Chapter 9  Headache

Questions on Headache

A.            In a patient with complaint of headache, it is most important that you find

1.             In the history:

2.             In the physical exam:

B.            The commonest cause of headache seen in the office patient and its two major distinguishing characteristics are:

1.            

a.            

b.            

C.            The other two common etiologies of headache seen in the office, and their distinguishing characteristics are:

1.            

a.            

b.            

2.            

a.            

b.            

D.            Your 30 y/o patient formerly had a migraine headache once every 2-3 months.  Ten years later, she now has a headache every day.  The kind of headache she most likely has now :

 

E.             You notice that your next patient is an attractive, well-dressed young woman who coughs as she enters your next examining room.  Your nurse notes that the patient says she has “the worst headache of her life.”

1.             The first thing you do is:

2.             The likely cause of her “worst ever” headache is:

3.             The characteristics on exam that you look for to confirm your initial suspicion are:

 

F.             Your patient is a healthy 75 y/o woman with her first tension-type headache.  Your management is:

 

G.            Your 37 y/o patient has headache that is “there all the time,” that is, chronic, daily headache.  Whenever you hear this complaint, frequently there is associated:


Answers on Headache

A.            Be certain of your patient’s symptom and its distinguishing characteristics

1.             Whether it is either new headache or episodic headache, and whether its onset to peak severity is abrupt, rapid (minutes) or gradual (hours-days)

2.             Ask the patient to touch her chin to her chest in order to determine she does not have nuchal pain or rigidity, suggesting meningitis or brain hemorrhage.  You quickly should palpate the head and neck region to be certain it is a usual “headache” and that there is no significant localized tenderness suggesting facial pain, neck myofascial pain, TMJ pain, referred pain

B.            Commonest cause of headache and its distinguishing characteristics

1.             Tension-type headache (TTH)

a.             Episodic headache

b.             The headache occurs in isolation, that is without other symptoms suggesting either a noncephalic infection or a migraine

C.            The other two common causes of headache and their characteristics

1.             Migraine

a.             Episodic headache (rarely, a patient may present with the first migraine)

b.             Any one of the following symptoms: either photophobia or phonophobia or nausea and vomiting or head and body movements aggravating the headache

2.             Infection (such as common cold or other causes of fever)

a.             New onset headache

b.             Other symptoms associated with the infection–myalgias, cough, rhinorrhea, sneezing, low grade fever, arthralgias, diarrhea

D.            Chronic daily headache is very common 

 Chronic daily headache or “rebound headache” commonly is due to analgesic abuse.  Patients are difficult to treat, because it requires stopping the offending analgesics, and starting a TCA

E.             A patient with a catastrophic disease e.g. SAH, does not walk into your office

1.             Open the door, ask how she is feeling, note that she is not in distress; she is sitting, reading a magazine

2.             Localized viral infection e.g. common cold, acute bronchitis

3.             Look for other systemic symptoms of a viral infection viz rhinorrhea, low-grade fever, myalgias

F.             Clues to an organic, or even dangerous, cause of headache

Note that it is a new or different headache in a patient >50 y/o, a clue to the possibility of a dangerous disease.  If she is well otherwise, with no dangerous symptoms and normal neurological exam, the minimal test is ESR for TA

G.            Chronic daily headache commonly is associated with a psychological disorder, either MD, GAD, somatization disorder, panic disorder.  Whether the disorder is primary or not is often difficult to determine, but it makes little difference therapeutically, because you need to treat the psychological problem as well as the headache


 

Headache

 

H.        Be certain of the symptom and its distinguishing characteristics. Your patient’s problem or chief complaint is “My head hurts” or  “I have a headache”Text Box: Differential diagnosis: the 3 common causes of headache
1.Tension-type headache (TTH)–episodic; no other significant symptoms with the HA
2.Migraine–episodic; photophobia or phonophobia or GI symptoms (anorexia, nausea, vomiting) or disabled by the pain
3.Localized infection–new onset headache; concomitant symptoms of the infection e.g. URI, GE
(For chronic daily headache, see Section G.3)

1.      First, be certain the problem is pain originating inside her head.  Have your patient show you the site of pain, where it begins and where it radiates.  “Show me everywhere it hurts.”  Don’t ask her to point with one finger, but if she does, it may suggest some localized cause, such as facial pain (neuralgia or sinusitis) or ear pain (TMJ) or eye pain or myofascial neck pain.  Always inspect and palpate these areas to elicit tenderness, which would divert you from further workup of the usual “headache” 

