Course 11- Headaches

Nelson Hendler, MD, MSFormer Assistant Professor of Neurosurgery Johns

Hopkins University School of MedicinePast president-American Academy of Pain

Management

Various Types of Headaches• Incidence of Migraine Headache-US Armed

forces 1998-2010 (1)• 3% of all men, 6% of all female with migraine, • 3.9% of men, 11.3% female with some sort of

headache• 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 diagnostic codes for migraine and other types of headaches, active component service members, 1998-2010.

Types of Headaches

• Most common headaches are:• Muscle Tension Headache• Migraine-common and classic• Trigeminal• Cluster• Chronic daily headache

Robbins MS, Lipton RB. The epidemiology of primary headache disorders.Semin Neurol. 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 a

trigeminal autonomic cephalalgia (TAC).• TACs include episodic & chronic cluster headache, episodic

& chronic paroxysmal hemicrania, SUNCT, & hypnic headache.

• If duration is > or =4 h, then CDH & differential diagnosis is chronic migraine, chronic tension-type headache, new daily persistent headache and hemicrania continua.

Bigal ME, Lipton RB. The differential diagnosis of chronic daily headaches: an algorithm-based approach, J Headache Pain. 2007 Oct;8(5):263-72.

Other Classifications

• Most common is muscle tension, then migraine, 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 descriptions

Monti AA, Rapela FJ, Bacile Bacile EA, Uberti D. Primary cephaleas. A revision of their diagnosis and therapeutic approach, Rev Fac Cien Med Univ Nac Cordoba. 2006;63(3):53-64.

Yet another classification • Chronic Daily Headache (CDH) defined as headache 15

days a month, for at least 3 months, lasting more than 4 hours. Not a diagnosis –merely a description

• CDH has 4 subtypes:• Chronic migraine, chronic tension type headache, new

daily persistent headache and hemicrania continua-again not diagnoses but descriptions

• Chronic migraine affects about 2% of population• Chronic migraine sufferers are 70% more likely to have

had strokeLipton RB. Chronic migraine, classification, differential diagnosis, and epidemiology. Headache.

2011 Jul-Aug;51 Suppl 2:77-83

Why Headaches Should Be Classified Based 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 to properly repair the tire. This is a DIAGNOSIS

• A single cause (DIAGNOSIS) may have multiple clinical manifestations, such as syphilis, Lyme disease, occlusive stroke, etc.

• Defining the origin allows a doctor to treat the causes and address multiple symptoms

Diagnostic Tests for Headache-I• Headaches are one of the most common symptoms and the list

differential diagnoses has over 300 different types and causes• The cause of most headaches can be determined by a careful history

and neurologic examination• The yield of 3026 neuroimaging scans in the evaluation of patients

with 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 chronic ischemic 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 of migraine, 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 scans

performed 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 spectrophotometry in the CSF at various times after a subarachnoid hemorrhage is: 12 hours, 100%; 1 week, 100%; 2 weeks, 100%; 3 weeks, more than 70%; and 4 weeks, more than 40%

• 30% to 90% of patients have various types of headaches after mild head injury. 1% to 3% of these patients have life-threatening pathology, including subdural and epidural hematomas, that are detected on CT and MRI scans

• Headaches are the most common symptom of Temporal Arteritis, reported by 60% to 90% of patients

Evans RW. Diagnostic testing for the evaluation of headaches.Neurol Clin. 1996 Feb;14(1):1-26.

Headache Classification- A meta analysis of all causes

Migraine –vascular origin

Common-no aura Classic-with aura

Muscle Tension

No secondary vascular entrapment

With vascular entrapment

With Nerve entrapmentNerve Compression

C2 Entrapment

Trigeminal neuralgia from intracranial 5th nerve compression

Cluster Headache

Chemical -tyramine, caffeine, etc.

Cable Frame Sunglasses Infectious

Post-herpetic trigeminal neuralgia Viral encephalitis

Stroke

SinusBRAIN TUMORS -CNS COMMPRESSION

Occipital NeuralgiaTemporal Arteritis

Post-concussion

Common Migraine Headache• Outstanding research from Denmark clearly

demonstrates that intra-cranial arteries on the side of the pain are more dilated during migraine attack, while extra-cranial arteries are not

• This clarifies the patho-physiology of common migraine

• 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. Magnetic

resonance angiography of intracranial and extracranial arteries in patients with spontaneous migraine without aura: 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 region

of the skull• No aura associated with the headache, i.e. no

antecedent nausea, no visual flashes, no unusual smells or tastes

• Described as sudden pain onset, which in 5 to 30 minutes which goes to a throbbing, pounding pain

• May last hours to a full day• If more frequent than once a week, use

prophylactic treatment, less frequently, use abortive treatments

Abortive Therapy for Common Migraine• Two types of therapy for common migraine• Abortive: used when migraines occur more

than once a week.• Prophylactic: used when migraines occur

more frequently than once a week• When steroids are added to standard abortive

therapy for migraine headaches, they are effective and safe for preventing moderate or severe headache recurrence.

