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Headache Insights for Neurologists

1. The document discusses the modern classification of headaches by the International Headache Society in 1988, which categorized 13 main types of headaches including migraine, tension-type headache, and cluster headache. 2. It provides details on the classification of migraine headaches, which affect 18-29% of women and 6-20% of men. Migraine attacks typically involve prodrome, aura, headache, resolution, and postdrome phases. 3. Visual auras are the most common type of aura experienced before the headache phase in classic migraines, and can involve sensations like flashing lights or fortification spectra illusions. The pulsating headache pain is usually unilateral.

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Ion Ungureanu
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0% found this document useful (0 votes)
102 views53 pages

Headache Insights for Neurologists

1. The document discusses the modern classification of headaches by the International Headache Society in 1988, which categorized 13 main types of headaches including migraine, tension-type headache, and cluster headache. 2. It provides details on the classification of migraine headaches, which affect 18-29% of women and 6-20% of men. Migraine attacks typically involve prodrome, aura, headache, resolution, and postdrome phases. 3. Visual auras are the most common type of aura experienced before the headache phase in classic migraines, and can involve sensations like flashing lights or fortification spectra illusions. The pulsating headache pain is usually unilateral.

Uploaded by

Ion Ungureanu
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPT, PDF, TXT or read online on Scribd

HEADACHE AND OTHER

CRANIOFACIAL PAINS

Prof. M. Gavriliuc,
Department of Neurology, Medical
and Pharmaceutical Nicolae
Testemitsanu State University,
Republic of Moldova
MODERN CLASSIFICATION of HEADACHE
1. Migraine
2. Tension-type Headache
3. Cluster Headache and Chronic Paroxysmal
Hemicrania
4. Miscellaneous headaches unassociated with
structural lesions
5. Headache associated with head trauma
Most of the research work 6. Headache associated with vascular disorders.
published in the past was 7. Headache associated with non-vascular
difficult to interpret because intracranial disorder
there were no clearly defined 8. Headache associated with substances or their
criteria established for the withdrawal
diagnosis of different types of 9. Headache associated with non-cephalic
headache. This problem was infections.
addressed by the 10. Headaches associated with metabolic disorders
International Headache 11. Headache or facial pain associated with disorder
Society (IHS) in 1988 when of cranium, neck, eyes, ears, nose, sinuses,
the "Classification and teeth, mouth or other facial or cranial structures.
Diagnostic Criteria for 12. Cranial neuralgias, nerve trunk pain and
Headache Disorders, Cranial deafferentation pain
Neuralgias and Facial Pain" 13. Non-classifiable Headache
was published.
Pain-Sensitive Cranial Structures
Only certain cranial structures are sensitive to pain:
(1) skin, subcutaneous tissue, muscles,
extracranial arteries, and periosteum of the
skull; (2) delicate structures of the eye, ear,
nasal cavities, and sinuses; (3) intracranial
venous sinuses and their large tributaries,
especially pericavernous structures; (4) parts of
the dura at the base of the brain and the arteries
within the dura and pia-arachnoid, particularly
the proximal parts of the anterior and middle
cerebral arteries and the intracranial segment of
the internal carotid artery; (5) the middle
meningeal and superficial temporal arteries; and
(6) the optic, ocular motor, trigeminal,
glossopharyngeal, vagus, and first three cervical
nerves. Interestingly, pain is practically the only
sensation produced by stimulation of these
structures; the pain arises in the walls of small
blood vessels which contain pain fibers (the
nature of vascular pain is discussed further on).
Migraine
Epidemiology
Contemporary data show that the
frequency of migraine is much higher
than suggested by earlier studies—18%
to 29% in women and 6% to 20% in
men (Stewart et al 1992). The first bout
evolves prior to age 40 years in about
90% of patients, with approximately half
of the cases appearing during childhood
or adolescence (Selby and Lance
1960). Before puberty, the prevalence
of migraine is slightly higher in boys
than in girls. Prevalence increases until
approximately age 40, after which it
declines.
Adult women are at greater risk for the development of migraine than adult
men, but estimates for the female-to-male ratio vary from approximately
two to one to approximately three to one. Marital situation, intelligence,
educational level, occupational category, and employment situation are
not correlated with migraine, but it appears that migraine is more frequent
in individuals from lower income groups (Stewart et al 1992).
Migraine
A bout of migraine may commence
at any time of the day or night. An
intense pounding headache may
awaken some individuals from
sleep; other individuals detect a
headache on awakening. Still
others develop an episode
gradually as the day progresses.
The frequency of attacks also
differs among individuals. For
example, over half of clinic patients
are estimated to endure one or
more attacks a month (Selby and
Lance 1960). In contrast, some
individuals only suffer two or three
bouts during their lives.
Migraine
The typical attack of migraine
consists of a sequence of
events that include
(1) prodrome,
(2) aura,
(3) headache,
(4) resolution, and
(5) postdrome (Blau 1987).