2.      Assuming no localized tenderness, determine whether the headache is new, episodic, or chronic.  Ask her “How many different kinds of headache do you get?” and  “Now we want to talk about this specific headache you are having”

a.      Ask her, “When is the first time you had a headache anything like this one, even though it might have been much less severe?”  and “How often do you get this specific headache?”  By now you should be confident it  is headache, and it is either new or episodic or chronic, daily headache

b.      “Think back to the first time you had this specific headache. Was there anything unusual you remember that preceded the headache?  Tell me about that first episode.”   Now, “Describe how this particular headache typically occurs–what brings it on, how you treat it.”  Very importantly, “From the moment the headache begins, about how long until it becomes as severe as it is gets?”  and, “What other symptoms precede or accompany the headache?”

c.       If your patient’s headache occurs on most days of the a month, by definition she has chronic daily headache and a different approach is needed (see below, Section G.3)

3.      Physical examination–In every patient complaining of headache, always do the following:

a.      Palpate the head and face for tenderness suggesting neuralgia, acute sinusitis, or TA; palpate the neck both for lymphadenectasis and for a TrP suggesting myofascial pain syndrome, and for typical areas of bilateral tender points of fibromyalgia

b.      Ask her to touch her chin to her chest to detect meningismus; if in question about interpretation, perform a Kernig maneuver

c.       Note vital signs, especially look for diastolic BP >110, fever >101EF, and appropriate pulse rate (for relative bardycardia– fever of 102EF should have a pulse rate of about 110 bpm; 103EF, 120 bpm; 104EF, 130 bpm; 105EF, 140 bpm.  A large deviation from the expected rate suggests a cause of relative bradycardia)

d.      Do funduscopy looking for a normal pulsating vein at the edge of the cup on the disc and a normal cup/disc ratio <0.5; and for papilledema; also be alert for photophobia or nystagmus when visualizing the fundusText Box: Be certain:
4.The symptom is headache and not localized facial pain or referred pain
5.Whether it is either new onset headache or episodic headache (If it is a chronic daily headache, see Section G.3)
6.Whether its onset to peak severity was abrupt, rapid (<1 hour) or gradual
7.If there are associated symptoms of an infection (fever, myalgias, diarrhea, rhinorrhea, cough) or photophobia, phonophobia, nausea, vomiting

                   By now you should be confident it  is headache, whether it is new, episodic or chronic daily headache, and if there are significant associated symptoms with the headache.  In almost all instances you now have the information you need for diagnosis of the obvious causes (Section C) and the common causes (Section E).  In the rare instance that your patient appears different clinically from the usual office patient with headache, be certain (Section B) that she does not have a symptom or sign of a dangerous disease.

I.          Be confident it’s not a dangerous disease.

1.             Send immediately to the ED by ambulance if there is:

a.             Evidence of catastrophic illness e.g. unbearable pain, hypotension, diaphoresis, pallor

b.             Abrupt onset of a new severe headache that incapacitates the patient (even if meningismus or nuchal rigidity are absent), think of brain hemorrhage, meningitis,  encephalitis                                                                    

c.             Abrupt onset of headache with syncope or dizziness, think of cerebellar hemorrhage, basilar migraine

d.             Altered consciousness or confusion or any one focal neurological deficit, think of intracranial hemorrhage, meningitis, stroke, encephalitis, sepsis

e.             Stiff or painful neck on flexion or rectal temperature >101EF, think of brain hemorrhage, meningitis

f.              Abrupt onset of severe neck and head pain with a focal neurological sign, think of carotid artery dissection

g.             Abrupt onset of severe, explosive headache (“thunderclap headache”), think of SAH, cervicocephalic arterial dissection, cerebral venous sinus thrombosis

h.             Ill patient with fever and rash, think of meningococcemia, RMSF, sepsis syndrome, infective endocarditis, toxic shock syndrome, Stevens-Johnson syndrome

2.             Admit at once to the ED or hospital if there is:

a.             BP > 200/120 with associated mental changes and retinopathy (flame hemorrhages, exudates, papilledema), think of emergent (malignant) HTN, pheochromocytoma

b.             Unilateral abrupt loss of vision or diplopia with headache; in a patient >50 y/o, think of TA or emboli from the ipsilateral carotid; in a younger person think of pseudotumor cerebri, complicated migraine, optic neuritis; any age, think of pituitary hemorrhage

c.             New or episodic headache with symptoms suggesting acute GE (headache, vomiting, diarrhea), but no concomitant abdominal pain or hyperperistaltic bowel sounds, think of carbon monoxide poisoning

d.             Symptoms suggesting acute GE, but high fever and gradual onset of a very severe headache, think of RMSF, bacterial meningitis

e.             The “worst headache ever,” think of SAH, meningitis, RMSF (but in the office almost always it is headache due either to new migraine or noncephalic infection)

f.              Abrupt onset of severe headache concomitant with any exertion or Valsalva maneuver (cough, sneeze, coitus, straining at stool), think of SAH, tumor, Chiari malformation

g.             Abrupt onset of unilateral eye pain and decreased ipsilateral visual acuity, think of acute glaucoma