Huang Y, Cai X, Song X, Tang H, Huang Y, Xie S, Hu Y. Steroids for preventing recurrence of acute severe migraine headaches: a meta-analysis Eur J Neurol. 2013 Apr 11.

Abortive Therapy for Common Migraine• Sumatriptan constricts extracerebral arteries

more than cerebral arteries, suggesting that sumatriptan may exert its anti-migraine action outside of the blood-brain barrier.

• Amin FM, Asghar MS, Ravneberg JW, de Koning PJ, Larsson HB, Olesen J, Ashina M, The effect of sumatriptan on cephalic arteries: A 3T MR-angiography study in healthy volunteers. Cephalalgia. 2013 Apr 15

• Other abortive treatments are oxygen which is minimally effective .

• Bennett MH, French C, Schnabel A, Wasiak J, Kranke P. Normobaric and hyperbaric oxygen therapy for migraine and cluster headache.Cochrane Database Syst Rev. 2008 Jul 16;(3)

• Aspirin, caffeine, non-steroidal anti-inflamatory drugs, & 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 more than once a week, then use prophylactic drugs

• Topiramate and valproic acid, both anti-convulsants, have been used equally effectively to reduce the number and severity of frequent common migraine

• Krymchantowski AV, Jevoux CC Topiramate vs divalproex sodium in the preventive treatment of migraine: a prospective "real-world" study. Headache. 2011 Apr;51(4):554-8.

• Propranolol, a beta blocker alone or in combination with amitryptiline, an anti-depressant have been used successfully for a number of years

Classic Migraine Headache• This has all the features of common migraine

headache, with other added features• Unilateral, usually in temple or parietal region• An aura event proceeds the headache• Auras may be flashing lights, nausea, unusual

smells or tastes, • Headache is throbbing, pounding, associated with

photopobia (fear of light) and phonophobia (fear of sound)

• May be relieved by vomiting, abortive medication

Chemically Induced Headache• Two major types-Tyramine and Cogeners• Tyramine is found in hotdogs, Cheddar cheese,

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 Freitas Gas

Chromatography in the Analysis of Compounds Released from Wood into Wine http://cdn.intechopen.com

Mixed Muscle Tension-Vascular Headaches• The nerves, arteries and veins pass through thick

fibrous fascia, and thick muscle tissue at the back of the neck –see the next slide

• When a muscle goes into spasm, from stress or injury, it gets shorted and thicker

• As the opening between the muscles is constricted, by the thickened muscle, this compresses the vessel which passes between, causing vessel spasm

• So the clinical presentation is a muscle spasm then about ½ hour later, a throbbing, pounding headache

• Treatment is muscle relaxers, not vascular drugs

Mixed Muscle Tension –Vascular Headache- Note how the blood supply passes through layers of heavy muscle

Cable Frame Sunglasses Headache• Many types of headache are caused by

compression of either arteries or nerves (1)• One of the easier to diagnosis is a headache at the

back of the ear,radiating up the side of the head, into the temple

• The onset of headache is precipitated by wearing cable frame sunglasses with a spring loaded earpiece to grip the ears, and not fall off, such as made by Rayban (my sunglasses of choice)

• Treatment is bending the ear pieces or padding (1) Krymchantowski AV. Headaches due to external compression, Curr

Pain Headache Rep. 2010 Aug;14(4):321-4

Headache due to Cable Frame Sunglassescausingcompression of the lesser and greater auricular nerve, with pain at the back of the ear, and back of the neck. Obviously, treatment is to get a pair of sunglasses which do not compress the nerve

Compression Points

Occipital Neuralgia http://www.hopkinsmedicine.org/neurology_neurosurgery/specialty_areas/

headache/conditions/occipital_neuralgia.html

• Irritation of occipital nerves anywhere along their course can cause a shooting, zapping, electric, or tingling pain with symptoms located on the back of the scalp (see next slide)

• Occipital neuralgia can be diagnosed—and temporarily treated—by an occipital nerve block

• In patients who do well with temporary “deadening” of the nerve, a more permanent procedure includes cutting the nerve surgically, “burning” the nerve with a radio-wave probe, or eliminating the nerve with a small dose of an injected toxin

OCCIPITAL NEURALGIA-pain starts at the back of the neck at the upper spine, and radiates up the back of the neck like a stripe, usually ending at the top of the head.