In an individual migraineur,
however, the attributes of a
specific bout can vary from a
limited number of symptoms Cured - Robert Zammerarchi
to a severe siege in which
the entire spectrum of the
disorder is present.
Approximately 25% of migraineurs
detect an assortment of premonitory
Migraine
symptoms (prodromes) that are the
initial events in the attack. These
frequently vague symptoms can
precede the aura or head pain by
several hours or even by days (Blau
1980; Amery et al 1986). Prodromal
symptoms typically include changes
in mood or behavior (eg, irritability,
depression, sluggishness, anxiety,
apathy, euphoria, excitement),
neurologic symptoms (eg,
excessive yawning, phonophobia
and photophobia, blurred vision),
constitutional symptoms (eg,
excessive fatigue, pallor, aching
muscles, fluid retention), and alimentary symptoms (eg, hunger, craving for
food, bulimia, nausea, anorexia). Prodromal symptoms vary widely among
individuals, but they are often consistent in a particular migraineur.
The absence or presence of an
Migraine
aura—an episode of focal,
transitory neurologic dysfunction—
in the preheadache phase of a
migraine attack distinguishes
common migraine (migraine without
aura) from classic migraine
(migraine with aura) (Headache
Classification Committee 1988).
Neurologic symptoms usually
develop over 5 to 20 minutes and
last less than 60 minutes.
Approximately 80% of migraine
sufferers have common migraine
(Selby and Lance 1960). About
70% of patients with classic
migraine also have attacks without
aura.
Migraine
Neurologic symptoms usually
develop over 5 to 20 minutes and
last less than 60 minutes. The
most common auras are visual,
but an aura may consist of
essentially any neurologic
symptom. Visual auras are of two
types: positive visual phenomena
with hallucinations and negative
visual phenomena (scotomas)
with either incomplete or
complete loss of vision in a
portion or the whole of the visual
field. Most visual auras have a
hemianoptic distribution.
Migraine
Photopsias are the simplest type of visual
hallucination, usually consisting of small
spots, dots, stars, unformed flashes or
steaks of light, or simple geometric forms
and patterns that typically flicker or sparkle.
Scintillating scotomas (also called
teichopsias or fortification spectra) are
considered to be the most distinctive
migrainous visual symptom. Such scotomas
consist of a scotomatous arc or band with a
shimmering or glittering, bright, zigzag
border (Richards 1971). The visual
alteration usually commences in the center
of the visual field and slowly extends
laterally. The scotoma frequently is
semicircular or horseshoe-shaped. On
occasion, objects may appear to change in
size and shape.
Patients may also have
Migraine
somatosensory auras consisting of
circumscribed feelings of
numbness or sensations of tingling
or pins and needles involving the
ipsilateral hand, face, and tongue
(cheiro-oral or digito-lingual
paresthesias) (Jensen et al 1986).
Minimal, brief hemiparesis is not
uncommon, but prolonged, severe
paresis is a rare aura. Dysarthria
and aphasia may be associated
with paresthesias or hemiparesis
in some patients, or aphasia may
occur as an isolated phenomenon.
True rotational vertigo frequently constitutes a migraine aura
(Savundra et al 1997). In some individuals, one type of aura
may follow another (eg, somatosensory symptoms may occur
as visual symptoms disappear).
Migraine
The headache phase of the bout follows
the aura and varies from mild discomfort
to intense and disabling. The pain of
migraine is typically described as
throbbing or pulsating. But the pain of
fewer than half of adult migraineurs has
a pulsating quality (Olesen 1978). The
head pain can last from a few hours to
several days, but it persists for less than
a day in most patients (Selby and Lance
1960). The unilateral nature of the
headache has been stressed
(Headache Classification Committee
1988), but migrainous head pain is
unilateral in only 56% to 68% of patients
(Lance and Anthony 1966; Olesen
1978; Sjaastad et al 1989).
Migraine
The pain may be bilateral at the onset of
the attack or begin on one side and
become generalized as the bout
continues. In patients with unilateral pain,
the side affected in different attacks may
vary or may invariably be the same in
each attack. The pain is usually located
in the frontotemporal region of the head
or in, around, or behind an eye. But any
region of the head or face may be
affected including the parietal region, the
upper or lower jaw or teeth, the malar
eminence, or the upper anterior neck.
Migrainous pain is typically diminished by
lying or sitting still and is increased by
any activity or effort or by any active or
passive head movement.
Migraine
Intolerance of light
(photophobia) and noise
(phonophobia) are the
most frequent symptoms
accompanying the head
pain. As a result of these
symptoms, most patients
seek a quiet, dark room.
Approximately 90% of
patients experience
nausea, and vomiting
affects more than half of
migraineurs (Lance and Serving Time - Nancy Ellen Wheeler
Anthony 1966; Olesen
1978).
Other gastrointestinal symptoms are common and
include anorexia, diarrhea, constipation, and
abdominal distension and cramps.
Migraine
Other patients complain of
blurry vision, facial pallor,
edema that is most
prominent in the temporal
and periorbital lobes, nasal
congestion, cold and
clammy hands and feet, and
polyuria. Ptosis and miosis
(Horner's syndrome) have
been observed during the
height of an attack in some
individuals, but in rare
patients the pupil dilates on
the side of the head pain
(De Marinis et al 1998).
Migraine
Many migraineurs suffer
from changes in their
psychological and mental
state during an attack.
Many feel depressed;
others feel irritable and
hostile. Many are
lethargic, drowsy, or
irresistibly sleepy. Minor
cognitive changes are
common during migraine
attacks and include
reduced ability to
concentrate, mildly Mentally Insufficient - Angela Mark
decreased memory, and
difficulty with abstract
thought.
Migraine
In most migraineurs, the pain
gradually diminishes over a
period of hours, but many
migraine attacks are concluded
by sleep (Blau 1982). Many
migraineurs have a postdromal
period after a headache lasting
several hours to several days.
Patients may feel fatigued,
weak, listless, or lethargic,
although some feel refreshed
or even euphoric.
Diagnostic Criteria for Migraine Without
Aura (Common Migraine)
• At least five attacks lasting 4 to 72 hours