3.             Admit to the hospital or have a specialist consult as soon as appropriate if there is:Text Box: Be certain in the first minutes of your exam that there is no evidence of any one dangerous symptom or sign associated with the headache:
8.Abrupt onset of a severely painful new headache
9.Any altered consciousness or confusion
10.Nuchal rigidity or pain on flexion
11.Any one focal neurological sign
12.New or different headache in a patient >50 y/o
13.Emergent hypertension, BP >220/120
14.Diplopia or amaurosis fugax
15.Rash or fever >101EF (38.3EC)
Any suggestion of a dangerous disease, send your patient to the ED.

a.             A new headache that has been constant since onset (syndrome of “new daily persistent headache”), think of SAH, post-trauma, chronic meningitis, low CSF volume, raised CSF pressure (J Neurol Neurosurg Psych 72 (Suppl II), 2, 2002)

b.             Headache that is persistent and unilateral (always the same side) or progressively worsening headache at any age, MRI must be done to exclude any mass lesion

c.             Papilledema or loss of venous pulsation on the  optic disc, think of increased intracranial pressure due to brain tumor, subdural hematoma, pseudotumor cerebri

d.             New onset of severe orbital pain and headache with decreased ipsilateral visual acuity: if >50 y/o, think of TA, acute glaucoma; if <40-50 y/o, think of  retrobulbar (optic) neuritis, migraine, cluster headache

e.             A new or different headache in an HIV-positive patient, think of CNS opportunistic infection

f.                              A new headache in a patient with coincident, progressive dementia or with any possible history of preceding head trauma, think subdural hematoma

g.             Onset of a new or different headache in a patient >50 y/o, always get a sed rate for TA; pain is not deep, but superficial, and it may be located holocephalic, or any unilateral site e.g. temporal or occipital

h.             Episodic headache associated with “spells” of spontaneous sweating, pallor (not flushing), palpitations, think of pheochromocytoma

i.                              Unilateral persistent headache, ipsilateral tinnitus, decreased hearing, imbalance or vertigo, think of acoustic neurinoma

j.                              Nocturnal or early morning headache, think of brain tumor, OSA, severe COPD, hypercarbia of any cause

k.             New headache with secondary amenorrhea, galactorrhea, sublibido, erectile dysfunction, or visual field deficit (bitemporal hemanopsia), think of pituitary tumor

l.                              Persistent fever and headache, think of CNS tumor, chronic meningo-encephalitis, relapsing fever, rat-bite fever, brucellosis, malaria, brain abscess (get rectal temp tid)

m.            New or different headache following trauma, think of subdural or epidural hematoma

n.             Persistent fever, headache and relative bradycardia, think of leptospirosis, psittacosis, central fever, typhoid fever, malaria

J.         Make a quick diagnosis if it’s obvious.  Is it an easily diagnosed cause or a medication that is responsible for the headache?  In most patients you will be highly confident of a common cause of the symptom by now.  If not, one or more of the following tests may exclude an organic disease and increase your confidence in your working diagnosis: ESR, WBC

1.             Spot diagnoses

a.             Post-lumbar puncture headache is provoked by sitting or standing, and entirely relieved by recumbency; onset is 2-14 days after LP, and rarely it may persist for months

b.             Post-traumatic headache occurs 1-2 days following any head trauma and may persist for years; you need a CT to exclude subdural hematoma; if associated symptoms (unilateral facial sweating, dilated pupil, tender at carotid bifurcation) occur, this is dysautonomic cephalgia and it is treated with propranolol

c.             “Ice pick” headache (idiopathic stabbing headache) has jabbing pain lasting seconds in the orbit or parietal area; prompt prophylactic response to indomethacin 50 mg. tid

d.             Headache after eating certain foods

(1)           Monosodium glutamate–“Chinese restaurant syndrome” occurs 20-30 minutes after eating Chinese food with symptoms of headache, facial flushing and sweating, paresthesias, pain or discomfort in chest and abdomen, dizzinessText Box: Recognize any easily diagnosed cause of headache–many are new headaches:
16.Spot diagnosis–post-lumbar puncture, post-trauma, “ice pick” headache, myofascial pain, GYN causes, acute sinusitis, fibromyalgia, ingestion of food products e.g. nitrates
17.Medication side effect