PAIN

C2 Entrapment & Other Cervical HeadachesLong, DM, Davis, RF, Speed, WG, and Hendler, NH, Fusion for Occult Posttraumatic Cervical

Facet 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 the eyebrow or the eye, is cervical in origin

• Effective diagnostic procedures were facet blocks, root blocks, and provocative discogram

• When properly diagnosed, a posterior cervical fusion was performed C1-C3 or C2-C4, or other combinations

• 79% of patients had complete relief, and 14% had satisfactory improvement

From:"Cervical Spine Trauma", Dr. John J. Carbone, M.D., Johns Hopkins University, Presented at the Johns Hopkins Orthopaedic Review Course, Baltimore, June, 2003. -- http://www.netorthodoc.org/1025/1025pp.pdf

Cervical Facet Blocks confirms, on a physiological basis, the cause of the pain.

If a provocative discogram is also positive, this confirms the anatomical picture, and a posterior fusion is performed

Ice Cream Headache (Brain Freeze)• 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 unusually

cold substance, consumed rapidly, passes over the palate and back of the throat

• Constriction or spasm of the sphenopalatine artery is thought to be the cause

• Treatment is running your tongue rapidly, for 60 seconds, on the roof of your mouth, to warm it

http://www.hopkinsmedicine.org/neurology_neurosurgery/specialty_areas/headache/conditions/ice_creamEditor_headache.html

Letter to the Editor, Slurpee Headache, due to Sphenopalatine Artery Spasm, New England Journal of Medicine

Hendler’s Ice Cream- The largest selling ice cream in the State of Maryland, from 1905 until 1970. Hendler’s Ice Cream was sold to Borden’s Ice Cream

Symptoms of Cluster Headache• excruciating severe stabbing and boring one sided

pain located in the temple and the orbit• The attacks can be associated with conjunctival

injection 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 neurological

examination are normal • unenhanced brain computed tomography had been

normalEdvardsson B, Persson S.Cluster headache and parietal glioblastoma multiforme. Neurologist. 2012

Jul;18(4):206-7

Cluster Headache Treatment? • The pain and autonomic symptoms of cluster

headache (CH) result from activation of the trigeminal parasympathetic reflex, mediated through the sphenopalatine ganglion (SPG)

• The efficacy of on-demand SPG stimulation for chronic cluster headache was tested

• Pain relief was achieved in 67.1% of full stimulation-treated attacks

• 36% had a ≥50% reduction in attack frequency Schoenen J, Jensen RH, Lantéri-Minet M, Láinez MJ, Gaul C, Goodman AM, Caparso A, May A. Stimulation of

the 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 of

pain and pressure sensation around the sinuses and periorbital area; most are misdiagnosed with sinusitis

• The aetiology of “sinus headache” in patients with negative endoscopic or computed tomography (CT) scan findings was allergic & vascular abnormalities

• Nasal obstruction, nasal discharge and sinus sensitivity are seen in other types of headaches

Kaymakci M, Cikriklar HI, Pay G.The aetiology underlying sinus headaches. J Int Med Res. 2013 Feb;41(1):218-23.

Headache from Encephalitis• Patients with viral infections of the central nervous

system (CNS) may present with a variety of neurological symptoms, most commonly dominated by either encephalitis or meningitis.

• The encephalitis headache is all over the head• Often it is accompanied by a low grade fever, some

photo sensitivity, and malaise• Sometimes features are non-specific• A spinal tap may prove a definitive diagnostic test Studahl 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: diagnosis and 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 more

types 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 was significantly 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 of intracranial aneurysms, J Headache Pain. 2013 Feb 20;14(1):15

Headache due to Brain Tumors• Sudden-onset headache is a common and often

alarming presentation. The location varies• If the headache persists, then more testing is

needed –CT, MRI, MRA, EEG and PET • A small proportion may be indicative of a

catastrophic etiology. Autopsy reports indicate: • Vascular events 60.4% ,primary brain tumours/cysts

16.7% , and meningitis 6.25%. • Aneurysms accounted for the majority of vascular

cases 22.9% Lynch KM, Brett F.Headaches that kill: a retrospective study of incidence, etiology and clinical features in

cases of sudden death. Cephalalgia. 2012 Oct;32(13):972-8.

Temporal Arteritis (Giant Cell Arteritis)• Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory

diseases 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 the

branches of the aortic arch. • Clinical manifestations of GCA may be a new headache, palpable tender vessel on

the 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 inflammatory

markers 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 GCA

require 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 and

giant 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 headache

following injury often complicates return to play decisions.

• Now evident that recurrent head trauma may be associated with the development of some chronic neurodegenerative disorders.

• Improved prevention and management of this injury will occur with ongoing educational and research efforts.

Seifert TD. Sports concussion and associated post-traumatic headache.Headache. 2013 May;53(5):726-36