• Headache has at least two of the following


characteristics:
unilateral location
pulsating quality
moderate or severe intensity
aggravation by routine physical activity

• At least one of the following during headache:


nausea or vomiting
photophobia and phonophobia
Hierboven "Buste van
een vrouw" van Pablo
• Normal neurologic exam and no evidence of
Picasso, van wie men
organic disease that could cause headaches vermoedt dat hij aan
migraine leed
Diagnostic Criteria for Migraine with Aura
• At least two attacks
(Classic Migraine)
• Aura must exhibit at least three of the
following characteristics:
fully reversible and indicative of
focal cerebral cortical or brainstem
dysfunction
gradual onset
lasts less than 60 minutes
followed by headache with a free
interval of less than 60 minutes or
headache
may begin before or
simultaneously with the aura
Headache I - Heidi Tobler
• Normal neurologic exam and no
evidence of organic disease that could
cause headaches
Differential
Migraine
Diagnosis

Tension-type
headache and
cluster headache
must be
differentiated from
migraine. The
International
Headache Society
promulgates
clear-cut criteria
for tension-type Migrain Duet - Jeanette
Abulafia
headache that
differentiate it
from migraine (Headache Classification Committee 1988), but there is
ongoing debate regarding the relationship between the two disorders.
Migraine
Management
The management of patients with
migraine headaches consists of several major
components:
1. The prevention of bouts by
identification and removal of known trigger
factors. Determination of trigger factors is
fundamental for effective migraine management
because many headaches may be prevented if a
particular migraineur abstains from alcohol,
eliminates chocolate, stops contraceptive pills,
obtains adequate sleep, or ingests three regular Take Two Aspirin and... -
meals a day. Edward LeSage
2. The use of nonpharmacologic treatments. Behavioral
procedures including biofeedback (both thermal and electromyographic),
simple relaxation therapy, autogenic training, and programs teaching
cognitive stress coping skills have been used successfully in migraineurs
(Andrasik et al 1984; Blanchard 1987; Andrasik 1996).
Migraine
Management
3. The pharmacologic
treatment of acute attacks.
Simple analgesics and
nonsteroidal anti-
inflammatory drugs are
capable of reducing pain in
many patients suffering from
acute migraine attacks.
Ergots (cafergot,
dihydroergotamine) are a
mainstay of symptomatic Relief - Deborah Barrett
treatment, but the efficacy of sumatriptan (Imitrex) and other newer triptans
(naratriptan, [Amerge], rizatriptan [Maxalt], zolmitriptan [Zomig]) has
changed that role (Goadsby 1998). Narcotics and analgesic/sedative drugs
should only be prescribed infrequently and in small quantities. They should be
used for a maximum of 2 to 3 days per week. The use of steroids for the
treatment of acute attacks should be discouraged because of the cumulative
nature of some of the side effects such as osteonecrosis.
Migraine
Management
4. The long-term treatment with prophylactic
medication to prevent recurring bouts. A considerable
number of potent medications (beta-blockers such as
propranolol and nadolol, calcium channel blockers
such as verapamil and diltiazem, antidepressants
such as amitriptyline and prozac, anticonvulsants
such as valproic acid and gabapentin, and
antiserotonergics such as methysergide) are
available for the prevention of attacks of migraine Visual Headache - Annette
(Silberstein 1996; 1997). LeBlanc
Preventive medications are capable of decreasing the frequency and
severity of migraine in most migraineurs.
All preventive drugs have side effects, however, and should be
prescribed with circumspection. Authorities disagree about guidelines for
administering preventative medication. Some physicians prescribe
prophylactic drugs for patients who have more than one headache each
month. Others feel that daily medication is warranted only if the frequency is
greater than one headache per week.
Migraine
Management
All preventive
drugs have side effects,
however, and should be
prescribed with
circumspection. Authorities
disagree about guidelines for
administering preventative
medication. Some physicians
prescribe prophylactic drugs Splitting Headache - Linda McCarthy
for patients who have more
than one headache each
month. Others feel that daily
medication is warranted only if
the frequency is greater than
one headache per week.
TENSION-TYPE HEADCHE
Tension-type headache falls into two distinct
categories, which can be specified as (1)
localized head pain (conforming to
International Headache Society classification
2.1.1 and 2.2.1) and (2) head pressure
awareness (conforming to International
Headache Society classification 2.1.2 and
2.2.2).

