The Differential Diagnosis and Boundaries of Migraine

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© JonesIntroduction & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC Differential Diagnosis for Migraine The Differential NOT FORWithout SALE Aura OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION Differential Diagnosis for Aura Diagnosis and Differential Diagnosis for Typical Migraine with Aura Differential Diagnosis for Boundaries of Migraine © Jones & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC Hemiplegic Migraine NOT FOR SALEConclusions OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION Michael Bjørn Russell, MD, PhD, Dr. Med Sci

© Jones & Bartlett Learning, LLC © Jones & Bartlett Learning, LLC NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION Introduction complementary; that is, migraine without aura is usually a unilateral pulsating moderate/severe headache that Migraine is the second most common pain condition, is aggravated by physical activities, while tension-type next to tension-type headache (Rasmussen et al., 1991; headache is usually a bilateral pressing mild/moderate Russell et al., 1995). Its diagnosis relies exclusively on © Jones & Bartlett Learning, LLC headache that© Jones is not aggravated & Bartlett by physicalLearning, activities LLC the patient’s history and exclusion of secondary causes (ICHD-II, 2004). However, it is the usual lack of accom- NOTruled outFOR by aSALE normal ORphysical DISTRIBUTION and neurologic exami- panying symptomsNOT FOR that makes SALE the differentialOR DISTRIBUTION diagnosis nation or appropriate investigations (ICHD-II, 2004). of tension-type headache easy, although tension-type The only objective marker in the most common types headache can also be accompanied by photophobia or of migraine is the increased excretion of 5-hydroxyin- phonophobia. The correct diagnosis of migraine without doleacetic acid in urine after a migraine attack (Sicuteri aura versus tension-type headache is important for correct © Jones & Bartlettet al., 1961), Learning, although LLC some individuals with the rare© Jonesmanagement & Bartlett of the formerLearning, condition, LLC as triptans are not NOT FOR SALEsporadic OR or DISTRIBUTION familial hemiplegic migraine might carry aNOT effectiveFOR SALE in tension-type OR DISTRIBUTION headache (Tfelt-Hansen, 2007). point mutation in the CACNA1A, ATP1A2, or SCN1A genes Cluster headache is another primary headache that (Ophoff et al., 1996; De Fusco et al., 2003; Dichgans is part of the differential diagnosis for migraine without et al., 2005). Thus the differential diagnosis and bound- aura. Usually it is easy to differentiate between cluster aries of migraine pose a real challenge. © Jones & Bartlett Learning, LLCheadache and migraine without© auraJones owing & to Bartlett their dif- Learning, LLC ferent attack patterns—that is, attacks in clusters versus NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION Differential Diagnosis for Migraine episodic attacks. Furthermore, cluster headache is char- Without Aura acterized by one or more associated symptoms, such as lacrimation, conjunctival injection, miosis, eyelid edema, Migraine without aura is characterized by headache ptosis, nasal congestion, rhinorrhea, facial sweating, and ©(symptoms) Jones & and Bartlett accompanying Learning, symptoms LLC (photopho- a sense of ©restlessness Jones &or agitationBartlett that Learning, is not seen LLCin bia and phonophobia, nausea and vomiting). The most migraine without aura (ICHD-II, 2004). At its onset, how- NOTcommon FOR differential SALE diagnosisOR DISTRIBUTION for migraine without ever, clusterNOT headache FOR might SALE be mistaken OR DISTRIBUTION for migraine aura is tension-type headache. The pain characteristics without aura because the associated symptoms might be of migraine without aura and tension-type headache are missed by the patient, may not be pronounced, or may

