Course 11- HeadachesNelson Hendler, MD, MSFormer Assistant Professor of Neurosurgery JohnsHopkins University School of MedicinePast president-American Academy of PainManagement
Various Types of Headaches• Incidence of Migraine Headache-US Armedforces 1998-2010 (1)• 3% of all men, 6% of all female with migraine,• 3.9% of men, 11.3% female with some sort ofheadache• If diagnosed with migraine, then less than 1%had other types of headaches.• However, what is a migraine?(1) MSMR. 2012 Feb;19(2):12-7.Outpatient encounters associated with diagnosticcodes for migraine and other types of headaches, active component servicemembers, 1998-2010.
Types of Headaches• Most common headaches are:• Muscle Tension Headache• Migraine-common and classic• Trigeminal• Cluster• Chronic daily headacheRobbins MS, Lipton RB. The epidemiology of primary headache disorders.SeminNeurol. 2010 Apr;30(2):107-19.
Chronic Daily Headache (CDH)• CDH occurs more than 15 days a month• Lasts more than 4 hours a day• If it lasts less than 4 hours a day, it is considered atrigeminal autonomic cephalalgia (TAC).• TACs include episodic & chronic cluster headache, episodic& chronic paroxysmal hemicrania, SUNCT, & hypnicheadache.• If duration is > or =4 h, then CDH & differential diagnosis ischronic migraine, chronic tension-type headache, new dailypersistent headache and hemicrania continua.Bigal ME, Lipton RB. The differential diagnosis of chronic daily headaches: an algorithm-basedapproach, J Headache Pain. 2007 Oct;8(5):263-72.
Other Classifications• Most common is muscle tension, thenmigraine, with and without auras.• 10% prevalence• Female to male ratio of 3 to 1• Migraine related to estrogen drop• Cluster Headaches highest morbidity• No basis of diagnosis…merely descriptionsMonti AA, Rapela FJ, Bacile Bacile EA, Uberti D. Primary cephaleas. A revision of their diagnosisand therapeutic approach, Rev Fac Cien Med Univ Nac Cordoba. 2006;63(3):53-64.
Yet another classification• Chronic Daily Headache (CDH) defined as headache15 days a month, for at least 3 months, lasting morethan 4 hours. Not a diagnosis –merely a description• CDH has 4 subtypes:• Chronic migraine, chronic tension type headache,new daily persistent headache and hemicraniacontinua-again not diagnoses but descriptions• Chronic migraine affects about 2% of population• Chronic migraine sufferers are 70% more likely tohave had strokeLipton RB. Chronic migraine, classification, differential diagnosis, and epidemiology.Headache. 2011 Jul-Aug;51 Suppl 2:77-83
Why Headaches Should Be ClassifiedBased On Origin• A single symptom may have multiple origins,such a flat tire, caused by a nail in the tread,cut sidewall, leaky valve stem, or bad bead• You have to know the cause in order toproperly repair the tire. This is a DIAGNOSIS• A single cause (DIAGNOSIS) may have multipleclinical manifestations, such as syphilis, Lymedisease, occlusive stroke, etc.• Defining the origin allows a doctor to treat thecauses and address multiple symptoms
Diagnostic Tests for Headache-I• Headaches are one of the most common symptoms and the listdifferential diagnoses has over 300 different types and causes• The cause of most headaches can be determined by a careful historyand neurologic examination• The yield of 3026 neuroimaging scans in the evaluation of patientswith headache and a normal neurologic examination is quite low.• Percentages of various pathologies: brain tumors, 0.8%;arteriovenous malformations, 0.2%; hydrocephalus, 0.3%; aneurysm,0.1%; subdural hematoma, 0.2%; and strokes, including chronicischemic processes, 1.2%.• 1440 scans of patients with migraine: brain tumor, 0.3%;arteriovenous malformation, 0.07%; and saccular aneurysm, 0.07%.• WMA have been reported on MRI studies of patients with all types ofmigraine, with a range from 12% to 46%.Evans RW. Diagnostic testing for the evaluation of headaches.Neurol Clin. 1996 Feb;14(1):1-26.