"Localized head pain" (International Headache Society classification


2.1.1. and 2.2.1) is comparable with osteoarthritic pain;
symptoms arise from the synovium, ligaments, or muscles moving
that joint. The pain is affected by movement and rest, showing diurnal
variations and responding in varying degrees to analgesics in the time
corresponding to drug absorption. The joints causing this type of
headache are the upper cervical (apophyseal) and the
temporomandibular joints, each with their own patterns.
TENSION-TYPE HEADACHE
The history extending over months or
years is of headache present initially on
awakening or on coming home after work
and lasting several hours; later the pain
can become continuous but, even then,
manifests diurnal varying intensities. The
distribution is indicated by the patient with
the index finger or the palm of the hand
affecting one or both sides of the head,
less frequently across the forehead;
indication of neck pain or stiffness usually has to be requested
specifically. Starting in the neck or the occiput, the pain sweeps
forward over the parietal region to the temple or forehead, or travels in
the opposite direction. Some indicate a line accurately outlining the
origin of the temporalis muscles, others the anterior fibers of that
muscle at the temple.
TENSION-TYPE HEADACHE
The patient has usually noted local
tenderness, eased or increased by finger
pressure. Heat when showering gives
temporary relief, but cold, especially icy
wind, accentuates the regional pain.
Neck movement influences the symptom
of pain arising from that region, but neck
crepitus, whiplash injury, or limitation of
neck movements (especially when reversing the car) need inquiry. If
the pain is predominantly in the temple or forehead, the physician
needs to ascertain whether there is bruxism, impacted wisdom teeth,
or jaw clicking. Neck pain often extends along the upper fibers of the
trapezius to one or both shoulders, whereas temporalis muscle pain
spreads downward in front of or behind the ear. Corroborative
evidence for organic pain origin derives from the response to
analgesics: partial or total relief in less than an hour, commonly in 20
to 30 minutes. Most of the history needs to be taken by direct (but not
leading) questions, because patients do not know what is relevant.
TENSION-TYPE HEADCHE
Physical examination is directed by the
history: if to the neck, then local unilateral
or bilateral muscle tenderness,
discomfort, or crepitus on neck
movements, especially restricted on
lateral flexion, are present. Precise
features to look for in examination of the
neck were recently specified by the
International Study Group on
Cervicogenic Headache (Sjaastad et al
1998). When the pain is anterior, the
temporomandibular joints may be tender,
crepitus or clicking may be heard through
a stethoscope, and jaw malalignment or
an overbite may be present; then it is
worthwhile asking about comments by the
patient's dentist, who may have
mentioned excess tooth wear from
bruxism not recognized by the patient.
TENSION-TYPE HEADACHE
The history extending for months to
years is vague. Head symptoms are
initially intermittent, but later occur 7
days a week, from the moment of
waking until sleep is attained. The
sensation is symmetrical, likened to a
weight on the vertex or a band around
the head, less frequently a tightness
across the forehead. In describing the
sensation the patient waves a hand
vaguely around the head, in contrast to
the precise indication given by patients
headache.
with type I Nothing relieves the ache except when the mind is diverted by