(2)           Nitrates, nitrites– A “hot dog headache” with facial flushing soon after eating meats cured with these substances (hot dogs, sausage, bacon)

(3)           Aspartame–artificial sweetener (soft drinks, desserts, prepared foods) is a migraine trigger

(4)           Alcohol may have headache 30-45 min after ingestion, and it is a trigger for migraine and cluster headaches

e.             Myofascial pain syndrome–unilateral focal painful site (TrP) in muscle (e.g. posterior cervical muscles, sternocleidomastoid); pain or spasm prevents normal ROM; treated with injection of the TrP, then preventive therapy by exercises

f.              GYN causes are all cyclic headaches or related to menses

(1)           Premenstrual migraine occurs from 7-2 days before menses

(2)           Menstrual migraine occurs from 2 days before to 3 days after onset of menses and at no other times

(3)           Menstrual-related migraine occurs during menses and also at other times

(4)           TTH increases in frequency 1 day before to 2 days after onset of menses

(5)           During pregnancy migraine commonly decreases or disappears, but in 10% there is new onset migraine

(6)           In some patients, menopause exacerbates migraine

g.             Acute sinusitis causes headache, but chronic sinusitis is rarely a cause of headache.  Attribute a new headache to bacterial sinusitis only if there are symptoms lasting >7 days (fever, purulent or sanguinopurulent nasal discharge, maxillary tooth pain), and there is focal unilateral tenderness over the infected sinus (compared to no tenderness over the ipsilateral zygomatic arch as control site where you press with your thumb at gradually increasing pressure)

h.             Fibromyalgia–generalized aches and pains along with headache; find bilateral tender points at typical sites e.g. back of the neck, back, chest

2.             Medications–up to 5% of people given placebo in drug studies complain of a new or different headache, so any new medication can be responsible for your patient’s new headache.  In addition, any one medication may  cause new headache in an individual patient, even though the medication is not included in lists of drugs causing headache.  Look for a temporal relationship between a new headache beginning after starting a new medication.  Below is a list of medications that “cause” headache in drug trials at rates greater than placebo

a.             Vasodilators–nitroglycerin, isosorbide dinitrate, hydralazine, dipyridamole

b.             Vasoconstrictors–bromocriptine, dopamine; sympathomimetics (pseudoephedrine, dextroamphetamine)

c.             Antihypertensives–captopril, atenolol, metoprolol, propranolol, reserpine, prazosin, minoxidil, clonidine, losartan, methyldopa, hydralazine

d.             Antiemetics–domperidone, metoclopramide

e.             NSAIDs–indomethacin, diclofenac, piroxicam

f.              Antidepressants–SSRIs, trazodone

g.             Triptans–after extended daily use; class effect

h.             $-agonists– terbutaline

i.              H2-receptor antagonists–cimetidine, ranitidine

j.              PPI– class effect

k.            CCB– nifedipine, verapamil

l.              Antiepileptic drugs–gabapentin, lamotrigine, tiagabine, barbiturates

m.            Hormones–contraceptives, danazol, estrogens, clomiphene, growth hormone, progestens e.g. Norplant-Z

n.             Anxiolytics–lorazepam, buspirone, zolpidem, hydoxyzine, benzodiazepines

o.             Antibiotics–trimethoprim-sulfamethoxazole, griseofulvin, itraconazole, tetracyclines

p.             Miscellaneous–all trans retinoic acid, cilostazol, interferon, sibutramine, sildenafil, amiodarone, rosiglitazone, thalidomide, azelastine, budesonide, cocaine, dipyridamole, atorvastatin, nicotinic acid (including Niaspan), aminophylline, theophylline, digoxin, quinidine, erythropoietin

q.             Withdrawal headaches due to glucocorticoids, clonidine, propranolol, vasodilators (caffeine, alcohol, ergots, amphetamines, methysergide)

K.        Identify your patient’s worries and wants. Headache occurs annually in 90% of people, and only a few of these consult about it.  During your interview and exam, try to intuit or discover why your patient is here today, and not previously, for this problem.  Don’t necessarily ask directly, but try to get some ideas from your patient’s responses to questions about the headache itself.  You can’t completely help your patient unless you know what she really worries about and wants from you.  That’s why she is here to see you--to satisfy her worries and wants!  Here are only some of the possible worries and wants of your patient with headache.  You must discover her specific ones

1.             Common worries are:Text Box: During your exam, discover your patient*s worries (brain tumor, stroke) and wants (pain relief, assurance the headache is benign), so that you can respond to her specific needs as soon as possible.