Diagnostic Tests for Headache -II• EEG is not useful in the routine evaluation of patients with headache.• The probability of detecting an aneurysmal hemorrhage of CT scansperformed at various intervals after the ictus is: day 1 0.95%; day 3,74%; 1 week, 50%; 2 weeks, 30%; and 3 weeks, almost nil• The probability of detecting xanthochromia with spectrophotometryin the CSF at various times after a subarachnoid hemorrhage is: 12hours, 100%; 1 week, 100%; 2 weeks, 100%; 3 weeks, more than70%; and 4 weeks, more than 40%• 30% to 90% of patients have various types of headaches after mildhead injury. 1% to 3% of these patients have life-threateningpathology, including subdural and epidural hematomas, that aredetected on CT and MRI scans• Headaches are the most common symptom of Temporal Arteritis,reported by 60% to 90% of patientsEvans RW. Diagnostic testing for the evaluation of headaches.Neurol Clin. 1996 Feb;14(1):1-26.
Headache Classification- A meta analysis of all causesMigraine –vascular originCommon-no aura Classic-with auraMuscle TensionNosecondaryvascularentrapmentWith vascularentrapmentWith Nerve entrapmentNerve CompressionC2EntrapmentTrigeminal neuralgiafrom intracranial 5thnerve compressionCluster HeadacheChemical -tyramine, caffeine, etc.Cable Frame Sunglasses InfectiousPost-herpetic trigeminal neuralgia Viral encephalitisStrokeSinusBRAIN TUMORS -CNS COMMPRESSIONOccipital NeuralgiaTemporal ArteritisPost-concussion
Common Migraine Headache• Outstanding research from Denmark clearlydemonstrates that intra-cranial arteries on theside of the pain are more dilated during migraineattack, while extra-cranial arteries are not• This clarifies the patho-physiology of commonmigraine• Treatment can now be directed appropriately• Amin FM, Asghar MS, Hougaard A, Hansen AE, Larsen VA, de Koning PJ, Larsson HB, Olesen J, Ashina M. Magneticresonance angiography of intracranial and extracranial arteries in patients with spontaneous migraine withoutaura: a cross-sectional study.Lancet Neurol. 2013 May;12(5):454-61• Need to examine the vaso-receptors on intra-cranial vessels.
Common Migraine Headache• Unilateral usually in the temple or parietal regionof the skull• No aura associated with the headache, i.e. noantecedent nausea, no visual flashes, no unusualsmells or tastes• Described as sudden pain onset, which in 5 to 30minutes which goes to a throbbing, pounding pain• May last hours to a full day• If more frequent than once a week, useprophylactic treatment, less frequently, useabortive treatments
Abortive Therapy for Common Migraine• Two types of therapy for common migraine• Abortive: used when migraines occur morethan once a week.• Prophylactic: used when migraines occurmore frequently than once a week• When steroids are added to standard abortivetherapy for migraine headaches, they areeffective and safe for preventing moderate orsevere headache recurrence.Huang Y, Cai X, Song X, Tang H, Huang Y, Xie S, Hu Y. Steroids for preventingrecurrence of acute severe migraine headaches: a meta-analysis Eur J Neurol.2013 Apr 11.
Abortive Therapy for Common Migraine• Sumatriptan constricts extracerebral arteriesmore than cerebral arteries, suggesting thatsumatriptan may exert its anti-migraine actionoutside of the blood-brain barrier.• Amin FM, Asghar MS, Ravneberg JW, de Koning PJ, Larsson HB, Olesen J, Ashina M, The effect of sumatriptan oncephalic arteries: A 3T MR-angiography study in healthy volunteers. Cephalalgia. 2013 Apr 15• Other abortive treatments are oxygen which isminimally effective .• Bennett MH, French C, Schnabel A, Wasiak J, Kranke P. Normobaric and hyperbaric oxygen therapy for migraineand cluster headache.Cochrane Database Syst Rev. 2008 Jul 16;(3)• Aspirin, caffeine, non-steroidal anti-inflamatorydrugs, & DHE 45 all have some degree of benefit• Gilmore B, Michael M. Treatment of acute migraine headache. Am Fam Physician. 2011 Feb 1;83(3):271-80.