some activity or sleep; exacerbations are provoked by stress or an
argument. If the patient is asked to describe the pain, "it is not a pain, it is
an ache" is the usual reply; if asked whether the headache is a pain or a
pressure, "it is a pressure" is the more usual response. Two analgesic
tablets taken regularly three or four times daily have "no effect
whatsoever," yet continue to be consumed for months in the vain hope
that "they might work" or because "you've got to do something."
TENSION-TYPE HEADACHE
Direct questioning may disclose fears of
organic diseases (usually brain
affections) or financial, social,
personal, or academic problems,
indicating the cause of the underlying
anxiety state. In children, parental
anxiety or bullying at school needs
sensitive inquiry. In others, asking
about depression provokes the
response that the condition is "getting
me down"; tears well up, and there
may be crying.

Formal neurologic examination reveals a Here It Comes Again -


Gerard Mackay
normally functioning nervous system, but
the demeanor of the patient during the
interview indicates anxiety or depression or
an agitated depression.
TENSION-TYPE HEADACHE
A typical visit to the doctor's office would go as follows:
Q. How long have you had your headache?
A. A long time.
Q. Would you say 1 month, 6 months, or a year?
A. Longer than that.
Q. Where is it?
A. All over (waving hand vaguely over the top of the
head).
Q. What is the pain like?
A. It is not a pain it is a pressure.
Q. When do you get it?
A. All the time—from when I wake to when I go to sleep.
Headache - John Crowley
Q. What do you take for it?
A. I have tired everything, nothing helps.
Q. What are you taking now?
A. Six to eight acetominophen tablets a day.
TENSION-TYPE HEADACHE
Q. Does this do any good?
A. (Promptly and
vehemently) Not a bit.
Q. How long have you been
taking six to eight tablets a
day?
A. For 6 months.
Q. If it's not working then
why are you taking them?
A. (pause) We'll you've got
do something haven't you.
Or, Well it might help.
At the end of the
history, when inquiring about 13 Year Old Headache - Val Akula
sleep difficulties it is
common to learn that the
patient has difficulty going to
sleep and staying asleep.
TENSION-TYPE HEADCHE
Diagnostic Workup
In cases of localized head
pain, brain scan may be
necessary to reassure the patient
or physician, although the test is
usually a waste of the patient's or
insurance company's money.
Some individuals cannot tolerate
the investigation because of
claustrophobia. Anemia can
contribute to headache, and many
menstruating women are on the Denatured Tension - Lisa LaMotte
borderline of anemia, so a blood
count needs to be considered. An ESR excludes most cases of temporal
arteritis and may be elevated in other systemic diseases, especially
those with pyrexia. Rarely can a plain skull x-ray, including the base,
reveal a secondary neoplasm, a myeloma deposit, or a pituitary tumor.
TENSION-TYPE HEADACHE
Management
The first line of
treatment is an explanation
of the condition in words that
the patient will be able to
understand, bearing in mind
that few can retain more than
three facts in any learning
session. Simple analogies
applicable to the patient's
daily activities help;
emphasis that the pain is
genuine and not imaginary or Twisted Mother -Nancy Ellen Wheeler
"in the mind," even when caused psychologically, is essential. Further,
"pinched" or "nipped nerves" or vessel obstruction by muscular
contraction are often voiced fears that need to be allayed gently, not
dismissed or denigrated.
TENSION-TYPE HEADACHE