a.             That the cause of the headache is a dangerous etiology e.g. brain tumor, stroke, severe systemic HTN, ruptured cerebral vessel, meningitis

b.             That the etiology is some rare disease e.g. WNV encephalitis, toxic chemicals

c.             What other fears or anxieties might be troubling your patient?  For example, she may have had TTH for many years, and only now does she come for help.  What has changed?  Perhaps a friend or relative, who had similar episodic headaches, recently has been diagnosed with a brain tumor.  Many other possibilities exist, so think of them

2.             Your patient wants:

a.             You to perform a thorough physical exam focused on possible causes of the headache and she wants appropriate tests and imaging studies

b.             A plausible explanation of the cause of the headache

c.             Confident reassurance from you that it is not a dangerous or rare etiology.  Tell her–“The good news, it’s not a brain tumor” (or whatever you believe her real worry is)

d.             To know the prognosis, short term (effects on job, home life, marriage; how soon she will be back to normal) and long term (if recurrences are possible; how to prevent recurrences; if it will cause any disability)

e.             Treatment to stop the headache now and to prevent recurrences e.g. propranolol for migraines

f.                              A specialist referral (neurologist) if she believes you are not confident of the diagnosis

L.         Know the symptoms that differentiate the 3 common causes of headache.

1.             The commonest cause is tension-type headache (TTH)

a.             Characteristics of TTH

(1)           Episodic headache

(2)           It occurs in isolation, that is, no other significant associated symptoms are present that repeatedly occur with the headache, unless the headache is just one feature of another disorder, e.g. MD, GAD, somatization disorder, fibromyalgia

(3)           TTH is never disabling, and the usual analgesics (aspirin or acetaminophen) relieve the headache for at least a few hours

b.             What suggests it might not be TTH

(1)           Anytime there is one associated symptom or sign suggesting a dangerous cause (see above, Section B)

(2)           If the headache is new and accompanied by systemic symptoms of a localized infection; or there is photophobia or phonophobia or GI (anorexia, nausea or vomiting) or worsening of the headache by head movements; or the headache  disables her

(3)           If it definitely is a new or different headache in an HIV-positive patient or in a patient >50 y/o, never attribute it to TTH without further studies e.g. ESR, imaging studies

2.             Common look-alikes and how each differs from TTH

a.             Migraine

(1)           It also is episodic and it has gradual onset (<1-2 hours) to peak severity

(2)           Two major types of migraine headaches exist: “migraine with aura” (formerly called classic migraine) is less common, and it is recognized by visual (flashing lights, scotomata) or sensory (unilateral paresthesias) phenomena preceding or accompanying the headache. “Migraine without aura” (formerly called common migraine) accounts for the overwhelming majority of patients with migraine.  Although migraine is described as very painful and often throbbing, starts unilaterally and may then affect the whole head, and begins on one side or the other at different times, none of these characteristics helps you separate it from TTHText Box: Common causes of most headaches in the office patient are:
18.Tension-type headache (TTH)–episodic; no other significant symptoms with the HA
19.Migraine–episodic; photophobia or phonophobia or GI symptoms (anorexia, nausea, vomiting) or disabled by the pain
20.Localized infection–new onset headache; concomitant symptoms of the infection e.g. URI, GE

(3)           What does differentiate migraine from TTH?  Migraine does not occur in isolation from other symptoms.  One or more of the following presents with the headache–sensitivity to light (photophobia) or sensitivity to loud sounds (phonophobia) or sensitivity to movements (especially moving the head), or GI of anorexia or nausea or vomiting

(4)           Again, different from TTH, migraine headaches at onset do not occur daily, and only rarely, even weekly; commonly they occur monthly,  or even less often

(5)           Different from TTH, the patient with migraine is disabled from the pain–she does not work through it. Rather, she seeks a dark, quiet room to sleep (to avoid lights, sounds, movements), after which the headache often is relieved within 24 hours

b.             Headache associated with localized infection

(1)           It is a new headache, not an episodic headache like tension-type headache or migraine. In winter, these may account for up to 30% of headaches in the office

(2)           It always is accompanied by some other symptoms of a localized infection (sneezing, rhinorrhea, myalgias, sore throat, cough, fever, diarrhea, abdominal pain, vomiting, dysuria) e.g. URI, acute bronchitis, GE, UTI

(3)           There may even be photophobia, nausea and vomiting as seen with migraine, or the headache may be so severe that she says  This is the worst headache I’ve ever had,” and it may be a throbbing pain over the whole head, suggesting migraine or something even worse.  But the important differentiating points are the systemic symptoms of a localized infection that accompany the headache

 

M.       Use this summary for efficient diagnosis of a common cause. With the information from Sections A-E in mind, here is the rapid approach to diagnosis of a new or episodic headache.

