H d h

Headache

Jennifer McCombe
PGY 3
PGY-3
September 15, 2006
Overview

„ Basics of headache „ Specific

p headache
‰ Epidemiology disorders
‰ Anatomy ‰ Thunderclap headache
‰ History ‰ Cerebral venous sinus
‰ Diagnostic work-up thrombosis
‰ Cervical artery dissection
‰ Mi i
Migraine
‰ Trigeminal neuralgia
‰ Post LP headache
‰ Idiopathic intracranial
hypertension
‰ Chronic dailyy headache
Epidemiology

„ Headache is the ppresenting

g symptom
y p in 1-2.5% of
emergency visits
‰ Patients present to the emergency with 2 types of
headache
1. “First or worst syndrome”
2. “Last straw syndrome”
„ Is one of the top 10 complaints in almost all
medical specialties
„ Most headaches don’t represent
p a serious medical
condition
‰ However, has one of the longest lists of differential
diagnoses in all of medicine
Anatomy

„ Pain sensitive structures include:

‰ Skin and its blood supply
‰ Muscles of the head and neck
‰ Great venous sinuses and tributaries
‰ Portions of the meninges including the dura mater at the
base of the skull
‰ D l arteries
Dural t i
‰ Intracerebral arteries
‰ Cervical nerves
‰ Select cranial nerves
„ Pain sensitive structures are affected by tension,
traction distension,
traction, distension dilatation
dilatation, and inflammation
Evaluation

„ Diagnosis of primary headache disorders

based on the International Headache Society
classification system
y updated
p in 2003
‰ Headache divided into two main types: Primary
and secondary
„ The most important diagnostic tool is a
detailed history
„ Diagnostic tests only help to establish or
exclude causes of secondaryy headache
Evaluation

„ History:
y „ Past headache history y
‰ Onset (including meds tried)
‰ Duration „ Past medical history
‰ Frequency „ M di ti
Medications
‰ Course
„ Family history
‰ Character
‰ Severity „ Social history (including
‰ Location alcohol and drugs,
‰ Associated symptoms
y p
sleep, eating and
‰ Precipitants or triggers
exercise
i h habits,
bit h how
headache affects
‰ Prodromes
function))
Evaluation

„ Asking the patient to complete a headache

diary documenting headaches, possible
triggers,
gg , and treatment tried is often very y
helpful in clarifying details of the history
Evaluation

„ Physical exam
‰ KEY POINTS:
„ Vitals
„ Fundoscopy
„ Palpation of areas of head and neck
„ Auscultation of eyes, neck
„ Nuchal rigidity and meningeal signs
„ Complete neurological exam
Further evaluation of headache

Reference:
Further evaluation of headache
When to consider neuroimaging
„ Temporal profile and headache features:
1. The first
Th fi t or worstt headache
h d h (thunderclap
(th d l h headache)
d h )
2. Subacute headache with increasing frequency or severity
3. Progressive or new daily persistent headache
4. Chronic daily headache
5. Side-locked
6. Headache not responding to treatment

‰ Demographics:
1. New headache in patient with cancer or HIV
2. New headache age > 50
3. Headache and seizures

‰ Associated symptoms and signs:

1. Fever, stiff neck, nausea and vomiting
2. Focal neurological symptoms or signs
3. Papilledema, cognitive impairment or personality change
Overview

„ Basics of headache „ Specific

p headache
‰ Epidemiology disorders
‰ Anatomy ‰ Thunderclap headache
‰ History ‰ Cerebral venous sinus
‰ Diagnostic work-up thrombosis
‰ Cervical artery dissection
‰ Mi i
Migraine
‰ Trigeminal neuralgia
‰ Post LP headache
‰ Idiopathic intracranial
hypertension
‰ Chronic dailyy headache
Thunderclap headache

„ Is a headache that is acute and severe at

onset
„ Originally used to describe the headache
associated with subarachnoid hemorrhage
(SAH)
„ If the work-up for SAH is negative, however,
there is a list of alternate possibilities for
etiology
Causes of thunderclap headache
Subarachnoid hemorrhage

„ Most common cause of secondary

thunderclap headache and should be the
focus of the initial investigations
g
‰ 11-25% of patients presenting with thunderclap
headache have SAH
„ Etiology of SAH:
‰ Ruptured
p aneurysm
y 85%
‰ Non aneurysmal perimesencephalic bleed 10%
‰ Other causes 5%
Subarachnoid hemorrhage