Pharmacological preparation
against anxiety and depression
may be necessary but should
be prescribed for a limited
period only. Others favor
diverse relaxation therapies,
with or without biophysical
measurements made visible or
audible to the patient.
Depression, if mild and recent,
can be treated by neurologists;
when severe or long-standing, Morning Heavyhead - Julia Knowlton
psychiatric help is needed, the
degree of urgency depending
on clinical assessment.
CLUSTER HEADACHE
Three major forms of cluster headache are
recognized, namely, episodic and chronic
varieties and the variant, chronic paroxysmal
hemicrania (Headache Classification
Committee of the International Headache
Society 1988). The terms used in describing
cluster headache include "attack," meaning
individual attacks of headache pain; "cluster
period," meaning the period of time during
which patients have repeated attacks;
"remission," indicating periods of freedom
from attacks; and "mini bouts," designating
bouts of attacks that last for less than 7 days. Loneliness, Pain, Tears - Denise
Auger
The attack profile consists of the rapid onset of headache that builds up to a
peak in about 10 to 15 minutes and lasts for approximately 30 to 45
minutes. The headache is almost always unilateral.
CLUSTER HEADACHE
The most common site of pain is orbital, retro-
orbital, temporal, supraorbital, and infraorbital in
order of decreasing frequency. On occasion the
head pain may switch sides, and in extremely
rare cases it can by bilateral. Typically the pain is
in the trigeminal nerve distribution, even though
extratrigeminal pain, especially in the
suboccipital area, is known to occur in 18% to
20% of attacks. The number of attacks per day
varies from one to three, but the range can be
from one a week to eight or more per day.
During the attacks, patients find it difficult to lie Attention Headache -
down as this position can aggravate the pain. Merana Cadorette
usually pace the floor, or sit up assuming postures that give them
Patients
maximum relief. Patients may behave in an uncontrolled and bizarre
way; they may moan, cry, yell, or scream and may even threaten
suicide. Some patients find relief by physical exercise such as jogging in
place. The pain is so excruciating that after attacks patients remain
exhausted for some time.
CLUSTER HEADACHE
Neurologic examination may reveal mild ptosis
and miosis on the side of the headache,
especially during the attack or immediately
following. Ipsilateral tenderness of the carotid
artery, periorbital swelling, and congestion of
the conjunctiva are also noted.
Alcohol, nitroglycerin, and histamine
can induce attacks during the cluster periods
(Horton et al 1939; Ekbom 1968).

Etiology
The etiology of cluster headaches is Anguish - George Dergalis
not known. Because it is predominantly a
disease of males, male hormone may have
some etiological role. No conclusive data are
available, however.
CLUSTER HEADACHE
Differential Diagnosis
Chronic paroxysmal hemicrania is a
recognized variant of cluster headache that
occurs predominantly in women. It is
differentiated from cluster headache by short-
lived attacks that typically last 5 to 10 minutes,
multiple attacks per day (up to 15 to 20 per day),
and absolute responsiveness to indomethacin.
Cases with a similar clinical picture but with
remissions have been recognized and are
referred to an episodic paroxysmal hemicrania
(Kudrow et al 1987; Newman et al 1993).
Florinal Call - Val Akula
Cluster headache is distinguished from migraine by the male
predominance, strict unilaterality of pain, short-lived attacks (45 minutes
to 1 hour), multiple attacks per day, associated autonomic features,
restlessness and inability to lie down during the attack, and the periodicity
of attacks, including circadian accuracy and clockwise regularity.
Migraines tend to occur primarily in females.
CLUSTER HEADACHE

