Dental referral top of head abscesses of teeth
present, gum disease
Chapter" 19 • Headache
, ~Ff[RENTIAL DIAGNOSIS OF Co111111011 C~ttll.'il!S o_f· l~elldac:ll
~'.--~~-H_ISll~O_RY~~~~~~~~~-P_HY~Sl_C_Al~F_IN_D_IN_G_S~~~-D_Wi~N_OSTJ~_C_STUD~_lES--1 Sore throat Fever; infection of Throat culture
~ posterior pharynx Ear pain. pain with Fever; red , bulgi n g None
p~ swallowing tympanic membrane
Severe headache. chills.. Positive Kernig and Lumbar puncture ~ myalgias. stiff neck; Brudzi nski s igns;
toxic child or adult fever. photophobia, petech ial rash may be present; mental status changes
IPJlOWllC ORJGIN People older than 55 yr; Normal physical exami None ~.inaJ
bursts of sharp pain over nation; stimulation of;ecraJgja face innervated by triggers may provoke affected nerve; triggered pain by stimulus to affected nerve
~neuritis Acute onset of pain with Diminished visual acuity, Ophthalmology OM followed by blurred decreased papillary referral vision reflex., hyperemia
of optic disc; pain with EOM
r.eMcal spine May have history of trauma; Normal physical exami Cervical spine disorders occipital pain, muscle nation findings or radiographs
stiffness pain associated with neck motion
Temporal Age older than 50 yr; Fever, weight loss; Elevated ESR arterrtis sharp, localized temporal tender over a nodular ( > 50); immedi
pain ; malaise, anorexia; temporal artery ate referral for history of polymyalgia treatment rheumatica
llTABOUC ORIGIN Carbon History of exposure; Nausea, vomiting, Blood gases and
rooooxide throbbing headache, change in mental carboxyhemo intSOning mild dyspnea status, lethargy, loss globi n level
of consciousness 'SMfe History of diabetes or medi Normal physical exami Blood glucose
hypoglycemia cation , alcohol, and food nation findings or level; may need ingestion; generalized pallor, sweating, and self-monitoring
headache, dizziness, weakness of blood glu sense of not feeling well cose to estab
1ish pattern Drug Withdrawal Pattern of headache associ Normal physical Blood chemistry
ated with stopping medi examination findings cation or substance use
Dietary Mild to moderately severe Normal physical Blood chemistry ingestion headache after ingestion examination findings
of foods or medication
Continued
------- • Headache
Chapter 19
coMD\llOM CuLAR ORIGIN d heCEREBROVAS Sudden-onset hea. ac
\ntracrania\ that is progressive. . tumor exacerbated by co~gh1ng
or exercise; worse in morning; history of. trauma increases nsk
Progressive headac.h.e, Hydrocephalus vomiting, irritab1l1ty
History of head trauma , . Subdural bleeding disorders, child
hematoma abuse; adult older than
35 yr; sudden onset o~. "worst headache ever, often over eye; transient loss of consciousness
Teens, menopausal women; Pseudotumor
history of vitamin A or cerebri
tetracycline ingestion; progressive headache
History of chronic ear Brain abscess infection or cyanotic heart disease
Risk factors: people older lntracerebral hemorrhage than 50 yr, with AIDS,
taking anticoagulation therapy, hypertension
AID.s, acqu.ired immune deficiency syndrome; CT, computed tomography; EOM, extraocular movement; ESR, erythroq'te sed1mentat1on rate; MRI, magnetic resonance imaging.
Papilledema , vomiting, asymmetrical reflexes, weakness, sensory deficit, or other neurologic deficit
Rapid enlargement of head, bulging fontanels
Unequal pupils, photo phobia, neurologic changes, seizure
Papi I I edema may be present
Fever, seizures, focal neurologic deficits
If conscious, abnormal neurologic findings correlated with extent of lesion
CT scan and referral
CT scan and neurosurgica1 referral
CT scan, neuroi. ogy referral to assess risk related to lum. bar puncture
MRI
Emergency trans port for immedi· ate evaluation (CT, MRI, and possible surgical treatment)