Prophylactic Therapy for Common Migraine• If common migraine headaches occur morethan once a week, then use prophylactic drugs• Topiramate and valproic acid, both anti-convulsants, have been used equally effectivelyto reduce the number and severity of frequentcommon migraine• Krymchantowski AV, Jevoux CC Topiramate vs divalproex sodium in the preventive treatmentof migraine: a prospective "real-world" study. Headache. 2011 Apr;51(4):554-8.• Propranolol, a beta blocker alone or incombination with amitryptiline, an anti-depressant have been used successfully for anumber of years
Classic Migraine Headache• This has all the features of common migraineheadache, with other added features• Unilateral, usually in temple or parietal region• An aura event proceeds the headache• Auras may be flashing lights, nausea, unusualsmells or tastes,• Headache is throbbing, pounding, associated withphotopobia (fear of light) and phonophobia (fearof sound)• May be relieved by vomiting, abortive medication
Chemically Induced Headache• Two major types-Tyramine and Cogeners• Tyramine is found in hotdogs, Cheddarcheese, yogurt, beer, red wine, or aged meat,• Congeners*, found in darker, amber liquors,aged in wood casks, cause headaches (1)• Wood casks have terpenes, norisoprenoids,eugenol *aldehydes, ketones, alcohols,phenols & esters (2).• Avoiding the wrong food is the best approach(1) http://headaches.about.com/od/headaches101/a/Understanding-Alcohol-Induced-Headaches.htm(2)Maria João B. Cabrita, Raquel Garcia, Nuno Martins, Marco D.R. Gomes da Silvaand Ana M. Costa FreitasGas Chromatography in the Analysis of Compounds Released from Wood into Winehttp://cdn.intechopen.com
Mixed Muscle Tension-Vascular Headaches• The nerves, arteries and veins pass through thickfibrous fascia, and thick muscle tissue at the back ofthe neck –see the next slide• When a muscle goes into spasm, from stress orinjury, it gets shorted and thicker• As the opening between the muscles is constricted,by the thickened muscle, this compresses the vesselwhich passes between, causing vessel spasm• So the clinical presentation is a muscle spasm thenabout ½ hour later, a throbbing, poundingheadache
Mixed MuscleTension –VascularHeadache-Note how theblood supplypasses throughlayers of heavymuscle
Cable Frame Sunglasses Headache• Many types of headache are caused bycompression of either arteries or nerves (1)• One of the easier to diagnosis is a headache at theback of the ear,radiating up the side of the head,into the temple• The onset of headache is precipitated by wearingcable frame sunglasses with a spring loadedearpiece to grip the ears, and not fall off, such asmade by Rayban (my sunglasses of choice)• Treatment is bending the ear pieces or padding(1) Krymchantowski AV. Headaches due to external compression, CurrPain Headache Rep. 2010 Aug;14(4):321-4
Headachedue toCableFrameSunglassescausingcompression of thelesser and greaterauricular nerve, withpain at the back of theear, and back of theneck. Obviously,treatment is to get apair of sunglasseswhich do notcompress the nerveCompressionPoints
Occipital Neuralgiahttp://www.hopkinsmedicine.org/neurology_neurosurgery/specialty_areas/headache/conditions/occipital_neuralgia.html• Irritation of occipital nerves anywhere along their coursecan cause a shooting, zapping, electric, or tingling painwith symptoms located on the back of the scalp (see nextslide)• Occipital neuralgia can be diagnosed—and temporarilytreated—by an occipital nerve block• In patients who do well with temporary “deadening” ofthe nerve, a more permanent procedure includes cuttingthe nerve surgically, “burning” the nerve with a radio-wave probe, or eliminating the nerve with a small dose ofan injected toxin