„ Need to have maximal headache within a few

minutes
„ Typically the headache lasts at least a few
days
„ 50-70% of patients can present with an
isolated headache
„ 10 43% of patients with aneurysmal SAH
10-43%
have a history of a sentinel headache days to
weeks before
Subarachnoid hemorrhage

„ CT head
‰ Sensitivity with new scanners nears 100% within
the first 12 hours
„ This is provided that the images are interpreted by an
experienced neuroradiologist (or Dr. Mike Saka)
‰ S
Sensitivity
iti it falls
f ll to
t 50% b
by 1 week
k
„ Therefore, in cases where clinical suspicion
i hi
is high,
h needd an LP tto rule
l outt SAH
‰ LP needs to be analyzed for xanthochromia
Cerebral venous sinus thrombosis

„ Generally present with a headache of gradual

onset exacerbated by increases in
intracranial p
pressure that occur with
coughing, sneezing, and valsalva
„ 15-30%
15 30% present with an isolated headache
„ Up to 10% of patients can present with a
thunderclap headache
Cerebral venous sinus thrombosis

„ CT head:
‰ Normal exam: CT normal 25% of the time
‰ Abnormal exam: CT normal 10% of the time
„ LP can have subtle abnormalities (↑RBC, ↑protein,
mild lymphocytic pleocytosis, ↑opening pressure)
„ Th f
Therefore, one needs
d a high
hi h iindex
d off suspicion
i i
especially in women who are pregnant or post-
partum, in patients with a history of DVT
„ Requires an MRI with MRV to establish or exclude
the diagnosis
Cervical artery dissection

„ Most commonly y associated with a headache of

subacute onset
„ 20% of patients present with a thunderclap
headache
„ Headache reported by 60-95% of patients with
carotid artery
y dissection and 70% of patients
p with
vertebral artery dissection
„ Headache generally ipsilateral to the dissection and
involves the face
face, jaw
jaw, ears
ears, periorbital
periorbital, frontal and
temporal regions; with neck pain in 25-50% of
patients
Cervical artery dissection

„ Median time from onset of headache to

neurological symptoms is 4 days with carotid
dissection and 14.5 hours with vertebral
artery dissection
„ Need a high index of suspicion in patients
with a history of TIA or stroke and a history of
trauma or chiropractic
p manipulation
p
Migraine

„ Genetic condition in which a person has a

predisposition to headaches, GI dysfunction,
and neurological
g dysfunction
y
„ Inherited in an autosomal dominant fashion
„ Migraine attack occurs when a trigger is
encountered; triggers include hormonal
changes stress,
changes, stress stress letdown
letdown, foods
foods,
alcohol, smells, sleep disturbance etc
Migraine

„ Typical headache is unilateral or bilateral

bilateral,
throbbing, worse with activity and associated
with nausea,, vomiting,
g, photophobia,
p p , and
sonnophobia
„ Can be variable, however, and some believe
that the HIS criteria are too strict
Migraine

„ Principles of Treatment:
‰ Goal is to stop headache and progression of the pain
‰ Are treatment options which are migraine specific and
th
those which
hi h are non-specific
ifi
‰ Is best to treat early in the course of the headache to
prevent central sensitization and treatment refractory pain
‰ Use different agents for different intensities of pain and limit
abortive therapies to 2 times per week to avoid medication
rebound headache
‰ If requiring more frequent abortive therapies need to
recommend prophylaxis
Migraine
„ Treatment of the acute headache
‰ NSAIDs

‰ Hydration/ iv fluids

‰ Triptans
„ Significant side effect is cardiac ischemia due to vasoconstriction of
the coronary arteries therefore contraindicated in those with a history
of CAD
‰ DHE
„ Mostt common side
M id effect
ff t iis nausea th
therefore
f pretreat
t t with
ith an
antiemetic
‰ Neuroleptics (metoclopromide, promethazine, prochlorperazine,
chlorpromazine)
„ Can cause hypotension when given iv therefore give with saline
‰ Corticosteroids
„ Used in status migrainosis
Trigeminal Neuralgia

„ The most common cranial neuralgia

„ More common in women with an average age of
onset at 50 years
„ Generally unilateral
„ Characterized by brief lancinating pain limited to the
distribution of one or more divisions of the trigeminal
nerve usually V2 or V3
nerve,
„ Pain intensity is excruciating, described as sudden,
sharp, superficial, stabbing or burning
„ Paroxysmal and usually lasts a few seconds
„ May occur in volleys with pain free periods lasting
seconds to hours
Trigeminal Neuralgia