Symptomatic cluster headaches


are cluster headache–like attacks
that occur as a result of an
underlying intracranial lesion
(Mathew 1993). Parasellar
meningiomas, adenomas of the
pituitary, calcified lesions in the
region of the third ventricle,
anterior carotid artery aneurysms,
nasopharyngeal carcinomas,
ipsilateral large hemispheric
arteriovenous malformations, and Through a Glass Darkly - Janet Morgan Mol
upper cervical meningiomas
haveall been reported to produce
symptomatic cluster headache.
CLUSTER HEADACHE
Symptomatic cluster
headache should be
suspected when the clinical
features of the headache are
atypical. Atypical features
include:
(1) absence of the typical
periodicity seen in episodic
cluster headache,
(2) a certain degree of
background headache which
does not subside between Suffering without Sin - Christine
attacks, Shaughnessy
(3) inadequate or unsatisfactory response to medications that are
effective in idiopathic cluster headache such as oxygen inhalation,
ergotamine, and verapamil, and
(4) the presence of neurologic signs other than miosis and ptosis.
CLUSTER HEADACHE
Diagnostic Workup
With a typical clinical
history, there is no need for
any specific diagnostic
workup. In atypical cases,
however, complete neurologic
workup is necessary, including
MRI and MRA, to exclude the
symptomatic variety of cluster
headache. EEG has no place
in the diagnostic workup of
cluster headache.
Management The Twilight Zone - Heather Dudley
Management of acute attacks: Acute attacks are of sudden
onset and of short duration. Therefore, the sue of agents that provide
immediate relief is essential. The most effective agents are oxygen
inhalation (Kudrow 1981) and subcutaneous sumatriptan (Sumatriptan
Cluster Headache Study Group 1991; Ekbom 1993).
CLUSTER HEADACHE
The recommended dose of oxygen inhalation is 7 L
per minute for 10 minutes using a facial mask at the
onset of headache. Approximately 60% to 70% of
patients respond to oxygen, the effect being evident
in approximately five minutes, however oxygen may
simply delay an attack rather than abort it
completely in some patients. The effect of oxygen
is purely a result of its cerebral vasoconstrictive
property.
With sumatriptan, the headache relief is
rapid, commencing within 5 minutes. The
recommended dose is 6 mg. Long-term, Sick - Karen LeBlanc

repeated use of sumatriptan for acute attacks of cluster headache has


been investigated. Sumatriptan is generally well tolerated. The current
opinion is that subcutaneous administration of 6 mg of sumatriptan is
rapidly effective and well tolerated in the long-term acute treatment of
multiple cluster headache attacks.
CLUSTER HEADACHE

Dihydroergotamine, which is available only in the


injectable form at the present time, is effective in the
relief of acute attacks of cluster headache.
Intravenous injection give rapid relief in less than 10
minutes, whereas intramuscular injection takes
longer to be effective. An intranasal preparation of
dihydroergotamine is under investigation and, when
available, may prove to be a useful agent in the
acute treatment of cluster headache.
Ergotamine is available only in tablet or suppository
form and thus is not particularly useful in the acute Cacophony - Rosemary
management of cluster headache. El'Hage
Ergotamine takes a substantial length of time to be effective, and the attack
may subside spontaneously before the medicine has had a chance to work.
However, some patients who may respond to the suppository form of
ergotamine fairly quickly. In general, use of oral or suppository ergotamine is
not highly effective in the management of acute attacks of cluster headache
because of the delayed action.
CLUSTER HEADACHE