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not be present (Russell & Andersson, 1995; Sjöstrand et moves across the cortex at a rate of 3–5 mm/min, and is al., 2005). Similarly, chronic cluster headache might be followed by prolonged depression of the neurons (Leão, difficult to differentiate© Jones from chronic & Bartlett migraine, Learning, given that LLC1944a, 1944b). This event is followed© Jones by a &reduction Bartlett in Learning, LLC chronic cluster headache is sometimes characterized by the regional cerebral blood supply in persons with typi- a milder headacheNOT between FOR the SALEmore severe OR attacks DISTRIBUTION of cal migraine with aura and hemiplegicNOT FOR migraine, SALE which OR DISTRIBUTION cluster headache. is not present in persons with migraine without aura The differential diagnoses for migraine without aura (Olesen et al., 1981; Lauritzen & Olesen, 1984; Olesen also include secondary migraine without aura. A major et al., 1990). ©reason Jones to suspect & Bartlett secondary Learning, migraine without LLC aura is The typical© Jones migraine & with Bartlett aura is characterizedLearning, byLLC its onset in close temporal relation to another disorder. visual aura followed by headache, with one-fourth and NOTMigraine FOR without SALE aura OR is caused DISTRIBUTION by a combination of one-third of NOTpatients FOR also experiencing SALE OR dysphasic DISTRIBUTION (speech) genetic and environmental factors (Russell et al., 1995). and sensory aura in some of their attacks (Russell & First-degree relatives of probands with migraine without Olesen, 1996a). The sequence observed usually consists aura have a 1.9-fold (statistically significant) increased of first visual, then dysphasic, and finally sensory aura. © Jones & Bartlettrisk of migraine Learning, without LLC aura compared to the general© JonesIf motor & Bartlett aura is present, Learning, the presentation LLC is classified population (Russell & Olesen, 1995). Interestingly, first- hemiplegic migraine (ICHD-II, 2004). Most persons with NOT FOR SALEdegree OR relatives DISTRIBUTION of probands with migraine withoutNOT hemiplegicFOR SALE migraine OR experience DISTRIBUTION all types of aura, includ- aura that occurred in relation to a head trauma have ing basilar-like aura, usually in the sequential order of no increased risk of migraine without aura (Russell & visual, sensory, motor, aphasic, and basilar-like aura, Olesen, 1996b). Thus head trauma in a susceptible indi- although the order is different in approximately 30% vidual might cause© Jones migraine & without Bartlett aura. Learning, However, LLCof affected individuals (Thomsen© Joneset al., 2002, & Bartlett 2003). If Learning, LLC head trauma is very common, and not all people experi- the sequential order of the aura symptoms is not logical ence onset of migraineNOT without FOR aura SALE after trauma.OR DISTRIBUTION Other according to the locations of NOTthe different FOR areas SALE in the OR DISTRIBUTION secondary causes include atriovenous malformation, brain, the symptoms might be due to memory bias, and MELAS (mitochondrial encephalomyopathy, lactic acido- prospective recordings of the aura might illuminate this sis, and stroke), and antiphospholipid antibody disease issue (Russell et al., 1994). ©(Pavalakis Jones et & al., Bartlett 1984; Chabriat Learning, et al., 1995; LLC Cervera et The gradual© developmentJones & Bartlettand duration Learning, of the migraine LLC al., 2002). aura over at least 20 minutes is unique for migraine with NOTBecause FOR migraine SALE without OR DISTRIBUTION aura is very common, co- aura—that is,NOT for typical FOR migraine SALE with OR aura DISTRIBUTION and hemiple- occurrence of migraine without aura and other disorders gic migraine. In contrast, an epileptic aura has a dura- is not rare. Thus, to establish a causal relationship, the tion of a few seconds, transient ischemic attacks have a occurrence of migraine without aura should be signifi- sudden onset with a duration of less than 24 hours, and cantly increased as compared to the risk of migraine stroke causes permanent neurologic signs. Thus a pre- © Jones & Bartlettwithout Learning, aura in the general LLC population (Weiss et al.,© Jonescise history & Bartlett of the aura Learning, will, in most LLC cases, provide the NOT FOR SALE1982; OR Khoury DISTRIBUTION et al., 1988). Suspicion of a secondaryNOT crucialFOR informationSALE OR necessary DISTRIBUTION for a precise diagnosis. cause for migraine without aura should be raised if the symptoms are always located on the same side, attack Differential Diagnosis for Typical frequency is dramatically changed, or age at onset is Migraine with Aura after age 40 years.© This Jones presentation & Bartlett requires Learning, a careful LLC © Jones & Bartlett Learning, LLC work-up that includes a physical and neurologic exami- The aura in typical migraine with aura can be followed nation as well as magneticNOT FOR resonance SALE imaging OR DISTRIBUTION(MRI) of by a migraine headache, a tension-typeNOT FOR headache, SALE or no OR DISTRIBUTION the brain. headache. Onset may occur at all ages, though it usually takes place within the first four decades of life; by com- Differential Diagnosis for Aura parison, the typical migraine with aura without headache © Jones & Bartlett Learning, LLC might have ©an onsetJones later & in Bartlettlife. Cautions Learning, should be taken LLC The migraine aura reflects reversible cerebral cortical with people in whom attack frequency is dramatically NOTdysfunction FOR ofSALE vision, OR speech, DISTRIBUTION sensory, and/or motor changed, asNOT this alteration FOR SALE in pattern OR may DISTRIBUTION be caused by function. The gradual development and sequential march reversible cerebral vasoconstriction (Ducros et al., 2007). of the aura symptoms are caused by cortical spreading The risk of typical migraine with aura is also increased depression, in which a brief excitation of the occipital in people with cerebral autosomal dominant arteriopa- © Jones & Bartlettcortical Learning, neurons initiates LLC a depolarization wave that© Jonesthy with & Bartlett subcortical Learning, infarcts and leukoencephalopathyLLC © Jones and Bartlett Publishers. NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION

00501_CH02_FINAL.indd 12 1/5/12 12:18:58 PM © Jones & Bartlett Learning, LLC Chapter© 2Jones | The Differential & Bartlett Diagnosis Learning, and Boundaries LLC of Migraine | 13 NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION

(CADASIL) (Tournier-Lasserve, 1993). Other secondary A series of secondary causes of hemiplegic migraine causes could be similar to those causing hemiplegic have been reported. In one case reported in the literature, migraine (discussed© Jones in the next & Bartlett section). Visual Learning, aura LLCa parasagittal meningeoma in© the Jones left parietal-occipital & Bartlett Learning, LLC is nearly always present in typical migraine with aura region caused alternating attacks of typical migraine with (Russell & Olesen,NOT 1996a). FOR Thus aSALE person ORwithout DISTRIBUTION visual aura and sporadic hemiplegic migraineNOT FOR for 17 SALE years in ORa DISTRIBUTION aura should be suspected to have a secondary cause. 42-year-old woman. The aura was strictly right-sided and spread in the sequential order of visual, aphasic, sensory Differential Diagnosis for Hemiplegic and motor aura, including dysarthria, and then headache ©M iJonesgraine & Bartlett Learning, LLC (Vetvik et al.,© 2005).Jones Other & Bartlett secondary Learning, causes of hemi LLC- plegic migraine may include Sturge-Webers syndrome, NOTStroke FOR is the firstSALE differential OR DISTRIBUTION diagnosis considered for Epstein-BarrNOT virus infection, FOR SALE avascular OR necrosis DISTRIBUTION associated hemiplegic migraine, especially if the aura description is with anticardiolipin antibodies, childhood lupus erythe- insufficient. A young age at onset points the diagnosis in matosus, progressive facial hemiatrophy, ornithine trans the direction of a hemiplegic migraine attack. Although carbamylase deficiency, MELAS, and CADASIL (Leavell et © Jones & Bartletttypical migraine Learning, with aura LLC is a risk factor for stroke, par-© Jonesal., 1986; & Bartlett Montagna Learning,et al., 1988; de LLC Grauw et al., 1990; ticularly in young women, the risk of stroke in hemiple- Seleznick et al., 1991; Klapper, 1994; Parikh et al., 1995; NOT FOR SALEgic migraine OR DISTRIBUTION is unknown (Tzourio et al., 1995). NOT HutchinsonFOR SALE et al., OR 1995; DISTRIBUTION Woolfenden et al., 1998; Dora & Alternating hemiplegia of childhood (AHC) is charac- Balkan, 2001). A secondary cause of hemiplegic migraine terized by paroxysmal episodes of hemiplegia, quadri- should be suspected if the aura symptoms always occur plegia, choreoathetotic movements, and nystagmus that on the same side, as nonsymptomatic attacks of hemiple- disappear immediately© Jones after sleep, & Bartlett along with Learning, progres- LLCgic migraine usually change side© Jonesfrom attack & Bartlettto attack. Learning, LLC sive mental retardation and development of permanent Other reasons to be suspicious for a symptomatic cause neurologic deficitsNOT such asFOR choreoathetosis, SALE OR dystonia, DISTRIBUTION and are atypical aura symptoms, NOT increasing FOR frequency SALE ofOR DISTRIBUTION ataxia (Bourgeois et al., 1993). AHC is usually a sporadic attacks, a change in the headache pain’s character, a disorder with onset prior to age 1½ year, whereas onset change in the efficacy of the usual medication, and occur- of hemiplegic migraine usually occurs at an older age. In rence of persistent neurologic symptoms or signs. ©addition, Jones co-occurrence & Bartlett of Learning,mental retardation LLC is rare in © Jones & Bartlett Learning, LLC hemiplegic migraine. Conclusions NOTComa FOR sometimes SALE occurs OR DISTRIBUTION in severe attacks of hemi- NOT FOR SALE OR DISTRIBUTION plegic migraine. Its emergence requires an extensive The paroxysmal nature of migraine and its reversible work-up to exclude other symptomatic causes such as neurologic symptoms usually make the diagnosis of this hypoglycemia, cerebral hemorrhages, mass lesion, and condition straightforward, especially if a precise head- infections. Fever and meningismus can be observed ache history and aura description are ascertained. The © Jones & Bartlettduring attacks Learning, of hemiplegic LLC migraine, and their pres-© Joneshealth-care & Bartlett provider shouldLearning, be suspicious LLC of a secondary NOT FOR SALEence OR requires DISTRIBUTION exclusion of bacterial and viral meningitis,NOT causeFOR if SALEthe migraine OR headacheDISTRIBUTION and/or aura symptoms sepsis, and inflammatory diseases from the diagnosis. are always located on the same side, if the attack fre- Permanent cerebellar signs occur in 40% of the families quency increases significantly, if the efficacy of the usual with familial hemiplegic migraine caused by point muta- medication changes, or if the neurologic symptoms or tions in the CACNA1A© Jones gene (Ducros & Bartlett et al., 2001). Learning, LLCsign become permanent. © Jones & Bartlett Learning, LLC NOT FOR SALE OR DISTRIBUTION NOT FOR SALE OR DISTRIBUTION References

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