„ Painful episodes associated with trigger

zones around the mouth and nostril
„ Can be triggered
gg by
y wind on the face,,
brushing teeth, shaving, chewing or even
talking
„ Majority of cases are idiopathic
„ Suspicion of a secondary cause should arise
when
h a chronic
h i continuous
ti pain
i iis punctuated
t t d
by paroxysms of pain or when the is signs of
trigeminal nerve dysfunction
Trigeminal Neuralgia

„ 90% of patients are over the age of 40

„ Diagnosis in younger patients should prompt
an evaluation for secondary causes such as
MS, posterior fossa tumours, or aneurysmal
compression of the trigeminal nerve
„ 10% of patients harbour an intracranial lesion
therefore an MRI is recommended for all
patients
Trigeminal Neuralgia

„ Treatment:
‰ Spontaneous
remissions are
common
‰ After 8 weeks of
successful therapy
therapy,
slow drug taper is
recommended
Trigeminal Neuralgia

„ Treatment
‰ Approximately 30% of patients will fail medical
therapy
py and may y require
q surgical
g or ablative
procedures
‰ Microvascular decompression is considered to be
the definitive process for idiopathic trigeminal
neuralgia
„ Success rate is approximately 90%
Post LP headache

„ Defined as a bilateral headache that

develops within 7 days after LP and
disappears within 14 days after LP
„ Headache worsens within 15 minutes of
assuming the upright position and disappears
or improves
i within
ithi 30 minutes
i t off resuming
i
the recumbent position
„ Headache is located in the frontal or occipital
areas, and may also involve the neck and
pp shoulders
upper
Post LP headache

„ Other common symptoms include nausea nausea,

vomiting, visual disturbances, and hearing
alteration
„ Visual symptoms most commonly due to 6th
nerve palsy
„ 10% of patients with LP have some alteration
in their hearing
hearing, usually in the low frequency
range
Post LP headache

„ The frequency of headache is inversely related to

age
„ The greatest influence on the incidence is technique
and choice of needle
‰ Ensuring the direction of the bevel is parallel to the
longitudinal axis of the spine decreases incidence of
headache
‰ Smaller needles also have a lower incidence of headache
„ Many recommend bedrest following dural puncture
however, studies do not support this
recommendation
Post LP headache

„ Treatment:
‰ Conservative measures:
„ Bedrest
„ Analgesia
„ Hydration
„ Caffeine
„ Sumatriptan
‰ Autologous epidural blood patch
„ High
Hi h success rate,
t llow complication
li ti rate
t
„ Performing the blood patch too early increases the risk
of failure; optimal time is 24 hours post LP
Idiopathic Intracranial Hypertension

„ Condition of increased intracranial pressure without

clinical, laboratory, or radiological evidence of
intracranial pathology
„ Typical patient is an obese, but otherwise healthy
woman of childbearing age with symptoms of
increased ICP
„ Seen in all ages with an incidence of 21:100000
„ Female : male
male, 4
4.3-15
3-15 : 1
„ Is a diagnosis of exclusion
„ Rare cases of familial occurrence
Idiopathic Intracranial Hypertension

„ Etiology and pathogenesis are unknown

„ Symptoms include headache, pulsatile tinnitus,
transitory visual obscurations, nausea, blurred vision
and diplopia
„ Headache is episodic in onset and usually develops
over weeksk to
t daily
d il pain
i off moderate
d t iintensity,
t it
worse in the morning and during physical activity,
with valsalva maneuvres and with postural changes
„ Quality and location of the headache are non-
specific
Idiopathic Intracranial Hypertension

„ Episodes
p of visual obscurations can be monocular
or binocular and usually last less than a minute
„ Episodes are provoked by postural changes and the
valsalva maneuvre
„ No way to predict those at risk of developing
permanent visual deficits
p
„ Transient sixth nerve palsy is the most common
cause of episodes of diplopia
„ Oth less
Other, l common symptomst experienced
i d iinclude
l d
transient sensory symptoms, decreased
concentration and memory y difficulties
Idiopathic Intracranial Hypertension

„ On examination, one usually sees bilateral

papilledema (blurring of the disc border, absent
venous pulsations, distended retinal veins, and later
protrusion of the optic disc, peripapillary
h
hemorrhages
h and
d exudates)
d t )
„ The risk of permanent deficit increases with the
duration of edema
„ Transient visual field deficits occur in 96% of
patients; enlargement of the blind spot being the
most frequent
„ Defect is usually asymptomatic and resolves when
the edema resolves
Idiopathic Intracranial Hypertension