Locally applied lidocaine nasal drops have been


reported to be effective in the treatment of acute
attacks of cluster headache (Kitelle et al 1985).
Patients are told to lie supine with the head tilted
backwards toward the floor at 30 degrees and
turned to the side of the headache. A nasal
dropper may be used and the dose (1 mL of 4%
lidocaine) repeated once after 15 minutes. The
beneficial effect arises from the local anesthetic
action interfering with the nociceptive circuits
involving the nasal mucosa and the
sphenopalatine ganglion. In turn, this action
decreases the afferent activity in the trigeminal Pain and Pain's Release -
system. Many physicians, however, do not find Terri Winding
lidocaine to be a very reliable agent.
CLUSTER HEADACHE
Prophylactic pharmacotherapy of cluster
headache: Prophylactic pharmacotherapy is the
mainstay of the management of cluster headache.
Medications are used daily during the cluster period
in the episodic variety and continuously for patients
with the chronic variety. The most effective agents
include ergotamine, verapamil, lithium carbonate,
corticosteroids, methysergide, and valproate.
Indomethacin is specific for paroxysmal hemicrania.
Beta-adrenergic blocking agents and tricyclic
antidepressants are of no particular value.
The Collage Life - Sheila Regan
The principles of prophylactic pharmacotherapy include: (1) initiation of
treatment early in the cluster period, (2) daily use of medications until the
patient is free of headache for at least 2 weeks, (3) tapering the
medications gradually, rather than abruptly withdrawing them towards the
end of the treatment period, and (4) reinstitution of medications at the
beginning of the next cluster period.
CLUSTER HEADACHE
The criteria for selection of a
particular medication for
prophylactic treatment will depend
on (1) the previous response to
prophylactic medications, (2) the
reactions, adverse or therapeutic,
to medications, (3) the presence of
contraindications to the use of a
particular medications, (4) the type
of cluster headache (episodic
versus chronic versus chronic
paroxysmal hemicrania), (5) the Pernicious Assault - Terri Russo
age of the patient, (6) the
frequency of attacks,
(7) the timing of attacks (nocturnal versus diurnal), and (8) the expected
length of the cluster period. Combinations of two or more medications
may be necessary for proper control in some patients.
CLUSTER HEADACHE
Ergotamine tartrate (1 mg twice a day) given
prophylactically is very useful. There is no
evidence that ergotamine causes rebound
phenomenon in cluster headache, unlike the
case of migraine. Ergotamine is particularly
useful in controlling nocturnal attacks when
taken at bedtime. Ergotamine is
contraindicated in patients with peripheral and
cardiovascular disease.
Verapamil (360 to 480 mg per day in
divided doses) is the prophylactic drug of
choice for both episodic and chronic cluster
headache. Constipation and water retention Roger Reacts to the Light -
are the most common side-effects. Verapamil Christine Lamb Toubeau
can be combined with ergotamine, and this
combination is very effective.
CLUSTER HEADACHE

Methysergide is useful as a prophylactic


agent especially in younger patients with
cluster headache. In older patients with
potential arthrosclerotic heart disease, this
agent should be used with care.
Methysergide has a number of side-effects,
including muscle cramps and muscle pains,
water retention, and fibrotic reactions
(retroperitoneal, pleural, pulmonary and
cardiac valvular). Because the duration of
periods of episodic cluster headache is
approximately 3 to 4 months, use of
methysergide is acceptable for that period of Headache #1 - Rebeckah
Raye
time.
CLUSTER HEADACHE
Lithium carbonate is mainly used for the
prophylactic treatment of chronic cluster
headache but is also helpful in management
of the episodic variety (Mathew 1978;
Manzoni et al 1983). The mechanisms of
the beneficial action of lithium in cluster
headache are not fully understood. Lithium
stabilizes and enhances serotonergic
neurotransmission within the CNS. The
usual dose of lithium is 600 mg to 900 mg
per day in divided doses. Lithium levels
should be obtained within the first week and
periodically thereafter. The serum level Gripping Headache -Raymond
required for therapeutic response is usually Dorow
0.4 to 0.8 mEq/L, which is less than the
standard recommended dose in cases of
manic-depressive psychosis.
CLUSTER HEADACHE
Corticosteroids, particularly prednisone,
are very useful agents in the treatment
of episodic cluster headache for short-
term use (2 to 3 weeks in tapering
doses). This usually helps to break the
cycle of headache especially in those
who don't respond to medications
such as ergotamine and verapamil.
Corticosteroids are useful in chronic
cluster headache; however, when the
medication dosage is tapered, the
headache tends to come back.

The Enemy Within - Vona Marengo

Sodium valproate (600 mg to 2000 mg per day in divided doses) has


been reported to be an effective agent in reducing the frequency of
cluster headache attacks (Hering and Kurtiz 1989).
CLUSTER HEADACHE
Indomethacin is particularly useful in the
treatment of paroxysmal hemicrania, both in
the chronic and episodic varieties. The
responsiveness is absolute and diagnostic
(Mathew 1980). Benefit usually appears
within 48 hours. Indomethacin is a powerful
prostaglandin inhibitor and reduces cerebral
blood flow.
Prioritization of prophylactic
therapy: For episodic cluster headache,
verapamil (360 mg to 480 mg per day) is the
first choice followed by ergotamine (1 mg
twice per day). In more resistant cases a The Eyes Have It - Antonia
combination of ergotamine and verapamil is Putman
recommended. Methysergide (2 mg three to four times a day) is an
effective alternative especially in younger patients. Methysergide should
not be combined with ergotamine. Corticosteroids may be used for short
periods to break the cycle of headache or to treat severe exacerbations.
THE END

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