OCCIPITALNEURALGIA-pain starts at theback of the neck atthe upper spine, andradiates up the backof the neck like astripe, usually endingat the top of thehead.PAIN
C2 Entrapment & Other Cervical HeadachesLong, DM, Davis, RF, Speed, WG, and Hendler, NH, Fusion for Occult Posttraumatic CervicalFacet injury, Neurosurgery Quarterly, Vol.16, No. 3, pp 129-138, Sept. 2006• Headache, at the back of the head, top of the head,or in C2 entrapment, over the ear, radiating to theeyebrow or the eye, is cervical in origin• Effective diagnostic procedures were facet blocks,root blocks, and provocative discogram• When properly diagnosed, a posterior cervicalfusion was performed C1-C3 or C2-C4, or othercombinations• 79% of patients had complete relief, and 14% hadsatisfactory improvement
From:"Cervical Spine Trauma", Dr. John J. Carbone, M.D., Johns HopkinsUniversity, Presented at the Johns Hopkins Orthopaedic Review Course,Baltimore, June, 2003. -- http://www.netorthodoc.org/1025/1025pp.pdfCervical FacetBlocksconfirms, on aphysiologicalbasis, thecause of thepain.If a provocativediscogram isalso positive,this confirmsthe anatomicalpicture, and aposterior fusionis performed
Ice Cream Headache (BrainFreeze)• A family favorite of mine (see next slide)• The pain is felt in the forehead, nose, and eyes• An ice cream headache is set-off when an unusuallycold substance, consumed rapidly, passes over thepalate and back of the throat• Constriction or spasm of the sphenopalatine arteryis thought to be the cause• Treatment is running your tongue rapidly, for 60seconds, on the roof of your mouth, to warm ithttp://www.hopkinsmedicine.org/neurology_neurosurgery/specialty_areas/headache/conditions/ice_creamEditor_headache.htmlLetter to the Editor, Slurpee Headache, due to Sphenopalatine Artery Spasm, New England Journal ofMedicine
Hendler’s Ice Cream- The largest selling ice cream in the State ofMaryland, from 1905 until 1970. Hendler’s Ice Cream was sold toBorden’s Ice Cream
Symptoms of Cluster Headache• excruciating severe stabbing and boring one sidedpain located in the temple and the orbit• The attacks can be associated with conjunctivalinjection and restlessness and migrainous features.• The duration of attacks can be 30 minutes• The frequency can be 4 to 5 per 24 hours.• Vital signs and physical and neurologicalexamination are normal• unenhanced brain computed tomography had beennormalEdvardsson B, Persson S.Cluster headache and parietal glioblastoma multiforme. Neurologist. 2012Jul;18(4):206-7
Cluster Headache Treatment?• The pain and autonomic symptoms of clusterheadache (CH) result from activation of thetrigeminal parasympathetic reflex, mediatedthrough the sphenopalatine ganglion (SPG)• The efficacy of on-demand SPG stimulation forchronic cluster headache was tested• Pain relief was achieved in 67.1% of full stimulation-treated attacks• 36% had a ≥50% reduction in attack frequencySchoenen J, Jensen RH, Lantéri-Minet M, Láinez MJ, Gaul C, Goodman AM, Caparso A, May A. Stimulation ofthe sphenopalatine ganglion (SPG) for cluster headache treatment. Pathway CH-1: A randomized, sham-controlled study. Cephalalgia. 2013 Feb 14.
Sinus Headaches are not Sinusitis• Patients with sinus headaches usually complain ofpain and pressure sensation around the sinuses andperiorbital area; most are misdiagnosed withsinusitis• The aetiology of “sinus headache” in patients withnegative endoscopic or computed tomography (CT)scan findings was allergic & vascular abnormalities• Nasal obstruction, nasal discharge and sinussensitivity are seen in other types of headachesKaymakci M, Cikriklar HI, Pay G.The aetiology underlying sinus headaches. J Int Med Res. 2013Feb;41(1):218-23.