1.             You already have determined:

a.             In Section A, by H&P, that it  is headache and not localized pain or referred pain.

b.             In Section B, that there is no evidence in the H&P of any one dangerous cause of the headache.

c.             In Section C, that there is neither an easily diagnosed cause nor a medication that is likely responsible for the headache.

2.             From the focused H&P almost always you should quickly be confident of a working diagnosis.  Confident final diagnosis is made only in followup the next few days or weeks when the response to treatment or nontreatment is appropriate to your working diagnosis.

a.             If it  is a new headache, look for associated systemic symptoms of an infection, such as myalgias, sneezing, coughing, fever, sore throat, rhinitis, dysuria, diarrhea, vomiting.  If such symptoms are present, almost always the diagnosis is headache secondary to an acute  infection.

b.             If it is an episodic headache and there is either photophobia or phonophobia or gastrointestinal symptoms (anorexia, nausea, vomiting) or aggravation of headache on head movements, then the diagnosis most likely is migraine headache.

c.             If it is an episodic headache and it occurs in isolation, that is, there are no symptoms suggesting either localized infection or migraine, then the patient most likely has TTH.

3.             Answer your patient’s worries and wants that you determined early on during the exam (Section D).  Have you done everything you can to help her?

4.             You now have a working diagnosis, but only with followup can you be highly confident it is correct.  In return visits you are always testing by H&P that your working diagnosis is correct.

 

N.        Abandon your working diagnosis and look for an uncommon or rare cause only if:

1.             Any one dangerous symptom or sign (see above, Section B) occurs in the following days, weeks, or months.  Quickly obtain a neurology consult or send the patient to the hospital as appropriate

2.             The headache does not respond to treatment as you expect or the natural history of your working diagnosis does not occur.  Patients with each of the three common causes (TTH, migraine, localized infection) should be “well” or back to their usual health when the headache is absent.  Headache associated with infection should get better at about the same time the other systemic symptoms fade.  The patient with migraine should be well when the headache is gone, almost always in 24-36 hours.   Although the patient with TTH may get frequent headaches, the headache should be relieved with aspirin or acetaminophen (1000 mg every 4-6 hr) and the patient otherwise well when the headache is absent

Text Box: If your patient’s symptoms do not respond as expected to your management, he may have an uncommon or rare cause listed in Section G.2 that you can diagnose and treat.  Or you may want to refer your patient to a specialist.                 Don’t spend time and money looking for one of the following etiologies until you are sure your patient doesn’t have one of the 3 common causes of episodic or new headache above.  If she doesn’t have one of those causes, it may be that the correct cause was one of the uncommon etiologies listed below.  These are important to recognize, because each has different treatment or prophylaxis.

a.             Headaches associated with autonomic symptoms viz lacrimation, nasal congestion, rhinorrhea, conjunctival injection, ptosis (Brain 120, 193, 1997):

(1)           Cluster headache is uncommon, perhaps one for every 500 patients with TTH.  They are episodic, with rapid onset to peak severity in <15 minutes; they are excruciatingly painful (“sharp, ice-pick”), occur mostly in men with onset <50 y/o, most last <3 hours, may occur 1-3 times daily, may recur in “clusters” for 4-6 weeks, and then remit for months or years.  The pain lasts such a short time that the patient rarely is seen in the office or the ED during the attack.  The pain is strictly unilateral and especially periocular.  Whereas the patient with migraine refuses to move about because it aggravates the pain, the cluster patient cannot sit still.  Correct diagnosis is important because there is effective treatment (oxygen) as well as preventive therapy (verapamil)

(2)           Paroxysmal hemicrania may be episodic or chronic headaches, last <1 hour and they are treatable with indomethacin

(3)           Short lasting unilateral neuralgiform headaches with conjunctival injection and tearing (SUNCT) syndrome has unilateral orbital or temporal pain lasting <2 min; no known specific treatment