„ Measurement of CSF pressure necessary for

diagnosis
‰ For accurate measurement, needs to be assessed in the
l t ld
lateral decubitus
bit position
iti with
ith llegs extended
t d d and
d as
relaxed as possible
‰ Pressure readings between 200 and 250 mm are a non-
diagnostic grey zone
„ 93% of patients have an elevated, steady state,
pressure; a small group of patients may require
pressure monitoring to catch transient pathological
elevations in pressure
p
Idiopathic Intracranial Hypertension

„ CT head normal by definition

„ CSF analysis within normal limits by definition
„ Recommend that a standard MRI with MRV
be carried out before the diagnosis of
Idiopathic intracranial hypertension can be
given
Idiopathic Intracranial Hypertension

„ Treatment
‰ Symptomatic: focused on lowering ICP and preventing
permanent visual deficits and headache
‰ I l d
Includes:
1. Weight loss
2. Acetazolamide (carbonic anhydrase inhibitor)
3. Furosemide
4. Topiramate
5
5. Short term oral corticosteroids
6. Surgical options: shunting and optic nerve sheet fenestration
7. Controversial: repeated lumbar punctures
Idiopathic Intracranial Hypertension

„ Prognosis:
g
‰ Spontaneous recovery is common
‰ Cessation of symptoms in 70-85% of patients within 2-3
months of medical therapy
‰ Up to 25% have a more protracted course
‰ Recurrence not unusual (10-40%)
‰ V
Very slight
li ht permanentt visual
i l fifield
ld d
deficits
fi it common ((normall
formal testing in only 43% of patients) but largely
asymptomatic
‰ 9% off patients
ti t experience
i severe permanentt visual
i l field
fi ld
deficits
‰ Up to 5% develop blindness in one or both eyes
Chronic daily headache

„ Refers to the presence of headache for more than

15 days per month for longer than 3 months
„ Is a category that contains many disorders
representing primary and secondary headaches
„ 70-80% of patients presenting to headache clinics
h
have d
daily
il or near-daily
d il hheadaches
d h
„ Transformed migraine and medication-overuse
headaches are among the most common
Chronic daily headache

„ Risk factors include obesity,

y, a historyy of frequent
q
headache (more than 1 per week), caffeine
consumption, and overuse (more than 10 days per
month) of acute headache medications
medications, including
analgesics, ergots, and triptans
„ Greater than 50% of patients have sleep
di t b
disturbances andd moodd di
disorders
d
„ Most patients with transformed migraine are women,
have a history pf episodic migraine
migraine, have a period of
transformation in which the headaches became
more frequent until the current pattern developed
Chronic daily headache

„ Reasons to image:
1. Development of progressively frequent and severe
headache within a period of 3 months
2. N
Neurologic
l i symptoms
t
3. Focal or lateralizing neurologic signs
4. Papilledema
5. Headaches aggravated or relieved by assuming an
upright or supine posture
6. H d h provoled
Headaches l db
by V
Valsalva
l l
7. Systemic symptoms or fever
8. Historyy of new headache after the age
g of fifty
y
Chronic daily headache

„ In medication overuse headaches,, there are varying

y g
intervals from the time of frequent intake of
medications to the development of chronic daily
headache:
‰ Triptans: 1.7 years
‰ Ergots: 2.7 years
‰ Si l analgesics:
Simple l i 4
4.8
8 years
„ Accurate diagnosis and treatment requires the
withdrawal
t da a o of tthese
ese medications;
ed cat o s; if a
an ep
episodic
sod c
pattern of headache recurs within 2 months of
withdrawal, medication-overuse headache is
diagnosed
Chronic daily headache

„ Treatment strategies:
‰ Lifestyle modification:
„ Limiting caffeine consumption
„ Regular exercise
„ Regular mealtimes
„ Regular
g sleepp schedule
‰ Preventative medications:
„ Tricyclic antidepressants, low dose
„ G b
Gabapentinti
„ Topiramate, low dose
„ Botulinum toxin type
y A
Chronic daily headache

„ With withdrawal from acute headache

medications, there are a few strategies to
limit withdrawal symptoms
y p
‰ Use NSAIDS and DHE to treat breakthrough
headaches as these are considered at lower risk
of medication overuse headache
‰ Prednisone 100 mg daily for 5 days may reduce
th number
the b off hours
h off severe withdrawal
ithd l
headache (Pageler et al., 2004)
‰ Antiemetics (metoclopramide or prochlorperazine)