Headache from Encephalitis• Patients with viral infections of the central nervoussystem (CNS) may present with a variety ofneurological symptoms, most commonly dominatedby either encephalitis or meningitis.• The encephalitis headache is all over the head• Often it is accompanied by a low grade fever, somephoto sensitivity, and malaise• Sometimes features are non-specific• A spinal tap may prove a definitive diagnostic testStudahl M, Lindquist L, Eriksson BM, Günther G, Bengner M, Franzen-Röhl E, Fohlman J, Bergström T,Aurelius E.Acute viral infections of the central nervous system in immunocompetent adults: diagnosisand management. Drugs. 2013 Feb;73(2):131-58.
Intra-Cranial Bleed• The old rule that the “first headache, or the worst headache”is dangerous applies to this• Rupture of a saccular intracranial aneurysm (SIA) causes“thunderclap headache”• Retrospective study of 199 consecutive patients with SIA• During the year before rupture, 124 (62.3%) had one or moretypes of headache.• These headaches were migraine without aura (39.2%),migraine with aura (1%), probable migraine (2%), tension-type headache (19.6%), and cluster headache (1%)• Only the prevalence of migraine without aura wassignificantly higher in patients with SIA (OR 6.7, 95% CI 3.8-11.9, p<0.0001) Lebedeva ER, Gurary NM, Sakovich VP, Olesen J. Migraine before rupture ofintracranial aneurysms, J Headache Pain. 2013 Feb 20;14(1):15
Headache due to Brain Tumors• Sudden-onset headache is a common and oftenalarming presentation. The location varies• If the headache persists, then more testing isneeded –CT, MRI, MRA, EEG and PET• A small proportion may be indicative of acatastrophic etiology. Autopsy reports indicate:• Vascular events 60.4% ,primary brain tumours/cysts16.7% , and meningitis 6.25%.• Aneurysms accounted for the majority of vascularcases 22.9%Lynch KM, Brett F.Headaches that kill: a retrospective study of incidence, etiology and clinical features incases of sudden death. Cephalalgia. 2012 Oct;32(13):972-8.
Temporal Arteritis (Giant Cell Arteritis)• Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatorydiseases affecting white individuals >50 years & women 2-3 times more than men.• PMR and GCA occur together more frequently than expected by chance.• GCA is a large-vessel and medium-vessel arteritis predominantly involving thebranches of the aortic arch.• Clinical manifestations of GCA may be a new headache, palpable tender vessel onthe side of the pain, jaw claudication and visual loss.• GCA usually remit within 6 months to 2 years from disease onset. Some patients,however, have a relapsing course and might require long-standing treatment.• Diagnosis of GCA is based on clinical features & elevated levels of inflammatorymarkers such as sed rate but check C3, C4, Rheumatoid factor, SSA, SSB,• Temporal artery biopsy remains the gold standard to support the diagnosis of GCA;• Imaging studies are useful to delineate large-vessel involvement in GCA.• Glucocorticoids remain the cornerstone of treatment, but patients with GCArequire high doses. Treatment is urgent to avoid blindness• Synthetic immunosuppressive drugs also have a role in disease managementSalvarani C, Pipitone N, Versari A, Hunder GG. Clinical features of polymyalgia rheumatica andgiant cell arteritis, Nat Rev Rheumatol. 2012 Sep;8(9):509-21.
Post-Concussion Headache• Incidence of approximately 3.8 million sports-related concussions per year• The presence of new onset or persistent headachefollowing injury often complicates return to playdecisions.• Now evident that recurrent head trauma may beassociated with the development of some chronicneurodegenerative disorders.• Improved prevention and management of thisinjury will occur with ongoing educational andresearch efforts.Seifert TD. Sports concussion and associated post-traumatic headache.Headache. 2013 May;53(5):726-36