(4)           Hemicrania continua is a unilateral headache that is “there all the time” and it frequently is associated with idiopathic (“ice pick”) stabbing headaches; prompt response to indomethacin 

b.             Orthostatic headaches occur with POTS (no orthostatic hypotension, but pulse rate on standing increases >30 bpm and absolute pulse rate is >120 bpm;  Neurology 61, 980, 2003)

c.             Hypnic headache occurs only at night.  It is a benign headache with onset almost always only in elderly patients.  The patient awakens with a pulsating headache that lasts <1-2 hr.  It may recur that night and then occurs at about the same time most nights.  Lithium at bedtime can prevent it

d.             Cervicogenic headache–In a patient >50 y/o, a new or different headache with characteristics that suggest a TTH may be due to OA (spondylosis) of the cervical spine.  Pain radiates from C1-3 vertebrae to the occipital and parietal regions as a dull ache, usually unilaterally.  Don’t think of cervicogenic headache if it has been gradually worsening, wakes her at night or has other danger signs. Most patients >50 y/o have cervical spine changes on imaging, so positive radiological findings are not helpful, i.e. many false positives.  Some respond to treatment with physical therapy exercises or steroid injections

e.             Carotodynia–not truly headache, but it may be confused with headache; pain is unilateral at the upper anterior neck and there is tenderness over the ipsilateral carotid or on swallowing

f.              Systemic diseases with associated headache:

(1)           Cardiovascular–during an attack of angina pectoris (“cardiac cephalgia,” Neurology 49, 813, 1997), after carotid endarterectomy, severe aortic insufficiency, chronic CHF

(2)           Pulmonary–sarcoidosis (granulomatous meningitis), any cause of hypercapnia, e.g. COPD, OSA, polycythemia, high altitude, any cause of hypoxia

(3)           Kidney–CRF, during or following dialysis, anemia

(4)           Endocrine–hypoglycemia, hypercalcemia, prolactinoma, “empty sella” syndrome

(5)           Immune-mediated disorders (collagen-vascular)–vasculitis, SLE, scleroderma, PAN, Wegener’s granulomatosis

3.             Chronic daily headache (CDH)–approach to diagnosis of its cause.  CDH means she has headache on most days of the  month for the previous few months.  Often the headache is described as being “there all the time”

a.             First, be certain she does not have a dangerous cause (see Section B) including “new daily persistent headache” (see above, Section  B.3.a), which demands vigorous workup for secondary causes

b.             The majority of CDH are chronic TTH or chronic (“transformed”) migraine, and most become CDH because of analgesic abuse.  Any analgesic taken multiple times daily in anticipation of a headache can be causative.  Patients frequently take the analgesic first thing in the morning and regularly through the day before they have a headache.  The  “rebound” headache occurs a few hours after taking the analgesic, that is, when the analgesic “wears off”

c.             Any one of the above causes of headache in Section G.2 may be a cause of CDH.  A convenient classification of CDH is the following (Neurology 47, 871, 1996):

(1)           Primary CDH

(a)           Headache duration >4 hours

(i)            Chronic migraine–70-80% of all CDH; as the migraine becomes more frequent, i.e. daily, the intensity of the characteristic symptoms of photophobia, phonophobia, vomiting, decrease or disappear.  Except for the history of migraine, or occasional migraine, the daily headache becomes indistinguishable from chronic TTH

(ii)           Chronic TTH

(iii)         New daily persistent headache (See Section B.3.a)

(iv)          Hemicrania continua

(b)           Headache duration <4 hours

(i)            Cluster headache

(ii)           Paroxysmal hemicrania

(iii)         Hypnic headache

(iv)          Idiopathic stabbing headache

(2)           Secondary CDH

(a)           Post-traumatic headaches

(b)           Cervical spine disorders

(c)            Intracranial disease–vascular and nonvascular

(d)           Extracranial disease–TMJ, sinus infection

d.             Anytime problems with sleeping are prominent in a patient with CDH think of OSA (30% are not obese; headache often occurs on awakening)

e.             CDH often is associated with a psychological disorder, even though it may not be causing the headache.  Still, treat the psychological problem along with headache therapy

(1)           MD–look especially for associated anhedonia, that is, unexplained decreased “appetite” for any one of her formerly pleasurable activities (food, drink, sex, hobbies, job, sports, music).  Helpful to find is associated depressed mood, early morning insomnia, mood swings, negativism, but if she does not have anhedonia as described above, MD is unlikely 

(2)           GAD–she worries almost constantly about some future event that could happen, but it is highly unlikely; she just can’t stop worrying about it, and the worrying interferes with her daily functioning

(3)           Somatization disorder--she has pain in a number of other organ systems along with headache

4.             New or different headache in a patient >50 y/o

a.             In 3% of all patients who get migraines, onset of the migraine will be >50 y/o.  For either TTH or cluster headache, the percentage of late onset headaches is 10%

b.             Even though the following causes of headache are uncommon, the incidence in patients >50 y/o increases markedly

(1)           Dangerous diseases–TA, acute glaucoma, exploding head syndrome, cerebrovascular disease, cardiac cephalgia, intracranial mass lesions (tumors, hematomas, infections)

(2)           Medication side effects, cervicogenic headache, hypnic headache, systemic diseases (see Section G.2.f)

O.        Use the glossary for precise thinking and accurate diagnosis.

1.             Amaurosis fugax (Greek amaurosis meaning “dark” or “obscure” and fugax, “swift.”)   Rapid onset, but transient total or partial blindness in one eye                                               

2.             Analgesic (Greek an meaning “without” and  algesis, “sense of pain.”) A medication that suppresses pain perception.

3.             Anorexia (Greek an meaning “lack of” and orexis, “appetite.”) Literally, lack of appetite; same meaning today, just as in Hippocrates’ time.

4.             Aura (Latin word meaning “a breeze or wind.”) Now used as a premonitory sign of changes to come, as a heightening breeze signals a change in the weather; aura of migraine headache.

5.             Carotidynia or carotodynia (Greek karotis meaning “deep sleep” and odyne, “pain.”) Ancients noted that deep pressure on the carotid artery, and not other arteries, produced “deep sleep.”

6.             Cephalgia (Greek kephale meaning “head” and algos, “pain.”) Another word for headache; as is cephalodynia (Greek odyne, “pain.”)

7.             Cervicogenic (Greek cervix meaning “neck” of anything  (including body, uterus, bladder) and gennan, “to produce.”) Headache produced by neck pathology

8.             Diaphoresis--ancient Greek writers used the same term for “profuse sweating”

9.             Glaucoma (Greek glaukoma meaning a “silvery swelling or tumor.”) Ancients applied the term to a group of degenerative eye diseases, all causing a dense lens opacity or cataract; later distinction between cataracts and degeneration inside the eye due to high intraocular pressure.

10.           Hemicrania (Greek hemi meaning “half” and kranion, “skull.”) A headache affecting one side of the head, but a different side at different times, in the particular case of migraine.

11.           Hypercapnia (Greek hyper meaning “above, excessive” and kapnos, “smoke.”) It refers to excess carbon dioxide in blood; a synonym is hypercarbia.

12.           Hypnic (Greek hypnos meaning “sleep.”) Anything that induced, or pertaining to, sleep.

13.           Meningismus (Greek meninx meaning “membrane” especially meninges.)  Now it refers to symptoms and signs of meningeal irritation with an acute febrile illness, but not actual infection of the meninges, which would be “meningitis.”

14.           Migraine (Greek word for “hemicrania.”) It was Latinized to migraena, and later to French, migraine.

15.           Miosis (Greek meiosis meaning “a lessening.”) Lessening of anything, including the size of the pupil, or pupillary constriction.

16.           Neuralgia, trigeminal (Greek neur meaning “nerve” and algia, “pain.”) Paroxysms of severe, stabbing pain along the distribution of the involved nerve.

17.           Nuchal (Arabic nukha meaning “back of the neck.”)

18.           Paresthesias (Greek para meaning “along with” and aisthesis, “perception.”) An abnormal feeling, such as numbness or tingling, in addition to normal perception

19.           Paroxysmal (Greek paroxysmos meaning “abrupt or rapid onset.”)  Applied to symptoms that occur as a group and that occur episodically. 

20.           Phonophobia (Greek phone meaning “voice” and phobos, “fear.”) Avoidance of loud sounds that aggravate the pain of migraine headache.

21.           Photophobia (Greek phos meaning “light” and phobos, “fear.”) Painful sensitivity to the usual intensity of light.

22.           Pleiocytosis, pleocytosis (Greek pleon meaning “more, excessive” and cytosis, “cells.”)  CSF containing more than 5 mononuclear cells or any RBC or PMN.

23.           Protean (Greek sea god, Proteus, who could change his appearance at will.)  We use the term for any disease with many varied appearances clinically, e.g. syphilis, AIDS, connective tissue diseases.  In contrast to pronunciation of the word “protein,” all the syllables in “protean” are pronounced.

24.           Ptosis (Greek word meaning “a falling.”) Now it refers only to a drooping eyelid.                                                                   

25.           Scotomata, plural of scotoma (Greek skotos meaning “darkness or gloom.”) It is a blind spot or focal area of decreased acuity in the visual field.

26.           Stroke (derived from the Greek word apoplexia meaning “seized by being struck down” by the gods as punishment.)  Stroked or struck down persists in our English usage–we’ve just dropped the gods.

27.         Teichopsia (Greek teichos meaning “city wall,” like a crenellated fortress, and opsis, “vision.”) Now it refers to a scintillating scotoma or flashing lights resembling jagged lines.